Familial Chylomicronemia Syndrome Presenting With Acute Necrotizing Pancreatitis in a Five Month Infant.

Familial chylomicronemia syndrome (FCS) is a rare disease characterized by severe fasting hypertriglyceridemia and chylomicronemia, which is inherited in an autosomal recessive manner. It is arisen from apolipoprotein C-ll deficiency or Lipoprotein Lipase(LPL) Deficiency.We report a 5-month-old male infant FCS presenting with acute abdominal pain and post surgical diagnosis of acute necrotizing pancreatitis.