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Amyloidosis in children with familial Mediterranean fever
Abstract:
The clinical and laboratory findings of 35 children with familial Mediterranean fever who developed amyloidosis are described. The types, frequency, and severity of attacks of familial Mediterranean fever in these children were no different from patients with this disease without amyloidosis. Although amyloid was widely deposited in all tissues, the major clinical manifestations of the amyloidosis were proteinuria, the nephrotic syndrome, and progressive renal failure. Only 20% of the patients were alive 5 years after the first appearance of proteinuria.
Insights
Familial Mediterranean fever attacks did not predict amyloidosis in children. Amyloidosis primarily caused kidney failure, with a poor 5-year survival rate after proteinuria onset in these pediatric patients.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Genetics
Background:
- Familial Mediterranean fever (FMF) is an inherited autoinflammatory disorder.
- Amyloidosis, a complication of chronic inflammation, can affect FMF patients.
- The clinical course of FMF-associated amyloidosis in children requires further elucidation.
Purpose of the Study:
- To describe the clinical and laboratory findings of pediatric FMF patients who developed amyloidosis.
- To correlate FMF attack characteristics with amyloidosis development.
- To assess the clinical manifestations and prognosis of childhood FMF-associated amyloidosis.
Main Methods:
- Retrospective analysis of clinical and laboratory data from 35 children with FMF and amyloidosis.
- Comparison of FMF attack patterns between patients with and without amyloidosis.
- Evaluation of tissue deposition and clinical outcomes related to amyloidosis.
Main Results:
- No significant difference in FMF attack type, frequency, or severity was observed between children with and without amyloidosis.
- Amyloid deposition was widespread across tissues, but renal involvement was predominant.
- Major clinical manifestations included proteinuria, nephrotic syndrome, and progressive renal failure.
Conclusions:
- Amyloidosis in pediatric FMF is characterized by significant renal pathology.
- The onset of proteinuria in FMF-associated amyloidosis portends a poor prognosis, with a 5-year survival rate of only 20% in this cohort.
- Early recognition and management strategies for FMF-associated renal amyloidosis are critical.