Serum biotinidase activity in children with chronic liver disease and its clinical significance

Basit Öğe Kaydı
dc.contributor.authorPabuccuoglu, A
dc.contributor.authorAydogdu, S
dc.contributor.authorBas, M
dc.date.accessioned2019-10-27T18:18:40Z
dc.date.available2019-10-27T18:18:40Z
dc.date.issued2002
dc.departmentEge Üniversitesien_US
dc.description.abstractBackground: 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. Method: 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 . min(-1) . mL(-1)) and of all patients with chronic liver disease (6.3 +/- 2.5 nmol min(-1) . 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. Conclusion: 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.en_US
dc.identifier.doi10.1097/00005176-200201000-00014en_US
dc.identifier.endpage62en_US
dc.identifier.issn0277-2116
dc.identifier.issue1en_US
dc.identifier.pmid11753166en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage59en_US
dc.identifier.urihttps://doi.org/10.1097/00005176-200201000-00014
dc.identifier.urihttps://hdl.handle.net/11454/35526
dc.identifier.volume34en_US
dc.identifier.wosWOS:000172873600014en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherLippincott Williams & Wilkinsen_US
dc.relation.ispartofJournal of Pediatric Gastroenterology and Nutritionen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectbiotin deficiencyen_US
dc.subjectbiotinidase activityen_US
dc.subjectchronic liver diseasesen_US
dc.titleSerum biotinidase activity in children with chronic liver disease and its clinical significanceen_US
dc.typeArticleen_US

Dosyalar

Koleksiyon

WoS İndeksli Yayınlar Koleksiyonu
PubMed İndeksli Yayın Koleksiyonu
Scopus İndeksli Yayınlar Koleksiyonu