Recurrent acute and chronic pancreatitis in two brothers with familial chylomicronemia syndrome

Truninger, Kaspar; Schmid, Peter A; Hoffmann, Michael M; Bertschinger, Philipp; Ammann, Rudolf W (2006). Recurrent acute and chronic pancreatitis in two brothers with familial chylomicronemia syndrome. Pancreas, 32(2), pp. 215-9. Hagerstown, Md.: Lippincott Williams & Wilkins 10.1097/01.mpa.0000202942.93578.dd

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The chylomicronemia syndrome is well recognized as a rare etiologic factor of acute pancreatitis; however, whether hypertriglyceridemia can cause chronic pancreatitis (CP) remains unclear. We describe the long-time course of 2 brothers with the familial chylomicronemia syndrome caused by identical compound heterozygous mutations in the lipoprotein lipase (LPL) gene with markedly reduced LPL activity. Other etiologic factors were excluded, including mutations in the PRSS1, SPINK1, and CFTR gene. Although both brothers had recurrent acute pancreatitis and the same LPL genotype, CP became evident in only one patient. Progression to CP was associated with a more severe disease course. Thus, the chylomicronemia syndrome may cause CP in the absence of other known causative factors, and similar to alcoholic and hereditary CP, a more severe disease course is associated with disease progression.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Gastroenterology
UniBE Contributor:	Truninger, Kaspar
ISSN:	0885-3177
ISBN:	16552344
Publisher:	Lippincott Williams & Wilkins
Language:	English
Submitter:	Factscience Import
Date Deposited:	04 Oct 2013 14:47
Last Modified:	05 Dec 2022 14:14
Publisher DOI:	10.1097/01.mpa.0000202942.93578.dd
PubMed ID:	16552344
Web of Science ID:	000241710200015
URI:	https://boris.unibe.ch/id/eprint/19771 (FactScience: 2711)