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Liver involvement in children with Familial Mediterranean fever

Fatih Unal1, Murat Cakir, Masallah Baran

  • 1Department of Pediatric Gastroenterology, Hepatology and Nutrition, Dörtçelik Pediatric Hospital, Bursa, Turkey.

Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
|February 16, 2012
PubMed
Abstract

Insights

Familial Mediterranean fever (FMF) can affect the liver in children, causing conditions like hepatitis and cirrhosis. Early recognition and colchicine treatment are crucial for managing FMF-related liver involvement.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Rheumatology

Background:

  • Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent febrile attacks.
  • Chronic inflammation in FMF, driven by cytokines, can lead to liver injury, including hepatitis and cirrhosis.
  • The role of liver involvement in pediatric FMF cases requires further investigation.

Purpose of the Study:

  • To investigate the prevalence and characteristics of liver involvement in children diagnosed with Familial Mediterranean fever.
  • To identify potential risk factors or genetic associations with liver disease in pediatric FMF patients.

Main Methods:

  • A cohort of 58 pediatric patients with Familial Mediterranean fever was studied.
  • Detailed examination of patients with confirmed liver involvement.
  • Analysis of demographic factors, laboratory findings, and genetic mutations (M694V).

Main Results:

  • Liver involvement was observed in 18.9% (11/58) of pediatric FMF patients.
  • Diagnoses included Budd-Chiari syndrome, chronic hepatitis/cirrhosis, and acute hepatitis.
  • Colchicine treatment led to clinical and laboratory improvement in all affected patients.

Conclusions:

  • Familial Mediterranean fever should be considered in pediatric cases of unexplained hepatitis or cirrhosis, particularly in endemic areas.
  • Pediatric hepatologists play a key role in diagnosing and managing FMF-associated liver disease.
  • Effective management with colchicine highlights the importance of early FMF diagnosis.

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