Publication: Familial Chylomicronemia Syndrome Presenting With Acute Necrotizing Pancreatitis in a Five Month Infant
Date
2010
Authors
Article Type
Case Report
Journal Title
Journal ISSN
ISSN 1990-7974 eISSN 1990-7982
Volume Title
Pages
Pages: 110 - 112
Publisher
Nepal Paediatric Society (JNPS)
Abstract
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.
Description
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
Identifier
https://doi.org/10.3126/jnps.v30i2.2431