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Familial Mediterranean fever in Arab children: the high prevalence and gene frequency
1Department of Paediatrics, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Insights
Familial Mediterranean fever (FMF) is prevalent in Arab children, with a gene frequency of 1:50. Early symptoms include abdominal pain, pleurisy, and arthritis, with potential for renal failure and amyloidosis.
Area of Science:
- Pediatrics
- Genetics
- Rheumatology
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Understanding FMF prevalence and characteristics in diverse populations is crucial for early diagnosis and management.
Purpose of the Study:
- To prospectively investigate the prevalence, clinical manifestations, and genetic frequency of familial Mediterranean fever in Arab children.
- To compare findings with previously reported data in Jewish and American populations.
Main Methods:
- Prospective study of 192 children diagnosed with familial Mediterranean fever over 3 years.
- Data collection included demographics, age at onset, clinical symptoms, family history, and specific complications like renal failure and amyloidosis.
Main Results:
- Prevalence of FMF was 1:2600 children, with a gene frequency of 1:50.
- Common symptoms included recurrent abdominal pain (82%), pleurisy (43%), and arthritis (37%).
- A positive family history was noted in 62% of cases, with 19 members across 12 families experiencing renal failure and 7 confirmed amyloidosis cases.
Conclusions:
- Familial Mediterranean fever exhibits a high prevalence and gene frequency in Arab children.
- The clinical presentation and genetic burden are comparable to findings in Jewish and American populations, highlighting the need for awareness in this demographic.
Unlabelled:
Over a period of 3 years, 192 children with familial Mediterranean fever were prospectively studied. Of these, 106 (55%) were girls and 86 (45%) were boys. The prevalence was 1:2600 children with a gene frequency of 1:50. The age at onset ranged between 4 months and 16 years. Of these patients 24% started their illness below the age of 2 years and 88% were symptomatic before the age of 10 years: 82% had recurrent abdominal pain, 43% had pleurisy, 37% had arthritis, 15% had cutaneous manifestations, 12% had splenomegaly and 4% had hepatomegaly. The presenting symptoms were abdominal pain in 51%, unilateral chest pain in 23% and arthritis in 26%. The family history was positive in 62%. Of 12 affected families 19 members had/have renal failure and amyloidosis was confirmed in 7 patients.
Conclusions:
Our data show a high prevalence of familial Mediterranean fever and a high gene frequency in Arab children similar to that reported in Jews and Americans.
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