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

  • Amirmasoud Borghei Resident in Paediatrics, Department of Paediatrics, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah
  • Mahba Azizi Department of Paediatrics, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah
Keywords: Pancreatitis, chylomicronemia, hyperlipidemia, lipoprotein lipase

Abstract

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.

Key words: Pancreatitis; chylomicronemia; hyperlipidemia; lipoprotein lipase.

DOI: 10.3126/jnps.v30i2.2431

J. Nepal Paediatr. Soc.
May-August, 2010 Vol 30(2) 110-112

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Abstract
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Published
2010-07-13
How to Cite
Borghei, A., & Azizi, M. (2010). Familial Chylomicronemia Syndrome Presenting With Acute Necrotizing Pancreatitis in a Five Month Infant. Journal of Nepal Paediatric Society, 30(2), 110-112. https://doi.org/10.3126/jnps.v30i2.2431
Section
Brief Reports/Case Reports/Case Series