OBJECTIVE: @entity827 ( @entity827 ) is an @entity74 of childhood characterized by attacks of fever and @entity11534 . @entity12042 is the most important complication of the disease that determines the prognosis. METHODS: Forty-eight Turkish @entity827 @entity1 with @entity2783 who have been followed at the two hospitals in Ankara were included in this study. RESULTS: All @entity1 with @entity2783 had been symptomatic for @entity827 at the time of the diagnosis (Phenotype I), none had received regular @entity3812 therapy and all presented with @entity644 . Ten of them had asymptomatic @entity644 ; 38 had @entity1985 and 8 of them had @entity177 (CRI) as well, at the time of the diagnosis. Regular @entity3812 therapy was commenced to all of the @entity1 . At the end of observation period of 4.5 +/- 2.23 years (range 2-12 yrs) on treatment, @entity1985 resolved in 13 @entity1 and @entity644 was lost in 5 of them. None but 2 of the @entity1 who were diagnosed at proteinuric stage progressed to end @entity177 (ESRF). Seven @entity16498 mutations ( @entity16502 , @entity52014 , @entity51049 , @entity16500 , @entity70478 , @entity40706 , @entity16499 ) were systematically investigated in 32 @entity1 . Six of the seven studied mutations were found in these @entity1 and clinical diagnosis was confirmed by mutation analysis in 24 @entity1 . Eight @entity1 were found to have mutations on one of the alleles. CONCLUSION: @entity2783 is the most serious complication of @entity827 . @entity3812 treatment ameliorates the progression of @entity8 in the @entity1 who presented with @entity644 and even with @entity1985 . No correlation between the outcome of the @entity1 with @entity1985 and the degree of @entity644 and/or serum albumin levels at the initiation of treatment were noted. Progression to ESRF seems inevitable despite @entity3812 therapy after the development of CRI in @entity1 with @entity827 associated @entity2783 .

XXXX ( @entity827 )-associated @entity2783 in childhood. Clinical features, course and outcome.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity644 :: ('MESH:D011507', 'Disease') :: ['proteinuria'] @entity16498 :: ('4210', 'Gene') :: ['MEFV'] @entity12042 :: ('MESH:C538249', 'Disease') :: ['Renal amyloidosis'] @entity8 :: ('MESH:D007674', 'Disease') :: ['renal disease'] @entity827 :: ('MESH:D010505', 'Disease') :: ['Familial Mediterranean fever', 'FMF'] @entity2783 :: ('MESH:D000686', 'Disease') :: ['Amyloidosis', 'amyloidosis'] @entity16502 :: ('p|SUB|M|694|V', 'ProteinMutation') :: ['M694V'] @entity52014 :: ('p|SUB|M|680|I', 'ProteinMutation') :: ['M680I'] @entity16500 :: ('p|SUB|M|694|I', 'ProteinMutation') :: ['M694I'] @entity51049 :: ('p|SUB|V|726|A', 'ProteinMutation') :: ['V726A'] @entity177 :: ('MESH:D051437', 'Disease') :: ['stage renal failure', 'renal insufficiency'] @entity40706 :: ('p|SUB|R|761|H', 'ProteinMutation') :: ['R761H'] @entity11534 :: ('MESH:D012700', 'Disease') :: ['serositis'] @entity74 :: ('MESH:D030342', 'Disease') :: ['autosomal recessive disorder'] @entity70478 :: ('p|SUB|K|695|R', 'ProteinMutation') :: ['K695R'] @entity3812 :: ('MESH:D003078', 'Chemical') :: ['colchicine', 'Colchicine'] @entity1985 :: ('MESH:D009404', 'Disease') :: ['nephrotic syndrome'] @entity16499 :: ('p|SUB|E|148|Q', 'ProteinMutation') :: ['E148Q']
@entity827 :: (MESH:D010505,Disease) :: ['Familial Mediterranean fever']
