Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bile salt metabolism was examined in 12 infants (ages 7 weeks to 10 months) who had recovered from protracted infantile diarrhea. Cholic acid kinetics were measured with the isotopic dilution technique. Cholate pool size was 938 +/- 89 mg/m2 (means +/- S.E.), synthetic rate was 478 +/- 52 mg/m2/day, and the fractional turnover rate was 0.537 +/- 0.060 day -1. Estimated chenodeoxycholate pool size was 607 +/- 103 mg/m2. similar cholic acid pool sizes were previously observed in older children; however, the synthetic and turnover rates were significantly lower, p less than 0.05 and 0.025, respectively. A significant inverse relationship between age and fractional turnover rate (r = -0.577, p less than 0.05) was observed in the first 10 months of life. Fasting serum cholylglycine measured by radioimmunoassay was significantly higher (p less than 0.01) in infants than in children. Peak postprandial concentrations were higher in infants than in older children. Accelerated hepatic bile salt synthesis rapidly increases the size of small pools at birth. Intraluminal bile salt concentrations concurrently increase, thereby normalizing fat solubilzation. Slow maturation of intestinal transport mechanisms may result in normal cycling of bile salts by 3 to 7 months of age. Persistent immaturity of hepatic uptake and/or excretion of bile salts leads to intrahepatic retention of a portion of the pool, with resultant regurgitation into the serum during the first months of life.
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PMID:Bile salt metabolism in the first year of life. 720 Oct

The 24h urinary bile acid excretion was prospectively studied during the neonatal period in healthy, fully breastfed, premature and full-term infants. The urinary bile acids were identified by gas-liquid chromatography (GLC)-mass spectrometry and quantified by GLC. The excretion of bile acids in urine increased after birth, reaching maximum levels by the 3-4th day. Taurine conjugates predominated and the excretion of bile acid sulphates was remarkably low. Cholic acid and atypical bile acids were the main bile acids in urine during the first week. Tetrahydroxylated bile acids carrying hydroxyl groups at C-1, C-2 and C-6 were common, and also other 1- and 6-hydroxylated bile acids, including hyocholic and hyodeoxycholic acids. Three tentatively identified 4-hydroxylated bile acids, including one ketonic bile acid, were also found. Ketonic bile acids constituted an average of 16% of total urinary bile acids during the first week. Unsaturated bile acids were scantily found only during the first days. The excretion of atypical bile acids decreased to 1 month of age, parallel with the total bile acid excretion. The data support earlier hypothesis of a physiological cholestasis in the newborn. Atypical hydroxylated and ketonic bile acids, as well as cholic acid, constituted the major part of the urinary bile acids. The persistent atypical pattern of bile acids in urine during the first month of life indicates a longer period of immaturity of bile acid metabolism in healthy infants than previously described.
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PMID:The urinary bile acid excretion in healthy premature and full-term infants during the neonatal period. 817 Dec 65