СИНДРОМ PFAPA
نویسندگان
چکیده
Як свідчать дані літератури, синдром РFАРА – періодична гарячка з тонзилофарингітом, афтозним стоматитом і шийним лімфаденітом вперше описали вчені G.S. Marshall et al. у 1987 р. Цей є поширеним автозапальним розладом дітей, але трапляється в дорослих, багатьох частинах світу. Його етіологія патогенез ще не з’ясовані. Триває пошук удосконалення діагностичних критеріїв.
 На сьогодні використовують стандартизовані схеми лікування хворих: 1) нестероїдні протизапальні препарати, 2) ГКС-абортивна терапія, 3) колхіцин або циметидин профілактична 4) тонзилектомія хірургічний метод. Однак трапляються рецидиви захворювання.
منابع مشابه
PW03-009 – Genetics of PFAPA syndrome
Introduction Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an autoinflammatory disorder of childhood and little is known about the underlying etiology. While mutations involving the IL-1 pathway have been identified in other recurrent fever disorders, including TNF-receptor associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)...
متن کاملPW03-003 – Altered neutrophil function in PFAPA
Introduction The PFAPA syndrome is a non-mendelian autoinflammatory disease of unknown aetiology characterized by Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis. In typical cases, attacks begin before the age of five and occur every 2-8 weeks, often with striking periodicity. We have previously profiled the blood cells and serum cytokine levels in a cohort study of pati...
متن کاملPFAPA syndrome: with regard to a case.
BACKGROUND PFAPA syndrome (Periodic Fever, Aphtas, Pharyngitis and cervical Adenopathies) is one of the causes of periodic fever in pediatrics and it is characterised by high fever, pharyngitis, cervical adenitis and aphtous stomatitis. Its etiopathogeny is unknown. The diagnosis is clinical and the findings of laboratory are unspecified. One or two doses (1 mg/kg) of oral prednisone are enough...
متن کاملPW03-001 - PFAPA syndrome in Turkish children
Results The median age of onset was 12 months, with a maleto-female ratio of 1,6:1. The length of fever attacks ranged between 2-8 days, and symptom-free intervals between 10-45 days. The most common associated symptoms were pharyngitis (100%), abdominal pain (45,1%), aphthous stomatitis (43,7%), and cervical lymphadenitis (32,4%). During fever flares, the leukocyte count and acute phase reacta...
متن کاملP03-001 - PFAPA and MEFV genes
Introduction Marshall Syndrome or PFAPA is an inflammatory periodic disease characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Restless, headache, abdominal pain, vomiting, hepatosplenomegaly and arthralgia are less common symptoms seeing in this disease. The diagnosis is established on the basis of clinical criteria that require the presence of a recurrent...
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ژورنال
عنوان ژورنال: Ìnfekcìjnì hvorobi
سال: 2023
ISSN: ['1681-2727', '2414-9969']
DOI: https://doi.org/10.11603/1681-2727.2022.3.13487