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Biomedical subjects

K Finni

Publications and source records attributed to K Finni.

11 recordsLinked to original sources

Deoxycholic and sulpholithocholic acid concentrations in serum during infancy and childhood.

The concentrations of the two secondary bile acids (deoxycholic (DCA) and sulpholithocholic (SLCA) acid) were determined by radioimmunoassays in the serum of infants and children at ages ranging from 1 hour to 15 years. The same bile acids were measured also in the umbilical cord serum. The concentrations of the secondary bile acids in the serum of 1-hour old infants corresponded to those in the umbilical cord serum. Secondary bile acid serum concentrations were after the age of 7 days and up to the age of 3 to 6 months significantly lower than those in the umbilical cord serum. After the age of 6 months a significant increase in DCA serum concentrations could be shown. During the first 6 months of life DCA concentrations were clearly lower than those of SLCA. Our observations suggest that in the perinatal period DCA is mainly of maternal origin and that an alternate hepatic pathway may exist for the synthesis of lithocholic acid in early infancy.

Adolescent↗

Cholic acid, chenodeoxycholic acid, alpha-1-fetoprotein and alpha-1-antitrypsin serum concentrations in breast-fed infants with prolonged jaundice.

Thirteen breast-fed one-month-old infants with prolonged jaundice not due to known causes were included in this study. All infants were investigated at one and twelve months of age. Serum concentrations of total (TB) and conjugated bilirubin (CB), aspartate (ASAT) and alanine aminotransferase (ALAT), alkaline phosphatase (AP), alpha-1-antitrypsin (alpha-1-AT), alpha-1-fetoprotein (AFP) and the two primary bile acids; cholic (CA) and chenodeoxycholic acid (CDCA) were determined at both ages. The Pi-phenotype of alpha-1-AT was determined at the age of twelve months. The serum concentrations of TB, CB, AP and AFP were elevated at the age of one month but were normal at the age of twelve months. No changes in the serum concentrations of ASAT or ALAT were observed between one and twelve months of age, and the values were within the reference ranges. The serum concentrations of alpha-1-AT were within the reference range at both ages. Two infants were heterozygous for MZ, and they had normal serum alpha-1-AT concentrations. The serum concentrations of CA and CDCA were elevated at the age of one month and were still significantly elevated at the age of twelve months indicating that the infants had slight cholestasis at the age of one month, and that the cholestasis had largely subsided by the end of the first year of life.

Alanine Transaminase↗

Intracranial calcifications in cerebro-oculo-facio-skeletal (COFS) syndrome.

Two brothers with typical clinical findings of the COFS-syndrome are described. In the cranial CT performed on the younger patient at the age of 2 years and 6 months, foci of intracranial calcification located symmetrically in the region of the lenticular nucleus and hemispheric white matter were noted. This finding is clearly distinguishable from the other known patterns of intracranial calcification and could be pathognomonic of the COFS-syndrome.

Abnormalities, Multiple↗

Effect of phototherapy and exchange transfusion on primary bile acids in the serum of hyperbilirubinaemic newborns.

Serum primary bile acid (cholic (CA) and chenodeoxycholic (CDCA) acid) concentrations were measured in 14 preterm and 11 full-term hyperbilirubinaemic newborns at the beginning and end of, and 24 and 72 hours following phototherapy. Only in the preterm newborns with gestational ages of 35-38 weeks there was a significant decrease of mean serum bile acid concentrations which could be shown 72 hours after the beginning of phototherapy. It can be hypothesized that the decrease was a result of a direct effect of light on the excretory liver function. Serum CA and CDCA concentrations were also measured in 5 hyperbilirubinaemic newborns at the beginning and end, and 24, 48 and 72 hours after the end of exchange transfusion. Exchange transfusion caused a clear immediate decrease in the mean serum primary bile acid concentrations. However, on day 2 after exchange transfusion the mean serum concentration of CA was about 150% and that of CDCA about 110% of the initial values. The most hyperbilirubinaemic newborns had extremely high primary bile acid serum concentrations before therapy. As bile acids compete with bilirubin for albumin binding it should be considered whether high bile acids in the serum of hyperbilirubinaemic newborns presuppose exchange transfusions.

Bilirubin↗

Alpha-1-antitrypsin: the PiM subtypes and serum concentrations in Finnish newborns.

Pi phenotypes were classified by isoelectric focusing of the sera of 200 full-term Finnish newborns at the age of 3--5 days. The phenotype PiM was found in 95% of the neonates. The frequencies of the alleles PiM1, PiM2 and PiM3 were 0.682, 0.189 and 0.129, respectively. The serum concentration (mean +/- SD) of alpha-1-antitrypsin (alpha 1-AT) in the whole study group was 2.72 +/- 0.61 g/l. The concentration of alpha 1-AT in the newborns with the phenotype PiM3 (1.79 +/- 0.65 g/l) was significantly lower (p less than 0.025) than in other PiM subtypes. No difference in serum concentrations of alpha 1-AT between boys (2.78 +/- 0.66 g/l) and girls (2.66 +/- 0.55 g/l) was observed.

Alleles↗

Serum cholic acid and chenodeoxycholic acid concentrations in neonatal hyperbilirubinemia.

Primary bile acid concentrations were measured in serum of 332 newborns with neonatal hyperbilirubinemia (serum total bilirubin level greater than 200 mumol/l) and compared with those of 95 nonhyperbilirubinemic neonates (serum total bilirubin level less than 200 mumol/l). The serum concentrations (mumol/l; mean +/- SEM) for cholic acid (8.78 +/- 0.44) and chenodeoxycholic acid (10.5 +/- 0.68) were significantly higher (p less than 0.001) in the hyperbilirubinemic group than in the controls (7.16 +/- 0.48 and 6.67 +/- 0.48, respectively). 80 (24%) of the hyperbilirubinemic newborns had true cholestasis (serum levels of cholic and/or chenodeoxycholic acid higher than mean +/- 2 SD in the reference group). The ratio of cholic to chenodeoxycholic acid was significantly higher (p less than 0.05) in the cholestatic group than in the hyperbilirubinemic newborns without cholestasis. There was no significant differences in the serum concentrations of alkaline phosphatase or lactate dehydrogenase between the cholestatic and noncholestatic groups. In the hyperbilirubinemic newborns, the primary bile acids were indiscriminately raised. Only 8 infants from the 332 newborns had jaundice at the age of 1 month. Of these 8 infants only 2 had neonatal cholestatic hyperbilirubinemia. It thus appears that measurement of serum primary bile acid concentrations has only limited diagnostic value in assessing the severity or prognosis of neonatal hyperbilirubinemia.

Chenodeoxycholic Acid↗

Cholic acid and chenodeoxycholic acid concentrations in serum during infancy and childhood.

Concentrations of two primary bile acids (cholic and chenodeoxycholic acids) were determined by radioimmunoassay in the serum of infants and children at ages ranging from 1 hour to 15 years. The same bile acids were also measured in umbilical cord serum. Concentrations of the primary bile acids were significantly higher in the serum of 1-hour old infants than those in the umbilical cord serum or the peripheral vein serum of adults. The levels of cholic and chenodeoxycholic acid remained high until the age of 6 months, being about 5-fold higher than those in the sera of adults. Primary bile acid concentrations reached the adult level by the age of 1-2 years. These results indicate that developmental changes occur in the metabolism and excretion of bile acids in man. The relatively high concentrations of the primary bile acids in serum during the first 6 months of life suggest that up to this age, the mature ability of the liver to excrete the bile salts into the bile and/or to clear them from the circulation has not yet been reached.

Adolescent↗

Serum bile acid concentrations in the course of benign recurrent intrahepatic cholestasis.

The patient reported here represents the classic features of the syndrome of benign recurrent intrahepatic cholestasis (BRIC). She had conjugated hyperbilirubinaemia during the neonatal period and early infancy. The concentrations of serum primary bile acids, cholic acid (CA) and chenodeoxycholic acid (CDCA) were determined by radioimmunoassay and were continuously extremely raised during the icteric, recovery, and also the anicteric phases of the disease. The ratio of CA to CDCA was always within the normal range. The secondary serum bile acid, deoxycholic acid (DCA), was only slightly raised. The oral cholate tolerance test was abnormal in the patient during the anicteric phase. These observations support the suggestion that a disturbance of the hepatocellular bile acid transport may be the primary defect in BRIC.

Bile Acids and Salts↗