Familial chylomicronemia syndrome presenting with acute necrotizing pancreatitis in a five month infant

Borghei, A. and Azizi, M. (2010) Familial chylomicronemia syndrome presenting with acute necrotizing pancreatitis in a five month infant. Journal of Nepal Paediatric Society, 30 (2). pp. 110-112.

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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.

Item Type: Article
Additional Information: Cited By :3 Export Date: 16 February 2020 Correspondence Address: Borghei, A.; Department of Paediatrics, Imam Reza Hospital, Kermanshah, University of Medical Sciences, Kermanshah, Iran; email: amirmasoud56@gmail.com
Uncontrolled Keywords: Chylomicronemia Hyperlipidemia Lipoprotein lipase Pancreatitis chylomicron fat soluble vitamin fibrin fresh frozen plasma medium chain fatty acid omega 3 fatty acid triacylglycerol unclassified drug vitamin abdominal tenderness acute abdomen acute hemorrhagic pancreatitis acute pancreatitis article ascites case report consanguinity disease severity exudate familial chylomicronemia syndrome fever genetic disorder human hypercholesterolemia infant laboratory test laparotomy low fat diet male peritonitis physical examination restlessness triacylglycerol blood level ultrasound vital sign vomiting
Subjects: WK Endocrine System
WS Pediatrics
Divisions: Mashhad University of Medical Sciences
Depositing User: mr lib4 lib4
Date Deposited: 03 Mar 2020 05:23
Last Modified: 03 Mar 2020 05:23
URI: http://eprints.mums.ac.ir/id/eprint/12952

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