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Urinary excretion of bile alcohols in normal children and patients with α 1 ‐antitrypsin deficiency during development of liver disease
Author(s) -
KARLAGANIS G.,
NEMETH A.,
HAMMARSKJÖLD B.,
STRANDVIK B.,
SJÖVALL J.
Publication year - 1982
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.1982.tb00687.x
Subject(s) - urine , medicine , cholestasis , excretion , cirrhosis , urinary system , liver disease , neonatal cholestasis , gastroenterology , endocrinology , bile acid , liver function tests , biliary atresia , transplantation , liver transplantation
. Healthy infants and children were found to excrete bile alcohol glucuronides in urine. Following isolation and hydrolysis, the bile alcohols were estimated by capillary gas‐liquid chromatography. The daily urinary excretion of the major compound, 27‐nor‐5 β ‐cholestane‐3α,7α,12α,24ξ,25ξ‐pentol (a C 26 bile alcohol), ranged from 0·1 to 1·1 μmol/24 h per m 2 body surface area for healthy infants and children. Two groups of patients with α 1 ‐antitrypsin deficiency (phenotype PiZ) were also studied during infancy and childhood, and biochemical liver function tests and liver morphology were compared to the excretion of bile alcohols. The highest excretion of the C 26 bile alcohol in urine was found in patients with α 1 ‐antitrypsin deficiency and juvenile cirrhosis (2·1–8·4 μmol 24 h ‐1 m ‐2 ) regardless of preceding neonatal cholestasis. Patients with α 1 ‐antitrypsin deficiency, neonatal cholestasis and subsequent fibrosis or normal liver morphology excreted bile alcohols within the normal range. The C 26 bile alochol constituted an average of 36% of the total bile alcohols in forty‐three urine samples. This percentage was about the same in the three groups studied. The findings suggest that determination of urinary bile alcohols may be a valuable non‐invasive diagnostic tool for patients with or at risk of developing liver cirrhosis.