Familial Mediterranean fever

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Prevalence of clinical parameters (%)

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Pubmed id number as a reference Organ system affected
Number of patients in the reference Percent affected patients (Between 0 and 1, eg. 0.1 = 10%)
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List of symptoms

Symptom/sign Organ system Percent affected Pubmed id Added on(yyyy-mm-dd) Edit/add reference
Abdominal pain digestive 90 % 9266193 2012-01-10
Fever multi 81 % 9266193 2012-01-10
Arthralgia skeletal 41 % 9266193 2012-01-10
Amyloidosis multi 30 % 9266193 2012-01-10
Arthritis skeletal 21 % 9266193 2012-01-10
Chest pain respiratory 14 % 9266193 2012-01-10
Hepatomegaly digestive 14 % 9266193 2012-01-10
Splenomegaly circulatory 8 % 9266193 2012-01-10

List of references:

Familial Mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis.
U Saatçi, S Ozen, S Ozdemir, A Bakkaloglu, N Besbas, R Topaloglu, S Arslan,

Familial Mediterranean fever (FMF) is a genetically transmitted disease characterized by recurrent attacks of fever and serositis. The most important complication of this disease is the development of amyloidosis. We present our analysis of 425 FMF patients without and 180 with amyloidosis (123 FMF having amyloidosis type 1 and 57 FMF having amyloidosis type II). The male female ratio was higher in the amyloidosis population (111/69) when compared to the FMF population (225 200) (P = 0.048). Consanguinity rate was the same among FMF and amyloidosis groups. However, a family history of amyloidosis was significantly more frequent in the amyloidosis group (P = 0.00001). Multivariate analysis has revealed that in FMF patients, the presence of a family history of amyloidosis plus consanguinity has a 6.04 fold increased risk of amyloidosis (P < 0.0001). The 5-year chronic renal failure free survival was 43.1% and 18.7% in type I and type II amyloidosis, respectively. The time interval to develop chronic renal failure after the development of amyloidosis was 4.8 in type I and 3.0 years in type II. respectively. We found ten cases of Henoch-Schönlein Purpura and nine of polyarteritis nodosa among our patients. The significance of the association between FMF and vasculitis awaits to be clarified. Among the FMF patients put on colchicine therapy (435), only 10 (2.3%) have developed amyloidosis confirming that this drug protects from amyloidosis.

European journal of pediatrics - Aug 1997