Turkish Journal of Gastroenterology
Case Report

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

1.

Department of Pediatric Gastroenterology, Akdeniz University Faculty of Medicine, Antalya, Turkey

2.

Department of Pediatric Neurology, Akdeniz University Faculty of Medicine, Antalya, Turkey

Turk J Gastroenterol 2014; 25: 216-219
DOI: 10.5152/tjg.2014.3667
Read: 497 Downloads: 89 Published: 25 July 2019

Abstract

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.

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