Low serum biotinidase activity in children with valproic acid monotherapy

dc.contributor.author	Σούλπη, Κλεοπάτρα	el
dc.contributor.author	Καρίκας, Γεώργιος-Αλβέρτος	el
dc.contributor.author	Τζαμουράνη, Ιωάννα	el
dc.contributor.author	Ρεγκούτας, Σπύρος	el
dc.contributor.author	Τσακίρης, Στυλιανός	el
dc.date.accessioned	2015-05-09T18:32:51Z
dc.date.available	2015-05-09T18:32:51Z
dc.date.issued	2015-05-09
dc.identifier.uri	http://hdl.handle.net/11400/10040
dc.rights	Αναφορά Δημιουργού-Μη Εμπορική Χρήση-Όχι Παράγωγα Έργα 3.0 Ηνωμένες Πολιτείες	*
dc.rights.uri	http://creativecommons.org/licenses/by-nc-nd/3.0/us/	*
dc.source	http://www.ncbi.nlm.nih.gov/pubmed/11737173?dopt=AbstractPlus	en
dc.subject	Βιοτινιδάση
dc.subject	Βαλπροϊκό νάτριο
dc.subject	βιοτίνη
dc.subject	Biotinidase
dc.subject	Valproic acid
dc.subject	Biotin
dc.title	Low serum biotinidase activity in children with valproic acid monotherapy	en
heal.type	journalArticle
heal.classification	Clinical biochemistry
heal.classification	Epilepsy
heal.classification	Κλινική βιοχημεία
heal.classification	Επιληψία
heal.classificationURI	http://id.loc.gov/authorities/subjects/sh85027062
heal.classificationURI	http://lod.nal.usda.gov/10276
heal.classificationURI	N/A-Κλινική βιοχημεία
heal.classificationURI	N/A-Επιληψία
heal.keywordURI	http://id.loc.gov/authorities/subjects/sh85141922
heal.keywordURI	http://id.loc.gov/authorities/subjects/sh85014267
heal.identifier.secondary	DOI: 10.1046/j.1528-1157.2001.47000.x
heal.language	en
heal.access	campus
heal.recordProvider	Τεχνολογικό Εκπαιδευτικό Ίδρυμα Αθήνας. Σχολή Επαγγελμάτων Υγείας και Πρόνοιας. Τμήμα Ιατρικών Εργαστηρίων	el
heal.publicationDate	2001-10
heal.bibliographicCitation	Schulpis, K.H., Karikas, G.A., Tjamouranis, J., Regoutas, S. &Tsakiris, S. (2001) Low serum biotinidase activity in children with valproic acid monotherapy. Epilepsia. [Online] 42 (10). pp.1359-1362. Available from: http://www.ncbi.nlm.nih.gov [Accessed 09/05/2015]	en
heal.abstract	Summary: Purpose: Valproic acid (VPA) is an effective antiepileptic drug (AED), which is associated with dose-related adverse reactions such as skin rash, hair loss (alopecia), etc. Profound as well as partial biotinidase deficiency causes dermatologic manifestations similar these. Therefore, it was of interest to evaluate serum biotinidase activity in patients receiving VPA monotherapy. Methods: Seventy-five patients with seizures, mean age, 8.6 years (±1.9 years) were divided into three groups. Group A (n = 25) was treated with VPA 28.7 ± 8.5 mg/kg/24 h, group B (n = 25) with 41.6 ± 4.9 mg/kg/24 h, and group C with 54.5 ± 5.8 mg/kg/24 h. Their “trough” VPA serum levels were 40.9 ± 13.2, 86.25 ± 11.5, and 137 ± 14.5 μg/ml, respectively. Fifty healthy children were the controls. Patients and controls underwent clinical and laboratory evaluations including liver function data, complete blood counts, NH3, and so on, after 45 days of VPA treatment. Biotinidase serum levels were evaluated fluorometrically. Results: Liver function data were found elevated in the groups B and C. On the contrary, biotinidase activity was significantly statistically lowered (p < 0.001) in groups B and C (1.22 ± 1.11, 0.97 ± 0.07 mmol/min/L respectively), as compared with controls (5.20 ± 0.90 mmol/min/L). Strong inverse correlations were observed between liver enzymes and VPA blood levels with the activity of the enzyme. Additionally, no inhibitory effect on biotinidase activity was found, when the enzyme was incubated in vitro with high (1.2 mM) concentrations of the drug. Skin lesions (seborrheic rash, alopecia) were improved in our patients after biotin (10 mg/day) supplementation. Conclusions: It is suggested that VPA impairs the liver mitochondrial function, resulting in a low biotinidase activity and or biotin deficiency. Biotin supplementation could restore some of the side effects of the drug.	en
heal.journalName	Epilepsia	en
heal.journalType	peer-reviewed
heal.fullTextAvailability	true