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1

Fancello, Virginia, Silvia Palma, Daniele Monzani, Stefano Pelucchi, Elisabetta Genovese, and Andrea Ciorba. "Vertigo and Dizziness in Children: An Update." Children 8, no. 11 (2021): 1025. http://dx.doi.org/10.3390/children8111025.

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Background: Vertigo and dizziness are relatively infrequent in paediatric patients, but specific data on the prevalence of these disorders are limited and influenced by various factors, including the age of the examined population. These conditions often have a significant impact on patients’ and parents’ quality of life. The aim of this paper is to investigate the prevalence of different aetiologies of vertigo in the paediatric population through a systematic review. Methods: According to PRISMA guidelines, a systematic review of the literature was performed. Medline and Embase were searched from January 2011 through to 10 September 2021. The search yielded 1094 manuscripts, which were reduced to 7 upon the application of inclusion criteria. Results: A total of 2470 paediatric patients were evaluated by the selected papers. Vestibular Migraine was the most frequently diagnosed condition, occurring alone or in association with other diseases. Overall, audio-vestibular disorders represented the second cause of vertigo, and the prevalence appears to increase according to age growth. Over the years, even though we assisted in the amelioration of diagnostic rates, partially related to an improvement in diagnostic tools, the aetiology of vertigo remains still unclear in a variable percentage of patients. Conclusion: Vertigo in children, despite being an uncommon symptom, requires a multidisciplinary approach, often involving Paediatricians, Neurologists and Otorhinolaryngologists. A comprehensive evaluation of children suffering from vertigo is crucial for establishing a successful therapy and reducing parental worries.
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Song, Cheng Hee, Mohammad Zikri Ahmad, Ab Hamid Siti-Azrin, and Wan Adnan Wan-Nor-Asyikeen. "The identification of key factors predictive of traumatic brain injury in paediatric patients with a minor blunt head injury." Hong Kong Journal of Emergency Medicine 27, no. 4 (2019): 211–16. http://dx.doi.org/10.1177/1024907919836568.

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Introduction: Traumatic brain injury (TBI) is a major public health concern, and contributes significantly to mortality and morbidity in paediatric patients. Objective: The study is aimed to identify the factors predictive of TBI in paediatric patients with a minor blunt head injury. Methods: This was a retrospective cohort study conducted on 274 paediatric patients with a minor head injury and registered to Hospital Universiti Sains Malaysia (USM) from the year 2009 to 2013. Patients with a minor blunt head injury aged less than 18 years who underwent computed tomography (CT) of the brain were included, while the patients with penetrating head injuries were excluded from the study. Simple and multiple logistic regression analysis were performed. Results: TBI occurred in 49% of the patients. Headache (adjusted odd ratio (AOR): 2.24; 95% confidence interval (CI): 1.24, 4.05, p = 0.008), dizziness (AOR: 3.08, 95% CI: 1.27, 7.51, p = 0.013) and the presence of scalp haematoma (AOR: 2.93, 95% CI: 1.60, 5.34, p < 0.001) were the most important clinical variables for predicting TBI following a minor blunt head injury in paediatric populations. Conclusion: Headache, dizziness and scalp haematoma were identified as important clinical variables that can be used to predict TBI on a CT scan of paediatric minor head injury. The presence of these factors should alert emergency physicians to the need to monitor such children closely.
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Bueno Gomez, Andrea, Mary Healy, Grazia Delle Donne, et al. "P10 Initiation of bisoprolol in paediatric patients – experience from a specialist paediatric cardiac centre." Archives of Disease in Childhood 108, no. 5 (2023): 5.2–6. http://dx.doi.org/10.1136/archdischild-2023-nppg.9.

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AimGiven the lack of published dosing information to guide the use of bisoprolol in our paediatric cardiology patients, we assessed the safety and tolerability of initiation and maintenance doses of bisoprolol in a cohort of children with congenital structural heart disease, cardiomyopathy, or structural normal heart, to treat heart failure, arrhythmias or hypertension, and prevention of arrhythmias in channelopathies.MethodA retrospective review of hospital records of all children who received bisoprolol between May 2014 and August 2019 in a tertiary paediatric referral centre. Patients were identified via pharmacy records and clinical informatic systems were used to identify the indication, dose at initiation (mg/kg), up-titration dose, and any documented side effects.Results157 children (mean 11.6 years; range 2 months – 17.8 years) were identified. 56 children (36%) had a cardiomyopathy, 23 (15%) had structural heart disease and 78 (50%) were receiving bisoprolol with a structural normal heart for the prevention or treatment of arrhythmias. 149 children had a weight documented. The starting dose of bisoprolol ranged from 0.02 mg/kg/day to 0.19 mg/kg/day (mean 0.06 mg/kg/day); independent of the indication. The dose was up titrated in 90 (57%) of children. The weight was documented in 77 of these patients and up-titrated doses in these patients ranged from 0.02 mg/kg/day to 0.4 mg/kg/day (mean 0.14 mg/kg/day). A total of 24 (15%) of children had documented adverse events attributed to bisoprolol. Fatigue or dizziness were the most common reported adverse events, with 67% of the total events. In total, 5 children (3%) stopped bisoprolol due to adverse events; 4 due to fatigue or dizziness and 1 because of wheeze in a known asthmatic. HR was documented before and after initiation of bisoprolol in 89 patients (57%). The mean change was a decrease of 8 beats per minute (bpm) and all were within normal limits. Systolic blood pressure (BP) was documented before and after initiation of bisoprolol in 61 patients (39%). The mean change was a reduction of 4.2 millimeters of mercury (mmHg) and all were within normal limits.ConclusionOur experience shows that initiating bisoprolol in paediatric cardiology patients with a cardiomyopathy, structural heart disease and normal structural heart, to treat arrhythmias, heart failure, outflow tract obstruction and systemic hypertension at a dose of 0.06 mg/kg once a day, up to a maintenance dose of 0.14 mg/kg/day was safe. However, this is limited by the sample size and retrospective nature of the study. Further studies are needed to be able to comment on the efficacy in this cohort of patients.
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Egunsola, Oluwaseun, Imti Choonara, and Helen Sammons. "SAFETY OF LAMOTRIGINE IN PAEDIATRICS: A SYSTEMIC REVIEW." Archives of Disease in Childhood 101, no. 1 (2015): e1.68-e1. http://dx.doi.org/10.1136/archdischild-2015-310148.70.

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BackgroundLamotrigine has been increasingly used for the treatment of paediatric epilepsy. It is the most commonly prescribed new generation antiepileptic drug in the UK.ObjectivesTo identify adverse drug reactions associated with lamotrigine in children, compare the safety profile with other antiepileptic drugs and identify all clinical studies and case reports involving lamotrigine safety in children.MethodsDatabases EMBASE (1974-November 2013), Medline (1946-November 2013), PubMed and the Cochrane library for randomised controlled trials were searched for original research or reports in which paediatric patients received at least a single dose of lamotrigine for the treatment of epilepsy, with safety as an outcome measure.ResultsA total of 76 articles involving 2,184 paediatric patients were identified. There were 1,952 adverse events (AEs) reported. Fifty two of these AEs were from 49 case reports. Rash was most commonly reported, with a risk of 8.96 per 100 patients. Other common AEs included headache, fever, somnolence, vomiting, seizure aggravation, dizziness, cough, aggression, ataxia and insomnia. There were significantly higher risks of dizziness [RR 4.57, 95% CI: 1.88–11.12, p<0.001], abdominal pain [RR: 2.53, 95% CI: 1.12–5.70, p=0.03] and nausea [RR: 5.94, 95% CI: 1.59–22.13, p=0.008] with lamotrigine than placebo. When compared with valproic acid, the risk of somnolence and vomiting was significantly lower for lamotrigine [RR: 0.35, 95% CI: 0.13–0.89, p=0.04] and [RR: 0.20, 95% CI: 0.04–0.89, p=0.03]. Discontinuation due to an adverse drug reaction was recorded in 72 children (3.2% of all treated patients). Rash was the most common reason, with 58% of treatment discontinuation attributed to different forms of rash.ConclusionRash is the most common adverse reaction of lamotrigine, seen in around 9% of patients, and it is the most common reason for treatment discontinuation.
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5

Kandogan, Tolga, Olgun Levent, and Gultekin Gurol. "Complications of paediatric cochlear implantation: experience in Izmir." Journal of Laryngology & Otology 119, no. 8 (2005): 606–10. http://dx.doi.org/10.1258/0022215054516331.

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Surgery for cochlear implantation (CI) bears the risks of complication associated with all major surgery, in addition to the particular risks associated with implanting a foreign body into the peripheral auditory system. Here we present a retrospective study involving 227 cochlear implant operations in 205 children to evaluate the rate of intra- and post-operative complications.Complications were defined as major complications, requiring explantation of the device or further operation, causing a significant medical problem, or leading to any degree of facial paralysis or requiring additional hospitalization for treatment; or defined as minor complications, namely those that settled spontaneously, with conservative treatment, with local care and/or with medication alone.In our study there were 15 (6.6 per cent) minor and 28 (12.33 per cent) major complications. The most frequent minor complication was dizziness and vomiting (3.08 per cent), followed by transient hemifacial oedema (1.76 per cent), head pain (1.32 per cent) and mild ataxia (0.4 per cent). The most frequent major complication was trauma to the device (9.69 per cent), followed by cerebrospinal fluid (CSF) gusher (2.2 per cent) and facial paresis (0.4 per cent). All of the device trauma cases were re-implanted. There were neither any life-threatening complications nor any facial nerve paralysis in our implanted children.This study confirms that CI is relatively safe and that major complications are few and within acceptable limits.
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Akhter, Naznin, Iffat Ara Shamsad, Rumana Islam, et al. "Post COVID Guillain Barre Syndrome (GBS) in paediatric COVID unit of Dhaka Medical College: A case series." Journal of Dhaka Medical College 31, no. 2 (2024): 232–36. http://dx.doi.org/10.3329/jdmc.v31i2.73195.

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SARS Cov-2 infection presents with mild respiratory illness but has evidence of multisystem involvement in little percentage, though the severity of infection is variable in children. Neurological manifestations may vary from headache, dizziness, olfactory or taste dysfunction to specific syndromes including meningoencephalitis, stroke, and acute transverse myelitis and Guillain- Barre syndrome (GBS). But there is little evidence worldwide about neurological complications of COVID-19 in children. Here we reporting five cases of post COVID-19 GBS with relevant literature review. J Dhaka Med Coll. 2022; 31(2) : 232-236
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7

T. B., Keerthana, Anuradha S., Madanika S. V., Mahendrappa K. B., Kumar G. M., and Chandana A. "Indoxacarb poisoning induced methemoglobinemia-a rare presentation in paediatric age group." International Journal of Contemporary Pediatrics 10, no. 4 (2023): 576–78. http://dx.doi.org/10.18203/2349-3291.ijcp20230740.

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Indoxacarb, an oxadiazine insecticide, has high insecticidal and low mammalian toxicity. The insecticidal activity of indoxacarb is attributed to its ability to block sodium channels in the insect nervous system. Several cases of indoxacarb-induced methemoglobinemia have been reported in adults, but presentation in paediatric age group is rare. Methaemoglobinaemia occurs after indoxacarb ingestion because its aromatic metabolites are biotransformed to active intermediates that produce methaemoglobin. The case involved is an adolescent girl who presented to the PICU 3 hours after suicidal ingestion of 10-15 ml of indoxacarb. After 15 min of consumption of poison she developed pain abdomen and vomiting. At PICU, she complained of nausea, vomiting and dizziness. Her vital signs were heart rate 96/min, BP 110/80 mmHg, oxygen saturation 78% on room air and GCS 15/15. Despite receiving 10L/ min of oxygen via NRBM mask. Her ECG showed normal sinus rhythm without ischaemic change, and CXR showed no specific abnormality. Initial arterial blood gas analysis revealed pH-7.413, pO2-123.6 mmHg, pCO2-37.7 mmHg, HCO3 23.5 mmol/L. Other laboratory results were normal. Methaemoglobinemia is a rare clinical presentation of indoxacarb poisoning. The treatment plan includes timely administration of injection methylene blue.
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Prem, Govindan Nair, Karukayil Sivadas Gayathri, and Dorasala Ramanna Srinivas. "Benign paroxysmal vertigo of childhood: A case report." GSC Biological and Pharmaceutical Sciences 22, no. 2 (2023): 136–40. https://doi.org/10.5281/zenodo.7919506.

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Benign Paroxysmal Vertigo of Childhood (BPVC) is a disorder characterized by recurrent episodes of dizziness which are often sudden, occurring in the first decade of life, last a few seconds to one minute, recover completely after the episode and often would re-occur several times a month for several years. The present case study was to highlight the clinical features of BPVC in a 3 year old female child. Audiologists must be well aware of the typical features and clinical perspectives of BPVC. 
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9

Eidlitz-Markus, T., O. Gorali, Y. Haimi-Cohen, and A. Zeharia. "Symptoms of Migraine in The Paediatric Population by Age Group." Cephalalgia 28, no. 12 (2008): 1259–63. http://dx.doi.org/10.1111/j.1468-2982.2008.01668.x.

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The revised criteria of the International Headache Society (IHS) for paediatric headache do not differentiate among age groups. This study aims to determine if different symptoms of migraine are specific or typical of different age groups of children. The files of 160 children (79 boys, 81 girls, mean age 10.39 ± 3.71 years) with migraine treated at the paediatric headache clinic of a tertiary centre were reviewed. The diagnosis was based on the criteria of the IHS (ICHD-II). The patients were divided by age into three groups according to educational status, ≤6 years (preschool, group 1), >6 to ≤12 years (elementary school, group 2) and >12 to ≤18 years (secondary school, group 3), and compared by symptoms and signs. Symptoms of migraine with and without aura were also compared. There was no significant difference among the groups in rates of unilateral headache, phonophobia, photophobia, awakening pain, nausea or worsening of pain during physical activity. The parameters found to be statistically significant were dizziness and duration of migraine, and aura which increased with time. Frequency of attacks increased with age. The single statistically significant parameter found to be more frequent in younger age was vomiting. The statistically significant parameters of nausea and duration of migraine were more frequent in migraine with aura compared with migraine without aura. In conclusion, most of the migraine symptoms included in the 2004 recommendations of the IHS are not typical for specific paediatric age groups, probably because brain maturity is a continuous process. A familial history of migraine is a frequent finding among all age groups and should be considered in the paediatric criteria, especially in younger children in whom diagnosis is more difficult. Vomiting may help the diagnosis of migraine in young children with a familial history of migraine.
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Murray, Nathan, Utsav Malla, Ruan Vlok, et al. "Buprenorphine versus Morphine in Paediatric Acute Pain: A Systematic Review and Meta-Analysis." Critical Care Research and Practice 2018 (August 7, 2018): 1–5. http://dx.doi.org/10.1155/2018/3792043.

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Introduction. In lab-based studies, buprenorphine appears to have a ceiling effect on respiratory depression but not on analgesia. There is increasing evidence in adult patients that buprenorphine has no ceiling effect on analgesia or side effects. The aim of this study was to investigate the efficacy and adverse effects of buprenorphine versus morphine in paediatric acute pain. Methods. A systematic review of five databases was performed until May 2018. Only randomised controlled trials were eligible for inclusion. The outcomes of interest included pain, respiratory depression, nausea, sedation, dizziness, and pruritus. Results. Four randomised controlled trials (n=195) were included. The only outcome measuring analgesic efficacy was time to breakthrough analgesia. Buprenorphine had a significant increase in time to breakthrough analgesia by 114.98 minutes compared to morphine (95% CI = 42.94 to 187.01; I2 = 0; p=0.002). There was no significant difference in the rates of adverse effects. Conclusions. Buprenorphine provided a longer duration of analgesia than morphine. This in combination with its unique sublingual preparation could prove particularly advantageous in the paediatric population. The studies included are likely underpowered to detect differences in the incidence of adverse effects; therefore, the same precautions should be taken as with any other opioid.
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Mahmood, Ashraf Nabeel, Osama Abulaban, and Arshad Janjua. "(Doctor…My child keeps falling over) unexpected MRI findings in children with history of frequent falls and dizziness: a case series." BMJ Case Reports 12, no. 7 (2019): e229849. http://dx.doi.org/10.1136/bcr-2019-229849.

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Frequent falls and dizziness are common complaints in children. These symptoms can be caused by wide range of underlying pathologies including peripheral vestibular deficits, cardiac disease, central lesions, motor skills delay and psychogenic disorders. We report three paediatric cases who presented with complaints of repeated falls and imbalance. MRI scan revealed underlying brain lesions (frontal lobe arteriovenous malformation, exophytic brain stem glioma and cerebellomedullary angle arachnoid cyst with cerebellar tonsillar ectopia). By reporting these cases, we would like to emphasise the importance of a thorough assessment of children with similar symptoms by detailed clinical history, physical examination and maintaining low threshold for investigations, including radiological imaging. Taking in consideration, the wide range of differential diagnosis, the challenge of obtaining detailed history and difficulty of performing reliable physical examination in this age group. Management of underlying disorders can be medical, surgical or just observational.
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Pasha, Waseem, Awais Amjad, Imran Ahmed, Usman Nawaz, Muhammad Usman Sajid, and Muhammad Usman Ali. "COMPARISON OF INTRAVENOUS DIAZEPAM AND INTRAVENOUS MIDAZOLAM FOR TREATMENT OF RECURRENT FEBRILE SEIZURES." Pakistan Journal of Physiology 20, no. 1 (2024): 12–14. http://dx.doi.org/10.69656/pjp.v20i1.1597.

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Background: Recurrent febrile seizures are seizures in children less than 5 years of age which reoccur within 24 hours. Benzodiazepines such as midazolam and diazepam are used to stop and decrease the recurrent rate of febrile seizures. The objective of study was to compare the efficacy of intravenous midazolam with intravenous diazepam for treatment of recurrent febrile seizures in paediatric patients. Methods: This observational cross-sectional study observed 60 children aged 6 to 59 months with recurrent febrile seizures who presented at paediatric clinic in Combined Military Hospital Kharian and THQ Hospital, Kharian from Sep to Nov 2023. The response time was noted for children who received intravenous midazolam 0.2 mg/Kg only and IV diazepam 0.2 mg/Kg only. The children were randomly allocated into group A (midazolam group) and Group B (diazepam group.) Study was done after taking informed consent from the parents. Both drugs were given during the fits. Results: The time interval from drug administration to cessation of febrile seizures was 2.50±0.94 minutes in the midazolam group and 2.4±1.12 minutes in the diazepam group. There was no significant difference between the two groups (p=0.567). Minor dizziness and sedation were the only main effects reported. Conclusion: Both diazepam and midazolam have same efficacy in the treatment of recurrent febrile seizures. Pak J Physiol 2024;20(1):12?4
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Koren, Tal Jonatan, and Matthew C. Kiernan. "Post-infectious acute cerebellar ataxia in a young adult." BMJ Case Reports 18, no. 1 (2025): e262043. https://doi.org/10.1136/bcr-2024-262043.

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Acute cerebellar ataxia is a clinical syndrome that involves loss of balance and coordination, typically within less than 72 hours. It usually presents in children and rarely affect adults. A woman in her early 20s presented with acute onset dizziness, vertigo, truncal ataxia and dysarthria 2 weeks following an acute viral illness. Cerebral MRI identified evidence of dural enhancement, and positron emission topography brain imaging showed cortical reduction of glucose metabolism, consistent with an encephalopathic process. Lumbar puncture established a normal cerebrospinal fluid protein and glucose, with seven white cells ×106/L. Subsequent investigations identified evidence of a recentEpstein–Bar virus, in keeping with a post-infectious acute cerebellar ataxia syndrome. Following treatment with intravenous methylprednisolone, symptoms resolved gradually over months. While post-infectious acute cerebellar ataxia is rare in adults, it is an important cause of an acute presentation of ataxia that should be recognised by paediatric and adult physicians.
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Cortez, Gustavo M., Andre Monteiro, Benjamin Ludwig, and Ricardo Hanel. "Reappraisal of haemorrhagic suprasellar pilocytic astrocytoma during adulthood." BMJ Case Reports 13, no. 10 (2020): e235662. http://dx.doi.org/10.1136/bcr-2020-235662.

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Pilocytic astrocytomas comprise the most common central nervous system tumour during childhood and have an excellent response to surgical treatment in this population. The tumour incidence decreases with age, whereas more aggressive behaviour tends to increase. Haemorrhage as a presenting feature of pilocytic astrocytomas is a rare phenomenon, especially in the adult population. We present a case of a 55-year-old patient with progressive headaches and dizziness. MRI confirmed a sellar and predominantly retrochiasmal suprasellar lesion with heterogeneous signal, enhancement and blood products. Management via transsphenoidal approach was performed, and histopathology revealed the unexpected diagnosis of haemorrhagic pilocytic astrocytoma. Haemorrhagic pilocytic astrocytoma is an infrequent entity in the adult population and it is essential to recognise the peculiarities regarding diagnostic evaluation and management, which differ from the paediatric population. During adulthood, this tumour carries an overall unfavourable prognosis, with higher rates of progression and recurrence.
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Dhamija, B., D. Kombogiorgas, I. Hussain, and G. A. Solanki. "Posterior Fossa Arachnoid Cyst Masking a Delayed Diagnosis of Hyperparathyroidism in a Child." Case Reports in Endocrinology 2012 (2012): 1–4. http://dx.doi.org/10.1155/2012/931371.

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Background. Primary hyperparathyroidism in childhood is a very rare entity, often being diagnosed late after the onset of its presenting symptoms. It most commonly affects patients in their fourth decade of life and beyond. The inclusion of primary hyperparathyroidism in the differential diagnosis is necessary when evaluating patients presenting with nonspecific symptoms such as polyuria, fatigue, weight loss, abdominal pain, nausea, and vomiting.Methods. We report the case of an eleven-year-old girl presenting with three years history of headaches, visual disturbance, along with episodes of emotional lability. Neuroimaging confirmed a large posterior fossa arachnoid cyst. It was decided to manage this lesion conservatively with surveillance. Only after further hospital admissions with recurrent loss of consciousness, dizziness, and nausea to add to her already existing symptoms, a full biochemical and endocrine assessment was performed to look for more specific causes for her presentation. These pointed to a diagnosis of primary hyperparathyroidism.Conclusions. The inclusion of primary hyperparathyroidism in the differential diagnosis should be considered when evaluating paediatric patients presenting with nonspecific (neurological, gastrointestinal, and renal) symptoms in order to establish a prompt diagnosis of the disorder and to avoid severe complications of prolonged hypercalcaemia and end-organ damage.
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Wang, Yufeng, Yi Zheng, Yasong Du, et al. "Atomoxetine versus Methylphenidate in Paediatric Outpatients with Attention Deficit Hyperactivity Disorder: A Randomized, Double-Blind Comparison Trial." Australian & New Zealand Journal of Psychiatry 41, no. 3 (2007): 222–30. http://dx.doi.org/10.1080/00048670601057767.

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Objective: To (i) test whether atomoxetine is non-inferior to methylphenidate in treating symptoms of attention deficit hyperactivity disorder (ADHD) in paediatric patients; and (ii) determine the tolerability of the two drugs. Method: This double-blind study was conducted in 6- to 16-year-old outpatients with ADHD (DSM-IV) in China, Korea and Mexico (January–October 2004). Patients were randomly assigned to once-daily atomoxetine (0.8–1.8 mg kg−1 day−1; n = 164) or twice-daily methylphenidate (0.2–0.6 mg kg−1 day−1; n = 166) for ∼8 weeks. Primary efficacy assessment was the comparison of response rates (≥40% reduction from baseline to end point in total score) on the Attention Deficit Hyperactivity Disorder Rating Scale-IV-Parent Version: Investigator-Administered and -Scored. Tolerability measures included, but were not limited to, the assessment of treatment-emergent adverse events (TEAEs) and weight. Results: Atomoxetine was non-inferior to methylphenidate in improving ADHD symptoms based on response rates (atomoxetine, 77.4%; methylphenidate, 81.5%; one-sided 95% lower confidence limit = −11.7%, p = 0.404). Treatment-emergent adverse effects experienced significantly more frequently in the atomoxetine group, compared with the methylphenidate group, included anorexia (37.2% vs. 25.3%; p = 0.024), nausea (20.1% vs. 10.2%; p = 0.014), somnolence (26.2% vs. 3.6%; p <0.001), dizziness (15.2% vs. 7.2%; p = 0.024) and vomiting (11.6% vs. 3.6%; p = 0.007), most of which were of mild or moderate severity. Atomoxetine-treated patients experienced a small but significantly greater mean weight loss from baseline to end point than methylphenidate-treated patients (−1.2 kg vs. −0.4 kg; p <0.001). Conclusions: This study suggests that atomoxetine is non-inferior to methylphenidate in the improvement of ADHD symptoms in paediatric outpatients. Although both of the drugs were well tolerated, atomoxetine was associated with a higher incidence of TEAEs than methylphenidate.
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Hookham, Lauren, Hillary C. Lee, Divya A. Patel, et al. "Vaccinating Children against SARS-CoV-2: A Literature Review and Survey of International Experts to Assess Safety, Efficacy and Perceptions of Vaccine Use in Children." Vaccines 11, no. 1 (2022): 78. http://dx.doi.org/10.3390/vaccines11010078.

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Introduction: The balance of risks and benefits of COVID-19 vaccination in children is more complex than in adults with limited paediatric data resulting in no global consensus on whether all healthy children should be vaccinated. We sought to assess the safety, efficacy, and effectiveness of childhood vaccination against SARS-CoV-2, as well as better understanding perceptions of vaccination in parents and vaccine experts. Methods: We performed a literature review for COVID-19 vaccine safety, efficacy, effectiveness, and perceptions. We searched international safety databases for safety data and developed an electronic survey to elicit country-specific COVID-19 immunisation data, including vaccine regulations, policies, rates, and public attitudes solicited from vaccine experts. Results: Nine studies were included in the final safety analysis. Local reactions were frequently reported across all studies and vaccine types. Adverse events reported to surveillance systems tended to be non-serious, and commonly included injection site reactions and dizziness. Twenty-three studies reported immunogenicity, efficacy, and effectiveness data. There were nine randomised control trials of six different vaccine types, which showed seroconversion of neutralising antibodies in vaccinated children ranging from 88% to 100%. The vaccine efficacy for Pfizer and Moderna vaccines ranged from 88% to 100%. There were 118 survey responses representing 55 different countries. Reported vaccination rates ranged from <1% to 98%. Most respondents described “mixed opinions” regarding paediatric vaccination policies in their country. By region, a more positive public attitude towards vaccination correlated with higher vaccination rates. Discussion: In this mixed-methods review, we have found evidence that vaccination against COVID-19 in children is safe, efficacious, and effective. Overall, the combined evidence from both the literature review and survey highlights the need for further data on both the safety and effectiveness of COVID-19 vaccinations in children.
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Jayasekera, Priyamali, Gayani Dassanayake, Kasthuri Bandara, Lakmali Jayawardhena, and K. M. S. Malkanthi. "A Study of the Pattern of Admissions to the Accident and Emergency (A&E) Department of a Tertiary Care Hospital in Sri Lanka." Emergency Medicine International 2020 (August 13, 2020): 1–12. http://dx.doi.org/10.1155/2020/6327293.

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Introduction. The latest national healthcare reform policies of Sri Lanka include the development of accident and emergency (A&E) departments in all major hospitals. Provincial General Hospital Kurunegala (PGHK) is a home to the first established A&E department in Sri Lanka. PGHK provides services to a population of 2.4 million spread out in the North Western Province and part of the Sabaragamuwa Province. This study was carried out to identify the pattern of all admissions to the A&E department of PGHK. Methods. The prospective observational study was carried out from July 1, 2016, to June 30, 2017 (one year) to identify the pattern of admissions to the A&E department. Results. There were 49,213 admissions to PGHK’s A&E department during the study period. The average number of admissions was 135 (±17.9) per day. The percentage of deaths in the A&E department was 0.21%. The mean age of admitted patients was 46.7 (±21.7) years. A further 62% of admitted patients were males. The number of medical, surgical, paediatric, and gynaecological and obstetrical admissions was 55%, 42%, 3.5%, and 0.22%, respectively. Among the common emergency medical presentations, 34% were chest pain, 11% patients presented with unilateral weakness and/or slurring of speech, 10% covered dyspnoea, and 9% complained of dizziness/giddiness. Among emergency surgical presentations, 83% were trauma due to accidents, of which 27% were road-traffic-related accidents (RTAs). Discussion. The A&E department of PGHK provides services to a significantly high number of health emergencies every day. The majority of these admissions was due to chest pain and trauma related to accidents. The lower recorded number of paediatric and gynaecological and obstetrical emergencies presented to the A&E department is a result of a government policy mandating the admission of these types of patients directly to their respective wards. Further infrastructure development, staff recruitment, and training have to be planned and implemented to address the significantly high number of admissions to the A&E Department of PGHK.
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Anwar, T. T. Muhammed, P. P. Muhamed Faris, and ,. Thansiha. "A Systematic Review on Lamotrigine Induced Skin Rashes." Journal of Drug Delivery and Therapeutics 11, no. 1 (2021): 146–51. http://dx.doi.org/10.22270/jddt.v11i1.4633.

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Lamotrigine is a newer anti-epileptic drug synthesised in 1980s and approved by USFDA in 1994. It is mainly used for treating paediatric epilepsy and one of the frequently prescribing newer anti- epileptic drugs in European countries. In English scenario, lamotrigine is suggested as monotherapy for recently identified partial seizures (localized seizure) in children as its first line drug. Lamotrigine is recommended for generalized seizures as second line drug and as add on therapy in intractable generalized epilepsy. It is a treatment option after sodium valproate and ethosuximide for petit-mal epilepsy. It can be used as either single or in combination with other anti-epileptic drugs. It has lot of adverse effects like other anti-epileptic drugs. Major adverse effect associated with lamotrigine is skin rashes in newly administered patients up to 8 weeks. About 3-10 % of the patients experience skin rashes in their therapy with lamotrigine. Lamotrigine doses should be titrated properly in combination with other anti-epileptic drugs especially with valproic acid. Other less common adverse effects with lamotrigine include dizziness, ataxia, blurred vision, headache, nausea and diplopia. Moreover, lamotrigine has a safe drug profile and effective in both children and adults for the treatment of various types of seizures. The dose escalation should be monitored regularly to avoid serious adverse reactions.
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Klein, A., D. Windschall, W. Emminger, et al. "POS1202 EXPERIENCE WITH COVID-19 IN GERMAN PAEDIATRIC RHEUMATOLOGY CENTRES." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 883.2–884. http://dx.doi.org/10.1136/annrheumdis-2021-eular.2045.

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Background:COVID-19 is a major challenge worldwide. Although the risk for a severe disease course is low among children with COVID-19, symptoms may be exacerbated by underlying disease and/or immunosuppressive medication. We analysed clinical data from COVID-19 cases in among pediatric patients with juvenile idiopathic arthritis (JIA) in Germany reported to the BIKER registry.Objectives:This is an analysis of clinical data for 56 COVID-19 cases reported to the German BIKER registry from 29 German pediatric rheumatology centers and clinics from February 2020 to January 2021.Methods:The major task of the German BIKER (Biologics in Paediatric Rheumatology) Registry is surveillance of biologics used in pediatric rheumatology patients. Following the start of the COVID-19 pandemic in Germany, a survey was established to proactively interview all participating centers regarding the occurrence, presentation and outcome of SARS-CoV-2-infected children with rheumatic diseases. Initially, the interviews were conducted in weekly intervals, later bi-weekly.A standardized Adverse Event of Special Interest form was developed requesting biographic data, pre-treatment, current medication, data on clinical presentation, course, treatment and outcome of COVID-19 pediatric rheumatology patients.Results:In all, 56 patients with JIA and SARS-CoV-2 infection were reported (Table 1). Of these patients, 71% were 12 or more years old.Table 1.Patient characteristics. COVID-19 positive patients.JIA patients, n=56n (%)Age 0-5 years / 6-11years / 12-18years3 (5.4) / 13 (23.2) / 40 (71.4)JIA category•Systemic JIA5 (8.9)•Oligoarthritis JIA9 (16)•Polyarticular JIA32 (57)•Enthesitis-related JIA2 (3.6)•Psoriatic JIA1 (1.8)•Unknown7 (12.5)Uveitis (concomitant)4 (7.1)Treatment•DMARD / MTX23/ 22 (41/39)•Biologics29 (52)•TNF inhibitors20 (36)•Tocilizumab5 (8.9)•Abatacept1 (1.8)•Anakinra1 (1.8)•Ustekinumab1 (1.8)•JAK inhibitors1 (1.8)•Steroids5 (8.9)Asymptomatic13 (23.2)Hospitalized/ICU/Ventilation/Death1/1/1/1 (1.8)At the time of infection, 41% of the patients received conventional DMARDs and 52% received biologics (Table 1). Forty-four patients (79%) received either a conventional DMARD or a biologic. Most patients had a polyarticular course of their JIA (57%).In 49 of the 56 cases (88%) COVID-19 was detected directly by PCR (n=46), by antigen test only (n=1) or an undisclosed method (n= 2). Six patients had detectable SARS-CoV2 antibodies and reported to have had typical symptoms. One patient tested negative but developed typical symptoms at approximately the same time a positive SARS-CoV-2 test was returned for a family member.Symptoms were reported in 43 of the 56 patients (77%): fever n=15, rhinitis n=14, cough n=12, headache n=10, loss of sense of taste and/or smell n=9, pharyngitis n=8, fatigue n=5, musculoskeletal pain n=5, GI symptoms n=2 (abdominal pain n=1, diarrhoea n=1), dizziness n=3, encephalitis/seizure/respiratory failure/death n=1. Thirteen patients (23%) were asymptomatic.A 3½ -year-old female patient initially diagnosed with systemic JIA developed intracranial oedema and respiratory failure. Her SARS-CoV2 PCR test was positive and pulmonary imaging displayed typical changes in lung texture. Before her SARS-CoV-2 infection, the patient was treated with methotrexate and low-dose steroids. Unfortunately, she died three days following hospital admission. Genetic testing revealed an inborn immunodeficiency. Except for this one patient, all other cases were treated as outpatients and no deaths were reported.Conclusion:Apart from one patient with an inborn immunodeficiency who died from her COVID-19 infection, no case of hospitalization or severe COVID-19 was reported in our cohort of JIA patients. At the time of COVID-19 diagnosis, nearly 80% of patients in our cohort had been treated with conventional DMARD and/or biologics. This seemed not to have a negative effect on severity or outcome of SARS-CoV2 infection.Acknowledgements:Thanks also for contributing Reports for this analysis to: Normi Brück, Frank Dressler, Ivan Foeldvari, Tilman Geikowski, Hermann Girschick, Johannes-Peter Haas, Tilmann Kallinich, Bernd-Ulrich Keck, Eggert Lilienthal, Anna-Hedrich Müller, Ulrich Neudorf, Nils Onken, Peggy Rühmer.Disclosure of Interests:None declared.
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Lembryk, I. S., I. V. Shlimkevych, A. B. Stefanyshyn, O. V. Zhyliak та N. I. Kostyrko. "Features of pyelonephritis course in adolescent females with co-existing anаemic syndrome". CHILD`S HEALTH 18, № 6 (2023): 423–29. http://dx.doi.org/10.22141/2224-0551.18.6.2023.1629.

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Background. Anaemic syndrome of complex origin is not uncommon in urinary tract infection, particularly in dietary non-adherence, menstrual cycle disorders, or concomitant digestive diseases, recurrent respiratory infections, etc. However, there is currently insufficient epidemiological data on this comorbidity in the literature. This study aims to establish the features of pyelonephritis course in its combination with anaemic syndrome in children. Materials and methods. We analysed the medical records of 200 children aged 0 to 17 years with acute non-complicated pyelonephritis, complicated and recurrent urinary tract infections for 2012–2017. In the second stage of our work, we comprehensively examined 85 girls aged 11 to 17 who underwent inpatient treatment between 2018 and March 2023. Thirty children of the same age and gender made up the comparison group. A paediatric gastroenterologist, a paediatric gynaecologist and/or urologist examined all patients. Immunochemical method with electrochemiluminescence immunoassay was used to assess ferritin content; also, serum iron and total iron-binding capacity were measured. Results. Anaemic syndrome is common in most girls with urinary tract infections (58.8 % of сases). Among the causes, the follo­wing are distinguished: menstrual cycle disorders with abnormal uterine bleeding (50.0 %), chronic digestive disorders, in particular malabsorption syndrome of unknown origin (25.0 %), recurrent bronchopulmonary disorders (15.0 %), and unbalanced diet (10.0 %). The features of urinary tract infections and concomitant iron deficiency in children include fatigue (55.0 % in acute non-complicated pyelonephritis vs. 40.0 % in complicated urinary tract infections, р < 0.05), dizziness (35.0 % in acute non-complica­ted pyelonephritis vs. 15.0 % in recurrent urinary tract infections, χ2 = 48.6532, р < 0.05), and pallor (25.0 % in complicated urinary tract infections vs. 10 % in recurrent urinary tract infections, χ2 = 0.7168, р > 0.05). The ferritin level was the highest in patients with complicated urinary tract infections (18.2 µg/mL). In patients with recurrent urinary tract infections, this indicator was the lo­west — 5.0 µg/mL. Conclusions. Retrospective analysis of medical records confirmed the presence of anaemia in 30.0 % of girls with inflammatory kidney diseases. During the physical examination, 58.8 % of female adolescents with urinary tract infections had signs of anaemic syndrome.
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Josef, Finsterer. "The Assessment of Neurological Complications in Paediatric SARS-CoV-2 Infections Requires Appropriately Designed Studies." Sarcouncil journal of Medical sciences 3, no. 3 (2024): 1–2. https://doi.org/10.5281/zenodo.10798338.

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We read with interest Han <em>et al</em>.&rsquo;s article on a retrospective, observational, single-centre study of the neurological complications of acute SARS-CoV-2 infections in 455 paediatric patients admitted to a tertiary Hospital in Singapore from December 2021 to June 2022 [Han, V. X. <em>et al</em>., 2023]. The study is impressive, but some points require discussion.&nbsp; The major limitation if the study is its retrospective design. A retrospective design does not allow control of the accuracy of the stored data, does not systematically apply the same examinations to all included patients, produces missing data, does not allow filling in missing data, and is not suitable for generating desirable new data.&nbsp; A second limitation is the inclusion/exclusion criteria [Han, V. X. <em>et al</em>., 2023]. The study population is not representative because those aged &gt;3 months to 18 years were admitted only if there was an appropriate indication (e.g. seizure, croup, dehydration, respiratory distress) [Han, V. X. <em>et al</em>., 2023]. Since neurological complications are not necessarily associated with the severity of a SARS-CoV-2 infection, it is conceivable that many neurological complications were missed. Furthermore, it is unclear why patients with MIS-C were excluded from the analysis. MIS-C is a complication of acute SARS-CoV-2 infections and can be associated with neurological impairments.&nbsp; Another limitation is that no subgroup analysis of vaccinated versus unvaccinated patients was performed. It is conceivable that the frequency, spectrum, and degree of neurological complications differed between the two groups in the sense that those vaccinated had fewer and less severe neurological complications.&nbsp; Since headaches can be primary or secondary, we should know the cause of headache in every patient with this symptom. How many had their headache secondary to stroke, venous sinus thrombosis, reversible, cerebral vasoconstriction syndrome, hypertension, or meningitis/encephalitis? Have these causes been adequately ruled out in all patients with headache? Were imaging and CSF tests performed on patients in whom the cause of headache remained unclear? Since dizziness is nonspecific and multicausal, we should know in how many cases it was due to the medications used, to otological involvement, and in how many cases to central nervous system or peripheral nervous system involvement in SARS-CoV-2 infection. It is incomprehensible how hospitalised patients could be compared with non-hospitalised patients. There were no records of nonhospitalised patients. This discrepancy should be explained.&nbsp; In summary, the frequency, type, and severity of neurological complications in SARS-CoV2 infections in paediatric patients can only be reliably assessed through an appropriate prospective, multicentre design with comprehensible inclusion/exclusion criteria. &nbsp;
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Monga, Suneeta, Natasha Saunders, Shivali Kapila, Evelyn Smith, Erin Romanchych, and Jordana Waxman. "Implementing an integrated care pathway for Somatic Symptoms and Related Disorders (SSRDs) in paediatric population." International Journal of Integrated Care 23, S1 (2023): 236. http://dx.doi.org/10.5334/ijic.icic23089.

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Introduction: Somatic symptom and related disorders (SSRDs) are commonly encountered in pediatric hospital settings. These children and youth, who present with physical symptoms (e.g., headache, abdominal pain, dizziness) of emotional distress (e.g., anxiety, stress) are diagnostically and psychosocially complex, posing significant challenges for medical and behavioral health care providers. Children and adolescents with SSRDs have high health system utilization and costs and present in multiple clinical settings, have high school absenteeism, and are at elevated risk of iatrogenic harm and missed diagnoses. Patients and their caregivers present with variable readiness to engage in mental health assessment and treatment, with only 40% receiving physician-based mental health care within a year of diagnosis. There is a lack of standardization of care across and within institutions for youth with these disorders. As part of the hospital’s broader strategic direction to integrate physical and mental health care, a SSRD pathway, was developed to address care needs of one of the highest resource intensive populations at a tertiary care hospital. &#x0D; Methods: In 2021, a survey was conducted by our hospital’s implementation team to understand hospital stakeholder priorities when considering the many areas where new integrated care project could be launched. Stakeholders engaged in the survey included physician and operational leaders, clinical staff across the spectrum of disciplines, and a youth and family advisory panel. Focus groups (n = 57) were also conducted to solicit input and feedback. The results highlighted the need to develop a specialized care pathway for children and youth with SSRD focused on integrating physical and mental health care and support with system navigation. Using a logic model with iterative presentations to key stakeholders, the design phase included an interdisciplinary team approach with project management and operational resources to support project implementation and evaluation. Care pathways were developed based on a patient’s readiness to engage in mental health treatment and where they we along their diagnostic odyssey and care journey and intensity of resources required to support their care needs.&#x0D; Integrated Care Pathway: As part of the pilot pathway, a patient’s care journey will be planned in accordance with their level of readiness to accept both a somatization diagnosis and a need for mental health treatment. Patients who are partially or non-receptive to mental health supports will have access to a care manager with both physical and mental health expertise. The care manager will engage with families in shifting their focus and understanding about the mind-body connection and provide extended support with care navigation. Patients who are receptive to mental health treatments, will be referred to a group treatment program led by psychiatry and psychology with a focus on psychoeducation relating to the mind-body connection. Patients in this pathway will also have access to occupational therapy and physiotherapy resources to support with restoring function and improving school attendance, when needed. Pilot evaluation will include process and outcome measures such as number of children engaged in this pathway, time to return to functioning, completion of various mental health measures, and health resource utilization."
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Abumehdi, Mohammad R., Andrew J. Wardle, Rewa Nazzal, et al. "Feasibility and safety of cardiopulmonary exercise testing in children with pulmonary hypertension." Cardiology in the Young 26, no. 6 (2015): 1144–50. http://dx.doi.org/10.1017/s1047951115001961.

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AbstractBackgroundCardiopulmonary exercise testing helps prognosticate and guide treatment in adults with pulmonary hypertension. Concerns regarding its feasibility and safety limit its use in children with pulmonary hypertension. We aimed to assess the feasibility and safety of cardiopulmonary exercise testing in a large paediatric pulmonary hypertension cohort.MethodsWe reviewed all consecutive cardiopulmonary exercise tests performed between March, 2004 and November, 2013. The exclusion criteria were as follows: height &lt;120 cm, World Health Organization class IV, history of exercise-induced syncope, or significant ischaemia/arrhythmias. Significant events recorded were as follows: patient-reported symptoms, arrhythmias, electrocardiogram abnormalities, and abnormal responses of arterial O2 saturation.ResultsA total of 98 children underwent 167 cardiopulmonary exercise tests. The median age was 14 years (inter-quartile range 10–15 years). Peak oxygen uptake was 20.4±7.3 ml/kg/minute, corresponding to 51.8±18.3% of the predicted value. Peak respiratory quotient was 1.08±0.16. All the tests except two were maximal, being terminated prematurely for clinical reasons. Baseline Oxygen saturation was 93.3±8.8% and was 81.2±19.5% at peak exercise. A drop in arterial O2 saturation &gt;20% was observed in 23.5% of the patients. Moreover, five patients (3.0%) experienced dizziness, one requiring termination of cardiopulmonary exercise testing; five children (3.0%) experienced chest pain, with early cardiopulmonary exercise test termination in one patient. No significant arrhythmias or electrocardiogram changes were observed.ConclusionExercise testing in non-severely symptomatic children with pulmonary hypertension is safe and practical, and can be performed in a large number of children with pulmonary hypertension in a controlled environment with an experienced team. Side-effects were not serious and were resolved promptly with test termination.
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Alkindi, Nour, Abdurahman Andijani, Samara Zavalkoff, and Karl Muchantef. "C4 (Clinical Case) Vascular Ehlers-Danlos Syndrome in the Paediatric Population: A Case Report of an Internal Bleed Caused by Ruptured Pseudoaneurysm." Paediatrics & Child Health 28, Supplement_1 (2023): e50-e51. http://dx.doi.org/10.1093/pch/pxad055.105.

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Abstract Introduction/Background Ehlers-Danols syndrome (EDS) is a connective tissue disease characterized by joint hypermobility, skin hyperextensibility and tissue fragility. EDS has six types. Type IV EDS: vascular EDS (vEDS), is rare but also the most malignant. Case Description We report a 15-year-old boy with a previous history of spontaneous colon perforation and two left shoulder dislocations. He presented with sudden onset abdominal pain, vomiting, dizziness, and lethargy with a Glasgow coma scale of 13. His abdomen was soft but mildly tender. He had a capillary refill of 3 seconds with cold extremities, was found to be in a hypotensive shock state requiring resuscitation with fluids and inotropes, as well as intubation and mechanical ventilation. His hemoglobin dropped by 30 g/L in 6 hours. Therefore, an abdominal ultrasound was done, revealing an intrabdominal hematoma. CT scan of the abdomen confirmed a ruptured fusiform pseudoaneurysm of the accessory left hepatic artery. This was successfully managed by coil embolization, see F1 and F2, below. Genetic workup was positive for the pathogenic COL3A1 gene that presents phenotypically as Ehlers-Danlos syndrome, particularly type IV. Discussion COL3A1 encodes pro-alpha1 chains of type III collagen. Inheritance is autosomal-dominant. It can present as a de novo variant with a negative family history, as was the situation for our patient. Estimated affected people with de novo variant vEDS is 33%-50%. Clinical diagnosis of vEDS involves one major and several minor criteria (2017). Major criteria include arterial aneurysms, dissection, or rupture; intestinal rupture; uterine rupture during pregnancy; family history of vEDS. Minor criteria include easy bruising; thin skin and visible veins; facial characteristics including prominent eyes, narrow nasal bridge, thin lips; club foot, congenital hip dislocation; inguinal hernia and pneumothorax. Our patient had two elements from the major criteria: arterial pseudoaneurysm and intestinal rupture. Conclusion vEDS has high morbidity and mortality due to the risk of spontaneous internal organ/vessel injury. This can be mitigated by increased awareness of systemic manifestations of vEDS and a high index of suspicion by both primary care and emergency physicians. Once diagnosed, surveillance is crucial, including regular blood pressure monitoring, frequent non-invasive imaging to identify emerging vascular lesions, and pregnancy counselling for potential uterine rupture and avoiding trauma, collision sports, and lifting heavy items.
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Gupta, S., L. Brazil, A. Swampillai, and O. Al-Salihi. "P11.38.A Case reports on aggressive Pilocytic astrocytoma in TYA and adults: patient tailored approach or standard of care?" Neuro-Oncology 24, Supplement_2 (2022): ii66. http://dx.doi.org/10.1093/neuonc/noac174.227.

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Abstract Background Pilocytic astrocytoma (piloA) is a low grade brain tumour and most of the literature is based on paediatric patients with limited data on adults. Surgical resection followed by adjuvant treatment for recurrent lesions is standard of care. While previous literature was mainly based on historical classification, molecular analysis of tumour is adding to the subgroup classification. Material and Methods We are reporting 3 cases of adult piloA treated at our institute. Results: Case1 31y female with headaches and visual problems was diagnosed with optic nerve glioma, biopsy confirmed piloA, and was treated with conventional radiotherapy 50Gy/30fr. A month later developed CSF dissemination requiring 10xTemozolamide. Two years later, developed L5 and S2 deposits which were resected and confirmed piloA. 5 years later there was progression of primary disease treated with 12xTemozolamide and a year later developed further progression and hydrocephalus. She received 1xPCV and succumbed to disease at the age of 42. Case 2: 29y female with severe dizziness on moving head, worsening headaches, word finding difficulty, and positive family history of anaplastic astrocytoma, was diagnosed with Tectal plate lesion. Surgical debulking was performed and pathology showed piloA with Methylated MGMT promoter, IDH wild-type, H3K27M negative, and no BRAF fusion. 3 years later developed recurrence which was treated with chemotherapy (5xPCV: 6th omitted due to recurrent infections) as patient was reluctant for radiotherapy. Follow up scans 6 months post chemotherapy remain stable. Case 3: 24y female with background of 2 haemorrhagic strokes, left spastic hemi-paralysis and left hemianopia, and negative family history, had a biopsy performed for Right Mesotemopral Cavenous Angioma. Pathology showed low grade neoplasm favouring Ganglioglioma with CD34 and IDH1 negative, ATRX retained, KI-67 focal index 5%, and no BRAF fusion or mutation. Surveillance imaging showed progressively increasing suprasellar mass and right temporal region enhancement which was surgically debulked at the age of 27. Repeat pathology examination favoured piloA considering the radiological progression. Methylation array failed but immunohistochemistry showed tumour cells positive with synaptophysin, ATRX and trimethylated H3K27 retained, H3K27M, NeuN, H3G34R and CD34 negative, p53 negative, and Ki-67 3-4%. Repeat imaging 10 months later showed further progressive suprasellar tumour and enhancing lesion in the right temporal area accompanied by rapid loss of right side vision. We have planned for radical radiotherapy 54Gy/30fr with IMRT. Conclusion There is a gap in management of piloA in adults with treatment extrapolated from paediatrics and does require further research. We want to approach other centres to establish consensus on treating such cases.
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Vadday, Komal. "P02 Use of intrathecal fluorescein to identify cerebrospinal fluid (CSF) rhinorrhea in paediatrics: a case report and literature review." Archives of Disease in Childhood 107, no. 5 (2022): e25.3-e25. http://dx.doi.org/10.1136/archdischild-2022-nppg.11.

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Early identification of CSF rhinorrhoea can reduce the risk of meningitis and potentially decrease the length of hospital stay. To determine the exact site of leak, intrathecal fluorescein (IF) is frequently used as a diagnostic tool adjunct to repair surgery in rhinorrhea. Although this is generally considered safe, there is a slight risk of seizures, radicular symptoms such as numbness and transient paraparesis.1Miss. AB, a 20 month old child weighing 11.6kg with history of traumatic subdural collections was admitted with episodes of absence seizures, ataxia and unresponsiveness. Initial investigations involved an electroencephalogram which reported a normal background rhythm. A follow up MRI scan reported no definite site of abnormal CSF leak to confirm the working diagnosis. Hence, IF was proposed as a diagnostic tool to identify the location of a possible leak. The pharmacist conducted a therapeutic review with the aim of appraising existing evidence for the use of IF in paediatrics.A total of 12 articles were identified using Medline and Embase. 5 case series and 1 case report were selected for further review to determine the safety profile, optimal dose and appropriate formulation for the diagnostic procedure. Studies showed at lower concentrations, with doses ranging from 25-100mg the rate of minor complications such as nausea/vomiting, headache and dizziness was negligible.1 2 No complications were accounted in using the lowest dose (&lt;25mg).2 Another study specifically mentioned no adverse reactions observed in children when 0.1ml/kg of 5% fluorescein was administered.3 The case report presented a paediatric patient (16 months) with CSF leak who was administered 0.125ml (6.25mg) of 5% IT to identify the leak.4 The potential dosage for Miss. AB was decided as between 10mg to 25mg balancing the increasing risk of adverse reactions with higher doses and possibility of false-negative result with lower doses.The neurosurgical team used this evidence to present the patient’s case to the chairman’s board for an off-label use approval at the trust. Upon enquiring various manufactures, the 5% unlicensed injection was unavailable to purchase and the 10% injection is unsuitable for intrathecal use. Therefore, the 20% fluorescence sodium injection which is an unlicensed ‘specials’ product usually used in adults was recommended by pharmacy. The smallest measurable dose of 0.1ml (20mg) of 20% fluorescein sodium, diluted in 10ml CSF with 5ml infused via a 0.2micron filter was recommended. The batch number and pyrogen free certificate was obtained from pharmacy procurement and application was submitted.Upon receiving the panel approval, IF was used and a CSF leak was identified. This has aided the surgeons to confirm diagnosis and repair the rhinorrhea. With this successful intervention, the use of IF can be an established option to diagnose CSF rhinorrhea prior to surgery in the trust. These findings will be used in submitting a formulary application and drafting trust guidance for extending the use of IF to paediatrics as a diagnostic tool in neurosurgery.ReferencesJavadi S, Samimi H, Naderi F, et al. The use of low- dose intrathecal fluorescein in endoscopic repair of cerebrospinal fluid rhinorrhea. Archives of Iranian Medicine 2013;16:264-266.Rainer K, Wienke A, Wolfgang D, et al. Use of sodium fluorescein solution for detection of cerebrospinal fluid fistulas: an analysis of 420 administrations and reported complications in Europe and the United States. Laryngoscope 2004;114:266-272.Crosara P, Becker C, Stamm A, et al. Chemical and cytological analysis of cerebral spinal fluid after intrathecal injection of hypodense fluorescein. Brazilian Journal of Otorhinolaryngology 2015;81:549-553Lue A, Manolidis S. Intrathecal fluorescein to localize cerebrospinal fluid leakage in Bilateral Mondini Dysplasia. Otology &amp; Neurotology 2004;25:50-52.
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Wiguna, Tjhin, Sasanto Wibisono, Sudigdo Sastroasmoro, and Fransiscus D. Suyatna. "Does 20 mg long-acting methylphenidate alter blood pressure and heart rate in children with ADHD?" Paediatrica Indonesiana 51, no. 5 (2011): 282. http://dx.doi.org/10.14238/pi51.5.2011.282-7.

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Objective To identify the cardiovascular effects of long-acting methylphenidate administered for twelve weeks in Indonesian children with ADHD.Methods This was an 18-week, time series study on children with ADHD who were given 20 mg of long-acting methylphenidate for twelve weeks. During the study period we made ten serial observations of the subjects, including before, during and 6 weeks following drug administration. We included drug naive children with ADHD between the ages of 7 – 10 years. Children with mental retardation and chronic physical or mental disorders were excluded. Blood pressure was measured by sphygmomanometer with a child’s cuff at the brachial artery. We also collected data on heart rate, side effects, complaints and other medications used during the study. Repeated analysis was performed on the data with a P level of 0.05.Results Twenty-one subjects were recruited for this study. Mean blood pressure fluctuated insignificantly during the research period, for both mean systolic and mean diastolic blood pressures (P=0.115 and P=0.059). Mean heart rate also fluctuated insignificantly (P=0.091). All fluctuations were within the normal ranges. During the study, there were complaints of dizziness, nausea, and gastrointestinal upset, but they were reportedly mild and disappeared before the second week of observation.Conclusion Administration of 20 mg long-acting methylphenidate for twelve weeks in children with ADHD altered mean blood pressures and heart rates, but within the normal range for children of their age. However, cardiovascular risk observation is still needed when administering methylphenidate to children with ADHD, especially for those using the medication long-term.[Paediatr Indones. 2011;51:282-7].
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Crunkhorn, Rosa, Suparna Dasgupta, Arnab Kumar Seal, and Soumit Dasgupta. "Fifteen-minute consultation: The dizzy child." Archives of disease in childhood - Education & practice edition, October 7, 2021, edpract-2020-320569. http://dx.doi.org/10.1136/archdischild-2020-320569.

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Vestibular disorders are often overlooked in children and may cause significant morbidity. About a third of children presenting with problems in balance show a vestibular pathology and the overall prevalence of paediatric vertigo is about 5%. Appropriate diagnosis and holistic management can have a significantly positive impact on a child’s quality of life and can be very rewarding. We present a structured approach to the assessment and management of a child presenting with dizziness in a general, non-neurological specialty or community paediatric outpatient setting.
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Elghaffar, Hossam Abd, Sherif Guindi, Mahmoud Mohamed Magdy, Mona Alakkad, and Reham Rafei El Shafei. "Common vestibular disorders in children in Fayoum governorate: a cross-sectional study." Egyptian Journal of Otolaryngology 38, no. 1 (2022). http://dx.doi.org/10.1186/s43163-022-00222-0.

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Abstract Background Vertigo in paediatric age is not a rare condition. However, a lack of awareness about causes of dizziness in children besides the absence of standard diagnostic workup often leads to unnecessary procedures in managing these cases. The purpose of this cross-sectional study was to determine the prevalence and frequency distribution of balance abnormalities in dizzy children. Additionally, the questionnaire’s utility as a screening tool and its predictive accuracy are discussed. Thus, an organized strategy can be allocated to assist the diagnostic process, which is likely to eliminate unnecessary and costly evaluations. Results This study comprised 848 children who presented with any of the dizzy symptoms referred to the Audio-Vestibular Unit, Fayoum University Hospital. The suggested diagnosis was 23.9% of the cases suffered from otitis media either alone or combined with other diseases, while 17.6% had benign paroxysmal vertigo of childhood. Migraine patients represented 11.2% of the cases. Only 5.5% of subjects had benign paroxysmal positional vertigo. 78.3% of cases with possible diagnosis showed agreement in diagnosis by both paediatric dizziness questionnaire and the final diagnosis reached by a full assessment of the patients. Conclusion Diagnosing dizziness in children is challenging; nevertheless, a structured history taken in conjunction with a vestibular examination is crucial for establishing an accurate diagnosis. The questionnaire appears to be an even-handed tool for diagnosing dizzy children.
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Picciotti, Pasqualina Maria, Giorgia Rossi, Stefano Settimi, Anna Rita Fetoni, Maria Coppola, and Jacopo Galli. "Validity of Italian adaptation of the Vanderbilt Paediatric Dizziness Handicap Inventory." Acta Otorhinolaryngologica Italica, July 2023, 1–7. http://dx.doi.org/10.14639/0392-100x-n2552.

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Dilber, Beril, Zeynep Gökçe Gayretli Aydın, Osman Yeşilbaş, et al. "Neurological manifestations of paediatric acute COVID infections: A single centre experience." Journal of Tropical Pediatrics, July 12, 2021. http://dx.doi.org/10.1093/tropej/fmab062.

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Abstract Background Coronavirus disease 2019 (COVID-19) usually leads to a mild infectious disease course in children, while serious complications may occur in conjunction with both acute infection and neurological symptoms, which have been predominantly reported in adults. The neurological complications in these patients vary based on patient age and underlying comorbidities. Data on clinical features, particularly neurological features, and prognostic factors in children and adolescents are limited. The present study provides a concise overview of neurological complications in pediatric COVID-19 cases. Materials and Methods The retrospective study reviewed medical records of all patients who were admitted to our hospital and were diagnosed with COVID-19 by real-time reverse-transcription polymerase-chain-reaction (RT-PCR) assay between March 11, 2020 and January 30, 2021. Patients with a positive PCR result were categorized into two groups: Out Patient Departments (OPD) patients and In Patient Departments (IPD). Results Of the 2,530 children who underwent RT-PCR during the study period, 382 (8.6%) were confirmed as COVID-19 positive, comprising 188 (49.2%) girls and 194 (50.8%) boys with a mean age of 7.14±5.84 (range, 0-17) years. Neurological complications that required hospitalization were present in 34 (8.9%) patients, including seizure (52.9%), headache (38.2%), dizziness (11.1%) and meningoencephalitis (%5.8). Conclusion The results indicated that neurological manifestations are not rare in children suffering from COVID-19. Seizures, headaches, dizziness, anosmia, ageusia, and meningoencephalitis major neurological manifestations during acute COVID-19 disease. Although seizures were the most common cause of hospitalization in IPD patients, the frequency of meningoensalitis was quite high. Seizures were observed as febrile seizures for children under six years of age and afebrile seizures for those over 6 years of age. Febrile seizure accounted for half of all seizure children.
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Singh, Madhusudan Prasad, Alok Singh, and Ekta Krishna. "Trial Sequential Analysis and Meta-analysis of Intranasal Ketamine Versus Intranasal Opioids for Analgesia in Paediatric Patients." Annals of Neurosciences, March 1, 2025. https://doi.org/10.1177/09727531251317292.

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Background Ketamine (KT) is known to have analgesic and sedative effects. Intranasal (IN)/inhalational KT has been used in different trials involving paediatric patients for analgesic and anxiolytic function. The present meta-analysis was conducted to establish the role of IN/inhalational KT compared to that of inhalational opioids (OPs). Summary A systematic literature search was performed through the Cochrane Library, Pub Med and ClinicalTrials.gov databases from inception to February 2024 using the following keywords: inhalational OR IN OR nebulised’ and ‘ketamine’ and ‘analgesia’. Randomised clinical trials published in English that analysed the efficacy and safety of inhalational KT either alone or as an adjunct to the standard of care (SoC) compared to OP in paediatric patients undergoing various procedures were included in the analysis. The important outcomes included patients who were pain responders, required rescue analgesics, achieved mild-moderate sedation and experienced any adverse events (AEs), dizziness, nausea/vomiting or unpleasant taste. A trial sequential analysis (TSA) was also performed. The analysis included seven studies with 489 paediatric patients. In the KT group, a smaller number of patients were pain responders and required rescue analgesics (RR = 0.94; 95% CI = 0.78–1.13; P = .52 and RR = 0.80; 95% CI = 0.44–1.43; P = .45, respectively). Similarly, more patients in the KT group achieved mild-moderate sedation and experienced any AEs (RR = 1.44; 95% CI = 0.95–2.18; P = .09; and RR = 1.99; 95% CI = 1.47–2.69; P &lt; .00001, respectively). A greater number of patients experienced dizziness (RR = 5.47; 95% CI = 3.12–9.58; P &lt; .00001) and an unpleasant taste (RR = 2.91; 95% CI = 1.51–5.61; P = .001) in the KT group. In the meta-analysis, the required information size (RIS) could not be obtained. Key Message KT had efficacy outcomes comparable to those of OP, but KT had very high adverse effects. OP seems to have better tolerability than KT. However, as the number of patients was less than the RIS, it was not possible to draw any conclusions.
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Olsson‐Åkefeldt, S., J. Luthander, L. Anmyr, et al. "Poor Association Between Clinical Characteristics and Seropositivity in Children With Suspected Long COVID—A Single‐Centre Study." Acta Paediatrica, February 19, 2025. https://doi.org/10.1111/apa.70034.

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ABSTRACTAimWe aimed to compare characteristics and clinical presentation of severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) antibody positive or negative children attending a specialist outpatient clinic for suspected paediatric long COVID.MethodsA cross‐sectional study was conducted of 113 children and adolescents enrolled between 1 December 2020 to 14 September 2021 in a multidisciplinary programme. Clinical and epidemiological data were collected with standardised interviews and laboratory tests including SARS‐CoV‐2 spike antibody measurement.ResultsA serological link to SARS‐CoV‐2 infection was found in 52%. Most patients (94.7%) reported several symptoms. Fatigue, post‐exertional malaise, dizziness, nausea, headache, and concentration difficulties were the most common. Seronegative children had a higher number of individual symptoms. School absence and drop‐out from leisure activities was substantial in both groups with higher numbers for the seronegative group. Self‐reported health was low in both groups.ConclusionChildren attending a specialist paediatric long COVID clinic experienced multiple symptoms and poor self‐reported health. The symptomatology was similar regardless of serological status, implying multifactorial causes. A multidisciplinary assessment of this cohort was essential considering the broad spectrum of symptoms displayed and their substantial impact on everyday functioning.
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Kataki, Rita Panyang, Bhaskar Borah Sonowal, Komal Agarwal, Chow Nifaseng Chiring, and Luhit Dhungel. "Unusual Presentations of Epilepsy: A Series of Paediatric Cases with Abdominal Epilepsy and Epileptic Angina." JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH, 2024. http://dx.doi.org/10.7860/jcdr/2024/67269.19207.

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Epilepsy, a common neurological disorder characterised by recurrent seizures, can manifest in unusual clinical presentations such as abdominal pain, nausea, vomiting, and bloating associated with diverse Central Nervous System (CNS) symptoms like confusion, fatigue, headache, dizziness, and syncope. The importance of recognising and documenting such unusual presentations lies in their potential to be misdiagnosed or overlooked, delaying appropriate treatment and causing undue distress to both the patients and their families. In the present case series, patients presented with gastrointestinal or chest pain for a prolonged period. As the symptoms did not subside with treatment, an Electroencephalogram (EEG) was performed to rule out epilepsy. In all three cases (10-year and 10-month-old female, 11-year-old male, seven-year-old male), EEG revealed a generalised seizure disorder. The present case series highlights unusual presentations of epilepsy in paediatric patients, including Abdominal Epilepsy (AE) and epileptic angina. These cases underscore the importance of considering epilepsy as a potential aetiology in patients with recurrent and atypical symptoms.
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Hiranput, Siwaporn, Kasthuri Nallathamby, Lucy Smith, and Paul Devakar Yesudian. "PA17 Paediatric-onset mastocytosis: a retrospective review." British Journal of Dermatology 188, Supplement_4 (2023). http://dx.doi.org/10.1093/bjd/ljad113.321.

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Abstract Mastocytosis is characterized by an abnormal accumulation of mast cells in different organs. The skin is the most frequently affected site. Most cases of paediatric-onset mastocytosis are benign, localized to the skin and regress spontaneously. We retrospectively reviewed the case notes of paediatric patients with a diagnosis of mastocytosis in dermatology departments within our region between 2021 and 2022. Seventeen patients were identified. The disease was more prevalent in males (male-to-female ratio 1.8s : 1). The median age at onset was &amp;lt; 1 year (range birth–4 years). The lesions were described as solitary (n = 9), macular (n = 6) and maculopapular (n = 2). The torso was involved in 16 patients. Other body sites affected were the face (n = 2), scalp (n = 1), limbs (n = 5) and buttock (n = 2). Most patients were asymptomatic (n = 12). Symptoms, including abdominal pain (n = 3), pruritus (n = 2), musculoskeletal pain (n = 1), diarrhoea (n = 1), flushing (n = 1), headache (n = 1) and dizziness (n = 1), were reported. Darier sign was positive in 11 patients and negative in three (not documented in three). None of the patients had bone marrow examination. One patient underwent a skin biopsy, which confirmed mastocytosis in the skin; the remaining 16 patients were diagnosed with cutaneous mastocytosis based on the characteristic clinical features. The disease was classified into mastocytoma (n = 8) and urticaria pigmentosa (n = 9). Five patients with urticaria pigmentosa were assessed for lymphadenopathy and organomegaly: four had blood investigations, including serum tryptase (n = 4), full blood count (n = 2) and liver function tests (n = 2); one had an abdominal ultrasound. No abnormality was found. Patients with mastocytoma were not examined for lymphadenopathy or organomegaly, or had further investigations for systemic involvement. Eight patients were managed by watchful monitoring. Nine patients received active treatments, including topical steroids (n = 6), antihistamines (n = 6), oral sodium cromoglicate (n = 1), phototherapy (n = 1), epinephrine autoinjector (n = 1) and cosmetic camouflage (n = 1). Partial regression was observed in seven cases at a median follow-up of 3 years (range 1–14), and seven had persistent disease. Six patients remain under long-term follow-up, and eight were discharged with a median follow-up period of 1.5 years (range 1–14). Five patients were followed to adolescence (disease was persistent in three cases and there was partial regression in two cases; none had complete resolution). While serum tryptase levels do not appear to have a prognostic value in the paediatric population, we would recommend checking these in patients whose disease persists beyond adolescence as this may indicate progression to systemic mastocytosis.
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Dasgupta, Soumit, Rosa Crunkhorn, John Wong, Annie McMahon, Sudhira Ratnayake, and Leonardo Manzari. "Suppression head impulse test in children—experiences in a tertiary paediatric vestibular centre." Frontiers in Neurology 15 (March 14, 2024). http://dx.doi.org/10.3389/fneur.2024.1297707.

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The suppression head impulse paradigm (SHIMP) involves suppression of the vestibulo-ocular reflex (VOR) and anticompensatory saccades generated thereof. SHIMP is gaining importance to understand vestibular compensation with its different parameters (VOR gain/peak saccadic velocity PSV/latency of saccades). SHIMP studies are emerging in adults, but pediatric studies have hardly been performed. This study is a retrospective case note audit over a period of 2 months in a tertiary pediatric vestibular center in the United Kingdom to investigate whether SHIMP is safe/robust to be used in children conforming to existing standards/norms in normal children and whether it yields any meaningful inferences in pediatric vestibular hypofunction. This is the largest pediatric SHIMP study to date. A total of 44 referred children (6–18 years, female children&amp;gt;male children) with a range of complaints from dizziness, imbalance, motor incoordination, postural instability, and hearing loss were included, and their SHIMP parameters were measured. All children underwent comprehensive functional/objective audiovestibular assessments. Two groups were defined—Group A with normal vestibular function and Group B with abnormal vestibular function. The normal population showed an average SHIMP VOR gain of 0.98+/−0.08 and latency of overt saccades at 215.68+/–46.16 milliseconds agreeing with published evidence. The PSV of overt saccades was 315.39+/−56.30/s, and there was a gain asymmetry of 7.42+/−4.68 between the sides. Statistically significant differences with moderate/large effect sizes were observed between the groups in terms of VOR gain and PSV but not in saccade latencies. Covert saccades were rare in SHIMP, while overt saccades were observed in 100% of children. VOR gain difference between the head impulse paradigm (HIMP) and the SHIMP was significant as well. We observed statistically significant differences in side asymmetry of VOR gain between the groups. Furthermore, we identified a group of children with cerebellar lesions where overt saccades in SHIMP were rather low in number. Further research is recommended to investigate pediatric PSV, asymmetry, and inability to generate overt saccades that may suggest useful means to assess compensation and central function. We conclude that SHIMP yields valuable information and is a safe, easy to perform, and a reliable test that should be used in children to supplement HIMP.
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Claraz, Pauline, Marie de Tersant, Valentine Feyants, Julie Roupret-Serzec, Thomas Storme, and Jean-Hugues Dalle. "Ethanol intoxication in paediatric patients receiving intravenous etoposide as conditioning regimen for allogenic hematopoietic stem cell transplant." Journal of Oncology Pharmacy Practice, April 16, 2025. https://doi.org/10.1177/10781552251330244.

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Introduction Total body irradiation associated with etoposide is widely used in conditioning regimens before hematologic stem cell transplantation (HSCT). The etoposide formulation exposes children to a high dose of ethanol, and data on its side effects are scarce. We investigated whether paediatric patients with acute lymphoid leukaemia and lymphoblastic lymphoma were experiencing hypersensitivity reactions or ethanol-related reactions after etoposide infusion before HSCT. Methods This study retrospectively analyzed all patients treated with a TBI 12 Gy-based-conditioning associated with etoposide 60 mg/kg before HSCT. Hypersensitivity reactions (tachycardia, hypotension, dyspnea, flushing and erythema) were opposed to ethanol-related symptoms (dizziness, impaired balance) in patients who presented clinical reactions during or after etoposide infusion. Ethanol-induced toxicity risk factors related to demographics and infusion data were analysed such as age, gender, etoposide and ethanol amount, etoposide or ethanol infusion rate. Results Between January 2015 and December 2020, 62 patients received etoposide as conditioning regimen and 48 patients were included in the study. The median etoposide dose was 1960 mg (414–3600), and the median amount of ethanol in the preparation was 24.3 g (5.1–44.6). Twenty patients exhibited etoposide-related symptoms. Eighteen patients presented ethanol-related symptoms, and 2 patients had hypersensitivity reactions and were admitted to the Intensive Care Unit. The incidence of ethanol-related symptoms was significantly higher in patients ≥ 7.5 years old at the time of HSCT ( p = 0.04). No other risk factors were found. Conclusion These data must raise awareness among physicians and pharmacists about the high dose of ethanol to which children are exposed during conditioning, and their adverse effects.
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Foley, Charlene, Kate Armon, Filippos Skarmoustsos, Brinda Muthusamy, and Peter Bale. "O26 More than meets the eye … When inflammation extends beyond the anterior chamber." Rheumatology Advances in Practice 5, Supplement_1 (2021). http://dx.doi.org/10.1093/rap/rkab067.025.

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Abstract Case report - Introduction The differential diagnosis of paediatric uveitis is extensive. Classification starts by determining infectious versus non-infectious causes, anatomic location and associated intra-ocular and extra-ocular features. A relatively common referral to Paediatric Rheumatology from our Ophthalmology colleagues is of a child with a diagnosis of uveitis. This case highlights the importance and benefits of multidisciplinary team working across our regional network when caring for children with complex and rare conditions. Case report - Case description An 8-year-old-girl was referred by her local ophthalmology team to the paediatric rheumatology clinic with a diagnosis of pars-planitis. She had presented to them with blurred-vision and eye-“floaters”. On review, the girl reported that she had a 4-month-history of headaches and blurred-vision, associated with dizziness. She denied any eye-pain. Corresponding to the onset of her symptoms, she had suffered with Chickenpox. Her mother felt that she had "not been right since then". She noted that she was generally quieter and more fatigued than normal. Over the course of the 4 months, she was noted to have become clumsier and was bumping into things on a regular basis. She reported increasing visual difficulties in her right eye and initially attended for an optician review. The optician was concerned and referred for urgent ophthalmology opinion. She was diagnosed with bilateral pars-planitis, commenced on oral prednisolone and referred for paediatric rheumatology and tertiary ophthalmology assessment. The headaches improved following commencement on steroids. Positive findings on systems review included occasional oral ulcers, 1—2 times-per-month. They started about 1-month prior to the onset of her chickenpox and continued for the following 3—4 months. She denied any history of genital-ulcers or skin-rash. She had new-onset muscle soreness and tiredness after activity. She also described non-specific abdominal pain since having chickenpox, but no associated change in bowel habit. Examination revealed normal skin, hair, nail and joint examination. She had a soft systolic murmur (echocardiogram normal). She had RUQ tenderness on abdominal palpation (abdominal-USS – spleen upper limit of normal. Nil else). Relevant blood and stool samples were sent as part of a uveitis work-up panel. The paediatric ophthalmologist found right-intermediate uveitis with vitreitis and peripheral retinal changes in keeping with a peripheral exudative detachment. Similar changes were seen in the peripheral retina of the left eye. The impression was of bilateral pan-uveitis with retinal involvement. A subsequent oral fluorescein angiogram showed a widespread retinal vasculitis with some occlusive changes in the right-eye, and a tuft of retinal vascular leakage at the 5-o'clock position in the left eye. Case report - Discussion The differential diagnosis for paediatric retinal vasculitis is broad. It includes collagen vascular disorders, Behçet's disease, Eales’ disease, post viral or post vaccination, acquired toxoplasmosis, multiple sclerosis, systemic immunosuppression and Henoch-Schönlein purpura. The ANA, ENAs, dsDNA, ANCA, ACE, Toxoplasma and Lyme serology were all negative for this little girl. Her inflammatory markers were also normal. She was not on any regular medications prior to her illness and had not had any recent vaccinations. She had no significant past medical or family history of note. However, the onset of her symptoms did correspond to her having chickenpox. She was subsequently found to be positive for HLA B51. In view of the ophthalmology findings and positive HLA B51, the little girl was admitted for an urgent MRI/MRI to exclude neuro- Behçets. This was reported as normal. She was treated with a three-day pulse of IV methylprednisolone and discharged on oral prednisolone 10mg OD (weight 33.4kg). At present Bechet’s retinal vasculitis remains high in the list of differentials for this little girl; however, currently she does not strictly meet the diagnostic criteria for Bechet’s disease. She has been commenced on a steroid sparing agent, azathioprine. Her follow-up plan is to be reviewed in the joint paediatric rheumatology and ophthalmology clinic in 1 months’ time. Case report - Key learning points Retinal vasculitis may occur secondary to a systemic disease or an infectious agent, or as an isolated retinal aetiology. Given that the differentials are vast, a detailed history and examination are important to identify signs and symptoms of systemic disease. Appropriate investigations should be chosen to help narrow the differentials and ensure pathology that could lead to significant morbidity and mortality is not missed. With a case such as this, close collaboration between the paediatric ophthalmologist and rheumatologist is paramount to ensure the best outcome for the patient. With regards to treatment, small case series have described a refractory nature of retinal vasculitis in paediatric patients. One study report that almost 80% of patients with paediatric idiopathic uveitis show manifestations of retinal vasculitis, which is associated with a lower probability of inflammation control resulting in a worse visual prognosis. Points for discussion
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Tunca Sahin, Gulhan, Cemil Cihad Kurt, Hasan Candas Kafali, et al. "Sinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience." Cardiology in the Young, November 18, 2024, 1–6. http://dx.doi.org/10.1017/s1047951124025952.

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Abstract Aim: This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction. Methods: This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022. Results: In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery–Dreifuss muscular dystrophy. Conclusion: Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.
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Calapai, Gioacchino, Marco Casciaro, Marco Miroddi, Fabrizio Calapai, Michele Navarra, and Sebastiano Gangemi. "Montelukast-Induced Adverse Drug Reactions: A Review of Case Reports in the Literature." September 9, 2014. https://doi.org/10.1159/000366164.

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&lt;b&gt;&lt;i&gt;Background:&lt;/i&gt;&lt;/b&gt; Montelukast, a leucotriene receptor antagonist, binds the cysteinyl leucotriene type 1 receptor. Montelukast is commonly prescribed to asthma patients as add-on therapy to inhaled corticosteroids. Several clinical trials emphasized that montelukast can be considered a safe drug. However, recent evidence reconsidered the benefit/risk ratio of the use of montelukast for both paediatric and adult patients. &lt;b&gt;&lt;i&gt;Summary:&lt;/i&gt;&lt;/b&gt; The present review analyzed the previous published case reports regarding montelukast-induced adverse drug reactions (ADRs). They included agitation, anxiety, depression, sleep disturbance, hallucinations, suicidal thinking and suicidality, tremor, dizziness, drowsiness, neuropathies and seizures. The immune system can be involved, in particular, cases of Churg-Strauss syndrome have been published. Furthermore, it can induce hypersensitivity reactions, including anaphylaxis and eosinophilic infiltration. In addition, hepatobiliary, pancreatic and uropoietic disorders have been observed. Some of these cases are characterized by severe prognosis (i.e. neurological deficit and fatal hepatotoxicity). &lt;b&gt;&lt;i&gt;Key Message:&lt;/i&gt;&lt;/b&gt; The use of montelukast can be burdened by several ADRs, of which physicians should be aware in their clinical practice. A better understanding of the mechanisms causing ADRs after using montelukast could help researchers and clinicians in defining a risk-reduction strategy aimed to lessen montelukast toxicity. More accurate epidemiological studies, in order to discover risk factors favouring montelukast-associated ADRs, are demanded.
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Adnan, Naqqash, Prachi Agarwal, Zoe Zagorac, Zohaib Jamal, and Imran Alam. "EGS P31 Small bowel obstruction in an elderly female secondary to congenital Ladd’s band. A rare case presentation." British Journal of Surgery 110, Supplement_8 (2023). http://dx.doi.org/10.1093/bjs/znad348.077.

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Abstract Background Intestinal malrotation is a congenital anomaly which arises during embryonic development. This can be associated with peritoneal tissue known as Ladd’s bands which can cause predisposition to intestinal obstruction. The incidence of intestinal malrotation is reported to be approximately 0.2% in the adult population [G]. Cases of intestinal obstruction secondary to malrotation commonly present in the paediatric population, but rarely may only be detected at adulthood. We present an elderly women who presented with features suggestive small bowel obstruction. Methods We present a rare case of a 78 year old female presenting with a three day history of coffee-ground vomiting, abdominal pain and dizziness. The patient had a notable background of a hiatus hernia and atrial fibrillation for which she was being treated with edoxaban. An abdominal X-ray film showed faecal loading. CT imaging revealed a high-grade small-bowel obstruction with an acute transitional point at the terminal ileum secondary to congenital Ladd's band which was confirmed at laparotomy. Subsequently, a Ladd’s procedure was performed and the patient was discharged after making a good recovery. Results In this case report, we highlight the importance of considering congenital anomalies in patients presenting with symptoms of bowel obstruction. We discuss the importance of appropriate investigations as well as multidisciplinary care to help facilitate early diagnosis. Conclusions Congenital Ladd’s band is a rare cause of small bowel obstruction in an elderly population. Although the diagnosis of intestinal malrotation can be challenging in adulthood, congenital anomalies should be considered as a differential in patients presenting with symptomatic obstruction.
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Nielsen, Bettina N., Steen W. Henneberg, Eva Malmros Olsson, and Stefan Lundeberg. "The use of intranasal sufentanil and/or s‐ketamine for treatment of procedural pain in children in an ambulatory setting: A retrospective observational study." Acta Anaesthesiologica Scandinavica, August 18, 2024. http://dx.doi.org/10.1111/aas.14510.

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AbstractBackgroundMinor but painful medical procedures are often handled at the operating room. If safe and effective treatment options are available many procedures may be performed outside of the operating room.Objective(s)The objective of this study is to assess the adverse events of intranasal s‐ketamine and/or sufentanil alone or as part of a multimodal analgesic regime for medical procedures outside of the operating room. Secondary outcomes included analgesic effect, doses and indications for use.DesignRetrospective observational study.SettingTertiary care paediatric hospital.PatientsChildren 1 year up till 18 years.Intervention(s)Intranasal (IN) sufentanil (S), intranasal s‐ketamine (K) or the free combination of the two drugs (SK).Main Outcome Measure(s)The frequency of adverse events including serious adverse events reported by intervention.ResultsBetween 2004 and 2014, 2185 medical procedures were registered, including 652 procedures with IN SK, 1469 procedures with IN S and 64 procedures with IN K. The children's median age was 5.6 years (range 1.0–17.9). Medical procedures with at least one adverse event were 18% with IN SK, 25% with IN K and 18% with IN S. Common adverse events included episodes of vomiting (9%), nausea (8%) and dizziness (3%). In two patients receiving IN S, serious adverse events occurred. One patient had respiratory depression and bronchospasm and another patient with cerebral palsy had a seizure. Both were handled immediately and did not result in any sequelae. The median doses of intranasal sufentanil were 38% lower when combined with s‐ketamine (IN SK free combination: sufentanil dose 0.5 μg/kg (range 0.2–1.3) and s‐ketamine dose 0.5 mg/kg (range 0.2–1.5). IN S monotherapy, sufentanil dose 0.8 μg/kg (range 0.2–2.7)). Similar analgesic effect was reported for S and SK.ConclusionsIntranasal sufentanil and/or s‐ketamine are feasible for the treatment of procedural pain in an ambulatory setting with appropriate per‐ and post‐procedural observations and trained staff.
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Dawson, Pamela, and Eslam Al-Abadi. "P50 A case of juvenile relapsing polychondritis with rapidly developing respiratory complications." Rheumatology 58, Supplement_4 (2019). http://dx.doi.org/10.1093/rheumatology/kez416.017.

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Abstract Background Relapsing polychondritis in childhood is a rare multi-system disease characterised by inflammation of cartilage and can be life-threatening with complications of the larynx, trachea and cardiovascular system. It is often a delayed diagnosis with a more severe course than in adults; there is higher incidence of laryngotrachobronchial involvement, which can occur early and is often unresponsive to conventional therapy. Methods A previously healthy 14-year-old of African descent presented to a children’s hospital with unilateral ear pain and swelling, lower anterior rib pain and red, painful eyes. Initial work-up showed microcytic anaemia and raised inflammatory markers. Initially treated with broad-spectrum antibiotics, markers of inflammation continued to rise and she became febrile. After discussion with tertiary paediatric rheumatology, polychondritis was suspected and high dose oral steroids were commenced, improving all symptoms. She was seen in tertiary paediatric rheumatology clinic and steroids were weaned. She quickly relapsed with fever, chest pain, cough, sore throat, dizziness and vomiting. On examination there was pain and swelling of the nasal bridge and cricoid tenderness. The left ear was swollen, warm and tender. She had tinnitus in the right ear. Further investigation showed high inflammatory markers, anterior uveitis and right sensorineural hearing loss. Management was pulsed intravenous methylprednisolone followed by oral Prednisolone as induction, with plans for methotrexate commencement. Parental concerns and missed appointments prevented methotrexate initiation; the family wished to withdraw steroids over two months before further treatment decision. On weaning steroids she developed stridor, barking cough, hoarse voice and dyspnoea. Saturations remained normal despite moderate work of breathing. Flexible nasendoscopy showed interarytenoid and subglottic oedema and likely trachobronchitis. She was commenced on CPAP, rituximab and a six-month course of cyclophosphamide. Due to parental concern and stress, relationships with healthcare professionals became increasingly challenging. Five months after initial presentation, she underwent semi-elective intubation owing to biphasic stridor and increased work of breathing. Subsequently extubation proved difficult and she was transferred to a specialist centre for ongoing management. Results The patient was subsequently extubated to BiPAP and completed cyclophosphamide infusions. Multi-speciality national teleconference agreed upon methotrexate and anti-TNF maintenance treatment with gradual wean of steroids. She has both saddle-nose and ear deformities, in addition to BiPAP-dependant airway stenosis. Discussions regarding airway reconstruction are ongoing with a national specialist team. Conclusion Relapsing polychondritis in childhood and adolescence is rare and more aggressive than in adults. This case demonstrates the speed destructive features can develop despite early treatment with steroids. Without aggressive and prompt treatment, life-threatening complications can develop rapidly and outcomes are poor. Also highlighted is potential airway reconstruction in collaboration with specialist centres. Excellent communication between healthcare professionals and with families is essential, especially when care of a complex and rare disease is shared between multiple centres. Conflicts of Interest The authors declare no conflicts of interest.
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Yershov, Danylo, Mona Seyed Esfahani, and Salman Heydari Khajehpour. "BN SO10 - Virtual Reality in Acute Pain Management of Adult Surgical Patients: Literature Review." British Journal of Surgery 111, Supplement_9 (2024). http://dx.doi.org/10.1093/bjs/znae271.181.

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Abstract Background Pain is an unavoidable side effect of medical procedures. British Pain Society (BPS) reports almost 10 million people in UK suffer from pain everyday. BPS reported an estimated €5 million per year is spent on managing back pain alone. Pharmaceutical pain management is costly and can cause side effects. Virtual Reality (VR) is a novel technology used to alleviate pain. We performed a literature review to assess the current advances in the pain management using VR. Method A literature search was done on the use of VR for pain management across 4 databases (Pubmed, Medline, Embase and Cinahl) using key words virtual reality, acute pain, adult, surgery. 144 non-duplicated studies were found. 66 studies were excluded: 41 were non-acute pain related, 16 involved chronic pain, 9 involved paediatric population. 78 studies with the total number of 1514 patients (n=1514) were included. All studies used VR to relieve acute pain symptoms experienced while undergoing a procedure. The data included sample size, gender, age, specialty, VR type, pain scale, pain score before and after, interventions, key findings and limitation. Results VR was tested to control pain in general surgery, plastics, obstetrics and gynaecology, cardiothoracics, orthopaedics, urology, and dentistry. In benign UGI surgery, including fundoplication, paraesophageal hernia repair, esophageal myotomy, and pyloroplasty requiring an overnight stay, the use of VR reduced pain, anxiety and nausea. Overall, no adverse outcomes were reported. No difference in nausea and dizziness between VR and control groups was observed. Active software has been shown to be superior to the passive one. Complete immersion with visual and audio stimulation replaced by VR environment is important. The results were similar in different age groups. Conclusion There is a good body of evidence to support the use of VR in pain management in clinical practice. It acts as a good distraction tool. The use of VR was demonstrated to be beneficial in reducing postoperative pain in benign UGI surgery. However, the main areas of application would be in short procedures where it not only delivers better pain management but also increases patient satisfaction and decreases the need for pharmaceutical analgesia, thus bringing down the overall cost.
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46

Rodrigo, D., U. Estandia, C. Perez, P. M. Perez, and E. Panera. "P837 Isolated cleft posterior mitral valve leaflet." European Heart Journal - Cardiovascular Imaging 21, Supplement_1 (2020). http://dx.doi.org/10.1093/ehjci/jez319.486.

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Abstract A 78-year-old man with a history of hypertension and diabetes mellitus was referred to the ER of our hospital due to an episode of shortness of breath of new onset, associated with dizziness and central thoracic pain of 5 minutes duration. Significant physical examination findings included a V/VI holosystolic murmur at fourth intercostal space with radiation to the axilla. ECG showed no significant abnormalities and cardiac enzymes were within normal ranges The TTE and 3D TOE showed dilated left atrial with normal ventricular size and function with an isolated cleft posterior mitral valve leaflet that bisected into two separate leaflets of identical morphology. Concomitant posterolateral leaflet prolapse was also present with two eccentric, posteriorly, and interatrial septum directed regurgitant jets visualized with colour flow Doppler. Cleft mitral valve leaflet (CMVL) is an uncommon congenital cause of mitral regurgitation. Clefts are slit-like holes or defects hypothesized to be a result of incomplete expression of an endocardial cushion defect and most commonly involve the anterior mitral valve leaflet with a paediatric incidence of 1:1340. Clefts affecting only the posterior mitral valve leaflet are extremely rare . Important co-existing anomalies with either posterior and/or anterior CMVL include counterclockwise rotation of the papillary muscles, the presence of an accessory papillary muscle or mitral valve leaflet, atrial septal defects, and mitral valve prolapse. Acquired causes of clefts include infective endocarditis or trauma from surgical exploration. Regurgitation in CMVL results from blood flow directly through the cleft itself or from malcoaptation from accessory chordae with or without papillary muscle distortion. Early detection through 3D TEE echocardiography can provide accurate anatomical images of the mitral valve structure and identify associated congenital anomalies. Conclusion Posterior CMVL is an extremely rare cause of mitral insufficiency. 3D TEE early recognition of this rare clinical entity and co-existent anomalies can identify afflicted patients who can be closely monitored for the progression of symptoms as well as ventricular dysfunction. 3D TEE permits a personalized medicine tailoring the medical treatment to the individual characteristics of each patient. Abstract P837 Figure. Isolated cleft posterior mitral valve
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Riera-Tur, Laura, Manuel Lubián-Gutiérrez, Isabel Benavente-Fernández, Simón Lubián-López, Antonio J. Martín-Mateos, and Alfonso M. Lechuga-Sancho. "Assessment of the perception of vertical subjectivity in children born preterm." European Journal of Pediatrics, March 1, 2023. http://dx.doi.org/10.1007/s00431-023-04863-y.

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AbstractChildren born preterm have increased rates of paediatric mortality and morbidity. Prematurity has been associated with impaired visual perception and visuo-motor integration. The alteration of the perception of verticality translates into alterations of the vestibular system at central and/or peripheral level, which may manifest itself in symptoms such as imbalance, dizziness or even vertigo. The aim of this study was to compare subjective visual vertical (SVV) test scores in children born preterm with those of children born at term at ages between 7 and 10. One hundred ten children with no neurodevelopmental disorder of 7 to 10 years of age were studied using a mobile application on a smartphone attached to a wall by means of a rotating plate. The SVV test was compared between two groups: a group of 55 preterm children (53 very preterm children born under 32 weeks of gestational age and 2 preterm with very low birth weight) and another group of 55 children born at term (after 37 weeks of gestational age). The SVV results were analysed for comparison with respect to prematurity, sex and age. We found no significant differences in the SVV study in the comparison between preterm and term children. In addition, no significant differences were observed regarding sex or age between 7 and 10 years. Conclusion: We found no alterations in the perception of vertical subjectivity in children between 7 and 10 years of age, with antecedents of very preterm birth and/or very low birth weight. What is Known: • The different studies published so far suggest the existence of balance disorders in premature children, although in most of these studies the children are examined at an age when the vestibular system is not mature and with non-specific tests for the study of the vestibular system. What is New: • We compared the results of the subjective visual vertical (SVV) test in a group of 55 preterm children (53 very preterm children born under 32 weeks of gestational age and 2 preterm with very low weight at birth) and in a group of 55 children born at term (after 37 weeks of gestational age), at the ages of 7 to 10 years and observed no differences. • We conclude that, if there had been any vestibular alterations due to very premature birth, these must have been compensated by the age of 7.
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48

Close, Rosie, Samantha Small, Olivia Murphy-Parry, and Alice Leahy. "P39 Virtual reality headset as an alternative to Entonox for intraarticular corticosteroid injection." Rheumatology Advances in Practice 6, Supplement_1 (2022). http://dx.doi.org/10.1093/rap/rkac067.039.

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Abstract Introduction/Background Intraarticular corticosteroid injection is an important therapeutic approach in paediatric rheumatology that results in targeted treatment of joint inflammation with minimal systemic side effects. In younger patients general anaesthetic is required but older children are generally able to tolerate the procedure well with Entonox. Entonox (a mixture of nitrous oxide and oxygen) provides mild analgesia and promotes relaxation. Nitrous oxide is a greenhouse gas, (nearly 300 times more potent than carbon dioxide) and enters the atmosphere after being exhaled by a patient. Therefore, a more environmentally responsible option is required and virtual reality is a potential option. Description/Method Virtual Reality (VR) is a computer-generated environment with scenes and objects that appear real, making the participant feel immersed in their surroundings. This environment is perceived through a device known as a Virtual Reality headset or helmet. This simulated experience can be similar to or completely different from the real world. Applications of virtual reality are expanding and no longer just include entertainment via video games. VR now has many medical applications. After practicing with a virtual reality system for six weeks, people with Parkinson's disease demonstrated improved obstacle negotiation and balance. Oxford VR’s social engagement program designed to help patients overcome anxious social avoidance is now available on the NHS. It applies cognitive behavioural therapy (CBT) techniques within an immersive virtual reality setting. Virtual reality headsets have been purchased by the play therapy team at Southampton Children’s Hospital. The paediatric rheumatology team was able to utilise one for a 10-year-old boy who required a left knee intraarticular corticosteroid injection. He had the procedure previously with Entonox and although tolerated it was anxious throughout resulting in trepidation at the prospect of a repeat procedure. Consent for a trial with virtual reality headset was obtained as well as consent to video the procedure. An accompanying iPad enabled the health professionals to be able to see the same images as the young person was experiencing enabling them to give relevant comments. The young person remained fully immersed in their virtual reality throughout the procedure and was not able to see what was occurring in ‘real life’. They tolerated the procedure perfectly with no movement of the leg and no verbal reaction. The only noticeable response observed from re-watching the video was the intake of a couple of deep breaths. Immediately after the young person provided verbal feedback: “That was amazing!” Discussion/Results Side effects of Entonox are short lasting and include nausea and light-headedness. Some young people cannot tolerate the sensation of feeling out of control with one diabetic patient likening it to experiencing a hypoglycaemic episode and requesting to discontinue. A study using functional magnetic resonance imaging of healthy patients using VR while exposed to a painful stimulus showed greater than 50% reduction in pain-related brain activity. Cochrane review in 2019 in VR distraction for acute pain in children was non confirmatory and concluded there is a need for larger studies in this area. It is postulated that interacting with immersive VR might divert attention, leading to a slower response to incoming pain signals. The minimum age limitation for VR gaming is seven but there is no clear consensus on the age recommendation of VR headsets. Reported side effects of VR include headaches, eye strain, dizziness and nausea. These are triggered by the vergence-accommodation conflict. If a child is susceptible to motion sickness they will likely experience virtual motion sickness. There was an increase in myopia with the rise of personal handheld devices. As VR screens are very close to the eyes this has raised concern. The American Academy of Ophthalmology state that staring at a VR screen (or any digital device) without breaks may cause eye fatigue due to blinking less often resulting in eye dryness. However, they also comment that although there are no long-term studies there is no reason to be concerned that VR headsets will damage eye development, health or function. As VR is in its infancy the long-term effects remain unknown. Regular breaks are recommended. As with other digital devices the “20-20-20” rule may be applied: every 20 minutes, adjusting gaze to look at an object at least 20 feet away, for at least 20 seconds. Key learning points/Conclusion Due to the environmental impact an alternative to Entonox is urgently required to support young people during invasive procedures. It is important that as many children as possible are able to avoid the risks associated with general anaesthetic. Virtual reality was very successful in enabling a young person to have a positive experience of a intraarticular corticosteroid injection having previously been anxious about this treatment. The encouraging feedback provided by this patient provides support for continued trial in other patients and also other procedures such as blood tests and subcutaneous medication administration in needle phobic patients. Although the long-term effects of VR on children are currently unknown the short time of exposure required for accompanying a clinical procedure is unlikely to have a long term impact. However, as always for any new therapeutic intervention, it will be important to continue to monitor the outcomming research with awareness that the VR exposure during medical interventions may not be the only VR experience and very likely not the only digital device the child is exposed to. The ongoing research into various medical applications including mental health suggests that virtual reality could also become a useful adjunct to paediatric chronic pain management in the future.
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Sharma, Abhinav Kumar, and Abdikayimova Gulzat. "Ataxia By Abdikayimova Gulzat." March 23, 2025. https://doi.org/10.5281/zenodo.15073092.

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<strong>Article on ataxia &ndash; classification and management</strong> <em>Abhinav Kumar Sharma</em> &nbsp; Under the guidance of <em>Abdikyimova G</em><em>ulzat Mam (Faculty of Neurology)</em> Professor - Osh State University,&nbsp; IMF&nbsp; Kyrgyzstan&nbsp; &nbsp; <strong>Abstract</strong> <em>Ataxia has been described as incoordination of voluntary movements and abnormal postural control. There are many different statements concerning the definition, range and nomenclature of ataxia. Different clinical findings, exposure to different neurological structures and different causes lead to the occurrence of each ataxia type. In most cases, treatment of ataxia is not available and supportive treatment should be applied for the management of ataxia symptoms. Ataxia usually follows a trauma to the cerebellum and its tracts like the vestibular, proprioceptive and visual systems.</em> <em>Clinically, the ataxias can be further classified into cerebellar, vestibular, sensory, frontal, optic, visual, mixed ataxia and ataxic-hemiparesis.</em> <em>Etiologically, the ataxias can be categorized into hereditary ataxias, sporadic degenerative ataxias and acquired ataxias.</em> <em>Genetic forms of ataxia must be distinguished from the acquired ataxias like chronic alcoholism, cerebrovascular disease, various toxins agents, immune-mediated inflammation, vitamin deficiency, and persistent central nervous system infections. Once the acquired causes are treated, as ataxia is generally resistant to therapy, the treatment is supportive but may involve physical, occupational, and speech therapy.</em> &nbsp; <strong><u>Introduction</u></strong> The word ataxia is greek in origin (a- negative prefix and taxia to set in order), meaning "Not in order." In medicine, it is utilized to describe loss of coordination and poor postural control. It's an inexact clinical indicator of dysfunction of the cerebellum and/or its connections such as the proprioceptive, visual, vestibular systems and interrelationships between the systems. There are numerous possible causes for these patterns of neurological dysfunction (1, 2). In this review, the neuroanatomic foundation, forms &nbsp;etiologies, and management of ataxia are detailed in the light of the literature. &nbsp; <strong><u>Pathogenesis </u></strong> Ataxia is an impairment of muscle coordination during voluntary movement and impaired postural control. Ataxia usually results from an injury to the cerebellum and its tracts. The main function of the cerebellum is to obtain balance and coordination. Based on the information presented by the vestibular, visual, somatosensory systems and cerebral cortex, the cerebellum generates postural control, balanced and coordinated movement by adjusting accordingly. The vestibulocerebellum acquires eye movements and balance by way of vestibuloocular, vestibulospinal and reticulospinal tracts through the regulation&nbsp;of information in the vestibular and the reticular nuclei. Spinocerebellum receives proprioceptive sensory inputs from the periphery, and regulates movement of body and limb, and is also responsible for locomotion, balance and tonus. Cerebrocerebellum is connected to the cerebral cortex, and is involved in the planning of movement and in evaluating sensory information for action, allowing for precise, coordinated distal movement. In contrast, the cerebrocerebellum has a role in motor control as well as in emotion and cognition. Therefore, the cerebellum is involved with balance and posture maintenance, control of eye movement, planning and execution of coordinated limb movements, motor performance adjustments, learning of new motor tasks, cognitive function and neuroimmunomodulation. Lesion of the cerebellum and/or its connections results in ataxia, oculomotor disturbances, dysmetria,&nbsp;dyssynergia, dysarthria, tremor, hypotonia,&nbsp;prolonged reaction time, and cognitive deficit&nbsp;termed &ldquo;dysmetria of thought&rdquo; (2-5). &nbsp; <strong><u>Types of ataxia</u></strong> Ataxia can result from damage to the cerebellum,&nbsp;proprioceptive, vestibular and visual pathways, and/or&nbsp;any interconnection between these pathways. Although&nbsp;there has been no consensus concerning classification of ataxias in&nbsp;literature, depending on system involvement, ataaxias are classified into following: <em>1. Cerebellar ataxia</em> <em>2. Vestibular ataxia</em> <em>3. Sensory ataxia</em> <em>4. Frontal ataxia</em> <em>5. Ataxic-hemiparesis</em> <em>6. Optic ataxia</em> <em>7. Visual ataxia</em> <em>8. Mixed ataxia</em> <strong>&nbsp;</strong> <strong>Cerebellar ataxia</strong> The designation cerebellar ataxia is employed to describe&nbsp;ataxia due to&nbsp;malfunction of the cerebellum.&nbsp;Ataxia, hypotonia, asynergy, dysmetria, dyschronometria,&nbsp;nystagmus, dysdiadochokinesia, tremor, and cognitive malfunction are features of cerebellar dysfunctions. Where and how these abnormalities&nbsp;manifest themselves will depend on which of the cerebellar structures, i.e.,&nbsp;vestibulocerebellum, spinocerebellum or cerebrocerebellum, have been damaged(6). &nbsp; Vestibulocerebellum (flocculonodular lobe) malfunction is marked by vertigo&nbsp;imbalance and abnormal eye movements. This&nbsp;appears as postural instability in an attempt&nbsp;to increase the base. Instability is thus aggravated&nbsp;while standing with the feet together, whether&nbsp;the eyes are open or closed. Some of the eye&nbsp;movement abnormalities like gaze-evoked&nbsp;nystagmus, rebound nystagmus, ocular dysmetria, inability to inhibit the vestibulo-ocular reflex and&nbsp;abnormalities of optokinetic nystagmus are also&nbsp;observed (7). Spinocerebellar (vermis and paravermis)&nbsp;dysfunction manifests as a broad-based "Drunken&nbsp;sailor" Gait referred to as truncal ataxia,&nbsp;characterized by uncertain starts and stops, lateral&nbsp;deviations, and unequal steps, and gait ataxia (8). Cerebrocerebellar dysfunction manifests as&nbsp;disturbances in the execution of voluntary, planned&nbsp;movements by the extremities. These include:&nbsp;Intentional tremor, writing difficulty, dysarthria,&nbsp;dysmetria, abnormality of alternating movements,&nbsp;loss of check reflex, and hypotonia. Intention&nbsp;tremor is a kinetic tremor that appears as a broad,&nbsp;course, and low frequency (less than 5 hz) tremor. The amplitude of an intention tremor increases as anextremity approaches the endpoint of voluntary and&nbsp;visually guided movement. Intention tremor is caused by&nbsp;dysfunction of the lateral zone of the&nbsp;cerebellum, and superior cerebellar peduncle. Intention tremors can also be seen as a result of &nbsp;damage to the brainstem or thalamus. Depending on the location of cerebellar damage, these tremors can be either unilateral or&nbsp;bilateral. Kinetic and postural tremors or titubations also occur in cerebellar diseases. There are also writing abnormalities in cerebellar ataxia characterized by large, unequal letters, and&nbsp;irregular underlining. Cerebellar&nbsp;dysarthria is characterized by slurred, monotonous&nbsp;or scanning&nbsp;speech. Dysmetria is inability to judge&nbsp;distances or ranges of movement, as undershooting&nbsp;(hypometria), or overshooting (hypermetria), the&nbsp;required distance or range to reach a target. Breakdown of alternating movements known as&nbsp;asynergia or dyssynergia characterizes defects&nbsp;in the&nbsp;sequence and timing of the constituent parts of a&nbsp;movement. Dysdiadochokinesia can entail rapid&nbsp;altering between pronation and supination of the&nbsp;forearm. Bradyteleokinesia is terminal&nbsp;slowing in reaching the target. Rebound&nbsp;phenomenon is also sometimes seen in&nbsp;patients with cerebellar ataxia. Hypotonia and&nbsp;hyporeflexia, pendular tendon reflexes are also&nbsp;seen in acute cerebellar lesion (2,6,8). &nbsp; <strong>Vestibular ataxia </strong> Vestibular ataxia follows as a result of&nbsp;vestibular dysfunction. Its clinical presentation is&nbsp;based on the speed of lesion development, extent&nbsp;of the lesion such as unilateral or bilateral, and&nbsp;the state of vestibular compensation. Vestibular&nbsp;dysfunction caused by acute-onset unilateral lesion&nbsp;is characterized by severe vertigo, nausea,&nbsp;vomiting, blurred vision and nystagmus. In&nbsp;slow-onset, chronic bilateral vestibular&nbsp;dysfunction cases, these symptoms are absent&nbsp;and dysequilibrium may be the sole presentation (6). Vestibular ataxia produces gross difficulties&nbsp;with gait and balance reactions in sitting and standing. Sudden vertigo may be&nbsp;associated with an inability to walk or even to stand.&nbsp;The patient stumbles on walking, has a&nbsp;broad base support and can lean backwards or&nbsp;to the side of the lesion. Head and trunk motion&nbsp;and subsequently arm motion are often diminished&nbsp;because of vertigo (9). The equilibrium in vestibular ataxia&nbsp;is perturbed when performing a head or eye&nbsp;movement. Ataxia may be provoked by asking them&nbsp;to move the head from side to side while walking. Balance on one foot or walking parallel&nbsp;with open or closed eyes may also be disrupted&nbsp;(10). In addition, the patient with vestibular&nbsp;dysfunction depend to a large degree on visual input,&nbsp;so shutting the eyes highlights the gait disorder.&nbsp;Since vestibular ataxia is gravity-dependent,&nbsp;limb movement incoordination cannot be&nbsp;elicited when the patient is tested in the recumbent&nbsp;position but appears when the patient attempts to stand or walk. Extremity ataxia is by no&nbsp;means evident in vestibular ataxia (11). Vestibular dysfunction also includes&nbsp;spontaneous or positional nystagmus, robotic gait&nbsp;ataxia with head turning, and on difficulty in balancing on one foot or on a complaint surface with eyes closed. Nystagmus is often encountered in unilateral peripheral vestibular lesion, typically&nbsp;unidirectional, and mostly most prominent on gaze away from the side of vestibular lesion. Head-shaking nystagmus is another helpful finding to diagnose patients with unilateral&nbsp;vestibular hypofunction. The head-thrust test is positive in peripheral Vestibular disorders. Dix-hallpike test is important,particularly when paroxysmal positional vertigois being tested. Central vestibular disorders also result indeficits in eye movement conjugation, saccadic pursuitand horizontal optokinetic abnormalities, spontaneous or positional central nystagmus,failure of suppression of fixation, slowing of thenystagmus fast phases, slowing of the nystagmus slow phases,retraction of nystagmus, perverted nystagmus, verticaloptokinetic abnormalities, and retraction Nystagmus. Deep tendon reflexes are normal, and romberg test is also negative in vestibular disorders (12). Vestibular ataxia can occur because of central vestibular lesions such as medullar stroke (wallenberg&rsquo;s syndrome), migraine, and multiple sclerosis; and peripheral vestibular diseases such as meniere&rsquo;s disease, benign paroxysmal positional vertigo, or vestibular neuronitis (1). &nbsp; <strong>Sensory ataxia</strong> The term sensory ataxia refers to ataxia due to&nbsp;loss of proprioception, loss of response to the&nbsp;positions of body and joint parts. The latter is&nbsp;generally due to an impairment of posterior&nbsp;columns&nbsp;of spinal cord. At times etiology of sensory&nbsp;ataxia would be impairment of cerebellum, thalamus,&nbsp;parietal lobes, and sensory peripheral nerves (1,13). Sensory ataxia presents itself with a clumsy&nbsp;&ldquo;stomping&rdquo; Gait with heavy heel strike, and a&nbsp;postural instability that is usually exacerbated when the&nbsp;insufficiency of proprioceptive input&nbsp;cannot be substituted&nbsp;by visual input. In sensory ataxic patients, they usually&nbsp;complain of loss of balance in darkness. With their eyes closed,&nbsp;instability is significantly increased, leading to large oscillations and possibly&nbsp;a fall (positive romberg&rsquo;s test). Aggravation of&nbsp;finger-pointing test with closed eyes is another&nbsp;feature of sensory ataxia. Further, when the patient stands&nbsp;arms and hands held out in the direction&nbsp;of the doctor, if the eyes are closed, the patient&rsquo;s finger will have a&nbsp;habit of &ldquo;dropping down&rdquo; And then coming back to&nbsp;the horizontal outstretched position by sudden&nbsp;muscular spasm, it is known as ataxic hand (2,3). Sensory&nbsp;ataxia differs from cerebellar ataxia by&nbsp;presence of near-normal coordination, and marked worsening of&nbsp;. Coordination when the eyes are closed. Sensory ataxia, on the other hand, likewise lacks the characteristic features of cerebellar ataxia&nbsp;such as&nbsp;pendular reflexes, cerebellar dysarthria, nystagmus and abnormal pursuit/saccadic eye movements (14). <strong>&nbsp;</strong> <strong>Frontal ataxia</strong> Frontal ataxia is also known as gait apraxia, and is observed in frontal lobe lesions such as tumors, abscesses, cerebrovascular disease and normal pressure hydrocephalus. Frontal ataxia patients exhibit inability to stand erect. Widened stance base, enhanced body sway and falls, truncal motion control loss, locomotor disability with gait ignition failure, hesitation in&nbsp;initiating, shuffling, short steps, and freezing are also observed in frontal Ataxia. A patient will still tend to hyperextend even with the use of support. Patients with frontal ataxia most frequently will push their foot rather than lift and place normally. This has also been described as a &ldquo;glue-footed&rdquo; Or &ldquo;magnetic&rdquo; Gait. Patient&rsquo;s legs are scissors-cross position while walking and there is incoordination between the trunk and legs. Frontal ataxia will be accompanied by dementia, urinary&nbsp;incontinence, and frontal release signs such as grasp,&nbsp;snout, palmomental and glabellar responses (1,15). Classically, normal pressure hydrocephalus is&nbsp;characterized by frontal gait disturbance, dementia&nbsp;and/or urinary incontinence, and ventricular&nbsp;enlargement. Broad-based, short-step, magnetic gait&nbsp;with start hesitation and increased instability on&nbsp;turning, which is commonly&nbsp;called apraxic/ataxic gait,&nbsp;are the cardinal signs of normal pressure&nbsp;hydrocephalus. The&nbsp;cerebrospinal fluid tap test is a&nbsp;primary diagnostic instrument because of the convenience&nbsp;and lower invasiveness. The programmable valves used in&nbsp;shunt surgeries are utilized in the&nbsp;treatment of normal&nbsp;pressure hydrocephalus (16). In differential diagnosis of frontal ataxia; the&nbsp;slowness of walking, lack of upper limb ataxia,&nbsp;dysarthria or nystagmus discriminates the wide&nbsp;stance base from cerebellar ataxia. A vibrant facial&nbsp;expression, normal voluntary movements of the&nbsp;upper limbs, upper motor neuron findings, and the&nbsp;lacks a rest tremor differentiate from parkinson&rsquo;s disease (17). &nbsp; <strong>Ataxia hemiparesis</strong>&nbsp; Ataxic-hemiparesis is a well-known clinical syndrome&nbsp;of homolateral ataxia with associated impairment&nbsp;of the corticospinal tract. Ataxia is typically a more&nbsp;bothersome symptom than weakness of the affected&nbsp;arm or leg. The face is spared. Since the fronto-ponto-cerebellar fibers may originate from the&nbsp;frontal cortex, including the precentral gyrus,&nbsp;most likely near the cortico-spinal tract, damage&nbsp;at this location could lead to ataxic-hemiparesis. Though&nbsp;ataxic- hemiparesis results from pontine &nbsp;or internal capsule/corona radiata lesions mainly, it also &nbsp;has been reported to result in the midbrain, diencephalic-mesencephalic &nbsp;junction, thalamus, parietal lobe, and the precentral &nbsp;gyrus lesions. Ischemic infarct is the most prevalent &nbsp;cause of the syndrome, although hemorrhagic, neoplastic&nbsp;and demyelinating disorders have also been noted (18,19). &nbsp; <strong>Optic ataxia</strong> Optic ataxia usually results from damage to the &nbsp;posterior parietal cortex, and is the inability to&nbsp;execute purposeful movement or movement to command in the absence of paralysis or other&nbsp;sensory and cerebellar impairments. Optic ataxia occurs when the patient has a deficit in reaching to visual command that cannot be explained by cerebellar, motor, somatosensory, visual field defect or loss of acuity. Optic ataxia patients execute an inaccurate reaching&nbsp;movement towards a target or object in space, this is especially true with&nbsp;their non-lesioned hand. Object grasping is also impaired in optic ataxia patients. The lesion also disturbs the proper shaping of the hand as a function of the objects&rsquo; configuration, and thereby creates a serious impairment in tool grasping or movement (20). Optic ataxia is one of the common symptoms of balint&rsquo;s syndrome. This syndrome comprises the clinical symptom triad of simultanagnosia, ocular apraxia and optic ataxia. These symptoms, visual&nbsp;oculus ataxia, an ocular apraxia; a deficit in ocular scanning, or simultanagnosia, or disorientation; optic ataxia, an impairment of pointing and reaching with vision as guidance; or ocular apraxia, a scanning deficit of vision, are rare and highly disabling since they are&nbsp;associated with disturbances of visuospatial ability, visual scanning and attentional operations. Bilateral border zone infarction occipitoparietal in location is the most frequent etiology of total balint&rsquo;s syndrome (20,21). &nbsp; <strong>Visual ataxia</strong>&nbsp; Visual ataxia is instability because of visual disturbances. The human being is very much reliant on vision for gait and balance. Foveal vision seems to be most significant for this purpose, but peripheral vision also plays a role in balance. The central part of the visual field in comparison with the peripheral retina is the one that dominates postural control. Visual acuity results in a linearly increasing postural instability. Visual acuity abnormalities or visual&nbsp;field defects result in exaggerated body sway, equilibrium disturbances, and predispose to the patient toppling over. Hemianopia amplifies lateral oscillations in the standing patient and the projection of the body&rsquo;s centre of gravity is shifted towards the hemianopia. Patients adapting to new bifocals may become unsteady or even topple over. Vision may also be disrupted by abnormalities of eye movement. Limitation of eye movements, particularly&nbsp;downward motion, diplopia or ossilopsia can &not;result in ataxia and falls. Conversely, &not;multisensory disequilibrium is present in deficits of &not;more than one sensory systems like visual, vestibular, &not;and proprioceptive (22,23). &nbsp; <strong>Mixed ataxia</strong>&nbsp; Mixed ataxia is present when the symptoms of two &not;or more ataxias like the presence of&nbsp;symptoms of sensory and cerebellar ataxia, areseen simultaneously. All forms of ataxia may have &not;overlapping etiologies and hence may coexist. In&nbsp;certain neurologic disorders can be frequently combined ataxia. Cerebellar, vestibular and sensory ataxia are combined in multiple sclerosis, for instance, and cerebellar and sensory ataxia are combined in spino-cerebellar ataxias. Vestibular and frontal and cerebellar ataxia can coexist in certain neurologic degenerative conditions such as multiple system atrophy. Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (canvas) is also&nbsp;mixed ataxia syndrome (24). &nbsp; <strong><u>Causes</u></strong> Congenital nonprogressive ataxia occurs early in life, is truly nonprogressive, i.e. The symptoms are not progressively aggravated. Motor development is usually retarded in such instances, and the accompanying mental retardation is common. They are sequelae of prenatal or perinatal injury, arrested hydrocephalus, and other nongenetic and genetic disorders of the cerebellum. Acute onset ataxia is usually due to cerebellar hemorrhage and cerebellar&nbsp;infarction. Diagnosis has to be made as an emergency by ct or mri. Virus or postinfectious cerebellitis, gait and limb ataxia, dysarthria, and pyrexia occurring over days or hours in young children or young adults. Paraneoplastic cerebellar syndromes from neuroblastoma in children, and lung or overian carcinoma in adults are also associated with subacute ataxia, dysarthria, nystagmus, opsoclonus,&nbsp;and myoclonus. Other etiologies of subacute ataxia&nbsp;are hydrocephalus, foramen magnum&nbsp;compression, posterior fossa tumors, abscess,&nbsp;multiple sclerosis, toxins and drugs. The miller-&nbsp;fisher syndrome also features subacute ataxia,&nbsp;ophthalmoplegia, and areflexia. The anti-gq1b igg&nbsp;nantibody titer is most frequently raised in miller-&nbsp;fisher syndrome (21). Chronic progressive ataxias are usually&nbsp;linked with inherited degenerative diseases. Conversely,&nbsp;chronic alcoholism, certain of drugs&nbsp;and toxic agents, chronic rubella panencephalitis,&nbsp;creutzfeldt-jacob disease, severe vitamin e&nbsp;deficiency, primary progressive multiple sclerosis,&nbsp;hypothyroidism, paraneoplastic cerebellar&nbsp;degeneration also are recognized as ataxia with&nbsp;chronic progressive course. Chronic alcoholism is&nbsp;one of the most common causes of cerebellar&nbsp;degeneration in adults (25). Episodic ataxias can be classically caused by drug&nbsp;use, transient vertebrobasilar ischemic attacks,&nbsp;multiple sclerosis, foramen magnum compression,&nbsp;colloid cyst, inherited periodic ataxias, and&nbsp;metabolic disorders such as mitochondrial&nbsp;encephalopathies, aminoacidurias, and leigh&rsquo;s&nbsp;syndrome. The attacks of ataxia in metabolic&nbsp;diseases may be precipitated by infection or diet,&nbsp;and can also be accompanied by lethargy, vomiting&nbsp;and seizures. Blood ammonia, pyruvate, lactate and&nbsp;amino acids are screening tests for metabolic&nbsp;diseases (26). Cerebellar ataxia may be hereditary or non-&nbsp;hereditary. Non-hereditary cerebellar ataxia is&nbsp;also known as sporadic cerebellar ataxia. The genetic&nbsp;forms of ataxia are identified on the basis of&nbsp;family history,&nbsp;physical examination, neuroimaging, and molecular&nbsp;genetic testing. Four patterns of inheritance are present&nbsp;for this genetic disease; <em>A</em><em>) autosomal dominant</em><em>&nbsp;</em><em>inheritance:</em> A faulty gene is received from one parent.&nbsp;Autosomal dominant cerebellar ataxias are also&nbsp;known as spinocerebellar ataxias (scas). Sca1 was&nbsp;initially identified as a dominant ataxia&nbsp;and sca36 was&nbsp;discovered in 2011. B)&nbsp;<em>Autosomal recessive</em><em>&nbsp;</em><em>inheritance:</em>&nbsp; Carriers are the parents. Most common&nbsp;recessive ataxia is friedreich&rsquo;s ataxia. C)&nbsp;<em>Mitochondrial ataxias: </em> These types of ataxias are&nbsp;passed to all offspring in the classical pattern by women. D)&nbsp;<em>X-linked:</em>&nbsp; Males alone are affected and&nbsp;females are carriers. The most common x-linked&nbsp;variant of ataxia is fragile x tremor ataxia syndrome&nbsp;(25,27).&nbsp; Hereditary episodic ataxia (ea) is an autosomal&nbsp;dominant disorder with sporadic spells&nbsp;of ataxia with or without myokymia. Seven inherited episodic ataxias have been identified thus far. Two common types of episodic ataxia syndrome have been described and are known as ea1 and ea2. Stress, startle, or intense exercise may cause ataxia. Progressive cerebellar degenerative illnesses, familial hemiplegic migraine, spinocerebellar ataxia, or familial vestibulopathy in the shape of episodic vertigo and migraine headache develop in some patients with episodic ataxia (26,28). &nbsp; <strong><u>Treatment of ataxia </u></strong> The management of ataxia involves a general assessment by an interdisciplinary team that may consist of neurologists, rehabilitation medicine specialists and physiotherapists. Once known acquired factors are corrected, care is symptomatic but can be supplemented by physical, occupational, and speech therapy. Ataxias that result from intrinsic conditions such as stroke, multiple sclerosis, hypothyroidism, vitamin e and b12 deficiency, wilson&rsquo;s disease, infections and certain tumours or exposure to an offending drug or chemical can&nbsp;be treated. Some examples of ataxia such as hereditary nataxias are treated with no specific treatments (29). However&nbsp;development of ataxia in some of the patients has been&nbsp;treated with decreased rate with the use of amantadine. On the other hand, it is reported that, in case series, riluzole has a range of&nbsp;actions among degenerative cerebellar ataxic patients. Riluzole activates calcium-&nbsp;dependent potassium channels, leading to inhibition of&nbsp;deep cerebellar nuclei and lessening cerebellar&nbsp;hyperexcitabililty. Riluzole administration (100 mg/day)&nbsp;has received level b recommendations from the&nbsp;european federation of neurological societies (30). Physical therapy employed for increasing the&nbsp;strength of muscles is of critical significance in&nbsp;the management of ataxia (29). The patients having ataxia due to&nbsp;vitamin e deficiency should be supplemented with&nbsp;vitamin e (800 mg daily). It leads to the cessation&nbsp;of progression of neurological symptoms and mild&nbsp;improvement in certain patients, especially in the nearly stages of the disease (31). Wilson&rsquo;s disease is an autosomal recessive genetic disorder of copper metabolism with resultant accumulation of copper in many organs. The most characteristic neurologic findings in wilson&rsquo;s disease are ataxia, dysathria, and extrapyramidal signs. Symptoms may be entirely reversible on zinc therapy or on copper chelators (32). Ataxia with coq10 deficiency&nbsp;observed in children and also adults is an&nbsp;apparently autosomal recessive disease with&nbsp;heterogeneous clinical presentation. Patients with&nbsp;this disorder benefit with coq10 supplementation&nbsp;in early stages (33). In friedreich&rsquo;s ataxia, there are&nbsp;oxidative stress damages as well as an accumulation&nbsp;of iron within the mitochondria. Due to these findings,&nbsp;there has been immense interest to assess the effect of&nbsp;antioxidants (eg, idebenone), vitamin e and iron&nbsp;chelators (e.g., deferiprone) and drugs with the ability to increase frataxin levels (34). Gluten ataxia has recently been defined as a sporadic cerebellar ataxia syndrome with the presence of antigliadin or endomysium or transglutaminase antibodies, and has been shown in a one-year controlled trial to be treatable with a gluten-free diet (35). Symptomatic treatment also addresses the treatment of the co-morbid conditions such as muscle&nbsp;cramps, stiffness, tremor, spasticity, dysphagia as well as depression, anxiety, sleep disorders, bowel, bladder, and sexual dysfunction, etc. Baclofen, tizanidine or botulinum toxin are medications for muscle stiffness, spasticity, cramps and pain. On the other hand, in episodic ataxia type 2 patients, relief of symptoms can be obtained by treatment with acetazolamide and aminopyridines, and also by the avoidance of precipitating factors such as stress, alcohol and caffeine (26,28). Oscillopsia and nystagmus can be treated with medications like gabapentin.&nbsp;Depression can be treated with antidepressant medications as well as cognitive-behavioral therapy. Clonazepam, beta-blockers like propranolol, or primidone can decrease the salience of certain&nbsp;cerebellar tremors (29). Surgical ablation or deep brain stimulation of the ventral intermediate nucleus of the thalamus can be beneficial in decreasing cerebellar tremor, however, they most often fail to decrease ataxia much, although there have been some reported cases with advantage (25). <strong><u>Conclusion</u></strong>&nbsp; Ataxia resulting from damage to the cerebellum&nbsp;and its connections, is characterized as incoordination&nbsp;and disturbance of balance in movements, and&nbsp;disturbed control of posture. Clinically, ataxias are&nbsp;subdivided into cerebellar, vestibular, sensory,&nbsp;frontal, optic, visual, mixed ataxia and ataxic-hemiparesis. Etiologically, the ataxias are being divided&nbsp;into hereditary, sporadic degenerative, and acquired&nbsp;ataxias. The genetic forms of ataxia must&nbsp;be distinguished from the acquired ataxias. 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