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Amyloidosis in children with familial Mediterranean fever
1Department of Pediatrics, Ondokuz Mayis University Faculty of Medicine, Samsun.
Insights
Colchicine effectively treats secondary amyloidosis in children with familial Mediterranean fever, relieving symptoms and improving protein levels. This study highlights colchicine
Area of Science:
- Pediatrics
- Rheumatology
- Genetics
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Secondary amyloidosis is a serious complication of FMF, particularly in children.
- Understanding the characteristics and treatment outcomes of FMF-associated amyloidosis is crucial.
Purpose of the Study:
- To review the clinical characteristics of children with secondary amyloidosis due to FMF.
- To evaluate the efficacy of colchicine in managing FMF-associated amyloidosis in pediatric patients.
- To identify key laboratory findings and patient demographics.
Main Methods:
- Retrospective review of 113 children diagnosed with secondary amyloidosis and FMF.
- Analysis of patient histories, physical examinations, and laboratory results.
- Assessment of treatment outcomes with colchicine, including symptom relief and biochemical markers.
Main Results:
- A considerable number of children presented with FMF-related amyloidosis, with a 4:3 male-to-female ratio.
- Amyloidosis incidence increased during adolescence, with 74.33% exhibiting phenotype I.
- Colchicine relieved periodic attack symptoms and significantly improved serum total protein and albumin levels, with 84.95% showing increased partial thromboplastin time.
Conclusions:
- Colchicine demonstrates significant benefits in managing secondary amyloidosis in children with FMF.
- Early diagnosis and consistent colchicine treatment are vital for improving patient outcomes.
- Further research into FMF amyloidosis phenotypes and long-term effects is warranted.
Abstract:
In this survey 113 children with secondary amyloidosis due to familial Mediterranean fever are reviewed in regard to their respective histories, and physical and laboratory findings. The beneficial effects of colchicine in the treatment of this condition are evaluated. The number of children presented with amyloidosis secondary to familial Mediterranean fever was considerable. The male-female ratio was 4/3. It was observed that the number of patients with amyloidosis increased through the adolescent period, and that most of the cases demonstrated phenotype I (74.33%). Another important finding was the increase of partial thromboplastin time in 96 out of 113 cases (84.95%). All the symptoms of the periodic attacks were relieved by colchicine. A significant difference was found between the serum total protein and albumin values before and after colchicine therapy.