Isolated Congenital Lipase—Colipase Deficiency
1984; Elsevier BV; Volume: 86; Issue: 6 Linguagem: Inglês
10.1016/s0016-5085(84)80175-4
ISSN1528-0012
AutoresFayez K. Ghishan, J. Roberto Moran, Peter R. Durie, Harry L. Greene,
Tópico(s)Diabetes and associated disorders
ResumoA 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 less than 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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