title: Serum biotinidase activity in children with chronic liver disease and its clinical significance.
abstract: BACKGROUND
  Biotinidase is the enzyme responsible for liberating the vitamin biotin from biocytin and dietary protein-bound vitamin. Individuals lacking biotinidase activity become biotin deficient. Because the liver is the major source of plasma biotinidase, chronic liver diseases can lead to decreased serum biotinidase activity and biotin deficiency. The aim of this study is to determine serum biotinidase activity values in children with chronic liver disease and to investigate the relation among enzyme activity, certain liver function tests, and degree of liver damage.
  
  
  METHODS
  In this study, using a spectrophotometric method, biotinidase activity was determined in sera from 62 children with chronic liver diseases (median age, 9.73 years; range, 8 months to 18 years) and from 27 healthy controls. Diagnoses of the patient group were as follows: noncirrhotic chronic hepatitis B virus infection (n = 12), metabolic liver diseases (n = 16), autoimmune hepatitis (n = 6), intrahepatic and extrahepatic cholestasis (n = 14), fulminant hepatitis (n = 5), cryptogenic cirrhosis n = 5), prehepatic portal hypertension (n = 4). Meanwhile, serum albumin, total bilirubin, alkaline phosphatase, alanine aminotransferase, and gamma-glutamyltransferase concentrations and prothrombine time were determined for each patient and the results were correlated with serum biotinidase activity.
  
  
  RESULTS
  There was significant difference between mean enzyme activity of the controls (7.6 +/- 1.2 nmol x min(-1) x mL(-1)) and of all patients with chronic liver disease (6.3 +/- 2.5 nmol x min(-1) x mL(-1)) ( P < 0.05). Serum biotinidase activity in patients with noncirrhotic chronic liver diseases (chronic viral hepatitis, prehepatic portal hypertension, glycogen storage disease, Gaucher disease) was within the normal ranges. However, serum biotinidase activity in patients with cirrhosis and Wilson disease was significantly less than that of the control group ( P < 0.05). The lowest enzyme activities were detected in patients with fulminant hepatitis.
  
  
  CONCLUSIONS
  In this study, serum biotinidase activity was significantly lower in patients with cirrhosis, particularly in the patients with decompensated cirrhosis and fulminant hepatitis who exhibited no clinical symptoms related to biotin deficiency. The decreased serum biotinidase activity in chronic liver diseases was associated with severe impairment of hepatocellular function.
authors.affiliation: Ege University Faculty of Pharmacy, Department of Biochemistry, Izmir, Turkey. papuccuoglua@pharm.ege.edu.tr
authors.forename: Aysun
authors.initials: A
authors.lastname: Pabuçcuoğlu
authors.forename: Sema
authors.initials: S
authors.lastname: Aydoğdu
authors.forename: Meral
authors.initials: M
authors.lastname: Baş
mesh_terms: D000293:Adolescent; D000328:Adult; D000581:Amidohydrolases / Q000097:blood*; D001710:Biotin / Q000097:blood / Q000172:deficiency; D043523:Biotinidase; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000201:enzymology* / Q000503:physiopathology; D008107:Liver Diseases / Q000097:blood / Q000201:enzymology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged
chemical_list: D001710:Biotin; D000581:Amidohydrolases; D043523:Biotinidase