Isolated Congenital Lipase—Colipase Deficiency

Fayez K. Ghishan; J. Roberto Moran; Peter R. Durie; Harry L. Greene

doi:10.1016/S0016-5085(84)80175-4

Isolated Congenital Lipase—Colipase Deficiency

Fayez K. Ghishan, J. Roberto Moran, Peter R. Durie, Harry L. Greene

Research output: Contribution to journal › Article › peer-review

46 Scopus citations

Abstract

A 5-yr-old child with isolated combined pancreatic lipase and colipase deficiency is described. The patient has a history of passing oily stools since birth. Pancreatic stimulation tests showed that both lipase and colipase activities were <2% of normal control values. Despite the total lack of both enzymes, the patient's fat absorption coefficient was 50%. Fat absorption coefficient increased to 82% with pancreatic enzyme supplementation. This is the first report of congenital combined lipase and colipase deficiency.

Original language	English (US)
Pages (from-to)	1580-1582
Number of pages	3
Journal	Gastroenterology
Volume	86
Issue number	6
DOIs	https://doi.org/10.1016/S0016-5085(84)80175-4
State	Published - 1984
Externally published	Yes

ASJC Scopus subject areas

Hepatology
Gastroenterology

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title = "Isolated Congenital Lipase—Colipase Deficiency",

abstract = "A 5-yr-old child with isolated combined pancreatic lipase and colipase deficiency is described. The patient has a history of passing oily stools since birth. Pancreatic stimulation tests showed that both lipase and colipase activities were <2% of normal control values. Despite the total lack of both enzymes, the patient's fat absorption coefficient was 50%. Fat absorption coefficient increased to 82% with pancreatic enzyme supplementation. This is the first report of congenital combined lipase and colipase deficiency.",

author = "Ghishan, {Fayez K.} and Moran, {J. Roberto} and Durie, {Peter R.} and Greene, {Harry L.}",

year = "1984",

doi = "10.1016/S0016-5085(84)80175-4",

language = "English (US)",

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pages = "1580--1582",

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TY - JOUR

T1 - Isolated Congenital Lipase—Colipase Deficiency

AU - Ghishan, Fayez K.

AU - Moran, J. Roberto

AU - Durie, Peter R.

AU - Greene, Harry L.

PY - 1984

Y1 - 1984

N2 - A 5-yr-old child with isolated combined pancreatic lipase and colipase deficiency is described. The patient has a history of passing oily stools since birth. Pancreatic stimulation tests showed that both lipase and colipase activities were <2% of normal control values. Despite the total lack of both enzymes, the patient's fat absorption coefficient was 50%. Fat absorption coefficient increased to 82% with pancreatic enzyme supplementation. This is the first report of congenital combined lipase and colipase deficiency.

AB - A 5-yr-old child with isolated combined pancreatic lipase and colipase deficiency is described. The patient has a history of passing oily stools since birth. Pancreatic stimulation tests showed that both lipase and colipase activities were <2% of normal control values. Despite the total lack of both enzymes, the patient's fat absorption coefficient was 50%. Fat absorption coefficient increased to 82% with pancreatic enzyme supplementation. This is the first report of congenital combined lipase and colipase deficiency.

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DO - 10.1016/S0016-5085(84)80175-4

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JO - Gastroenterology

JF - Gastroenterology

IS - 6

ER -

Isolated Congenital Lipase—Colipase Deficiency

Abstract

ASJC Scopus subject areas

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