Scopus
YÖKSİS Eşleşti
Evaluation of the international severity score for FMF (ISSF) scores in Turkish children diagnosed with FMF: a single-center experience
Clinical Rheumatology · Ağustos 2021
YÖKSİS Kayıtları
Evaluation of the international severity score for FMF (ISSF) scores in Turkish children diagnosed with FMF: a single-center experience
Clinical Rheumatology · 2021 SCI-Expanded
DOKTOR ÖĞRETİM ÜYESİ FATİH MEHMET AKİF ÖZDEMİR →
Makale Bilgileri
DergiClinical Rheumatology
Yayın TarihiAğustos 2021
Cilt / Sayfa40 · 3219-3225
Scopus ID2-s2.0-85101259325
Özet
Aims: The aim of this study is to evaluate our patients with the newly developed international severity score for FMF (ISSF) and make comparisons with the literature. Methods: This cross-sectional study included patients with FMF, were between 6 months and 18 years old, and were using colchicine/colchicine+IL-1 inhibitor for at least 6 months. The patients were classified as mild, intermediate, and severe based on their scores between 1 and 10. In addition to scoring, those who have additional pathological or silent mutation were compared based on these classifications. Results: Our patients consist of 88 women 72 men total 160. The mean age, age of onset, and age of diagnosis were 12 ± 4.3, 4.9 ± 3.7, and 7.5 ± 4 years, respectively, and the mean diagnostic delay was 2.6 ± 3years. When our large patient population is evaluated with ISSF, the disease severity is mostly intermediate. According to ISSF, mild, intermediate, and severe diseases were 21.3%, 70.5%, and 8.1%, respectively. The duration of attacks shorten with increasing age (p > 0.05), and there is an increase in the involvement of other organs during the attacks among patients older than 7 years. Conclusions: ISSF appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients.Key Points•Our article is the first study to evaluate ISSF performance in the pediatric population.•International severity score for FMF (ISSF) appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients.•We think that the addition of pathogenic mutations and inheritance model to the ISSF scoring system as important determinants of disease severity, especially when comparing different patients, can be evaluated in future studies.
Yazarlar (3)
1
Fatih Mehmet Akif Özdemir
ORCID: 0000-0003-4820-1234
2
Nesrin Gülez
ORCID: 0000-0002-3343-6949
3
Balahan Makay
ORCID: 0000-0001-6193-0402
Anahtar Kelimeler
Disease severity
FMF
ISSF
Kurumlar
Dr. Behçet Uz Children Research and Training Hospital
Izmir Turkey
University of Health Scienes Dr. Behcet Uz Children’s Education and Research Hospital
Konak/Izmir Turkey
Metrikler
7
Atıf
3
Yazar
3
Anahtar Kelime