A very rare cause of acute pancreatitis: Berardinelli-Seip congenital lipodystrophy

Islek A. , Sayar E., YILMAZ A., DUMAN Ö., ARTAN R.

TURKISH JOURNAL OF GASTROENTEROLOGY, cilt.25, ss.216-219, 2014 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 25
  • Basım Tarihi: 2014
  • Doi Numarası: 10.5152/tjg.2014.3667
  • Sayfa Sayıları: ss.216-219


Pancreatitis is among rare diseases in pediatrics clinics. It is usually presented with a sign of underlying systemic disease. Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare disease characterized by near absence of adipose tissue resulting in apparent muscle hypertrophy from birth or early infancy associated with severe insulin resistance. Common clinical features are hypertriglyceridemia, acanthosis nigricans, hepatomegaly with or without splenomegaly and high stature. Acromegaloid features, cardiomyopathy and mental retardation can also be present. We describe a 7-year-old Turkish boy with these clinical features of BSCL and presented with acute pancreatitis.