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[Familial Mediterranean fever in childhood (author's transl)]
Abstract:
Most cases of familial mediterranean fever (periodic disease) begin in childhood. However reports of this disorder in the pediatric literature are rare. This diagnosis is too often missed in pediatric practice. His symptomatology is the same as in adulthood with some particularities. Familial history is often the corner-stone of the diagnosis. Assessment of C'4 fraction of complement seems of good help for the diagnosis. The frequent occurrence of premonitory symptoms heralding the attacks allows us in many cases to start an intermittent colchicine therapy. Long term colchicine therapy should be used very cautiously in children and should be restricted to those children whose activities are disrupted by crisis occurring without alarm or to cases associated with amyloidosis.
Insights
Familial Mediterranean Fever (FMF) is often missed in children, despite symptoms appearing in childhood. Early diagnosis and cautious colchicine use are key for managing this periodic disease.
Area of Science:
- Pediatric Rheumatology
- Genetic Inflammatory Disorders
Context:
- Familial Mediterranean Fever (FMF) diagnosis is frequently delayed in pediatric practice.
- Pediatric FMF cases are underreported despite symptom onset in childhood.
Purpose:
- To highlight diagnostic challenges of FMF in children.
- To discuss diagnostic aids and therapeutic strategies for pediatric FMF.
Summary:
- FMF presents similarly in children and adults, with familial history being crucial for diagnosis.
- Complement C'4 fraction assessment aids in diagnosis.
- Intermittent colchicine therapy is recommended for FMF attacks without warning symptoms.
Impact:
- Improved recognition of FMF in pediatric patients.
- Guidelines for cautious long-term colchicine use in children with FMF.
- Potential reduction in FMF-associated complications like amyloidosis.