Pediatric Hematology/Oncology and Immunopathology

Вопросы гематологии/онкологии и иммунопатологии в педиатрии

1726-17082414-9314

Fund Doctors, Innovations, Science for Children

638

10.24287/1726-1708-2022-21-3-56-64

ORIGINAL ARTICLES

ОРИГИНАЛЬНЫЕ СТАТЬИ

An autoinflammatory disease – PFAPA syndrome: a single-center experience

Аутовоспалительное заболевание – синдром PFAPA: опыт одного Центра

https://orcid.org/0000-0002-2869-6535

Kozlova

A. L.

Козлова

А. Л.

Russian Federation

Anna L. Kozlova, Cand. Med. Sci., a senior researcher at the Department of Treatment Optimization for Immunodeficiencies

1 Samory Mashela St., Moscow 117997, Russia

Козлова Анна Леонидовна, канд. мед. наук, старший научный сотрудник отдела оптимизации лечения иммунодефицитов

117997, Москва, ул. Саморы Машела, 1

annamax-99@mail.ru

https://orcid.org/0000-0002-0799-1025

Leontieva

M. E.

Леонтьева

М. Е.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0001-9275-4261

Velieva

P. T.

Велиева

П. Т.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-6839-3918

Kalinina

M. P.

Калинина

М. П.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-8208-2075

Deordieva

E. A.

Деордиева

Е. А.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0001-5347-7150

Shvets

O. A.

Швец

О. А.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-1669-8621

Kuzmenko

N. B.

Кузьменко

Н. Б.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-3305-1694

Mukhina

A. A.

Мухина

А. А.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-4451-3233

Grachev

N. S.

Грачев

Н. С.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-2322-5734

Novichkova

G. A.

Новичкова

Г. А.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

https://orcid.org/0000-0002-3113-4939

Shcherbina

A. Yu.

Щербина

А. Ю.

Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

Москва

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России

15102022

213

56642607202229082022

2022

«D. Rogachev NMRCPHOI»

ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России

https://creativecommons.org/licenses/by/4.0

https://hemoncim.com/jour/article/view/638

The aim of this study was to analyze the clinical and laboratory data of 101 patients (61 boys, 40 girls) diagnosed with PFAPA syndrome. The age of onset of PFAPA syndrome ranged from 8 to 36 months (the median age of onset was 18 months). The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. In most cases, clinical manifestations included recurrent fevers, acute tonsillitis (in 84% of cases, the tonsils were covered with a whitish coating), and cervical lymphadenitis. More rarely, the patients had diarrhea, vomiting, myalgia, arthralgia, and rash. All of the patients met the criteria proposed by G.S. Marshall. Our management strategies for patients with PFAPA syndrome include symptomatic treatment with antipyretics, short-term glucocorticosteroid therapy, and tonsillectomy. The majority of patients have complete resolution of PFAPA symptoms by the age of 7 regardless of the type of therapy.

Целью настоящей работы был анализ лабораторных и клинических данных 101 пациента (61 мальчик и 40 девочек) с диагнозом «синдром PFAPA/синдром Маршалла». Возраст манифестации синдрома PFAPA составил от 8 до 36 месяцев (медиана – 18 месяцев). Данное исследование одобрено независимым этическим комитетом и утверждено решением ученого совета НМИЦ ДГОИ им. Дмитрия Рогачева. В большинстве случаев клинические проявления включали рецидивирующие лихорадки, признаки острого тонзиллита, у 84% пациентов с налетом на миндалинах, шейным лимфаденитом. В меньшем количестве встречались такие клинические проявления, как диарея, рвота, миалгия, артралгия, сыпь. Все пациенты соответствовали критериям G.S. Marshall. Наш опыт ведения пациентов с синдромом PFAPA складывается из симптоматической терапии жаропонижающими препаратами, кратковременной терапии глюкокортикостероидами и тонзиллэктомии. В большинстве случаев к 7 годам наступает полное разрешение клинических проявлений синдрома PFAPA, независимо от варианта проводимой терапии.

autoinflammatory syndromePFAPAMarshall syndromerecurrent tonsillitisperiodic syndromechildren

аутовоспалительный синдромсиндром PFAPAсиндром Маршалларецидивирующий тонзиллитпериодический синдромдети

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