ABSTRACT
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-H-dehydrogenase (NADH-D) and lactatdehydrogenase (LDH) was measured using the quantitative cytochemical method in the peripheral lymphocytes. RESULTS: It was revealed decrease of SDH- (p < 0.001) and GPDH-activities (p < 0.001), along with increase of the NADH-D activity (p < 0.05) in all patients with GSD, (SDH/ NADH-D) index was decreased (p < 0.001). LDH activity was increased in groups 1 (p < 0.05) and 3 (p < 0.01), compared with comparison group. The most pronounced intracellular enzymes activity deviations were observed in children with GSD type I, that correspond to more severe clinical form of GSD. It was found strong correlation between intracellular enzymes activity and both hepatomegaly level (R = 0.867) and metabolic acidosis severity (R = 0.987). CONCLUSION: Our investigation revealed features of mitochondrial dysfunction in children with GSD, depending on the GSD type. Activities of lymphocytes enzymes correlates with the main disease severity parameters and can be used as an additional diagnostic criteria in children with hepatic form of GSD.
Subject(s)
Glycogen Storage Disease Type III , Glycogen Storage Disease Type I , Glycogen Storage Disease Type VI , Liver , Lymphocytes/metabolism , Mitochondria/metabolism , Carbohydrate Metabolism , Child , Cytological Techniques/methods , Female , Glycogen Storage Disease Type I/diagnosis , Glycogen Storage Disease Type I/metabolism , Glycogen Storage Disease Type I/physiopathology , Glycogen Storage Disease Type III/diagnosis , Glycogen Storage Disease Type III/metabolism , Glycogen Storage Disease Type III/physiopathology , Glycogen Storage Disease Type VI/diagnosis , Glycogen Storage Disease Type VI/metabolism , Glycogen Storage Disease Type VI/physiopathology , Humans , Liver/metabolism , Liver/pathology , Liver/physiopathology , Male , Oxidoreductases/analysis , Oxidoreductases/classification , Oxidoreductases/metabolism , Severity of Illness Index , Statistics as TopicABSTRACT
The enzymatic status of leukocytes with respect to succinate- and alpha-glycerophosphate dehydrogenase activity, which reflects the degree of hypoxia in the organism is changed by 30% by the action of natural physical parameters of the environment. Using mathematical modelling, it was shown that the largest changes in the enzymatic status occur upon increased geomagnetic perturbations at a high atmospheric pressure. Chronic tissue hypoxia of the fetus and newborn is the most likely disease that can develop under these conditions. This is most important to expectant mothers older than 32 years. The effect of environmental influences is more pronounced during the second half of pregnancy.
Subject(s)
Pregnancy/physiology , Adult , Female , Glycerolphosphate Dehydrogenase/blood , Humans , Hypoxia , Leukocytes/enzymology , Models, Theoretical , Pregnancy/blood , Succinate Dehydrogenase/bloodSubject(s)
Colon/metabolism , Energy Metabolism/drug effects , Hirschsprung Disease/surgery , Intestinal Mucosa/metabolism , Child , Citric Acid Cycle/drug effects , Colon/drug effects , Hirschsprung Disease/etiology , Hirschsprung Disease/metabolism , Humans , Intestinal Mucosa/drug effects , Linoleic Acids/administration & dosage , Niacinamide/administration & dosage , Preoperative Care , Thiamine Pyrophosphate/administration & dosageABSTRACT
Eleven informative signs significant in differential diagnosis were revealed on the basis of correlation analysis by Wald's consecutive analysis among 182 signs characterizing the manifestation of nonrheumatic cardiopathies in children. A diagnostic table with the level of significance coefficients for each principal sign was complied.
Subject(s)
Cardiomyopathies/diagnosis , Adolescent , Child , Child, Preschool , Clinical Enzyme Tests , Diagnosis, Computer-Assisted , Diagnosis, Differential , Female , Heart Defects, Congenital/diagnosis , Heart Function Tests , Humans , Leukocytes/enzymology , Male , Mathematics , Myocarditis/diagnosisABSTRACT
The activity of succinate dehydrogenase, alpha-glycerophosphate dehydrogenase and acid phosphatase in the lymphocytes of peripheral blood was studied in 28 patients with hypertensive disease and vasorenal hypertension (the diagnosis was made on the basis of the findings of angiographic examination) by the method of quantitative cytochemical analysis suggested by R.P. Nartsissov. It was revealed that alpha-glycerophosphate dehydrogenase activity was significantly lower in vasorenal hypertension than in hypertensive disease. The calculation of the difference between the value of succinate dehydrogenase activity and that of alpha-glycerophosphate dehydrogenase activity in these groups was also significant. It is concluded that a complex of cytochemical analysis may be used in differential diagnosis of vasorenal hypertension and hypertensive disease.
Subject(s)
Acid Phosphatase/blood , Hypertension, Renal/diagnosis , Hypertension, Renovascular/diagnosis , Hypertension/diagnosis , Lymphocytes/enzymology , Oxidoreductases/blood , Adolescent , Adult , Clinical Enzyme Tests , Diagnosis, Differential , Female , Glycerolphosphate Dehydrogenase/blood , Humans , Male , Middle Aged , Succinate Dehydrogenase/bloodSubject(s)
Asphyxia Neonatorum/diagnosis , Lymphocytes/enzymology , Asphyxia Neonatorum/enzymology , Clinical Enzyme Tests , Female , Glucosephosphate Dehydrogenase/blood , Glutamate Dehydrogenase/blood , Glycerolphosphate Dehydrogenase/blood , Humans , Infant, Newborn , Pregnancy , Succinate Dehydrogenase/bloodABSTRACT
A study was made of the activity of succinic dehydrogenase (SDH) and of the alpha-glycerophosphoric dehydrogenase (alpha-GPDH) in the peripheral blood lymphocytes and the transplant myocardium in the CBA mice in transplantation to them of the heart of newborn C57BL/6 mice into the pinna, subcutaneously. It appeared that the (see article) ratio in the myocardium and the lymphocytes of intact animals and also in the recipients of the isolinear transplant of the heart was greater than 1, but in allogenous transplantation this ratio became less than 1 on account of alpha-GPDH activation in the blood lymphocytes, which preceded the inversion in the transplant myocardium. It is supposed that the mentioned phenomenon was associated with the development of the immunological conflict.
Subject(s)
Glycerolphosphate Dehydrogenase/blood , Heart Transplantation , Lymphocytes/enzymology , Succinate Dehydrogenase/blood , Animals , Mice , Mice, Inbred C57BL , Mice, Inbred CBA , Transplantation, HomologousABSTRACT
The activity of succinatedehydrogenase, mitochondrial alpha-glycerophosphatedehydrogenase and NADH2-diaphorase was studied cytochemically and histochemically in the myocardium and lymphocytes of rats and mice after the effect of hypoxia in a hyperbaric chamber. A short-duration effect of hypoxia and step-wise adaptation during a month was shown to result in a growing activity of the enzymes both in the lymphocytes and in the myocardium. Hypoxia during a month and readaptation during the same period caused a monotonous decrease of the activity of dehydrogenases. The conducted analysis permitted to reveal a distinct correlation between the activity of dehydrogenases in the lymphocytes and in the myocardium. The authors emphasize the similarity of the changes in the activity of the enzymes in the lymphocytes and in the myocardium.
