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Khilendra, Kumar Sahu Ankita Damahe* Antra Sahu Nilesh kumar Devki Markande Chunesh kumar Janvi NilMarkar. "A Review on Novel Approaches for Cure, Diagnosis, Treatment and Future Direction in Polycythemia." International Journal of Scientific Research and Technology 2, no. 2 (2025): 102–10. https://doi.org/10.5281/zenodo.14852627.
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An excess of red blood cells in the body is known as polycythemia. The blood becomes thicker due to the additional cells, which raises the risk of blood clots and other health problems. There are various causes of polycythemia, and each one has a unique course of treatment. Discover more about polycythemia's causes, symptoms, and available treatments in this article. An rise in hemoglobin or hematocrit levels over the standard values is known as polycythemia. The majority of its causes are linked to the development of hyper viscosity, and the majority of cases are classified as primary or secondary polycythemia (SP). The underlying disease of the former, often referred to as polycythemia vera (PV), affects the bone marrow itself, whereas the latter is typified by an overabundance of stimulation of cell creation in the normal bone marrow. This study included patients who were diagnosed with PV or SP after undergoing additional testing at our hospital from January 2020 to December 2023 after polycythemia was discovered. The 2016 WHO criteria (hemoglobin > 16.5 mg/L for men and >16.0 mg/L for women, and/or hematocrit > 49% for men and >48% for women) were followed in determining the laboratory thresholds used to diagnose polycythemia. A case of PV exacerbated by cardiac hypertrophy is presented in this paper. It provides a full summary of the patient's long-term follow-up and a detailed account of their experience using antihypertensive medication. With an emphasis on clinical case studies, a thorough literature analysis was also carried out to look into any potential connections between PV and the risk of cardiovascular disease. The goal is to offer fresh viewpoints and understandings for evaluating cardiovascular risk and predicting prognosis in MPN patients. These findings have important ramifications for future clinical care guidelines in addition to improving our understanding of the relationship between PV and cardiovascular disease
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Válkova, Z., and M. Vymazal. "Intraokuläre Hypertension bei Polycythaemia vera." Spektrum der Augenheilkunde 8, no. 6 (1994): 284–85. http://dx.doi.org/10.1007/bf03163720.
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Coulthard, M. G. "Polycythaemia and hypertension caused by renal artery stenosis." Archives of Disease in Childhood 86, no. 4 (2002): 307–8. http://dx.doi.org/10.1136/adc.86.4.307.
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Tachi, Kenneth, Victor Ekem, and Yvonne Dei-Adomakoh. "Delayed diagnosis of polycythaemia vera in an adult female with non-cirrhotic portal hypertension." Ghana Medical Journal 56, no. 1 (2022): 38–41. http://dx.doi.org/10.4314/gmj.v56i1.6.
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Polycythaemia vera (PV) is a rare myeloproliferative neoplasm characterized primarily by erythrocytosis and an in-creased risk of thrombosis. We report a case of PV in a 60-year-old female with diabetes mellitus (DM) and a past history of recurrent abdominal pain and documented oesophageal varices who was followed up for 2 years as a case of non-cirrhotic portal hypertension of unknown cause. PV was only diagnosed after persistent complaints of vaso-motor symptoms and better scrutiny of full blood count results.
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Khandait, Vinod, Sneha S. Bhujle, and Nandita Bagchi. "Acute Myocardial Infarction in a Patient of Polycythaemia Vera." Vidarbha Journal of Internal Medicine 32 (January 31, 2022): 59–62. http://dx.doi.org/10.25259/vjim_8_2021.
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Polycythaemia Vera is the most common form of myeloproliferative neoplasm.The median age of diagnosis is 60 years. Atherosclerosis is the most common cause of myocardial infarction, however other causes too should be looked for in the patients. Here, we present a case of 62 year old female who was a known case of hypertension and Ischemic heart disease on regular medications. She came with complaints of chest pain for the past 2 days. ECG showed NSTEMI with raised cardiac enzymes. She developed left side hemiparesis after admission, and the CT head showed acute infarct in right frontal lobe. She was started on treatment for NSTEMI and cerebrovascular event. Her CBC showed Hb-17.1g/dl, TLC-51600/mm3 and platelet count-625000/mm3, hence we suspected her to have polycythemia Vera with trilineage involvement. Serum erythropoietin was normal. JAK2(V617) profile was positive for homozygous (TT) mutation. Bone marrow biopsy findings were suggestive of trilineage hyperplasia. She was diagnosed as a case of polycythemia Vera. Our patient came into the age group wherein various risk factors for ischemic heart disease could be present like hyperlipidaemia, hypertension, diabetes mellitus but a careful look out for other causes too should be kept in mind.
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Moore-Gillon, J. C., and I. R. Cameron. "Right ventricular hypertrophy and polycythaemia in rats after intermittent exposure to hypoxia." Clinical Science 69, no. 5 (1985): 595–99. http://dx.doi.org/10.1042/cs0690595.
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1. Six groups of 20 male adult rats were maintained in an environmental chamber, each group for a period of 28 days. One group breathed air throughout its experimental period, and a second group breathed a normobaric atmosphere of 12% oxygen. The other four groups were exposed to this hypoxic atmosphere for only a proportion of each 24 h cycle: 2, 4 and 12 h daily, and eight periods of 30 min daily. 2. After 28 days, measurement was made, in each rat, of right ventricule (RV) weight and of red cell mass (RCM) by using 51Cr-labelled rat erythrocytes. 3. In the normoxic control group, RV weight corrected for log body weight in grams was 63.2 ± 1 mg/log body wt. and RCM was 2.02 ± 0.05 ml/100 g body wt. This was significantly less than in the group hypoxic for only 2 h each day for 28 days: RV weight 66.6 ± 0.8 mg/log body wt. (P < 0.05) and RCM 2.27 ± 0.05 ml/100g body wt. (P < 0.05). Greater increases compared with control were observed in all the other hypoxic groups. There was no significant difference in the increases in RV weight and RCM produced by daily hypoxia in a 4 h continuous period and daily hypoxia in eight 30 min periods. 4. The possible role of intermittent hypoxia in producing polycythaemia and pulmonary hypertension has been the subject of much speculation. Our results show that intermittent hypoxia is a potent stimulus to erythropoiesis and to pulmonary hypertension, reflected in RV hypertrophy. They support the view that abnormalities of respiration during sleep may be responsible for the polycythaemia and cor pulmonale seen in some patients with sleep apnoea syndromes and with chronic obstructive airways disease.
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Girish, T., M. P. Lamb, T. P. Rollason, and L. J. R. Brown. "An endometrioid tumour of the ovary presenting with hyperandrogenism, secondary polycythaemia and hypertension." BJOG: An International Journal of Obstetrics and Gynaecology 108, no. 3 (2001): 330–32. http://dx.doi.org/10.1111/j.1471-0528.2001.00063.x.
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Girish, T., M. P. Lamb, T. P. Rollason, and L. J. R. Brown. "An endometrioid tumour of the ovary presenting with hyperandrogenism, secondary polycythaemia and hypertension." British Journal of Obstetrics and Gynaecology 108, no. 3 (2001): 330–32. http://dx.doi.org/10.1016/s0306-5456(00)00063-2.
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Rao, Raunak, Spoorthy Kulkarni, and Ian B. Wilkinson. "Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms." Case Reports in Vascular Medicine 2020 (November 7, 2020): 1–6. http://dx.doi.org/10.1155/2020/8887423.
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Background. Myeloproliferative neoplasms are a heterogeneous group of disorders resulting from the abnormal proliferation of one or more terminal myeloid cells—established complications include thrombosis and haemorrhagic events; however, there is limited evidence to suggest an association with arterial hypertension. Herein, we report two independent cases of severe hypertension in JAK2 mutation-positive myeloproliferative neoplasms. Case Presentations. Case 1: a 39-year-old male was referred to our specialist hypertension unit with high blood pressure (BP) (200/120 mmHg), erythromelalgia, and headaches. We recorded elevated serum creatinine levels (146 μM) and panmyelosis. Bone marrow biopsy confirmed JAK2-mutation-positive polycythaemia vera. Renal imaging revealed renal artery stenosis. Aspirin, long-acting nifedipine, interferon-alpha 2A, and renal artery angioplasty were employed in management. BP reached below target levels to an average of 119/88 mmHg. Renal parameters normalised gradually alongside BP. Case 2: a 45-year-old male presented with high BP (208/131 mmHg), acrocyanosis, (vasculitic) skin rashes, and nonhealing ulcers. Fundoscopy showed optic disc blurring in the left eye and full blood count revealed thrombocytosis. Bone marrow biopsy confirmed JAK2-mutation-positive essential thrombocytosis. No renal artery stenosis was found. Cardiac output was measured at 5 L/min using an inert gas rebreathing method, providing an estimated peripheral vascular resistance of 1840 dynes/s/cm5. BP was well-controlled (reaching 130/70 mmHg) with CCBs. Conclusions. These presentations highlight the utility of full blood count analysis in patients with severe hypertension. Hyperviscosity and constitutive JAK-STAT activation are amongst the proposed pathophysiology linking myeloproliferative neoplasms and hypertension. Further experimental and clinical research is necessary to identify and understand possible interactions between BP and myeloproliferative neoplasms.
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Janender Baghel, Dhiraj Jhamb, Rajesh Kumar, and Kaushik Chatterjee. "Prevalence of Lifestyle Diseases in Non-Acclimatized Lowlanders at High Altitude in subdivision Darjeeling district of Eastern Himalayan Region, India." Indian Journal of Public Health Research & Development 15, no. 2 (2024): 136–41. http://dx.doi.org/10.37506/1qg0a273.
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Background: Lifestyle diseases including overweight, obesity, hypertension and metabolic syndrome leads toheart diseases, increased risk for insulin resistance, diabetes and stroke. Obesity is one of the lifestyle diseases declared as worldwide epidemic which is a major health burden. The study determine the association of lifestylediseases with Body Mass Indexinnon-acclimatized lowlanders at altitude of 7500 ft above sea level and to estimate effect of altitude on anthropometric and biochemical parameters.Methods: The total of 300 male age between 20-57 years studied. The present retrospective study was done using Annual Medical Examination documents which were recorded at plains and at high altitude. The anthropometric indexes observed using Asia-pacific criteria from the World Health Organization and biochemical parametersobserved using metabolic syndrome according to the American Heart Association.Conclusion: The present study comprises of maximum cases of young adults at plains and middle-aged at high altitude. The maximum overweight cases were found at high altitude and general obese cases found at plains. There is a slight risk of hypertension and deranged lipid profile at this altitude but no risk of polycythaemia. The present study’s indicator of abdominal obesity at high altitude is the waist-hip ratio. Increase in altitude causes changes in body mass index, blood glucose levels and lipid profile. Additionally, positive correlation found between lifestyle diseases such as central or abdominal obesity(r=0.33,P<0.05) and diastolic blood pressure (r=0.19,P<0.05) with Body Mass Index at high altitude. The present study agreed with the various studies from India and abroad.
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Jain, Neeraj, Dnyaneshwar Jadhav, Akash Chheda, et al. "Major Neurological Syndromes with COVID-19: Lessons to Learn." SciMedicine Journal 4, no. 1 (2022): 13–24. http://dx.doi.org/10.28991/scimedj-2022-0401-02.
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Objective: Covid-19 is a highly infectious viral disease, and our understanding of the impact of this virus on the nervous system is limited. Therefore, we aimed to do a systematic analysis of the neurological manifestations. Methods: We retrospectively studied the clinical, laboratory, and radiological findings of patients with major neurological syndromes (MNS) in Covid-19 over 6 months. Results: We had 39 patients with major neurological syndromes (MNS). The most common MNS was cerebrovascular disease (CVD) (61.53%), in which ischemic stroke (83.33%), cortical sinus thrombosis (12.50%), and haemorrhagic stroke (4.16%) were seen. Among ischemic stroke patients, 50% had a large vessel occlusion, and 66.66% of patients with CVD had a significant residual disability. Cranial neuropathy (15.38%), GBS (10.26%), encephalitis (7.26%), and myelitis (5.12%) were the other MNS. Among the three encephalitis cases, two had CSF-Covid-19 PCR positivity and had severe manifestations and a poor outcome. Associated comorbidities included hypertension (30.76%), diabetes mellitus (12.82%), chronic kidney diseases (7.69%), and polycythaemia vera (2.56%). Lung involvement was seen in 64.1% of patients. Mortality was 17.94% in MNS with Covid-19. Conclusions: The most common major neurological syndrome associated with Covid-19 is CVD with increased frequency of large vessel occlusion causing significant morbidity and mortality. Simultaneous lung and other systemic involvement in MNS results in a deleterious outcome. Doi: 10.28991/SciMedJ-2022-0401-02 Full Text: PDF
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Omoniyi, O. S., I. B. Fajolu, A. Adediran, E. O. Temiye, and J. I. Ladele. "Clinical and haematological features of newborns of mothers with hypertensive disorders in pregnancy in Lagos, Nigeria." Nigerian Journal of Paediatrics 47, no. 3 (2020): 252–57. http://dx.doi.org/10.4314/njp.v47i3.10.
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Background: Newborns of mothers with hypertensive disorders in pregnancy have an increased risk of preterm delivery, low birth weight, perinatalasphyxia and haematological derangements such as polycythaemia, thrombocytopenia and neutropenia. These morbidities are associated with uteroplacental insufficiency. The haematological derangements however have not been studied in detail in African neonates.
 Objective: To determine the clinical and haematological features of newborns of hypertensive mothers
 Methods: Cross-sectional study involving 250 newborns; 125 newborns each of hypertensive mothers (cases), and normotensive mothers (controls). The babies were examined following delivery, their clinical data were recorded, and umbilical cord blood samples were analysed for haematological indices.
 Results: Preterm deliveries were significantly higher amongst infants of hypertensive mothers (31.2%)compared with controls (12.0%);p = 0.000. Similarly, the birth weight, length and head circumference of the cases were significantly lower than the controls; p = 0.008, 0.003 and 0.004 respectively. Low fifth minute APGAR scores occurred more frequently in cases (8.0%) than controls (0.8%), p=0.010; whilst the mean haematocrit was also significantly higher in cases than the controls, p = 0.013. The median absolute neutrophil count and platelet count were significantly lower in cases than controls; p=0.023 and 0.047 respectively. Thrombocytopenia was identified in 40.0% of the cases compared to 27.2% of the controls, p = 0.041
 Conclusion: The present study has shown that newborns of hypertensive mothers have an increased risk of neonatal morbidities such as preterm birth, LBW and thrombocytopenia compared to the newborns of mothers with normal blood pressure in pregnancy, hence close attention needs to be paid to them with emphasis on their haematological system.
 Key words: newborn, pregnancy, hypertension, hypertensive disorders, haematological, clinical
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Augustine, Amie-Anne, Jin Hui Ho, and Hwee Ching Tee. "A RARE PRESENTATION OF MEDULLARY THYROID CARCINOMA." Journal of the ASEAN Federation of Endocrine Societies 40, S1 (2025): 103–4. https://doi.org/10.15605/jafes.040.s1.176.
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INTRODUCTION/BACKGROUNDMedullary thyroid carcinoma (MTC) is a rare neuroendocrine tumour arising from the parafollicular C cells of the thyroid gland, accounting for approximately 4% of all thyroid malignancies. We present a case of MTC with an unusual and life-threatening initial manifestation — cardiac tamponade — which led to the diagnosis. CASEA 63-year-old Kadazan male with a medical history of myocardial infarction with non-obstructive coronary arteries (MINOCA) in 2017, intracranial haemorrhage in 2018, polycythaemia rubra vera, dyslipidaemia, hypertension, and type 2 diabetes mellitus, presented with a three-day history of exertional dyspnoea and chest tightness. He also reported a gradual neck swelling and unintentional weight loss over the past year. Initial chest radiography revealed a right lower zone lung opacity, and he was empirically treated for pneumonia. However, a neck ultrasound demonstrated a right thyroid nodule categorized as TIRADS 4, raising suspicion for malignancy. A contrast-enhanced CT (CECT) of the thorax revealed a suspicious right thyroid nodule with bilateral cervical, supraclavicular, and mediastinal lymphadenopathy, multiple pulmonary nodules, a segment VIII liver lesion, and a significant global pericardial effusion measuring 2.8 cm. Fine needle aspiration cytology (FNAC) of the right thyroid nodule and left cervical lymph node confirmed medullary thyroid carcinoma, with positive staining for calcitonin and amyloid deposits identified via Congo red staining. Transthoracic echocardiography showed right atrial and right ventricular collapse, consistent with cardiac tamponade. Emergency pericardiocentesis was performed, and cytology of the pericardial fluid confirmed metastatic MTC. Further laboratory evaluation revealed markedly elevated serum calcitonin and carcinoembryonic antigen (CEA), along with raised urinary levels of normetanephrine, metanephrine, and 3-methoxytyramine, suggesting a paraneoplastic neuroendocrine profile. Germline RET mutation analysis could not be performed due to resource limitations. Given the presence of distant metastases and extensive locoregional disease, the patient was scheduled for systemic therapy with Cabozantinib with plans for total thyroidectomy following tumour debulking. CONCLUSIONThis case highlights a rare and aggressive presentation of medullary thyroid carcinoma (MTC), manifesting as cardiac tamponade — a life-threatening complication seldom associated with thyroid malignancies. The diagnosis was confirmed through cytological evaluation and supported by elevated tumour markers and imaging. This case underscores the importance of considering metastatic MTC in patients with unexplained pericardial effusion and systemic symptoms, especially in the presence of a suspicious thyroid lesion. Prompt recognition and multidisciplinary management are crucial in optimizing outcomes in such advanced and atypical presentations.
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Mohan Rao, Preethi, Nishant Ranjan, and Thomas Hugh Jones. "PMON266 The Effect of Testosterone Replacement Therapy on Cardiovascular Safety and Other Adverse Effects – a Randomised Double Blinded Placebo Controlled Trial (STRIDE Study)." Journal of the Endocrine Society 6, Supplement_1 (2022): A704. http://dx.doi.org/10.1210/jendso/bvac150.1451.
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Abstract Background Concerns about testosterone treatment and its safety ranging from cardiovascular risk to prostate cancer risk to polycythaemia and obstructive sleep apnoea has always been controversial especially cardiovascular safety. To tip the balance, there were 3 trials that were published in the recent past which induced more controversy by demonstrating that testosterone therapy increased cardiovascular disease and mortality. Our RCT evaluates the safety of testosterone Undecanoate in patients with type 2 diabetes and hypogonadism. Research design and methods: This is a randomised double blinded placebo-controlled add-on trial of testosterone undecanoate (Nebido®) administered every 12 weeks in 65 hypogonadal men with poorly-controlled type 2 diabetes. Phase 1 patients were randomly assigned to either treatment or placebo arm for 6 months of TRT. Phase 2 was an open-labelled phase for 6 months and patients on placebo moved on to the treatment group wherein patients in the treatment group continued. Adverse events, hospitalizations and mortality were recorded and biochemical parameters were measured at baseline and every three months for a year. Results Mean age of the cohort was 59(42-77)years. Baseline characteristics were comparable between active/placebo groups apart from cardiovascular disease and hypertension which were significantly higher in the active group compared to placebo group (n=28 vs n=18, p=0.05), (n=23 vs n=13, p=0.008) respectively. There was no mortality during the period of the trial. There were no cardiovascular adverse effects in either of the phases of the trial in the active arm. One patient had TIA in the placebo arm. There were 3 prostrate related hospitalisations - 2 in those receiving TRT. One gentleman on TRT was diagnosed to have unmasked prostate cancer in the phase-2 of the trial. There was non-significant increase in PSA between active and placebo group at 6 months (p=0.756). There was no significant increase in mean PSA between baseline, 3, 6, 9 and 12months post treatment in active group. There was no significant increase in number of patients with &gt;0.75 µg/l PSA rise between active and placebo group at 6 months (p=0.902). An increase in mean haematocrit value at 6 months post treatment with testosterone (Active group- pre-treatment 00.42±0.033 l/l, post treatment 0.435±0.024 l/l Vs Placebo group – pre-treatment 0.42±0.027 l/l, post treatment 0.46±0.034 l/l, p=0.000) was noted. Similar rise was seen in mean RBC and haemoglobin levels (p=0.000, p=0.001 respectively). Such increase in haematocrit and haemoglobin was observed to persist at 12 months in the active arm of the trial. However, no participant had haematocrit value of more than 0.54l/l in both first and second phase of the trial. Conclusions Results from our study demonstrates that testosterone replacement therapy in physiological dose was not associated with any serious adverse effects. However, a close monitoring of adverse events is highly advisable. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
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Fogliatto, Laura, Raquel Breunig, Tito Vanelli Costa, et al. "The Assessment of the European Leukemianet Criteria for Clinicohematologic Resistance and Intolerance to Hydroxyurea in Polycythemia Vera Is Not Easily Applicable in Daily Practice." Blood 124, no. 21 (2014): 5550. http://dx.doi.org/10.1182/blood.v124.21.5550.5550.
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Abstract Background Polycythemia vera (PV) is a subgroup of myeloproliferative neoplasm (MPN) BCL-ABL1 negative. The current therapy of PV should be aimed at preventing vascular complications and avoid increasing the risk of leukemic transformation. The therapy response monitoring is based in the European LeukemiaNet (ELN) unified definition of clinical resistance and intolerance to hydroxycarbamide in polycythaemia vera consensus process, published by Barbui T et al in Br J Haematol 2010;148(6):961-963. Objectives We conducted a study to assess in our clinical practice the aplicability of the standard criteria for resistance and intolerance proposed by ELN in patients (pts) with PV that have been treated with hydroxycarbamida (HU). Methods This is a retrospective study in a cohort of pts with PV enrolled in a single Hematology University center in South Brazil. All pts were treated according to PV guidelines, and the response monitoring was based on clinical practice. All database was compared to standard criteria proposed by ELN. Intolerance /resistance was defined by: a) need for phlebotomy to keep hematocrit < 45% after 3 months of at least 2 g/d of HU or b) uncontrolled myeloproliferation (ie, platelet count > 400 x109/L and white blood count (WBC) > 10 x109/L) after 3 months of at least 2g/d of HU or c) failure to reduce massive splenomegaly by 50% as measured by palpation or failure to completely relieve symptoms related to splenomegaly after 3 months of at least 2 g/d of HU or d) absolute neutrophil count < 1.0 x109/L or platelet count < 100 x109/L or hemoglobin < 10 g/dL at the lowest dose of HU required to achieve a complete or partial clinicohematologic response or e) presence of leg ulcers or other unacceptable HU related nonhematologic toxicities, such as mucocutaneous manifestations, GI symptoms, pneumonitis or fever at any dose of HU. Results We analyzed data from 33 patients with PV assisted in the last five years in our outpatient clinical data. The ELN criteria for resistance and intolerance were accessed in these patients. At diagnosis, 42,4% of pts were younger than 61yo, and 54,5 were male. Arterial hypertension, diabetes mellitus and dyslipidemia were identified on 21, 2 and 7 pts, respectively. Only one patient was tobacco smoker at diagnosis. Total of 5 pts showed WBC > 15 x109/L, and 7 pts showed platelets > 450 x109/L. Massive splenomegaly is a rare PV manifestation in our series, occurring in 2 pts. Five patients complained of symptoms related to PV as pruritus and vasomotor phenomena at diagnosis. Less than 5% of patients had been treated with 2 g of HU for more than 3 months. In daily practice, when the patient presented hematologic toxicity, the HU was decreased and, if the hematocrit was over 45%, an occasional phlebotomy was performed. In relation to platelets (less than 400 x 109/L) and leucocyte (less than 10 x109/L) counts, these targets were not used exclusively in the clinical practice to change treatment. The intolerance was easily discriminated in patients with leg ulcers and other non-hematological events. Conclusion These criteria were done based on an expertise consensus for international criteria standardization for clinical studies. Its application in a retrospective study, using clinical daily practice data is not adequate. The reason is that in the last years the main target to treat patients was the hematocrit above 45%, the exact number of platelets and leucocytes is still not a consensus to define resistance, so different counts had been used to guide treatment, we rarely used HU doses above 1500 mg/daily to treat our patients and massive splenomegaly was observed in very few patients. These criteria should be used most for prospective studies. Disclosures No relevant conflicts of interest to declare.
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Mehta, Dipal, Holly Theaker, Donal P. McLornan, et al. "A Real World, Single-Centre Study of Young Adult Patients with Philadelphia Negative Myeloproliferative Neoplasms." Blood 142, Supplement 1 (2023): 6402. http://dx.doi.org/10.1182/blood-2023-189502.
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Classical ‘Philadelphia negative’ myeloproliferative neoplasms (MPNs) are clonal haematopoietic disorders and include essential thrombocythaemia (ET), polycythaemia vera (PV) and myelofibrosis (MF). MPNs have a heterogenous phenotype with an inherent risk of thromboembolic and haemorrhagic complications, and risk of blastic transformation. MPNs are commonly diagnosed in the 6 th decade or later, although up to 20% of patients are diagnosed &lt; 40 years. Current risk stratification and treatment guidance, however, is frequently extrapolated from older cohorts. Here, we present a comprehensive retrospective analysis of 107 MPN adolescent and young adult (AYA) patients diagnosed &lt;40 years, representing 19.6% of the total outpatient MPN cohort at University College London Hospital. Median follow up was 78 months (range (r), 1 - 507). Table 1 displays clinicopathological details of the entire cohort. Median age at diagnosis was 32 years (r,13-40). There was a female preponderance (66%); 57.9% of patients were Caucasian. Most patients had a diagnosis of ET (57.9%), with 24.3% PV and 9.3% MF, respectively. The most common driver mutation was JAK2 V617F, observed in 60.7%, followed by CALR (15.9%) and MPL (1.9%); 17% of patients were classified as triple negative (TN). Where data was available (n=27), median variant allelic frequency (VAF) for JAK2 V617F was 19.7% (r, 4-75%) and for CALR 43% (r, 5.7 - 53). 9/107 (8.4%) of patients had additional pathogenic gene abnormalities, most commonly variants of NRAS (22.2%), TET2 (22.2%) and ASXL1 (22.2%). We observed a high thrombosis rate of 19.6% (21/107); the majority had a diagnosis of ET (61.9%).. In 80% of such cases, this was the heralding event leading to the MPN diagnosis. Venous thrombosis was more frequent than arterial thrombosis (68% vs 33%). In particular, there was a disproportionate number of venous thromboses at unusual sites including cerebral venous sinus and splanchnic venous thromboses, representing 40% and 33% of total events, respectively. Only 3 (2.8%) patients were found to have a co-existent thrombophilia. Younger age at diagnosis appeared to associate with a higher rate of thrombosis (60% in patients aged &lt;20, 18.2% in patients aged 20-30, and 17.2% in patients aged 31-40). Thrombotic events were most common in those with a JAK2V617 mutation (23.4%), followed by those who were TN (20.0%). Only 5.9% of CALR mutated cases had a thrombotic event. In terms of cardiovascular risk factors, 13.0% (14/107) and 6.5% (7/107) of patients had diagnoses of hypertension and type 2 diabetes, respectively. Haemorrhagic events were reported in 8.4% (9/107) of patients; 3.7% (4/107) had grade 3 or 4 bleeding complications, requiring transfusion and/or surgical intervention. All who experienced haemorrhagic events were on treatment with an anti-platelet or anti-coagulant agent. 4.7% (5/107) were diagnosed with acquired von Willebrand's disease, of whom one experienced a grade 3 haemorrhagic event. No patients transformed during follow up. 33 patients reported pregnancies, with 7/33 (21%) reporting antenatal or perinatal medical complications. Over 50% (55/107) of patients were commenced on cytoreduction. Most common agents employed were pegylated interferon (45.5%; 25/55) and hydroxycarbamide (43.6%%; 24/55). No patients required combination therapy. 92/107 (86%) patients were treated with an antiplatelet agent and 9/107 (8.4%) were on anti-coagulant treatment with the majority (88.8%) receiving warfarin rather than a DOAC. AYA MPN patients have unique characteristics and heterogeneous clinico-biological features differentiating them from older MPN cohorts. Our data highlights a female preponderance, higher incidence of venous thrombosis (particularly atypical sites), and lower incidence of arterial thrombosis. Whilst traditionally younger patients are considered to have ‘low risk’ disease, this real-world study highlights a significant thrombosis rate of ~ 20% which may have associated morbidity considering the relatively longer duration of the disease course and need for cytoreductive therapy. Specific AYA MPN directed risk stratification and therapeutic algorithms are required to inform holistic and age-appropriate patient management.
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Pires De Lima, Marta, Maria Eduarda Couto, Jorge Coutinho, Maria Gomes da Silva, and Fernanda Leite. "Januskinase-2 V617F Positive Myeloproliferative Neoplasms and Haemostatic Disorders: Importance of Obesity in Prediction of Thrombotic Events." Blood 132, Supplement 1 (2018): 5466. http://dx.doi.org/10.1182/blood-2018-99-112297.
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Abstract Introduction : Myeloproliferative neoplasms (MPN), namely polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) have been associated with an increased risk of thrombotic and haemorrhagic events, mainly when januskinase-2 (JAK 2) V617F mutation is detected1. This association is higher for thrombotic events (TE) contributing for overall mortality in MPN patients, although the pathophysiological mechanisms remain unclear. Although age and history of thrombosis have been considered the most important factors for thrombosis risk assessment, cardiovascular risk factors have recently been included in prognostic scores for thrombosis in individuals with MPN. Aim : The primary end-point of this study was to evaluate the prevalence of thrombotic and haemorrhagic events in a population with MPN (PV, ET, PMF) harbouring the JAK-2 V617F mutation. A secondary end-point was to identify possible risk factors for these events. Methods: We retrospectively analysed the records of MPN patients with JAK-2 V617F mutation, identified between January 2010 and September 2013. Variables analysed included blood counts, gender, age at diagnosis, therapy, vascular events and cardiovascular risk (body mass index obesity-defined (WHO), diabetes, dyslipidemia, hypertension and smoking status). Multiple linear regression analysis was performed to identify the variables that best predict thrombosis after controlling for potential confounders. Variables significant in the univariate analysis were included in the regression. Analysis was performed using the SPSS version 22.0 (SPSS, Chicago, IL, USA). p-value lower than .05 was considered significant. Results: Excluding blood counts, MPN subgroups did not differ considering the studied variables, namely, thrombotic and bleeding events. In this MPN JAK-2 V617F mutated population (N=99; PV:38, ET:56, PMF:5), the prevalence of thrombotic and bleeding events was 35.4% (PV: 42.1%; ET: 30.4%; PMF: 40%) and 19.2% (PV: 15.8%; ET: 17.9%; PMF: 60%), respectively. With patients having more than one TE (1-5), there were 49 episodes, presented in either arterial (53.1%) or venous (46.9%) territories. The commonest sites of arterial thrombosis (N=26) were cerebral (50%), coronary (15.4%), peripheral arteries (15.4%) and ophthalmic (11.5%); venous thrombosis locations (N=23) were deep venous thrombosis (31.1%), superficial venous thrombosis (21.7%), pulmonary embolism (17.4%) and splanchnic (17.4%) (3 portal and 1 splenic). Prediction of TE was explained in 75% by a model including the number of thrombosis and obesity [R2 =.747; F (2, 36) = 53.255; p<.001; β (number of TE) = .410; β (obesity) = -.198]. The group with thrombosis showed lower number of platelets in comparison with the counterpart [F (1, 94) = 4.836; p= .030; η2 = .05]. None of other studied parameters differed significantly between groups with and without thrombosis. Bleeding episodes (N=28) occurred mostly under antiplatelet and/or anticoagulant therapy (N=22) and local/traumatic causes were identified in 42.9% of the events. In 2 haemorrhagic cases platelet blood count was >1x106/mL. The commonest affected sites were gastrointestinal tract (21.4%), nasal (21.4%), subdural (14.3%). In our study population, mortality was associated to older age (p= .002), male gender (p=.026), smoking (p=.007) and number of TE (p=.029). Mortality was found to be correlated with venous but not with arterial thrombosis (r=0.232, p=.021; r=0.105, p=.299, respectively). Conclusions and Discussion: In this MPN JAK-2 V617F mutated population we found higher prevalence of thrombotic events then bleeding episodes. The prediction of TE was explained in 75% by a model that includes the number of thrombosis episodes and obesity. Since JAK-2 V617F is a driver mutation in MPN acting through JAK/STAT inflammatory pathway and the finding of a TE prediction model that includes obesity, a chronic inflammatory condition, the present study underlies: 1) the importance of inflammation in the pathophysiology of thrombosis-MPN associated; 2) the relevance of the recent inclusion of cardiovascular risk factors as variables in prognostic scores for thrombosis in individuals with MPN. References Falchi L, Kantarjian HM, Verstovsek S (2017). Assessing the thrombotic risk of patients with essential thrombocythemia in the genomic era. Leukemia 31, 1845-1854. Disclosures Silva: Gilead Sciences: Research Funding; Abbvie, Gilead Sciences, Janssen, BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Roche, Janssen, Celgene: Other: Travel Support; Roche, Janssen: Other: Institution's payment for consultancy.
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Chaudhary, Rajvi, Neel Vora, Darsh Patel, and Kartikey G Parmar. "CASE STUDY: JAK2 V617F NEGATIVE POLYCYTHAEMIA CAUSING PORTAL VENOUS HYPERTENSION WITHOUT EVIDENT PORTAL VEIN THROMBOSIS." INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH, August 1, 2022, 13–15. http://dx.doi.org/10.36106/ijsr/8506192.
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Polycythaemia is dened as an increase in the haemoglobin above normal. This increase may be real or only apparent because of a decrease in plasma volume (spurious or relative polycythaemia). Often patients with polycythaemia are detected through an incidental nding of elevated haemoglobin or haematocrit level. Patients with polycythaemia may be asymptomatic or experience symptoms related to the increased red cell mass or the underlying disease process that leads to the increased red cell mass. The dominant symptoms from an increased red cell mass are related to hyper viscosity and thrombosis (both venous and arterial), because the blood viscosity increases logarithmically at haematocrits >55%. We are presenting a case of a patient aged 30 years presenting with fatigue, headaches, dizziness, recurrent multiple joint pain.
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van de Sande, Margot J. A., Femke Slaghekke, Arjan B. te Pas, Ruben S. G. M. Witlox, Enrico Lopriore, and Lisanne S. A. Tollenaar. "Increased risk of persistent pulmonary hypertension of the newborn in twin anaemia polycythaemia sequence donors." Fetal Diagnosis and Therapy, November 12, 2024, 1–19. http://dx.doi.org/10.1159/000542493.
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Introduction This study aimed to describe the prevalence and risk factors for respiratory complications in monochorionic twins with twin anaemia polycythaemia sequence (TAPS). Methods All neonates diagnosed with postnatal TAPS at our centre between 2002 and 2023 were included in this retrospective study. The primary outcome was the prevalence of respiratory complications, including respiratory distress syndrome (RDS), bronchopulmonary dysplasia (BPD) and persistent pulmonary hypertension of the newborn (PPHN). Secondary outcomes included need of respiratory support during admission and a risk-factor analysis for adverse respiratory outcome. Results In our study of 100 postnatally diagnosed TAPS pregnancies, 32% (62/199) experienced RDS and 13% (25/199) had BPD, with no difference between donors and recipients. PPHN occurred in 7% of cases, more frequently in donors (11%, 11/100) than in recipients (3%, 3/100) (OR = 1.3, 95%CI 0.2-2.6). Lower gestational age at birth and severe fetal anaemia were found to be significant independent risk factors associated with PPHN in TAPS twins (OR = 0.3, 95%CI 0.1-0.5), respectively (OR = 1.9, 95%CI 0.8-3.1). Conclusion TAPS donor twins have a four-fold increased risk of PPHN due to anaemia compared to recipient twins. Given the life-threatening nature of PPHN, TAPS twins should be born in hospitals equipped to treat it.
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Forsell, S., A. Najeb, H. Habel, T. Jerberg, R. Hofmann, and P. Svensson. "Abnormal hb-levels associate differently with type 1 and type 2 myocardial infarction in patients visiting the emergency department with chest pain." European Heart Journal 42, Supplement_1 (2021). http://dx.doi.org/10.1093/eurheartj/ehab724.1451.
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Abstract Background Previous studies have found an association between myocardial infarction (MI) and abnormal haemoglobin (Hb) but it is unknown whether Hb-levels are associated with type 1 and type 2 MI in unselected patients with chest pain visiting the emergency department (ED). Purpose To investigate the association between abnormal Hb-levels and type 1 and type 2 MI in patientens visiting the ED with chest pain. Methods The study population comprised all consecutive patients visiting four urban ED:s for chest pain between 2013–2016 with available data on Hb. Clinical data from the ED visit were cross-referenced to compulsory national registries retrieving information on previous diagnoses and treatments to identify history of cardiovascular disease (defined as previous MI, stroke or peripheral vascular disease), hypertension, hyperlipidaemia and diabetes mellitus. Patients were categorized depending on the exposure (Hb-level) to anaemia, normal and polycythaemia. The primary outcome (type 1 and type 2 MI) was identified in the Swedish Web-System for Enhancement and Development of Evidence-Based Care in Heart Disease Evaluated According to Recommended Therapies (SWEDEHEART). Relative risk ratio (RRR) was calculated using multinomial logistic regression, with 95% confidence interval (CI) using no infarction as reference. The robust sandwich estimator was used to estimate standard errors. Adjustments were made for risk factors according to HEART-score. Results A total of 64 606 patient with chest pain were included with a mean (SD) age of 56 (19) years and 48% were women. Anaemia was present in 10 204 (15.8%) and polycythaemia in 1598 (2.5%). Overall, type 1 MI occurred in 2 296 patients and type 2 MI in 145. The risk for type 1 MI was higher in both anaemia (RRR 1.8, 95% CI 1.6–2.0) and polycythaemia (RRR 1.6, 95% CI 1.3–2.0) compared to normal Hb. For type 2 MI the risk was markedly higher for both low and high Hb compared to normal (RRR 4.0, 95% CI 2.8–5.6) and (RRR 3.0, 95% CI 1.4–6.9). Taking age, gender and risk factors into account, patients with anaemia had a lower risk (RRR 0.8, 95% CI 0.7–0.9) for type 1 MI compared to patients with normal Hb whereas patients with polycythaemia still had a higher risk (RRR 1.6, CI 1.2–2.0). For type 2 MI, the risk remained higher for both low (RRR 1.9, 95% CI 1.3–2.8) and high Hb (RRR 2.8, 95% CI 1.3–6.2) compared to normal. Conclusion Abnormal Hb-levels in chest pain patients in the ED were significantly associated with an increased risk of type 1 or type 2 MI, however when accounting for risk factors, in a differential pattern. These novel findings indicate that Hb-level may be important when assessing patients for MI symptoms in the ED, however, further investigations are needed to establish the definite predictive value. Funding Acknowledgement Type of funding sources: None. Adjusted results type 1 and type 2 MI
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Balcar, Lorenz, Lina Degenfeld‐Schonburg, Can Hopp, et al. "Non‐Invasive Stratification of Portal Hypertension in Patients With BCR::ABL1‐Negative Myeloproliferative Neoplasms." Liver International 45, no. 6 (2025). https://doi.org/10.1111/liv.70098.
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ABSTRACTBackground & AimsThe course of BCR::ABL1‐negative myeloproliferative neoplasms (MPN) is frequently complicated by thromboembolic events in the splanchnic venous system, resulting in portal hypertension (PH). Therefore, the introduction of spleen stiffness measurement (SSM) might improve the diagnosis of PH. The aim of this study was to evaluate the clinical utility of SSM (performed by using the 100 Hz probe) in non‐invasive stratification of PH in these patients.MethodsWe performed a retrospective, monocentric, cross‐sectional analysis including consecutive patients with BCR::ABL1‐negative MPN attending the haematological outpatient clinic at the Medical University of Vienna with available liver stiffness (LSM)/SSM from 10/2023 to 09/2024. LSM/SSM were linked to signs and events of PH.ResultsFifty‐five patients were included (mean age 57.9 ± 14.2 years, 69% females, polycythaemia vera as main entity). One fourth of patients had splanchnic vein thrombi. Nineteen patients (34.5%) had specific and 29 patients (52.7%) had unspecific signs of PH. Twelve patients (21.8%) experienced PH events prior to study inclusion.SSM correlated with disease severity (i.e., JAK2 V617F allele frequency). LSM/SSM adequately stratified patients with vs. without PH. While SSM was strongly linked with splenomegaly, it yielded independent information regarding PH on top of splenomegaly. The implementation of sequential LSM (< 5 & ≥ 15 kPa) and SSM (< 21 & ≥ 50 kPa) for ruling in and out PH reduced the grey zone (24%) with adequate sensitivity/specificity.ConclusionsWhile SSM is strongly correlated with splenomegaly and disease severity, it is independently associated with PH in patients with BCR::ABL1‐negative MPN. Implementation of LSM/SSM might improve patient management.
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Dunn, Matthew, Joshua Dawe, Beng Eu, Kevin Lee, Timothy Piatkowski, and Mark Stoové. "The health effects of non‐prescribed anabolic–androgenic steroid use: Findings from The Performance and image‐enhancing drugs UseRS' Health (PUSH) audit." Drug and Alcohol Review, July 7, 2024. http://dx.doi.org/10.1111/dar.13899.
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AbstractIntroductionTo ascertain the adverse health outcomes experienced by those using prescribed testosterone and non‐prescribed anabolic–androgenic steroids presenting to general practitioner (GP) clinics.MethodsRetrospective clinical audit from nine GP clinics in major metropolitan areas across three Australian states. Data included demographic and individual characteristics (age, sexuality, body mass index, smoking status and HIV status); performance and image‐enhancing drug use (type, reasons for use, patient‐reported adverse effects); and blood biochemistry measurements (lipid profiles, liver function tests and red blood cell tests). Adverse health outcomes included evidence of polycythaemia, hypertension, liver abnormalities and hypercholesterolemia.ResultsThree hundred men were identified as either using prescribed testosterone (66%; n = 197) or non‐prescribed anabolic–androgenic steroids (AAS) (34%; n = 103). Individuals in the prescribed group were more likely to be older (p < 0.001), gay or bisexual (p < 0.001) and living with diagnosed HIV (p < 0.001) compared to individuals in the non‐prescribed group. Abnormal liver function, polycythemia and gynecomastia were the top three adverse events experienced. When adjusting for age, sexuality, HIV status and smoking status, those who used non‐prescribed AAS were more likely to experience any adverse event (aPR = 1.28; 95% CI 1.01–1.60; p = 0.038), hypertension (aPR = 1.86; 95% CI 1.19–2.91; p = 0.006) and liver abnormalities (aPR = 1.51; 95% CI 1.04–2.20; p = 0.030) compared to those using prescribed testosterone.Discussion and ConclusionFor GPs who have clients who may be using, or who they suspect of using, AAS, these findings highlight the importance of not only exploring a patient's history of the adverse effects they have experienced, but that measuring for these other conditions may provide a more accurate clinical picture.
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Gunawan, R. Y., and H. Medishita. "C42. Eisenmenger Syndrome, Could Its Pathophysiological Changes Serve as Protective Factors Against Severe COVID-19 Infection?" European Heart Journal Supplements 23, Supplement_F (2021). http://dx.doi.org/10.1093/eurheartjsupp/suab125.041.
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Abstract Background Congenital heart disease (CHD) and pulmonary artery hypertension (PAH) are comorbidities to severe COVID-19 infection. We reported a case of adult congenital heart disease (ACHD) with Eisenmenger Syndrome (ES) who survived COVID-19 without progression to severe infection. Case Description A 39 years old male with history of uncorrected ACHD complained of low-grade fever and cough for 3 days. Initial oxygen saturation was 85%, no tachypnoea, no tachycardia, and blood pressure was normal. SARS-CoV2 RT-PCR results was positive. Thorax CT showed lung fibrosis, minimal GGO, and prominent pulmonary notch. Notable laboratory result was haemoglobin: 20.4g/dL. Echocardiography showed a large secundum ASD with bidirectional shunt, dilated RA and RV, normal ventricular systolic functions, mild TR with increased tricuspid gradient (TR V max 4.27m/s) which concluded the diagnosis of ES. The patient was treated according to moderate COVID-19 guidelines and ES management. His response to treatment was favourable and was discharged in 3 weeks uneventfully. Discussion Studies have documented CHD and PAH as comorbid to severe COVID-19 infection, but our patient had moderate COVID-19 infection and recovered without progression to severe disease. We proposed several pathophysiological characteristics of ES which could serve as protective factors against severe COVID-19, which are: 1) Pre-existing PAH, where pulmonary vasculopathy and remodelling lessened endothelial response to inflammation, and reduced expression of ACE2 receptors, 2) Chronic hypoxia, which prepared cells and tissues against hypoxic environment, and 3) Polycythaemia, might be beneficial in haemoglobinopathy directly caused by SARS-CoV2.
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Gunawan, R. Y., and H. Medishita. "C42. Eisenmenger Syndrome, Could Its Pathophysiological Changes Serve as Protective Factors Against Severe COVID-19 Infection?" European Heart Journal Supplements 23, Supplement_F (2021). http://dx.doi.org/10.1093/eurheartjsupp/suab124.041.
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Abstract Background Congenital heart disease (CHD) and pulmonary artery hypertension (PAH) are comorbidities to severe COVID-19 infection. We reported a case of adult congenital heart disease (ACHD) with Eisenmenger Syndrome (ES) who survived COVID-19 without progression to severe infection. Case Description A 39 years old male with history of uncorrected ACHD complained of low-grade fever and cough for 3 days. Initial oxygen saturation was 85%, no tachypnoea, no tachycardia, and blood pressure was normal. SARS-CoV2 RT-PCR results was positive. Thorax CT showed lung fibrosis, minimal GGO, and prominent pulmonary notch. Notable laboratory result was haemoglobin: 20.4g/dL. Echocardiography showed a large secundum ASD with bidirectional shunt, dilated RA and RV, normal ventricular systolic functions, mild TR with increased tricuspid gradient (TR V max 4.27m/s) which concluded the diagnosis of ES. The patient was treated according to moderate COVID-19 guidelines and ES management. His response to treatment was favourable and was discharged in 3 weeks uneventfully. Discussion Studies have documented CHD and PAH as comorbid to severe COVID-19 infection, but our patient had moderate COVID-19 infection and recovered without progression to severe disease. We proposed several pathophysiological characteristics of ES which could serve as protective factors against severe COVID-19, which are: 1) Pre-existing PAH, where pulmonary vasculopathy and remodelling lessened endothelial response to inflammation, and reduced expression of ACE2 receptors, 2) Chronic hypoxia, which prepared cells and tissues against hypoxic environment, and 3) Polycythaemia, might be beneficial in haemoglobinopathy directly caused by SARS-CoV2.
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Wouter, Schutyser, Budts Werner, and Verhamme Peter. "Percutaneous embolization of pulmonary arteriovenous malformations in adult patient with Rendu-Osler-Weber: a case report." European Heart Journal - Case Reports, November 3, 2023. http://dx.doi.org/10.1093/ehjcr/ytad533.
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Abstract Background Hereditary haemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is a rare genetic disorder characterized by the development of telangiectasias and arteriovenous malformations throughout the body. We present a case of percutaneous embolization of pulmonary arteriovenous malformations in an adult patient. Case summary A 26-year-old male patient with polycythaemia of unknown origin and a family history of secundum atrial septal defect underwent cardiac evaluation which revealed clubbing as a sign of peripheral cyanosis. Transthoracic echocardiography showed no intracardiac shunting, but further imaging revealed pulmonary arteriovenous malformations in the lower lobe of the left lung. Magnetic resonance imaging of the brain detected vascular-ischemic lesions, likely due to embolization through the pulmonary malformations. Right heart catheterization and pulmonary angiography confirmed the presence of large arteriovenous malformations in the left lower pulmonary lobe. Percutaneous closure using Amplatzer devices was performed, followed by temporary anticoagulation therapy. Oxygen saturation improved and follow-up imaging confirmed successful closure of the arteriovenous malformations. Genetic testing using whole exome sequencing identified a mutation in the ENG gene, confirming the diagnosis of hereditary haemorrhagic telangiectasia. Discussion Our case highlights the importance of investigating both intra- and extracardiac shunting in patients with clinical features of right-to-left shunting. Arteriovenous malformations can serve as a pathway for paradoxical emboli, potentially leading to ischemic brain events, and might cause pulmonary arterial hypertension. Screening for arteriovenous malformations in various organs and embolization of significant shunts are essential aspects of managing hereditary haemorrhagic telangiectasia. Genetic testing aids in confirming the diagnosis and guides family testing.
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Fialho, I., M. Passos, J. Lima Lopes, et al. "Clinical and echocardiographic features of platypnea-orthodeoxia syndrome: a single-centre experience." European Heart Journal - Cardiovascular Imaging 23, Supplement_1 (2022). http://dx.doi.org/10.1093/ehjci/jeab289.317.
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Abstract Funding Acknowledgements Type of funding sources: None. Background Platypnea-orthodeoxia syndrome (POS) is an uncommon condition characterized by dyspnoea and hypoxemia in the upright position that improves with recumbency.1 Possible causes are intracardiac shunt, pulmonary arteriovenous shunt, and V/Q mismatch.1,2 Echocardiography is the cornerstone of POS diagnosis, with special focus on atrial septal defect (ASD) morphology and right-to-left shunt confirmation.3 Purpose To evaluate the clinical and echocardiographic features of patients presenting with POS due to a cardiac cause. Methods We performed a retrospective analysis of patients diagnosed with POS in our centre between 2015 January and 2021 August. Routine blood tests and transthoracic echocardiogram (TTE) were performed in all patients. Demographics, clinical presentation, blood test results, TTE information, and PFO closure procedure details were recorded. Results Seven patients were included, 85.7% female (n = 6). The median (IQR) age was 78 (72-85) years. The most prevalent cardiovascular risk factors were hypertension (100%; n = 7) and overweight/obesity (85.7%; n = 6). Two patients (28.6%) had chronic pulmonary disease. The most common symptoms were fatigue and exercise intolerance (n= 5; 71.4%) and the most frequent sign was persistent hypoxemia (n = 7; 100%), although 28.6% (n = 2) patients did not present the typical positional changes in peripheral oxygen saturation. Haemoglobin levels [14.1 (13.3-15.2)] were within the normal range and serum NTproBNP levels [656 (287-1196)] were slightly elevated. Left ventricle function was preserved in all patients; right ventricle morphology and function were normal in 85.7% (n = 6) patients, low probability of pulmonary hypertension in TTE was found in 85.7% (n = 6), and exuberant Eustachian valve was observed in 14.3% (n = 1). All patients presented atrial septal hypermobility, 87.5% (n = 6) meeting atrial septal aneurysm criteria. Patent foramen ovale was found in 85.7% of patients (n = 6) and ostium secundum ASD in 14.3% (n = 1). POS precipitating factors were aortic root dilation (28.6%; n = 2), chest trauma (14.3%; n = 1), right hip arthroplasty (14.3%; n = 1), atrial septal stretching regarding right volume overload (14.3%; n = 1). The underlying mechanism was unknown in 28.6% (n = 2) of patients. ASD closure was performed in 57.1% (n = 4) of patients: 75% (n = 3) showed residual shunt, but clinical improvement was reported by all. No acute complications were described, except for paroxysmal atrial fibrillation (14.3%; n = 1). Conclusion POS diagnosis depends on high clinical suspicion: the most common manifestations are fatigue and persistent hypoxemia. Typical positional changes in oxygen saturation are not present in all patients. Polycythaemia, right chambers dilation, and pulmonary hypertension are not common. Echocardiography is fundamental for diagnosis, allowing right-to-left shunt confirmation and ASD morphology evaluation to outline a successful closure procedure.
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Lambden, Simon, Andrew S. Cowburn, David Macias, et al. "Endothelial cell regulation of systemic haemodynamics and metabolism acts through the HIF transcription factors." Intensive Care Medicine Experimental 9, no. 1 (2021). http://dx.doi.org/10.1186/s40635-021-00390-y.
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Abstract Background The vascular endothelium has important endocrine and paracrine roles, particularly in the regulation of vascular tone and immune function, and it has been implicated in the pathophysiology of a range of cardiovascular and inflammatory conditions. This study uses a series of transgenic murine models to explore for the first time the role of the hypoxia-inducible factors, HIF-1α and HIF-2α in the pulmonary and systemic circulations as potential regulators of systemic vascular function in normoxic or hypoxic conditions and in response to inflammatory stress. We developed a series of transgenic mouse models, the HIF-1α Tie2Cre, deficient in HIF1-α in the systemic and pulmonary vascular endothelium and the L1Cre, a pulmonary endothelium specific knockout of HIF-1α or HIF-2α. In vivo, arterial blood pressure and metabolic activity were monitored continuously in normal atmospheric conditions and following an acute stimulus with hypoxia (10%) or lipopolysaccharide (LPS). Ex vivo, femoral artery reactivity was assessed using wire myography. Results Under normoxia, the HIF-1α Tie2Cre mouse had increased systolic and diastolic arterial pressure compared to litter mate controls over the day–night cycle under normal environmental conditions. VO2 and VCO2 were also increased. Femoral arteries displayed impaired endothelial relaxation in response to acetylcholine mediated by a reduction in the nitric oxide dependent portion of the response. HIF-1α L1Cre mice displayed a similar pattern of increased systemic blood pressure, metabolic rate and impaired vascular relaxation without features of pulmonary hypertension, polycythaemia or renal dysfunction under normal conditions. In response to acute hypoxia, deficiency of HIF-1α was associated with faster resolution of hypoxia-induced haemodynamic and metabolic compromise. In addition, systemic haemodynamics were less compromised by LPS treatment. Conclusions These data show that deficiency of HIF-1α in the systemic or pulmonary endothelium is associated with increased systemic blood pressure and metabolic rate, a pattern that persists in both normoxic conditions and in response to acute stress with potential implications for our understanding of the pathophysiology of vascular dysfunction in acute and chronic disease.
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Ratish Chandra, G., Shyam Sundar. S, and Praveen Kumar Sindhur. "A STUDY OF POLYCYTHEMIA IN 34-41 WEEK SMALL FOR GESTATIONAL AGE(SGA) /INTRAUTERINE GROWTH RESTRICTED (IUGR) NEONATES." GLOBAL JOURNAL FOR RESEARCH ANALYSIS, March 15, 2023, 128–31. http://dx.doi.org/10.36106/gjra/7110023.
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Background: Polycythemia in neonates is dened by hematocrit value more than or equal to 65% on venous sample. Polycythemia is associated with hyperviscosity syndrome. In clinical setting,as it is not feasible to measure viscosity of blood, hematocrit value is used as a surrogatefor hyperviscosity. There is a need to evaluate the incidence of polycythemia in SGA babies as SGA is the commonest risk factor for PC followed by pregnancy induced hypertension (PIH) and infant of diabetic mother (IDM). Objectives: To estimate the incidence of polycythemia in 34 to 41week SGA/IUGR neonates and its relation with severity of polycythemia and birth weight-for gestational age-s.d score and its associated morbidity with polycythemia Methodology: Allasymptomatic enrolled neonates will be screened for polycythemia at 2,12, 24, 48 hours of life. Blood sample for hematocrit is obtained by venipuncture (venous hematocrit). These babies will be screened for hypoglycemia, hypocalcemia and where ever applicable for sepsis. The highest hematocrit value of the polycythemic neonates during the course of stay will be considered to study the relationship between severity of polycythemia and birth weight -for gestational-age-s.d score. Results: The Incidence of polycythemia in our study was 16.3%, Distribution based on morbidities associated with polycythemia, the odds of having polycythemia is 4.3 times in those with transient tachypnea of newborn, and it was 8.04 times in respiratory distress syndrome is compared to those without respiratory distress syndrome. Where it was 6.38timesin sepsis compared to those without sepsis, and in meconium aspiration it was 15.02 times compared to those without meconium aspiration. This observation were statistically signicant.Distribution based on morbidity prole of symmetrical and asymmetrical small for gestational age babies, in this study hypothermia was observed in 38 study participants out of which 10 were symmetrical 28 were asymmetrical small for gestational age babies. This observation was not statistically signicant. Conclusions: The prevalence of polycythaemia among SGA babies in the present study was 16.3%.The main problems need to be anticipated in the newborn are perinatal asphyxia, hypothermia, apnea, jaundice, sepsis and hypoglycemia. Improvement of perinatal care is required in order to prevent the birth of SGA babies and also to manage the problems associated with them.
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Ogunsalu, Christopher. "The Physiological Basis Of The Oral Surgical Management Of A Patient With Polycythemia Rubra Vera: A Pathway For The Development Of A Protocol." Journal of Dentistry and Oral Sciences, February 1, 2022. http://dx.doi.org/10.37191/mapsci-2582-3736-4(1)-120.
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Various oral surgical procedures are currently performed under local anaesthesia and conscious sedation as a day surgery procedure. Both oral and intravenous conscious sedation are available as a choice. Oral sedation with Alprazolam and Codeine is very effective for most oral surgical procedures especially the removal of impacted third molars and surgical placement of implants. It is this sedation regimen that comprises the synergistic use of both aprazolam and codeine to effect sedation with amnesia that is the standard procedure at the Faculty of Dentistry of the International Postgraduate Medical College. The purpose of this case report is to appraise the possible negative effect of Alprazolam and Codeine sedation mentioned above on a patient who presented with polycythaemia rubra vera (a very rare haematological condition). This is because the codeine component of this combination will depress the respiratory centre in the medulla oblongata putting the patient at risk. The case study is that of a 70-Year-old male patient who had been a regular patient for approximately 10 years ago at the Faculty of Dentistry, International Postgraduate Medical College (IPMC), where a total upper denture and a partial lower denture were placed. The patient returned in 2019 with the chief complaint of halitosis and pain in the mandibular posterior right region. The patient gave past medical history of PRV, epilepsy and hypertension and history of being on multiple medications for these conditions. The treatment plan advised was total extraction of the remaining mandibular dentition and excisional biopsy of the suspicious lesion mesial to the mandibular right first molar, together with a complete lower denture replacement. The procedure was done without sedation and at no time was the patient considered to be in danger as it relates to oxygen saturation, carbon dioxide drive to the brain and bleeding. Every hematologist managing patients with PRV will be very concerned as to how these patients are managed by dentists, particularly as it relates to oxygen saturation, carbon dioxide drive to the brain and hemostasis. Similarly, most dental practitioners that are managing such patients may not be familiar with the technicalities surrounding the clinical and operative management of these patients. It is for this reason that this case report focusing on oral surgical management of a patient with PRV is of significance to the dental literature, particularly as it aims to develop a working group for the development of the protocol for the management of PRV.