Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment

Familial Mediterranean fever is a typical monogenic disease with an autosomal recessive inheritance pattern; caused by mutations in the MEFV gene, which encodes the pyrin protein. It is a relatively rare pathology in the practice of paediatricians and rheumatologists of the Russian Federation. The a...

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Main Authors: Mikhail M. Kostik, Olga V. Zhogova, Natalia V. Lagunova, Sergey V. Ivanovskiy, Oksana L. Kolobova, Larisa N. Melnikova
Format: Article
Language:English
Published: "Paediatrician" Publishers LLC 2018-12-01
Series:Вопросы современной педиатрии
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Online Access:https://vsp.spr-journal.ru/jour/article/view/1953
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author Mikhail M. Kostik
Olga V. Zhogova
Natalia V. Lagunova
Sergey V. Ivanovskiy
Oksana L. Kolobova
Larisa N. Melnikova
author_facet Mikhail M. Kostik
Olga V. Zhogova
Natalia V. Lagunova
Sergey V. Ivanovskiy
Oksana L. Kolobova
Larisa N. Melnikova
author_sort Mikhail M. Kostik
collection DOAJ
description Familial Mediterranean fever is a typical monogenic disease with an autosomal recessive inheritance pattern; caused by mutations in the MEFV gene, which encodes the pyrin protein. It is a relatively rare pathology in the practice of paediatricians and rheumatologists of the Russian Federation. The article provides up-to-date data on the disease prevalence, presents a complete clinical picture of the auto-inflammatory  syndrome, discusses diagnostic criteria and methods for treating patients with familial Mediterranean fever.
format Article
id doaj-art-8decaa6a1006450cbc7f1f3250ef1d47
institution Kabale University
issn 1682-5527
1682-5535
language English
publishDate 2018-12-01
publisher "Paediatrician" Publishers LLC
record_format Article
series Вопросы современной педиатрии
spelling doaj-art-8decaa6a1006450cbc7f1f3250ef1d472025-08-20T03:59:53Zeng"Paediatrician" Publishers LLCВопросы современной педиатрии1682-55271682-55352018-12-0117537138010.15690/vsp.v17i5.19531780Familial Mediterranean Fever: Current Approaches to Diagnosis and TreatmentMikhail M. Kostik0Olga V. Zhogova1Natalia V. Lagunova2Sergey V. Ivanovskiy3Oksana L. Kolobova4Larisa N. Melnikova5St. Petersburg State Pediatric Medical UniversityV.I. Vernadskiy Crimean Federal University; Republican Pediatric Clinical HospitalV.I. Vernadskiy Crimean Federal UniversityV.I. Vernadskiy Crimean Federal University; Republican Pediatric Clinical HospitalSt. Petersburg State Pediatric Medical UniversitySt. Petersburg State Pediatric Medical UniversityFamilial Mediterranean fever is a typical monogenic disease with an autosomal recessive inheritance pattern; caused by mutations in the MEFV gene, which encodes the pyrin protein. It is a relatively rare pathology in the practice of paediatricians and rheumatologists of the Russian Federation. The article provides up-to-date data on the disease prevalence, presents a complete clinical picture of the auto-inflammatory  syndrome, discusses diagnostic criteria and methods for treating patients with familial Mediterranean fever.https://vsp.spr-journal.ru/jour/article/view/1953familial mediterranean feverperiodic feverfever of unknown origininterleukin 1colchicineanakinracanakinumab
spellingShingle Mikhail M. Kostik
Olga V. Zhogova
Natalia V. Lagunova
Sergey V. Ivanovskiy
Oksana L. Kolobova
Larisa N. Melnikova
Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
Вопросы современной педиатрии
familial mediterranean fever
periodic fever
fever of unknown origin
interleukin 1
colchicine
anakinra
canakinumab
title Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
title_full Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
title_fullStr Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
title_full_unstemmed Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
title_short Familial Mediterranean Fever: Current Approaches to Diagnosis and Treatment
title_sort familial mediterranean fever current approaches to diagnosis and treatment
topic familial mediterranean fever
periodic fever
fever of unknown origin
interleukin 1
colchicine
anakinra
canakinumab
url https://vsp.spr-journal.ru/jour/article/view/1953
work_keys_str_mv AT mikhailmkostik familialmediterraneanfevercurrentapproachestodiagnosisandtreatment
AT olgavzhogova familialmediterraneanfevercurrentapproachestodiagnosisandtreatment
AT nataliavlagunova familialmediterraneanfevercurrentapproachestodiagnosisandtreatment
AT sergeyvivanovskiy familialmediterraneanfevercurrentapproachestodiagnosisandtreatment
AT oksanalkolobova familialmediterraneanfevercurrentapproachestodiagnosisandtreatment
AT larisanmelnikova familialmediterraneanfevercurrentapproachestodiagnosisandtreatment