The first part of the article describes a rare case of the onset and course of juvenile dermatomyositis. In the second part the comparative clinical analysis of results of own supervision with other similar reports is carried out. The main attention is focused on atypical variants of the onset of the disease, in particular signs of severe intoxication syndrome, hemorrhagic-necrotic rash, anasarca, lesions of the oral cavity in the form of stomatitis and ulcerative-necrotic glossitis. The difficulties of early diagnosis of the disease on the background of delayed manifestation of pathognomonic skin signs and the absence of reliable features of myopathic syndrome are shown. Clinical and laboratory characteristics of amyopathic juvenile dermatomyositis with persistent skin lesions have been defined. There are three differentiated plans-recommendations of the Childhood Arthritis and Rheumatology Research Alliance for the treatment of patients who have never had muscle damage and those who have had clinical manifestations of myopathy only in the first months of the disease. Current trends in achievements and gaps in the classification, diagnosis and treatment of various forms of juvenile dermatomyositis have been observed. On the basis of scientific publications the possible prognostic consequences of the amyopathic form of this disease in children are highlighted. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: juvenile dermatomyositis, juvenile amyopathic dermatomyositis, atypical course, ulcerative necrotic glossitis, anasarca, psoriasis, treatment.
В історичній послідовності викладено основні етапи розвитку дитячої ревматології з особливим ухилом на передумови виникнення терміну «ювенільний ревматоїдний артрит». Зазначено причини виокремлення цього захворювання з аналогічної патології суглобів у дорослих. Показано еволюцію номенклатури ювенільного ревматоїдного артриту, починаючи з 1946 року і до наших днів. Окремо розглядаються причини введення терміну «ювенільний ідіопатичний артрит». Викладено класифікаційні та термінологічні розбіжності щодо патології у різних країнах світу. Показано внесок визначних вчених та міжнародних організацій у вивчення цієї проблеми. Звертається увага на проблемні питання трактування номенклатури хронічних захворювань суглобів дітей в Україні і світі. Зроблено припущення, що вже найближчим часом буде запропоновано нову номенклатурну та класифікаційну платформу ювенільних артритів. Автори заявляють про відсутність конфлікту інтересів. Ключові слова: діти, ревматологія, ювенільний ревматоїдний артрит, ювенільний ідіопатичний артрит.
The article provides basic information about the syndrome of prolonged QT interval, which is a rare pathology of the cardiovascular system and can cause sudden death. The mutations in the genes responsible for dysfunction of ion channels are the key in the pathogenesis of this disease. Jerwell and Lange—Nielsen, Romano—Ward, Andersen—Tavil and Timothy syndromes are described as the main hereditary variants of this syndrome. A own case of the disease in a patient was given. The peculiarity of this case was on the acute, atypical onset of the disease, which started with convulsions and sudden cardiac death. The clinical picture of this case, features of a current are described, indicators of the basic methods of research are resulted. Information on the treatment of this clinical case at the local level and in the department of surgical treatment of arrhythmias, where the patient underwent implantation of endocardial bicameral cardioverter-defibrillator, іs presented. Data on further observation and treatment of the child at the place of residence are given. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: syndrome, QT interval, etiology, pathogenesis, clinical picture, diagnosis, examination, treatment.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.