Evidence map›Paper›PMID 33619627›Full record

ArticleClinical rheumatology2021

Evaluation of the international severity score for FMF (ISSF) scores in Turkish children diagnosed with FMF: a single-center experience.

Fatih Mehmet Akif Özdemir, Nesrin Gülez, Balahan Makay

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Article in Clinical rheumatology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.6field-weighted citation impact, top 36% of its field
1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed, 10 citations in OpenAlex.

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4 · The record

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5 · Who and what money

Authors and funding

3 authors at 1 institution in 1 country.

Fatih Mehmet Akif ÖzdemirDepartment of Pediatrics, University of Health Scienes Dr. Behcet Uz Children's Education and Research Hospital, İsmet Kaptan Neighborhood, Sezer Doğan Street, 11, 35210, Konak/Izmir, Turkey. fatihmehmetakif@hotmail.com.ORCID http://orcid.org/0000-0003-4820-1234
Nesrin GülezDepartment of Pediatric Immunology and Allergy, University of Health Scienes Dr. Behcet Uz Children's Education and Research Hospital, Izmir, Turkey.ORCID https://orcid.org/0000-0002-3343-6949
Balahan MakayDepartment of Pediatric Rheumatology, University of Health Scienes Dr. Behcet Uz Children's Education and Research Hospital, Izmir, Turkey.ORCID https://orcid.org/0000-0001-6193-0402
Dr. Behçet Uz Çocuk Hastalıkları Hastanesi · TR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

aimsThe 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.

methodsThis 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.

resultsOur 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.

conclusionsISSF 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.

Indexed as

Delayed DiagnosisFamilial Mediterranean FeverAdolescentChildColchicineCross-Sectional StudiesFemaleHumansMaleMutationSeverity of Illness IndexColchicineDisease severityFMFISSF

Identifiers

PMID33619627
OpenAlexW3133345668

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.