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Decrease in the rate of secondary amyloidosis in Turkish children with FMF: are we doing better?
Victoria Akse-Onal1, Erdal Sağ, Seza Ozen
1Department of Pediatrics, Hacettepe University, Ankara 06100, Turkey.
European Journal of Pediatrics
|February 25, 2010
Summary
Secondary amyloidosis, a complication of Familial Mediterranean Fever (FMF), significantly decreased in Turkey. Improved FMF treatment and potentially environmental factors contributed to this decline.
Area of Science:
- Rheumatology
- Nephrology
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a prevalent autoinflammatory disorder.
- Secondary amyloidosis is a severe complication of FMF, influenced by genetic and environmental factors.
Purpose of the Study:
- To assess the change in secondary amyloidosis rates in Turkey over time.
- To identify potential factors contributing to observed changes in amyloidosis incidence.
Main Methods:
- Comparative analysis of clinical features of FMF patients with secondary amyloidosis.
- Data collected from two distinct periods: 1978-1990 and 2000-2009.
- Utilized a modified scoring system for assessing disease severity in pediatric patients.
Main Results:
- A significant reduction in secondary amyloidosis was observed, decreasing from 12.1% (1978-1990) to 2% (2000-2009) of renal biopsies.
- No significant differences in patient demographics (gender, age, onset, duration, severity) were found between the two groups.
- The incidence of secondary amyloidosis among FMF patients in Turkey has markedly decreased.
Conclusions:
- Improved FMF management, including increased awareness and treatment, is the primary driver for reduced secondary amyloidosis.
- Potential positive impact of an improved infectious environment on this monogenic autoinflammatory disease warrants further investigation.
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