Bile salt kinetics in cystic fibrosis: influence of pancreatic enzyme replacement

Journal Article · Sat Jan 01 04:00:00 EST 1977 · Gastroenterology; (United States)

OSTI ID:6803611

Watkins, J B; Tercyak, A M; Szczepanik, P; Klein, P D

Bile acid kinetics was investigated by stable isotope dilution technique in 6 children (ages 3/sup 1///sub 2/ months to 4/sup 1///sub 2/ years) with previously untreated cystic fibrosis. All of the patients had clinical and laboratory evidence of malabsorption, normal intestinal mucosal function, as judged by glucose absorption, intestinal histology, disaccharidase levels, and normally functioning gallbladders. The children were maintained on a constant diet throughout the study period; fat intake averaged 4.2 g per kg per day. Before administration of pancreatic enzyme replacement, fat excretion equalled 50 +- 4% (mean +- SE) of intake and was reduced to 20 +- 1.0% of intake after therapy. Total bile acid pool size nearly doubled during enzyme replacement from 379 +- 32 ..mu..moles per kg to 620 +- 36 ..mu..moles per kg with secondary bile acids comprising 57% of the total pool before therapy and 40% after therapy. The data indicate that both primary and secondary bile acids are conserved within the enterohepatic circulation during enzyme therapy, and that the mechanism for the regulation of hepatic bile acid synthesis is intact in cystic fibrosis. However, the demonstration that large amounts of bile acid continue to be excreted during therapy suggests that interruption of the enterohepatic circulation continues and that deficiencies of the intraluminal phase may persist during enzyme therapy in this disease.

• Research Organization: Children's Hospital Medical Center, Boston
• OSTI ID: 6803611
• Journal Information: Gastroenterology; (United States), Journal Name: Gastroenterology; (United States) Vol. 73:5; ISSN GASTA
• Country of Publication: United States
• Language: English

Related Subjects

550200* -- Biochemistry
59 BASIC BIOLOGICAL SCIENCES
ANIMALS
BILE ACIDS
BIOCHEMICAL REACTION KINETICS
BIOSYNTHESIS
BODY
CARBOXYLIC ACIDS
CHILDREN
CLEARANCE
DIGESTIVE SYSTEM
DISEASES
ENDOCRINE GLANDS
ENZYMES
EXCRETION
FIBROSIS
GLANDS
HYDROXY COMPOUNDS
KINETICS
MAMMALS
MAN
METABOLISM
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANS
PANCREAS
PATHOLOGICAL CHANGES
PRIMATES
REACTION KINETICS
STEROIDS
STEROLS
SYNTHESIS
VERTEBRATES