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Familial Mediterranean fever in children: the expanded clinical profile

H A Majeed1, M Rawashdeh, H el-Shanti

  • 1Department of Pediatrics, Faculty of Medicine, University of Jordan, Amman, Jordan. pal@go.com.jo

QJM : Monthly Journal of the Association of Physicians
|January 5, 2000
PubMed

Insights

Familial Mediterranean fever (FMF) presents with a wider range of symptoms than previously recognized, including significant gastrointestinal and joint issues. This study highlights a familial tendency towards kidney problems in FMF patients.

Area of Science:

  • Pediatrics
  • Genetics
  • Rheumatology

Background:

  • Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
  • The clinical spectrum of FMF has been observed to expand over recent years.

Purpose of the Study:

  • To investigate the expanded clinical profile of FMF in a large cohort of pediatric patients.
  • To identify familial predispositions to specific complications, particularly nephropathy.

Main Methods:

  • Retrospective analysis of clinical data from 476 children with FMF over an 8-year period.
  • Detailed documentation of presenting symptoms, complications, and family history.

Main Results:

  • The study identified a broad range of clinical manifestations including abdominal pain (81%), chest pain (41%), and arthritis (42%).
  • Renal complications were observed in 0.4% of children, with a notable familial predisposition to nephropathy in 36 family members.
  • Other reported symptoms included myalgia, skin manifestations, scrotal swelling, and recurrent fever.

Conclusions:

  • FMF exhibits a more diverse clinical presentation in children than previously documented.
  • A familial predisposition to nephropathy associated with FMF warrants further investigation.
  • This research provides valuable data for genotype-phenotype correlation studies in FMF, particularly within the Arab ethnic group.

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