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1
Turovskaya, N. G. "Mental Development of Children with Non-epileptic Paroxysmal States in Medical History." Psychological-Educational Studies 7, no. 3 (2015): 82–95. http://dx.doi.org/10.17759/psyedu.2015070309.
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The author studied mental functions disorders in children with a history of paroxysmal states of various etiologies and compared mental development disorder patterns in patients with epileptic and non-epileptic paroxysms. Study sample were 107 children, aged 6 to 10 years. The study used experimental psychological and neuropsychological techniques. According to the empirical study results, non-epileptic paroxysms unlike epileptic much less combined with a number of mental functions disorders and intelligence in general. However, non-epileptic paroxysmal states as well as epileptic seizure associated with increasing activity exhaustion and abnormal function of the motor analyzer (dynamic and kinesthetic dyspraxia). Visual memory disorders and modal-nonspecific memory disorders have more pronounced importance in the mental ontogenesis structure in children with convulsive paroxysms compared to children with cerebral pathology without paroxysms history.
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Voitiuk, Anna A., Tetyana A. Litovchenko, Olena N. Borodai, and Nataliia A. Rudkivska. "DIFFERENTIAL DIAGNOSIS OF PAROXYSMAL STATES: LITERATURE REVIEW AND ANALYSIS OF A CLINICAL CASE ON THE EXAMPLE OF CLOCCS-SYNDROME IN A YOUNG MAN." Wiadomości Lekarskie 75, no. 4 (2022): 907–13. http://dx.doi.org/10.36740/wlek202204127.
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Diagnosis of paroxysmal conditions in neurology is one of the most difficult problems. Particular difficulties are caused by differential diagnosis of epileptic and non-epileptic paroxysmal states. There are no absolutely pathognomonic signs of epileptic and non-epileptic seizures. False positive diagnosis of epilepsy occurs in 2-71% of cases. Diagnosis of paroxysmal conditions requires an integrated approach to the problem and includes not only a clinical examination, but also a thorough history taking, neurophysiological, neuroimaging, laboratory research methods, involves the involvement of other specialists. The article presents a clinical case of 27-year-old young man who was initially misdiagnosed. Using the methods of functional and laboratory diagnostics, the patient was diagnosed correctly. Instead of idiopathic epilepsy, he was diagnosed with cytotoxic lesions of the corpus callosum (CLOCCs-syndrome associated with an infectious process) with motor paroxysms of non-epileptic genesis. Thus, using the example of this clinical case, it has been shown that the differential diagnosis of epileptic and non-epileptic paroxysmal states presents significant difficulties for a practicing neurologist.
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Жиенбаева, Б. С., and Т. Б. Мажирова. "PAROXYSMAL STATES IN ADULTS (LITERATURE REVIEW)." Vestnik, no. 3 (December 15, 2021): 269–72. http://dx.doi.org/10.53065/kaznmu.2021.91.15.051.
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В данной статье автор рассматривает вопросы пароксизмального расстройства сознания у взрослых, которые выражаются в эпилептических припадках, потери сознания, обморок, панические атаки, расстройства сна. Оценка временных нарушений сознания имеет решающее значение для диагностики эпилептических припадков, обмороков, парасомний, органических энцефалопатий и психогенных непилептических припадков A temporary change in consciousness is the main clinical problem of neurology. Assessment of transient disorders of consciousness is crucial for the diagnosis of epileptic seizures, syncope, parasomnias, organic encephalopathies, and psychogenic non-pictorial seizures. Attacks and other disorders of consciousness converge on a common set of cortical and subcortical structures. These structures constitute the "system of consciousness."Paroxysmal disorders are one of the most important problems of modern clinical medicine, which is characterized by a steady increase in the frequency of these pathological conditions in people of young and middle age and the diagnostic complexity of many conditions. The analysis of modern publications, presented the results of their own observations on the studied problem.
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Fomina, M. Y., T. V. Melashenko, A. B. Palchik, O. I. Pavlova та D. A. Malekov. "Hyperekplexiа. Clinical observation". Pediatrician (St. Petersburg) 12, № 4 (2021): 83–88. http://dx.doi.org/10.17816/ped12483-88.
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The differential diagnosis of paroxysmal conditions, as well as disorders of muscle tone (hypertension) in the neonatal period and in young children is quite complicated. Various states of the nervous system in newborns are transient and permanent, optimal and suboptimal, normal and pathological. Among them, we can mention non-epileptic paroxysmal states of early childhood. In some cases, non-epileptic paroxysmal states of early childhood is accompanied by motor disorders, manifested by an excessive increase in limb tone in newborns. This pathological condition of muscle tone in the English-language literature is referred to by the term stiffness baby (the syndrome of a rigid or fettered baby). Neonatal pathological muscle hypertonicity, unlike physiological hypertonicity of muscles of a newborn, is a rather rare condition. The article presents literature data and a description of the clinical observation of a patient with hyperekplexia. Hyperekplexia is a rare paroxysmal movement disorder in young children. The main clinical variants of the disease, methods of diagnosis and correction, the main mutations associated with this condition are considered. The article describes the own clinical observation of an early-age patient with hyperekplexia, its clinical picture, features of paroxysmal states and therapy, neuroimaging data, electroencephalographic phenomena recorded in the patient and genetic testing that confirmed the diagnosis of non-epileptic paroxysmal disorders. The child has a mutation in the ATAD1 gene associated with type 4 Hyperekplexia (618011).
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Turovskaya, N. G. "Pathology of memory in the structure of mental development disorders in children with convulsive paroxysms." Experimental Psychology (Russia) 8, no. 3 (2015): 145–55. http://dx.doi.org/10.17759/exppsy.2015080313.
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Memory disorders are a common pathology in children with convulsive paroxysms. The present study tested the hypothesis that the pathology of memory in children with paroxysmal states have quantitative and qualitative specificity. The study involved 107 children aged 6-10 years. 59 people had a history of paroxysmal state, 12 people with epileptiform activity on EEG without seizures in history. A comparison group comprised 36 people with residual cerebral pathology without a history of seizures. The study used experimental psychological and neuropsychological research methods memory. The results of empirical studies have shown that increasing importance in the picture of violations mnestic activity in children with convulsive paroxysms addition to short-term verbal memory disorders have impaired short-term visual memory, the phenomenon of amnestic aphasia and modal-nonspecific memory disorders. The degree of short-term verbal memory disorders correlates with the age of onset of seizures, visual memory - with the number of attacks in history. Consideration of the results will allow to organize the process of providing psychological assistance to sick children more effectively.
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Schyolkova, O. Yu, and D. A. Eremina. "Psychosocial and clinical factors of cognitive functioning of patients with coronary heart disease after coronary stent." Experimental Psychology (Russia) 8, no. 3 (2015): 156–72. http://dx.doi.org/10.17759/exppsy.2015080314.
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Memory disorders are a common pathology in children with convulsive paroxysms. The present study tested the hypothesis that the pathology of memory in children with paroxysmal states have quantitative and qualitative specificity. The study involved 107 children aged 6–10 years. 59 people had a history of paroxysmal state, 12 people with epileptiform activity on EEG without seizures in history. A comparison group comprised 36 people with residual cerebral pathology without a history of seizures. The study used experimental psychological and neuropsychological research methods memory. The results of empirical studies have shown that increasing importance in the picture of violations mnestic activity in children with convulsive paroxysms addition to short-term verbal memory disorders have impaired short-term visual memory, the phenomenon of amnestic aphasia and modal-nonspecific memory disorders. The degree of short-term verbal memory disorders correlates with the age of onset of seizures, visual memory - with the number of attacks in history. Consideration of the results will allow to organize the process of providing psychological assistance to sick children more effectively.
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Kollar, B., Z. Carnicka, P. Siarnik, et al. "The importance of interictal electroencephalography in paroxysmal states." Bratislava Medical Journal 115, no. 03 (2014): 168–70. http://dx.doi.org/10.4149/bll_2014_168.
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Gromov, S. A., and V. Е. Mashukova. "Differential diagnosis of epilepsy and paroxysmal states of nonepileptic genesis in the early stages of the disease." Neurology Bulletin XXIX, no. 1-2 (1997): 27–30. http://dx.doi.org/10.17816/nb79872.
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There discussed and systematized different diagnostic mistakes in patients, suffering epilepsy and nonepileptic paroxysmal conditions at early stage of disease. There given the analysis of clinical and paraclinical examination data of 525 patients. Decisive factors in diagnosis are revealing basic clinical symptocomplex and its analytical understanding in comparsion with the findings of investigation methods, conducted by special devices.
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Tatiana, Roshchupkina. "Cognitive violations of persons with alcoholic encephalopathy and paroxismal states." ScienceRise: Medical Science, no. 6(33) (November 29, 2019): 43–46. https://doi.org/10.15587/2519-4798.2019.185782.
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The article addresses the issues of cognitive impairment among persons with alcohol dependence, aggravated by alcoholic encephalopathy and paroxysmal conditions. The <strong>aim</strong> of the study was to identify the levels of impaired short-term memory, to identify cognitive impairment of patients with alcohol addiction with alcoholic encephalopathy and paroxysmal conditions. <strong>Materials and methods:</strong> 132 people from the contingent of alcohol addicts (AА) and from the contingent of healthy and 4 comparison groups have been identified and examined over the two years on the basis of KNP CHOR «Regional Clinical Narcological Hospital No. 3». The following techniques were used to assess psychosocial and cognitive impairment: “Jacobson Short-Term Memory Measurement Technique”; "The methodology for determining the index of short-term memory proposed by L. S. Muchnik and V. M. Smirnov (1968)". <strong>Result.</strong> According to the results of the researches, the cognitive and mnemonic sphere of persons with AА, AE and PS were expressed in the form of significant reduction of short-term memory and cognitive impairment. The presence of significantly "deeper" and "gross" degenerative-organic lesions of the central nervous system in chronic alcoholic lesions with the development of alcoholic encephalopathy and paroxysmal states of alcoholic genesis has been proved. <strong>Conclusions. </strong>Thus, the obtained research results only confirm the data of numerous world sources on the development of cognitive decline in individuals with alcohol dependence
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Yevtushenko, S. К., O. Yu Sukhonosova, and A. A. Omelianenko. "The use of topiramate in paroxysmal and non-paroxysmal states in children (scientific review and personal observation)." INTERNATIONAL NEUROLOGICAL JOURNAL, no. 3.97 (July 9, 2018): 59–68. http://dx.doi.org/10.22141/2224-0713.3.97.2018.133683.
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Ganeev, K. G. "Integral health improvement system for adolescent patients with paroxysmal conditions." Neurology Bulletin XXXIII, no. 1-2 (2001): 66–69. http://dx.doi.org/10.17816/nb79748.
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The development of pathological mechanisms of paroxysmal states of epileptic and non-epileptic nature of the pubertal period was studied. A systematic approach to identifying the interest of many body systems that provide homeostasis (mental, autonomic, immune) revealed their inconsistency. Taking into account the dominant role of the central nervous system in the implementation of adaptive, adaptive functions of the body, the formation of a paroxysmal state (PS) is considered as one of the manifestations of central nervous system dysadaptation in the period of hormonal imbalance.
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Sizov, S. V., I. V. Oleichik, and P. A. Baranov. "Endogenous Manic-Paraphrenic States." Psikhiatriya 19, no. 1 (2021): 90–101. http://dx.doi.org/10.30629/2618-6667-2021-19-1-90-101.
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Purpose of the work: analysis of changes in the views of domestic and foreign researchers on the clinical and biological features of manic-paraphrenic states developing within the framework of endogenous paroxysmal states psychoses.Material and method: by the keywords “mania, paraphrenia, manic, paraphrenic”, “manic-paraphrenic state”, publications available to authors were selected in the databases MEDLINE/PubMed, Scopus, Webofscience, eLibrary.Conclusion: the following areas of research have been identified: 1) the prevalence of manic-paraphrenic states at the present time; 2) evolution of researchers’ opinions regarding these psychoses throughout the entire period of the study of the issue; 3) a modern point of view on the nosology of manic-paraphrenic states and their place in current classifications of mental disorders; 4) topical issues of the pathogenesis of such disorders.
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Korsunska, L. L., and A. V. Meshcheryakova. "Clinical characteristics of benign paroxysmal positional vertigo (DPPG) in perimenopausal women." NATIONAL JOURNAL OF NEUROLOGY, no. 8 (December 1, 2018): 61–65. http://dx.doi.org/10.28942/nnj.v2i8.89.
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The article presents the structural features of benign positional paroxysmal vertigo (BPPV) to gender differences in the comprehensive assessment of the epidemiology of vestibular dysfunction in women of perimenopausal period, allowing for flow of advanced reproductive disorders and medical background correction dishormonal states.
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Korsunska, L. L., and A. V. Meshcheryakova. "CLINICAL CHARACTERISTICS OF BENIGN PAROXYSMAL POSITIONAL VERTIGO (DPPG) IN PERIMENOPAUSAL WOMEN." National Journal of Neurology 2, no. 08 (2015): 85–89. http://dx.doi.org/10.61788/njn.v2i15.13.
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The article presents the structural features of benign positional paroxysmal vertigo (BPPV) to gender differences in the comprehensive assessment of the epidemiology of vestibular dysfunction in women of perimenopausal period, allowing for flow of advanced reproductive disorders and medical background correction dishormonal states.
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Privorotskaya, V. V., A. B. Palchik, A. E. Ponyatishin, and G. A. Mashevskiy. "Non-Epileptic Paroxysmal Events in Infants: stucture, manifestation and risk factors." V.M. BEKHTEREV REVIEW OF PSYCHIATRY AND MEDICAL PSYCHOLOGY, no. 2 (July 9, 2020): 80–86. http://dx.doi.org/10.31363/2313-7053-2020-2-80-86.
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During 3 years we observed in specialized neurological department of St. Olga Children’s City Hospital 708 babies aged up to 42 months with different paroxysmal disorders. Non-epileptic paroxysmal events (NEPE) were diagnosed in 98 cases. These disorders were qualified (according to ILAE criteria) as differentiated and undifferentiated NEPE. In most cases neurophysiological assessment data, neurovisualization data, and neurological status of infants with NEPE and babies from comparison group varied marginally. It was shown that NEPE development correlated with perinatal factors, and with infants’ developmental diseases (functional gastrointestinal disorders, iron deficiency states, chronic or persistent infections). The obtained data was interpreted according to the developmental neurology principles (optimality concept, in particular).
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Marulina, V. I., and F. K. Safiullina. "Paroxysmal conditions in the practice of a pediatric neuropathologist." Neurology Bulletin XXX, no. 3-4 (2021): 56–57. http://dx.doi.org/10.17816/nb81064.
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Paroxysmal states are interpreted ambiguously by different authors. A different assessment of their character creates additional difficulties for a practitioner, primarily a pediatric neuropathologist. One of the most difficult tasks that a specialist has to solve in everyday work is the differentiation of epilepsy and conditions of a non-epileptic nature that are similar in some features.
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Djuric, Ivica, Boris Dzudovic, Bojana Subotic, et al. "New-Onset Paroxysmal Atrial Fibrillation in the Setting of Acute Pulmonary Embolism Is Associated with All-Cause Hospital Mortality in Women but Not in Men." Diagnostics 13, no. 11 (2023): 1829. http://dx.doi.org/10.3390/diagnostics13111829.
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Background: Patients with acute pulmonary embolism (PE) may have various types of atrial fibrillation (AF). The role of AF in hemodynamic states and outcomes may differ between men and women. Methods: In total, 1600 patients (743 males and 857 females) with acute PE were enrolled in this study. The severity of PE was assessed using the European Society of Cardiology (ESC) mortality risk model. Patients were allocated into three groups according to their electrocardiography recordings taken during hospitalization: sinus rhythm, new-onset paroxysmal AF, and persistent/permanent AF. The association between the types of AF and all-cause hospital mortality was tested using regression models and net reclassification index (NRI) and integrated discrimination index (IDI) statistics with respect to sex. Results: There were no differences between the frequencies of the types of AF between men and women: 8.1% vs. 9.1% and 7.5% vs. 7.5% (p = 0.766) for paroxysmal and persistent/permanent AF, respectively. We found that the rates of paroxysmal AF significantly increased across the mortality risk strata in both sexes. Among the types of AF, the presence of paroxysmal AF had a predictive value for all-cause hospital mortality independent of mortality risk and age in women only (adjusted HR, 2.072; 95% CI, 1.274–3.371; p = 0.003). Adding paroxysmal AF to the ESC risk model did not improve the reclassification of patient risk for the prediction of all-cause mortality, but instead enhanced the discriminative power of the existing model in women only (NRI, not significant; IDI, 0.022 (95% CI, 0.004–0.063); p = 0.013). Conclusion: The occurrence of paroxysmal AF in female patients with acute PE has predictive value for all-cause hospital mortality independent of age and mortality risk.
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Mendelevich, Vladimir D., Gazinur A. Sitdikov, and Radzhab R. Abakarov. "Clinical mixed dissociative and post-traumatic stress disorders in a 10-year-old boy after a traffic accident." Neurology Bulletin LVI, no. 1 (2024): 49–58. http://dx.doi.org/10.17816/nb625486.
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The article presents the clinical case of 10-year-old Karim, whose behavior and mental status changed after a traffic accident in which the boy was hit by a car (without loss of consciousness and without traumatic brain injury). In addition to various motor paroxysms (convulsions) that did not meet the criteria for epileptic symptoms, the clinical picture included wave-like psychopathological symptoms and inappropriate behavior. The clinical picture could not be clearly attributed to the manifestation of dissociative disorders or post-traumatic stress disorder. Within two months after the accident, the clinical picture worsened — the "light intervals" during which Karim was completely adequate and critical became less frequent. Neurologists rejected the organic (epileptic) basis of the paroxysmal states. Examination and treatment by psychiatrists did not lead to stabilization of the mental state. In the opinion of the authors, the clinical case belongs to a rare polysyndromic variant of the dissociative subtype of PTSD, not described in the scientific literature, in which a mixture of various psychopathological symptoms was found - dissociative motor paroxysms, Ganser's syndrome, astasia-abasia, onirism, and flashbacks.
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Eninya, G. I., and G. V. Zagoryanskaya. "Paroxysmal syndromes in patients with discirculation in vertebral-basilar system." Neurology Bulletin XXV, no. 1-2 (1993): 26–30. http://dx.doi.org/10.17816/nb105919.
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As many as 450 patients with vertebral-basilar discirculations accompanied by attacks: syncopal, diencephalic, amnestic, sudden fall are examined using dopplerography correlated with other methods. The dependence of these states on arterial hypotension, extravasal stenoses, anomalies of vessels and vertebrae, extravasal commissures is established.
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Rehorn, Michael, Naomi Sacks, Philip Cyr, Maia Emden, and Sean Pokorney. "RATES OF CATHETER ABLATION FOR PAROXYSMAL SUPRAVENTRICULAR TACHYCARDIA IN THE UNITED STATES." Journal of the American College of Cardiology 77, no. 18 (2021): 330. http://dx.doi.org/10.1016/s0735-1097(21)01689-2.
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Rehorn, Michael, Naomi C. Sacks, Maia R. Emden, et al. "Prevalence and incidence of patients with paroxysmal supraventricular tachycardia in the United States." Journal of Cardiovascular Electrophysiology 32, no. 8 (2021): 2199–206. http://dx.doi.org/10.1111/jce.15109.
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Kuzenkova, L. M., V. M. Shaytor, O. V. Globa, and R. F. Tepayev. "EPILEPTIC AND NON-EPILEPTIC PAROXYSMAL STATES IN CHILDREN. PRINCIPLES OF DIAGNOSIS AND THERAPY." Pediatric pharmacology 13, no. 1 (2016): 38–43. http://dx.doi.org/10.15690/pf.v13i1.1513.
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Smith, James A., Mary Lynn Baeck, Long Yang, Julia Signell, Efrat Morin, and David C. Goodrich. "The Paroxysmal Precipitation of the Desert: Flash Floods in the Southwestern United States." Water Resources Research 55, no. 12 (2019): 10218–47. http://dx.doi.org/10.1029/2019wr025480.
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Wong, Raymond S. M. "Safety and efficacy of pegcetacoplan in paroxysmal nocturnal hemoglobinuria." Therapeutic Advances in Hematology 13 (January 2022): 204062072211146. http://dx.doi.org/10.1177/20406207221114673.
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, hematologic disease characterized by complement-mediated hemolysis, thrombosis, and various degrees of bone marrow dysfunction. Until recently, C5 inhibition with eculizumab or ravulizumab represented the only therapies approved for patients with PNH by the United States Food and Drug Administration (US FDA). Although C5-inhibitors reduce PNH-related signs and symptoms, many patients continue to exhibit persistent anemia and require frequent blood transfusions. In May 2021, pegcetacoplan became the third US FDA-approved treatment for adults with PNH, and the first to target C3, a complement component upstream of C5. The novel strategy of inhibiting proximal complement activity with pegcetacoplan controls C5-mediated intravascular hemolysis and prevents C3-mediated extravascular hemolysis. Here, we review the results from multiple pegcetacoplan clinical studies on the efficacy and safety of pegcetacoplan treatment in adults with PNH. This review summarizes findings from three studies in complement-inhibitor-naïve patients with PNH (PADDOCK [phase Ib], PALOMINO [phase IIa], PRINCE [phase III; pegcetacoplan versus standard treatment excluding complement-inhibitors]), and one phase III study (PEGASUS) that compared eculizumab to pegcetacoplan in patients who remained anemic (hemoglobin levels < 10.5 g/dL) despite stable eculizumab treatment (⩾3 months). These studies found that pegcetacoplan contributed to superior improvements in primary and secondary endpoints related to hemoglobin levels and other hematologic parameters and provided effective management of anemia and anemia-related complications (i.e. transfusion burden, reticulocyte production, and fatigue). Furthermore, we summarize results from the 32-week open-label period from the PEGASUS trial, which confirmed the long-term safety and durable efficacy of pegcetacoplan as demonstrated by sustained improvements in clinical and hematologic outcomes in pegcetacoplan-treated patients. Pegcetacoplan is approved for the treatment of adults with PNH in the United States (Empaveli™) and for adult patients who remain anemic after at least 3 months of stable C5-inhibitor therapy in the European Union (Aspaveli®) and Australia (Empaveli; also approved for patients intolerant to C5-inhibitors).
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Abrashev, Hristo, Julian Ananiev, and Ekaterina Georgieva. "Paroxysmal Finger Hematoma—A Probable Vascular Disorder in Post-COVID-19 Condition: Two Clinical Case Presentations." Medicina 58, no. 7 (2022): 915. http://dx.doi.org/10.3390/medicina58070915.
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Background and Objectives: Achenbach’s syndrome is usually a benign, self-limiting clinical condition presented with finger discoloration, pain, and edema. Etiology, pathogenesis, and incidence remain unknown due to the variety of clinical features and the diversity of disease states leading to digital ischemia. COVID-19 primarily affects microcirculation, causing endothelial damage and disseminated microthrombosis. Materials and Methods: We reviewed two cases of Caucasian women with Achenbach’s syndrome after COVID-19 infection recovery between April and May 2021. Results: Here are presented two extremely rare cases of paroxysmal finger hematoma in two female patients after COVID-19 infection recovery. Conclusions: The exact etiology and pathophysiology of Achenbach’s syndrome remain unclear. It is assumed that SARS-CoV-2 infection could be the triggering factor in the pathophysiological mechanism of paroxysmal finger hematoma. We highly recommend the implication of the synthetic prostacyclin receptor agonist (Iloprost) as a first-line conservative treatment in patients with Achenbach’s syndrome and COVID-19 infection recovery.
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Billard, C., A. Autret, S. Markabi, et al. "The influence of vigilance states on paroxysmal EEG activities and clinical seizures in children." Electroencephalography and Clinical Neurophysiology 75, no. 3 (1990): 127–35. http://dx.doi.org/10.1016/0013-4694(90)90165-g.
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Novitskaya, Yulia, Katrin Götz-Trabert, and Andreas Schulze-Bonhage. "Recurrent episodes of falls and amnestic confusional states as diagnostic challenge in the elderly." BMJ Case Reports 12, no. 6 (2019): e228842. http://dx.doi.org/10.1136/bcr-2018-228842.
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New-onset paroxysmal events in patients over 60 years of age are often diagnostically challenging owing to atypical presentation. Recurrent falls and transient states of confusion are especially common in the elderly population, yet their causes often remain undiagnosed due to concomitant cognitive deficits and motor impairments. We present an elderly patient with newly occurring ‘blackouts’ without obvious triggers and transient states of confusion for which he was amnestic. All neurological exams including brain MRI scan and routine electroencephalography (EEG) were normal. Long-term ECG monitoring using an event recorder captured an asystole during a habitual episode, leading to the diagnosis of syncope and pacemaker implantation. A subsequent video EEG monitoring performed due to ongoing unexplained confusional states revealed both bradycardia and long-lasting confusional states to be caused by unrecognised temporal lobe seizures. Ictal video EEG monitoring may play a crucial role in establishing a diagnosis of atypical temporal lobe seizures in the elderly.
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Tomassetti, Sarah, Nicholas Kuypers, Jincy Paulose, et al. "Current Real-World Treatment Landscape for Patients with Paroxysmal Nocturnal Hemoglobinuria in the United States." Blood 144, Supplement 1 (2024): 5675. https://doi.org/10.1182/blood-2024-202528.
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Introduction: Treatment options have historically been limited for patients with paroxysmal nocturnal hemoglobinuria (PNH), a rare, life-threatening hematological disorder. However, since the first complement-inhibitor (CI) approval in 2007, therapeutic complement inhibition has revolutionized the treatment of PNH. In December 2023, iptacopan was approved as the first orally administered monotherapy for patients with PNH in the United States (US). Since then, two more CIs have been approved (danicopan and crovalimab). With an increasing number of CIs available for PNH, timely real-world evidence is needed to inform treatment decision-making. The objective of this study was to describe the current real-world treatment landscape among patients with PNH in the US. Methods: This retrospective, observational cohort study used both US closed (insurance provider records) and open claims data (health system and pharmacy records) from Komodo's Healthcare Map™. The study included adults (age ≥18 years) with ≥2 distinct claims with a PNH diagnosis between January 1, 2018 and June 14, 2024. Patients were classified into two mutually exclusive cohorts based on their CI therapy use in the identification period (June 14, 2023 to June 14, 2024): CI-treated (those with ≥2 consecutive paid claims for CI therapy) and CI-untreated (those with ≥1 paid claim associated with a PNH diagnosis and ≤1 paid claim for CI therapy). CI therapies included eculizumab, ravulizumab, pegcetacoplan, iptacopan, danicopan, and crovalimab. The index date was defined as the start date of the most recent CI therapy received for the CI-treated cohort and the date of the earliest claim with a PNH diagnosis within the identification period for the CI-untreated cohort. All patients were required to have continuous enrollment for ≥90 days prior to the index date. It should be noted that this study will be updated with additional patients and clinical outcomes, including laboratory data, which will be available at the time of presentation. Results: A total of 1,090 patients met the study inclusion criteria, of whom 369 (34%) were CI-treated (median age 49 years [interquartile range (IQR) 35-64 years], 58% female, 36% White, 10% Black, 8% Asian, and 55% covered by commercial insurance) and 721 (66%) were CI-untreated (median age 52 years [IQR 34-67 years], 56% female, 44% White, 10% Black, 7% Asian, and 48% covered by commercial insurance). The distribution of claims for the most recent CI therapy were as follows: pegcetacoplan (41.7%), ravulizumab (34.7%), iptacopan (13.8%), eculizumab (9.5%), danicopan (0.3%), and crovalimab (0%). Of the three most recently approved CI therapies, iptacopan had the highest number of patients with claims (n=51) and real-world data available; one patient had claims for danicopan and none for crovalimab. Among the 51 patients with iptacopan claims, the median age was 53 years (IQR 35-66 years); 65% were female, 41% were White, 6% were Black, 18% Asian, and 63% were covered by commercial insurance. At the start of iptacopan therapy, patients who had a claim for prior CI therapy were most commonly patients switching from pegcetacoplan and ravulizumab. The remaining patients did not have any claim for CI therapy in the 6 months prior to the index date. Conclusions: This study provides early insights into the current real-world treatment landscape for patients with PNH. Following its approval, uptake of iptacopan among patients with PNH has been rapid in US real-world clinical practice, with 14% of CI-treated patients currently receiving iptacopan as their most recent therapy. Nonetheless, the majority of included patients with PNH did not have a claim for a CI therapy during the study period, despite the availability of six approved CI therapies.
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Dingli, David, Joana E. Matos, Kerri Lehrhaupt, Sangeeta Krishnan, Sujata P. Sarda, and Scott B. Baver. "Clinical Burden of Paroxysmal Nocturnal Hemoglobinuria Among Patients Receiving C5 Inhibitors in the United States." Blood 136, Supplement 1 (2020): 2. http://dx.doi.org/10.1182/blood-2020-141844.
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INTRODUCTION Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, hematologic disease characterized by chronic complement-mediated hemolysis. Treatment with the C5 inhibitor eculizumab has resulted in a reduction in intravascular hemolysis (IVH) and improvements in morbidity and mortality. However, in a single-center cohort of patients with PNH receiving treatment with eculizumab, 72% remained anemic and 36% continued to require transfusions due to ongoing IVH and extravascular hemolysis (McKinley CE, et al.Blood. 2017;130(Suppl 1):3471; Risitano AM, et al.Front Immunol. 2019;10:1157). This study aims to describe the burden of illness in patients with PNH currently being treated with C5 inhibitors (eculizumab and ravulizumab). Overall, the study aims to understand the clinical and hematological outcomes associated with burden of illness in about 150 patients with PNH globally. In these preliminary analyses, the impact of PNH on hematologic and clinical measures is assessed from patients in the United States. METHODS A cross-sectional survey was administered to adult patients ≥18 years of age in the United States with a self-reported diagnosis of PNH, recruited through a patient advocacy group. Inclusion criteria to complete the secure online survey included current treatment with either eculizumab or ravulizumab, informed consent, and agreement to adverse event reporting. This study was initiated in July 2020 and is ongoing. Results presented herein are preliminary. Impact of PNH on hematologic and clinical measures will be assessed using the following variables: diagnosis levels; and any patient history of blood transfusions, thrombotic events, renal impairment, fatigue, and other PNH-associated symptoms as well as dosing frequency and treatment patterns. For these preliminary analyses, descriptive statistics will be reported for patients who have completed the survey. RESULTS As of August 6, 2020, 58 adult patients with a median age of 52 years (range, 21-88) completed the survey, among which 78% were female. Current medications included eculizumab (n = 20 [34.5%]) or ravulizumab (n = 38 [65.5%]), as well as concurrent anticoagulants (n = 9 [15.5%]) and/or anti-thrombotics (n = 2 [3%]). Most patients initiated treatment with eculizumab (n = 20 [100%]) or with ravulizumab (n = 34 [90%]) ≥3 months before. Median (interquartile range) last known hemoglobin level for patients on eculizumab and ravulizumab was 9.3 g/dL (8.0-11.1) and 10.1 g/dL (8.9-11.5), respectively. Overall, 45 (82%) patients reported hemoglobin values &lt;12 g/dL (eculizumab: 90%; ravulizumab: 78%). Forty (69%) patients reported having ≥1 red blood cell transfusion at any point during their disease. Within the previous 12 months, 53% and 26% of eculizumab- and ravulizumab-treated patients, respectively, had ≥1 transfusion, and 12% and 17% were unsure. Among those patients who had ever received ≥1 transfusion, 6% and 13% had &gt;4 transfusions in the previous 12 months for eculizumab and ravulizumab, respectively. Seventeen patients (29%) reported ≥1 thrombotic event at any point during their disease. Seven patients reported thrombotic events over the previous 12 months; six were receiving ravulizumab. The majority (77%) of patients reported fatigue. Fatigue was reported by nearly 95% of eculizumab-treated patients and 68% of ravulizumab-treated patients. CONCLUSIONS Preliminary results from this burden of illness survey demonstrate that a majority of patients with PNH report remaining anemic, despite treatment with C5 inhibitors eculizumab and ravulizumab for a period of ≥3 months. Disclosures Dingli: Sanofi-Genzyme:Consultancy;Karyopharm Therapeutics:Research Funding;Bristol Myers Squibb:Research Funding;Millenium:Consultancy;Alexion:Consultancy;Apellis:Consultancy;Rigel:Consultancy;Janssen:Consultancy.Matos:Kantar:Current Employment.Lehrhaupt:Kantar:Current Employment.Krishnan:Apellis:Current Employment, Current equity holder in publicly-traded company.Sarda:Apellis:Current Employment, Current equity holder in publicly-traded company.Baver:Apellis:Current Employment, Current equity holder in publicly-traded company.
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Steriade, M. "Impact of Network Activities on Neuronal Properties in Corticothalamic Systems." Journal of Neurophysiology 86, no. 1 (2001): 1–39. http://dx.doi.org/10.1152/jn.2001.86.1.1.
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Data from in vivo and in vitro experiments are discussed to emphasize that synaptic activities in neocortex and thalamus have a decisive impact on intrinsic neuronal properties in intact-brain preparations under anesthesia and even more so during natural states of vigilance. Thus the firing patterns of cortical neuronal types are not inflexible but may change with the level of membrane potential and during periods rich in synaptic activity. The incidences of some cortical cell classes (defined by their responses to depolarizing current pulses) are different in isolated cortical slabs in vivo or in slices maintained in vitro compared with the intact cortex of naturally awake animals. Network activities, which include the actions of generalized modulatory systems, have a profound influence on the membrane potential, apparent input resistance, and backpropagation of action potentials. The analysis of various oscillatory types leads to the conclusion that in the intact brain, there are no “pure” rhythms, generated in simple circuits, but complex wave sequences (consisting of different, low- and fast-frequency oscillations) that result from synaptic interactions in corticocortical and corticothalamic neuronal loops under the control of activating systems arising in the brain stem core or forebrain structures. As an illustration, it is shown that the neocortex governs the synchronization of network or intrinsically generated oscillations in the thalamus. The rhythmic recurrence of spike bursts and spike trains fired by thalamic and cortical neurons during states of decreased vigilance may lead to plasticity processes in neocortical neurons. If these phenomena, which may contribute to the consolidation of memory traces, are not constrained by inhibitory processes, they induce seizures in which the neocortex initiates the paroxysms and controls their thalamic reflection. The results indicate that intact-brain preparations are necessary to investigate global brain functions such as behavioral states of vigilance and paroxysmal activities.
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Nishimura, Jun-Ichi, Yuzuru Kanakura, Russell E. Ware, et al. "Clinical Course and Flow Cytometric Analysis of Paroxysmal Nocturnal Hemoglobinuria in the United States and Japan." Medicine 83, no. 3 (2004): 193–207. http://dx.doi.org/10.1097/01.md.0000126763.68170.46.
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Mulherin, Brian, Lawton Laurence, Carl Friddle, et al. "Patient Satisfaction with Use of Complement Inhibitor Injector in Paroxysmal Nocturnal Hemoglobinuria in the United States." Blood 144, Supplement 1 (2024): 5673. https://doi.org/10.1182/blood-2024-200983.
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Introduction: Pegcetacoplan is a C3/C3b inhibitor approved for paroxysmal nocturnal hemoglobinuria (PNH), a rare disease characterized by complement-mediated hemolysis that can lead to anemia and thrombosis. The previously approved C5 inhibitors (C5is) eculizumab and ravulizumab for PNH are administered in health care settings as intravenous infusions. In contrast, pegcetacoplan can be self-administered as a subcutaneous (SC) injection with an at-home infusion pump. On October 2, 2023, a wearable, single-use, SC pegcetacoplan injector with a hidden needle was approved by the US Food and Drug Administration after being validated for ease of use, safety, and effectiveness in a human factors study. This analysis of 2 US studies describes patients' experience with the new injector in the real world. Methods: A US cross-sectional, mixed methods study is ongoing. Adults with PNH diagnosed for ≥6 months, who self-administered pegcetacoplan for &gt;3 months and switched to the new injector for ≥6 weeks could fill out a preliminary web-based survey (including 17 items with 1-7 Likert scale ratings [1-worst response, 7-best response]) before completing a qualitative, 45-minute telephone interview to discuss and rate their administration experience with the new injector compared with prior device and C5i therapies. Interview results were summarized in 7 items with Likert scale ratings from 1 (worst) to 7 (best), and 1 item (“overall administration experience”) rated from 1 (worst) to 100 (best). Interim findings on patients' experience with the new injector are presented ahead of full enrollment and final analysis. In a separate US study, email and text messaging surveys (7 questions; 6 with yes/no answers) were sent out by care-coordinators from the ApellisAssist support program to patients who recently (~2 weeks) converted from an infusion pump to the new injector for their pegcetacoplan treatment. Results: As of July 15, 2024, 7 patients have been recruited in the real-world injector users' experience study. Of the 6 patients who filled out the preliminary web-based survey, 5 reported they were “very confident” (7-rating) and 1 was “confident” (6-rating; 1-7-scale) overall about administering pegcetacoplan with the new injector at home; all (n=6) reported that it was “very easy” (7-rating; 1-7-scale) to administer pegcetacoplan with the new device without assistance. Among 6 of the 7 patients who participated in the telephone interviews, the mean of 6 individual “overall administration experience” ratings was 81 (1-100-scale from worst to best experience); the mean experience satisfaction ratings (1-7-scale) were 6.3 for convenience, 6.2 for ability to complete tasks around the house, 5.8 for ability to move freely and confidence in the administration, 5.3 for ease of use, and 5.2 for ability to run errands. Notably, 5 of the 6 patients generally gave high ratings for their experience with the new injector, and all 7 interviewed patients were very (n=1) or extremely (n=6) likely to recommend the new device to other patients with PNH (mean rating = 6.9; 1-7-scale [1-not at all likely; 7-extremely likely]). In the email/text messaging study, 58 patients completed and returned the survey. Of these, 42/58 (72%) had used the injector ≥5 times. Almost all patients (56/58; 97%) were satisfied with the new injector and preferred it over their previous device (52/58; 90%); compared with their previous device, 79% (46/58) felt more confident about self-administration with the new device, 86% (50/58) stated it was faster to set up, 90% (52/58) felt the hidden needle helped them be more at ease with the infusion, and 90% (52/58) felt more mobile during the infusion. Conclusions: Preliminary real-world findings suggest that most patients with PNH were satisfied with various aspects of their experience with the new single-use, wearable injector and were likely to recommend it to other patients with PNH. All patients found it easy to self-administer pegcetacoplan with the new injector without assistance and most were confident in the administration. Surveyed patients seemed to prefer the new device and feel more confident using it than their previous device. Although responses might have been impacted by recall and social desirability biases, these findings align with those from the device human factors evaluation study, suggesting that real-world evidence supports laboratory results on usability of the new injector.
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Stern, Robert M., and Nathan T. Connell. "Ravulizumab: a novel C5 inhibitor for the treatment of paroxysmal nocturnal hemoglobinuria." Therapeutic Advances in Hematology 10 (January 2019): 204062071987472. http://dx.doi.org/10.1177/2040620719874728.
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare stem cell disorder characterized by hemolytic anemia, bone marrow failure, and thrombosis. Until recently, the complement inhibitor, eculizumab, was the only United States Food and Drug Administration (US FDA)-approved therapy for the treatment of PNH. Although effective, eculizumab requires a frequent dosing schedule that can be burdensome for some patients and increases the risk of breakthrough intravascular hemolysis. Ravulizumab, an eculizumab-like monoclonal antibody engineered to have a longer half-life, is intended to provide the same benefits as eculizumab but with a more convenient and effective dosing schedule. In two recently published phase III non-inferiority trials, ravulizumab was found to be non-inferior to eculizumab both in efficacy and safety for the treatment of patients with PNH. Based on these results, ravulizumab was approved by the US FDA on 21 December 2018 and is currently under regulatory review in both the European Union and Japan.
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Glazunova, L. A., A. R. Musina, A. A. Yurchenko, Y. V. Glazunov, and E. M. Gagarin. "Spread of alimentary-toxic paroxysmal myoglobinuria-haff disease (literature review)." E3S Web of Conferences 254 (2021): 09002. http://dx.doi.org/10.1051/e3sconf/202125409002.
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In 1924, Haff disease was first detected in East Prussia. Till now, cases of Haff disease have been recorded in Sweden, Russia, the United States, China, Brazil, Japan, and China among people and animals. During the last 40 years, there has been a significant expansion in the geographical range of Haff disease. From 1924 to 2019, 31 outbreaks were recorded in various parts of the world. The total number of victims was about 3,000 people. In Russia, the last cases of human disease were registered in 2019-2020. In fact, the source of the toxin is fish (crucian carp, carp, pike, burbot, walleye, perch, ruff, ide, yellowtail, black sea bass, eel, silver dollar, brown paku, red paku, cowfish, etc.) or crayfish. Today, the problem of the disease etiology has not been solved; the toxin with the corresponding features has not been isolated, and as a result, causal and pathogenetic treatment of alimentary-toxic paroxysmal myoglobinuria has not been developed. Over this period, several hypotheses were made that are leading in the study of the etiology of the occurrence of Haff disease (thiaminase theory, tannic, arachidonic). This disease-causing substance is known to be heat-resistant and break down the metabolism of skeletal muscles, resulting in the release of myoglobin, which disorders kidney function. It has also been found that toxic substances themselves gradually resolve from the fish, according to its diet (depending on what prevails - plankton, zooplankton or larvae, mollusks, crustaceans). For finding out the origin of the disease, it is essential to conduct comprehensive research by biologists, hydrologists, doctors, and veterinarians.
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Voronina, Tatyana A., and Svetlana A. Litvinova. "The possibility of pharmacological correction of disorders arising from cerebral ischemia and paroxysmal conditions. Derivatives of Dibenzofuran." Reviews on Clinical Pharmacology and Drug Therapy 17, no. 1 (2019): 65–70. http://dx.doi.org/10.17816/rcf17165-70.
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The review examines the role of brain hypoxia in the development of stroke and convulsive States and the possibility of using antihypoxants and antioxidants in various hypoxic conditions, including strokes and convulsions; provides information about the original compound from the class of dibenzofuran with antihypoxic, neuroprotective and anticonvulsant action.
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Paisey, John, Joe Moss, Jason Andrade, et al. "Economic evaluation of first-line cryoballoon ablation versus antiarrhythmic drug therapy for the treatment of paroxysmal atrial fibrillation from an English National Health Service perspective." Open Heart 11, no. 1 (2024): e002423. http://dx.doi.org/10.1136/openhrt-2023-002423.
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IntroductionThree recent randomised controlled trials have demonstrated that pulmonary vein isolation as an initial rhythm control strategy with cryoablation reduces atrial arrhythmia recurrence in patients with symptomatic paroxysmal atrial fibrillation (PAF) compared with antiarrhythmic drug (AAD) therapy. The aim of this study was to evaluate the cost-effectiveness of first-line cryoablation compared with first-line AADs for treating symptomatic PAF in an English National Health Service (NHS) setting.MethodsIndividual patient-level data from 703 participants with PAF enrolled into Cryo-FIRST (Catheter Cryoablation Versus Antiarrhythmic Drug as First-Line Therapy of Paroxysmal Atrial Fibrillation), STOP AF First (Cryoballoon Catheter Ablation in an Antiarrhythmic Drug Naive Paroxysmal Atrial Fibrillation) and EARLY-AF (Early Aggressive Invasive Intervention for Atrial Fibrillation) were used to derive the parameters applied in the cost-effectiveness model (CEM). The CEM comprised a hybrid decision tree and Markov structure. The decision tree had a 1-year time horizon and was used to inform the initial health state allocation in the first cycle of the Markov model (40-year time horizon; 3-month cycle length). Health benefits were expressed in quality-adjusted life years (QALYs). Costs and benefits were discounted at 3.5% per year. Model outcomes were generated using probabilistic sensitivity analysis.ResultsThe results estimated that cryoablation would yield more QALYs (+0.17) and higher costs (+£641) per patient over a lifetime than AADs. This produced an incremental cost-effectiveness ratio of £3783 per QALY gained. Independent of initial treatment, individuals were expected to receive ~1.2 ablations over a lifetime. There was a 45% relative reduction in time spent in AF health states for those initially treated with cryoablation.DiscussionAF rhythm control with first-line cryoablation is cost effective compared with first-line AADs in an English NHS setting.
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Kulasekararaj, Austin G., Robert A. Brodsky, Jun-ichi Nishimura, Christopher J. Patriquin, and Hubert Schrezenmeier. "The importance of terminal complement inhibition in paroxysmal nocturnal hemoglobinuria." Therapeutic Advances in Hematology 13 (January 2022): 204062072210910. http://dx.doi.org/10.1177/20406207221091046.
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, chronic hematologic disorder associated with inappropriate terminal complement activity on blood cells that can result in intravascular hemolysis (IVH), thromboembolic events (TEs), and organ damage. Untreated individuals with PNH have an increased risk of morbidity and mortality. Patients with PNH experiencing IVH often present with an elevated lactate dehydrogenase (LDH; ⩾ 1.5 × the upper limit of normal) level which is associated with a significantly higher risk of TEs, one of the leading causes of death in PNH. LDH is therefore used as a biomarker for IVH in PNH. The main objective of PNH treatment should therefore be prevention of morbidity and mortality due to terminal complement activation, with the aim of improving patient outcomes. Approval of the first terminal complement inhibitor, eculizumab, greatly changed the treatment landscape of PNH by giving patients an effective therapy and demonstrated the critical role of terminal complement and the possibility of modulating it therapeutically. The current mainstays of treatment for PNH are the terminal complement component 5 (C5) inhibitors, eculizumab and ravulizumab, which have shown efficacy in controlling terminal complement-mediated IVH, reducing TEs and organ damage, and improving health-related quality of life in patients with PNH since their approval by the United States Food and Drug Administration in 2007 and 2018, respectively. Moreover, the use of eculizumab has been shown to reduce mortality due to PNH. More recently, interest has arisen in developing additional complement inhibitors with different modes of administration and therapeutics targeting other components of the complement cascade. This review focuses on the pathophysiology of clinical complications in PNH and explores why sustained inhibition of terminal complement activity through the use of complement inhibitors is essential for the management of patients with this chronic and debilitating disease.
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Ulcickas Yood, Marianne, Susan Jick, Catherine Vasilakis-Scaramozza, et al. "Baseline Characteristics of Patients with Paroxysmal Nocturnal Hemoglobinuria Identified in the Department of Defense Database." Blood 132, Supplement 1 (2018): 5830. http://dx.doi.org/10.1182/blood-2018-99-113478.
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Abstract Background: Rare diseases are often characterized by misdiagnosis resulting in delays in critical and potentially lifesaving treatment. Awareness of first signs and symptoms of rare diseases can provide clinical evidence for early and accurate diagnosis. Medical records are the primary source of clinical information from first signs and symptoms to key clinical disease related events, yet access to records can be difficult and time consuming. We conducted a study using a large electronic medical record (EMR) and claims database to identify and describe characteristics of Paroxysmal Nocturnal Hemoglobinuria (PNH) patients at the time of their PNH diagnosis. We present baseline findings of the PNH population identified in the Department of Defense (DOD) healthcare system, where all clinical details were readily available. The DOD healthcare system is a US-based, longitudinal EMR and claims database with health information on approximately 10 million active beneficiaries throughout the country. Methods: We identified all people in the DOD database from January 01, 2007 through May 31, 2017 who had an ICD-10-CM code for PNH or a NDC or HCPCS code for eculizumab, the drug used to treat PNH. There is no ICD-9-CM code for PNH. Cases had no other indication for eculizumab use and had to have appropriate symptoms, comorbidities or lab results to be considered a case. We reviewed the electronic record for each patient and classified each as definite/likely, probable/possible or unlikely PNH based on attributes of the available health data including codes for eculizumab, flow cytometry, lab results, hemoglobinuria, aplastic anemia, pancytopenia and other comorbidities. Our ruling was validated by medical record review by a clinical expert for all equivocal cases and a sample of definite/likely cases. Individuals classified as unlikely PNH were excluded. Patient characteristics at PNH diagnosis are presented using descriptive statistics. Results: We identified 73 PNH patients (55% female) after review of all available electronic data; 41 had a diagnosis of PNH and received eculizumab, 17 had a diagnosis of PNH only, and 15 received eculizumab only. From these, 61 patients were determined to have definite/likely PNH and 12 had probable/possible PNH. The use of eculizumab ranged from 0 to 378 (median=18) prescriptions, dependent in part on the amount of follow-up in the database. There were 19 patients (26%) who had prevalent PNH and 54 (74%) who were newly diagnosed with PNH during the study period. Among newly diagnosed patients, the median age at PNH diagnosis was 46 years (range 13 - 86). Most patients had codes for hemoglobinuria at or before the PNH diagnosis date (69%), and many had codes for aplastic anemia (46%), thrombocytopenia (56%), and pancytopenia (28%). At any time after the PNH diagnosis, 85% had unspecified anemia, 52% had aplastic anemia, 53% had thrombocytopenia, and 33% had pancytopenia codes in their records. See table. Conclusion: From a population of around 10 million actively enrolled patients, across a 10-year study period, we identified 73 patients with PNH. Data from these patients will be used to identify patterns of health encounters leading up to diagnosis and relevant outcomes following diagnosis. This information can be used to help diagnose other patients with this rare disease and to improve their medical outcomes. Disclaimer Statement: Research data were derived from an approved Naval Medical Center, Portsmouth, VA IRB protocol (NMCP.2017.0080). The views expressed in this abstract are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense or the United States Government. Copyright Notice: CAPT Brian Feldman is a military service member. This work was prepared as part of his official duties. Title 17 U.S.C. 105 provides that 'Copyright protection under this title is not available for any work of the United States Government.' Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person's official duties. Table. Table. Disclosures Ulcickas Yood: Alexion Pharmaceuticals, Inc.: Other: Employee of EpiSource, LLC, which was contracted by Alexion Pharmaceuticals, Inc. EpiSource had the final decision on content. . Jick:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data. . Vasilakis-Scaramozza:Alexion Pharmaceuticals, Inc.: Other: Employee of the Boston Collaborative Drug Surveillance Program, which was contracted and paid by Alexion Pharmaceuticals, Inc. to work on a study of PNH using DOD data.. Donato:Alexion Pharmaceuticals Inc: Employment, Equity Ownership. Tomazos:Alexion Pharmaceuticals, Inc.: Employment, Equity Ownership. L'Italien:Alexion Pharmaceuticals, Inc.: Equity Ownership, Other: Former employee and current stockholder of Alexion Pharmaceuticals, Inc. . Sicignano:Alexion Pharmaceuticals, Inc.: Other: Employee of Health ResearchTx, which has a business relationship with Alexion Pharmaceuticals, Inc.. Feldman:Alexion Pharmaceuticals, Inc.: Other: Employee, Department of Navy, United States Government..
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Nosov, S.G., L.М. Yuryeva, T.Y. Shusterman, and O.V. Nekrasova. "Clinical and pathogenetic prediction of the dynamics of the course of psychoses in epilepsy." Medicni perspektivi 27, no. 3 (2022): 97–102. https://doi.org/10.26641/2307-0404.2022.3.265945.
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Transient epileptic psychoses in 30-40% of cases turn into psychotic states with a long, paroxysmal or chronic course. The objective of this article is to conduct a systematic analysis of modern literature sources to clarify the factors, clinical and pathogenetic patterns of transformation of transient (short-term) epileptic psychosis into psychotic states with prolonged and chronic course, as well as to identify pathogenetically oriented principles of treatment of such patients. The prognostic value of clinical and neurophysiological factors regarding increasing of the psychotic process duration has been noticed, clinical patterns of transformation of short-term psychosis into long-term (changes in the dynamics and structure of psychosis, features of the relationship with organic personality disorder and dementia in epilepsy, as well as epileptic seizures) have been shown. A number of important neurophysiological pathogenetic mechanisms of increasing the psychosis duration (growing cerebral hypofrontality, temporal localization and regular spread of the epileptic process) have been identified. Pathogenetically oriented principles of treatment tactics of patients with epileptic psychoses have been described and analyzed taking into account the revealed regularities of increasing of their duration.
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Lugovskaya, Svetlana, Fyodor Dyukov, Elena Naumova, Margarita Pochtar, Olga Plehanova, and Vladimir Dolgov. "Screening for Paroxysmal Nocturnal Hemoglobinurea with Cytodiff Analysis." Blood 124, no. 21 (2014): 4962. http://dx.doi.org/10.1182/blood.v124.21.4962.4962.
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Abstract Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder resulting from the somatic mutation of the X-linked phosphatidylinositolglycan complementation Class A (PIG-A) gene. PIGA mutations in PNH patients lead to a glycosylphosphatidylinositol (GPI)-linked membrane proteins expression deficiency.In PNH, there is a partial or absolute inability to make GPI-anchored proteins including complement-defense structures such as CD55 and CD59 on RBCs and WBCs. Clinical features of PNH include intravascular hemolysis, bone marrow failure, and thrombosis, all major causes of morbidity and mortality. Flow cytometry (FCM) plays a key role in the laboratory investigation of PNH, and rapid diagnosis of this condition is highly desirable. A definitive diagnosis of PNH can be established by demonstrating the absence of cell membraneGPI-anchored proteins from granulocytes or red blood cells (RBC) according to ICCS Guidelines for the diagnosis and monitoring of PNH by flow cytometry. It has been also described that the expression of CD16 can be decreased on PNH-affected granulocytes. Recently new method for extended flow WBC differential was introduced by Beckman Coulter. This method uses flow cytometric analysis with CytoDiff™** reagents which is a 5-color/6-marker reagent that provides a 10-part cytometric differential from whole blood specimens and comprises CD36-FITC, (CD2+CD294)-PE, CD19-ECD, CD16-PC5, and CD45-PC7 (Beckman Coulter). The protocol allows detection of mature neutrophils, total lymphocytes, total monocytes, eosinophils, basophils, immature granulocytes, B lymphocytes, CD16-negative T/NK lymphocytes, CD16-positive T/NK lymphocytes, CD16 positive and CD16 negative monocytes, and blasts cells with lineage orientation. This method also allows the detection of the abnormal antigen expression on WBC, for example low CD16 expression on neutrophils. In case of abnormal low CD16 expression on segmented neutrophils they will be classified as Immature Granulocytes (Imm Gran). The aim of the study was to evaluate the efficacy of CytoDiff** analysis of peripheral blood for PNH screening detecting low CD16 expression on neutrophils. Methods: EDTA-anticoagulated blood samples from 53 patients with PHN suspicion were prospectively included in the study. Analysis of the PNH clones was conducted in according with international protocol, using CD235a for RBC gating, CD15-PE/CD45-PC7 for granulocyte gating, CD64-PC5/CD45-PC7 for monocyte gating and GPI-anchored proteins CD59-PE, FLAER-FITC/CD24-PC5 and FLAER-FITC/CD14-PE for RBCs, granulocytes and monocytes accordingly. For extended flow WBC differential analysis the blood samples were stained with the CytoDiff** panel, lyzed with Versalyse (Beckman Coulter) and 20 000 leucocytes were analyzed on a FC500 Flow Cytometer (Beckman Coulter) using CytoDiff** CXP software. Results:Totally 53 patients with PHN suspicion were prospectively included in the study. All these patients were characterized by anemia, thrombocytopenia and/or leucopenia. PNH diagnosis was confirmed in 6 patients and in other 7 patients the final diagnosis was aplastic anemia with PNH clone. For all 13 patients (4 males, 9 females, with median age of 41.5 years) with confirmed presence of PNH clone CytoDiff** reported increased number of Imm Gran (range 3-45%). Microscopy analysis did not detect the presence of Imm Grans in the slide, so we concluded that falsely reported increased Imm Gran count was due to the decreased expression of CD16 on Neutrophils. Good correlation (r=0.9257) was observed between Imm Gran count and the size of granulocytic PNH clone. Conclusion: Our data demonstrate that CytoDiff** analysis, which is able to detect a wide spectrum of normal and pathological cells in peripheral blood allows determination of CD16-low expression on neutrophils and thus provide efficient screening for suspicious of PNH in patients with anemic syndrome. ** Not available in the United States and other geographies. Disclosures Lugovskaya: Beckman Coulter: Research Funding.
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Patlolla, Sri Harsha, Sriharsha Kandlakunta, Sanjana Boyapalli, Aravind Reddy Kuchkuntla, and Harika Kandlakunta. "PREVALENCE OF TRICUSPID AND MITRAL VALVE REGURGITATION IN PATIENTS WITH NON-PAROXYSMAL ATRIAL FIBRILLATION IN THE UNITED STATES." Journal of the American College of Cardiology 81, no. 8 (2023): 1964. http://dx.doi.org/10.1016/s0735-1097(23)02408-7.
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Nosov, S. G., L. М. Yuryeva, T. Y. Shusterman, and O. V. Nekrasova. "Clinical and pathogenetic prediction of the dynamics of the course of psychoses in epilepsy." Medicni perspektivi 27, no. 3 (2022): 97–102. http://dx.doi.org/10.26641/2307-0404.2022.3.265945.
Full textAbstract:
Transient epileptic psychoses in 30-40% of cases turn into psychotic states with a long, paroxysmal or chronic course. The objective of this article is to conduct a systematic analysis of modern literature sources to clarify the factors, clinical and pathogenetic patterns of transformation of transient (short-term) epileptic psychosis into psychotic states with prolonged and chronic course, as well as to identify pathogenetically oriented principles of treatment of such patients. The prognostic value of clinical and neurophysiological factors regarding increasing of the psychotic process duration has been noticed, clinical patterns of transformation of short-term psychosis into long-term (changes in the dynamics and structure of psychosis, features of the relationship with organic personality disorder and dementia in epilepsy, as well as epileptic seizures) have been shown. A number of important neurophysiological pathogenetic mechanisms of increasing the psychosis duration (growing cerebral hypofrontality, temporal localization and regular spread of the epileptic process) have been identified. Pathogenetically oriented principles of treatment tactics of patients with epileptic psychoses have been described and analyzed taking into account the revealed regularities of increasing of their duration.
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D.M. Akpanova, D.A. Ospanova, S.F. Berkinbayev, A.T. Mussagaliyeva, and A.M. Grjibovsk. "Predictors of Hospital Mortality in Patients with Atrial Fibrillation and Stroke." Journal of Pharmacy and Nutrition Sciences 9, no. 1 (2019): 49–59. http://dx.doi.org/10.29169/1927-5951.2019.09.01.9.
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Atrial fibrillation is one of the most common cardiac arrhythmias; it accounts for about a third of all hospital admissions for cardiac arrhythmias. Currently, there is a tendency of aging of the population and an increase in overall life expectancy, which will further lead to an increase in the number of patients with atrial fibrillation.Usually atrial fibrillation is associated with a number of symptoms such as palpitations, interruptions, shortness of breath, pain in the heart area, fatigue, dizziness and syncopal states, but at the same time the course of both paroxysmal and permanent atrial fibrillation may not be accompanied by obvious symptoms or a noticeable decrease in quality life. Such asymptomatic atrial fibrillation is usually diagnosed by chance during an examination and can be considered a clinical finding. According to a number of studies, every third to fifth patient with atrial fibrillation was asymptomatic, and in a recently completed study in patients with paroxysmal atrial fibrillation, more than 50% of all episodes of arrhythmia were asymptomatic. When newly diagnosed atrial fibrillation, the asymptomatic form may occur in 83.2% of cases [1].The purpose of the research is on the basis of studying the clinical features of the course of atrial fibrillation, determine the effect of asymptomatic arrhythmia on the development of fatal complications and patient survival in various forms of atrial fibrillation and develop a therapeutic strategy for managing patients with asymptomatic atrial fibrillation for the first time.
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van Bijnen, Sandra, Konnie Hebeda, and Petra Muus. "Bone Marrow Histology in Patients with Paroxysmal Nocturnal Hemoglobinuria." Blood 114, no. 22 (2009): 4215. http://dx.doi.org/10.1182/blood.v114.22.4215.4215.
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Abstract Abstract 4215 Introduction Paroxysmal Nocturnal Hemoglobinuria (PNH) is a disease of the hematopoietic stem cell (HSC) resulting in a clone of hematopoietic cells deficient in glycosyl phosphatidyl inositol anchored proteins. The clinical spectrum of PNH is highly variable with classical hemolytic PNH at one end, and PNH in association with aplastic anemia (AA/PNH) or other bone marrow failure states at the other end. It is still largely unknown what is causing these highly variable clinical presentations. Immune-mediated marrow failure has been suggested to contribute to the development of a PNH clone by selective damage to normal HSC. However, in classic PNH patients with no or only mild cytopenias, a role for immune mediated marrow failure is less obvious. No series of trephine biopsies has been previously documented of patients with PNH and AA/PNH to investigate the similarities and differences in these patients. Methods We have reviewed a series of trephine biopsies of 41 PNH patients at the time the PNH clone was first detected. The histology was compared of 27 patients with aplastic anemia and a PNH clone was compared to that of 14 patients with classic PNH. Age related cellularity, the ratio between myeloid and erythroid cells (ME ratio), and the presence of inflammatory cells (mast cells, lymphoid nodules and plasma cells) were evaluated. The relation with clinical and other laboratory parameters of PNH was established. Results Classic PNH patients showed a normal or hypercellular marrow in 79% of patients, whereas all AA/PNH patients showed a hypocellular marrow. Interestingly, a decreased myelopoiesis was observed not only in AA/PNH patients but also in 93% of classic PNH patients, despite normal absolute neutrophil counts (ANC ≥ 1,5 × 109/l) in 79% of these patients. The number of megakaryocytes was decreased in 29% of classic PNH patients although thrombocytopenia (< 150 × 109/l) was only present in 14% of the patients. Median PNH granulocyte clone size was 70% (range 8-95%) in classic PNH patients, whereas in AA/PNH patients this was only 10% (range 0.5-90%). PNH clones below 5% were exclusively detected in the AA/PNH group. Clinical or laboratory evidence of hemolysis was present in all classical PNH patients and in 52% of AA/PNH patients and correlated with PNH granulocyte clone size. Bone marrow iron stores were decreased in 71% of classic PNH patients. In contrast, increased iron stores were present in 63% of AA/PNH patients, probably reflecting their transfusion history. AA/PNH patients showed increased plasma cells in 15% of patients and lymphoid nodules in 37%, versus 0% and 11% in classic PNH. Increased mast cells (>2/high power field) were three times more frequent in AA/PNH (67%) than in PNH (21%). Conclusion Classic PNH patients were characterized by a more cellular bone marrow, increased erythropoiesis, larger PNH clones and clinically by less pronounced or absent peripheral cytopenias and more overt hemolysis. Decreased myelopoiesis and/or megakaryopoiesis was observed in both AA/PNH and classic PNH patients, even in the presence of normal peripheral blood counts, suggesting a role for bone marrow failure in classic PNH as well. More prominent inflammatory infiltrates were observed in AA/PNH patients compared to classical PNH patients. Disclosures: No relevant conflicts of interest to declare.
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Jiang, Yunqiu, Sunny S. Po, Faris Amil, and Tarun W. Dasari. "Non-invasive Low-level Tragus Stimulation in Cardiovascular Diseases." Arrhythmia & Electrophysiology Review 9, no. 1 (2020): 40–46. http://dx.doi.org/10.15420/aer.2020.01.
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Low-level tragus stimulation (LLTS) is a non-invasive approach of transcutaneous vagus nerve stimulation. LLTS has applications in diseases of multiple systems, including epilepsy, depression, headache and potentially several cardiovascular diseases. LLTS has shown promising results in suppressing AF, alleviating post-MI ventricular arrhythmias and ischaemia-reperfusion injury along with improving diastolic parameters in heart failure with preserved left ventricular ejection fraction (HFpEF). Preliminary pilot clinical studies in patients with paroxysmal AF, HFpEF, heart failure with reduced ejection fraction and acute MI have demonstrated promising results. The beneficial effects are likely secondary to favourable alteration of the sympathovagal imbalance. On-going exploratory work focused on underlying mechanisms of LLTS in cardiovascular disease states and larger scale clinical trials will shed more light on the non-invasive modulation of the neuro-immune axis.
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Jaul, Efraim, and Oded Meiron. "Advanced Dementia: Brain-State Characteristics and Clinical Indicators of Early Mortality." Journal of Alzheimer's Disease 81, no. 3 (2021): 933–41. http://dx.doi.org/10.3233/jad-201563.
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There is an urgent need in advanced dementia for evidence-based clinical prognostic predictors that could positively influence ethical decisions allowing health provider and family preparation for early mortality. Accordingly, the authors review and discuss the prognostic utility of clinical assessments and objective measures of pathological brain states in advanced dementia patients associated with accelerated mortality. Overall, due to the paucity of brain-activity and clinical-comorbidity predictors of survival in advanced dementia, authors outline the potential prognostic value of brain-state electroencephalography (EEG) measures and reliable clinical indicators for forecasting early mortality in advanced dementia patients. In conclusion, two consistent risk-factors for predicting accelerated mortality in terminal-stage patients with advanced dementia were identified: pressure ulcers and paroxysmal slow-wave EEG parameters associated with cognitive impairment severity and organic disease progression. In parallel, immobility, malnutrition, and co-morbid systemic diseases are highly associated with the risk for early mortality in advanced dementia patients. Importantly, the authors’ conclusions suggest utilizing reliable quantitative-parameters of disease progression for estimating accelerated mortality in dementia patients entering the terminal disease-stages characterized by severe intellectual deficits and functional disability.
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Levy, A. R., I. Tomazos, Y. Patel, B. M. K. Donato, and A. Briggs. "PSY15 COMPARISON OF LOST PRODUCTIVITY DUE TO ECULIZUMAB AND RAVULIZUMAB TREATMENTS FOR PAROXYSMAL NOCTURNAL HEMOGLOBINURIA IN THE UNITED STATES." Value in Health 22 (May 2019): S377. http://dx.doi.org/10.1016/j.jval.2019.04.1840.
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Shammo, Jamile, Julia Kim, Mahnouch Georget, Thirupathi Pattipaka, and Jilles M. Fermont. "P796: HOSPITALIZATION IN PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: A RETROSPECTIVE ANALYSIS OF OBSERVATIONAL STUDY DATA FROM THE UNITED STATES." HemaSphere 7 (August 2023): e22585a2. http://dx.doi.org/10.1097/01.hs9.0000970088.22585.a2.
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Bunn, H. Franklin, David G. Nathan, George J. Dover, et al. "Pulmonary hypertension and nitric oxide depletion in sickle cell disease." Blood 116, no. 5 (2010): 687–92. http://dx.doi.org/10.1182/blood-2010-02-268193.
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During the past decade a large body of experimental and clinical studies has focused on the hypothesis that nitric oxide (NO) depletion by plasma hemoglobin in the microcirculation plays a central role in the pathogenesis of many manifestations of sickle cell disease (SCD), particularly pulmonary hypertension. We have carefully examined those studies and believe that the conclusions drawn from them are not adequately supported by the data. We agree that NO depletion may well play a role in the pathophysiology of other hemolytic states such as paroxysmal nocturnal hemoglobinuria, in which plasma hemoglobin concentrations are often at least an order of magnitude greater than in SCD. Accordingly, we conclude that clinical trials in SCD designed to increase the bioavailability of NO or association studies in which SCD clinical manifestations are related to plasma hemoglobin via its surrogates should be viewed with caution.
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Xu, Yinfang, Yan Zhang, Ivan A. Lopez, et al. "Identification of a genetic variant underlying familial cases of recurrent benign paroxysmal positional vertigo." PLOS ONE 16, no. 5 (2021): e0251386. http://dx.doi.org/10.1371/journal.pone.0251386.
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Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigo in humans, yet the molecular etiology is currently unknown. Evidence suggests that genetic factors may play an important role in some cases of idiopathic BPPV, particularly in familial cases, but the responsible genetic variants have not been identified. In this study, we performed whole exome sequencing [including untranslated regions (UTRs)] of 12 families and Sanger sequencing of additional 30 families with recurrent BPPV in Caucasians from the United States (US) Midwest region, to identify the genetic variants responsible for heightened susceptibility to BPPV. Fifty non-BPPV families were included as controls. In silico and experimental analyses of candidate variants show that an insertion variant rs113784532 (frameshift causing truncation) in the neural cadherin gene PCDHGA10 (protocadherin-gamma A10) is an exceedingly strong candidate (p = 1.80x10-4 vs. sample controls; p = 5.85x10-19 vs. ExAC data; p = 4.9x10-3 vs. NHLBI exome data). The mutant protein forms large aggregates in BPPV samples even at young ages, and affected subjects carrying this variant have an earlier onset of the condition than those without [average 44.0±14.0 (n = 16) versus 54.4±16.1 (n = 36) years old, p = 0.054]. In both human and mouse inner ear tissues, PCDHGA10 is expressed in ganglia, hair cells and vestibular transitional epithelia. Fluorescent RNA in situ hybridization using mouse inner ear tissues shows that expression increases with age. In summary, our data show that a variant in the PCDHGA10 gene may be involved in causing or aggravating some familial cases of recurrent idiopathic BPPV.