Mitochondrial Dysfunction in Children with Hepatic Forms of Glycogen Storage Disease
| Autor: | A.N. Surkov, Miroshkina Lv, Samokhina, Izmaĭlova Td, S.V. Petrichuk, O. V. Kurbatova, Poliakova Si, G.F. Semenova, Z.N. Dukhova, Leyla Namazova-Baranova, Potapov As, Kapustina EIu |
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| Rok vydání: | 2014 |
| Předmět: |
Male
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Cytological Techniques Statistics as Topic Glycogen Storage Disease Type I Carbohydrate metabolism Biology Severity of Illness Index Glycogen Storage Disease Type III Disease severity Internal medicine Severity of illness medicine Humans Glycogen storage disease In patient Lymphocytes Glycogen Storage Disease Type VI Child Succinate dehydrogenase nutritional and metabolic diseases Metabolic acidosis General Medicine medicine.disease Mitochondria Endocrinology Liver biology.protein Carbohydrate Metabolism Female Ldh activity Oxidoreductases |
| Zdroj: | Annals of the Russian academy of medical sciences. 69:78-84 |
| ISSN: | 0869-6047 |
| Popis: | Aim: The purpose of the study was to assess mitochondrial dysfunction severity in patients with hepatic forms of glycogen storage disease (GSD). Patients and methods : We examined 53 children with GSD in the dynamics. Distribution of children by disease types was: 1st group — children with GSD type I, 2nd group — children with GSD type III, 3rd group — children with GSD type VI and IX; comparison group consisted of 34 healthy children. Intracellular dehydrogenases activity: succinate dehydrogenase (SDH), glycerol-3-phosphate-dehydrogenase (GPDH), nicotinamideadenin-Н-dehydrogenase (NADH-D) and lactatdehydrogenase (LDH) was measured using the quantitative cytochemical method in the peripheral lymphocytes. Results : It was revealed decrease of SDH- (р |
| Databáze: | OpenAIRE |
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