Abstract
Objective: To study metabolic alterations in the organisms of children with hereditary hemolytic anemia (HHA). Methods: Authors studied metabolic conditions in 54 children aged 3 to 15 years suffering from HHA: deficit of glucose-6-phosphate dehydrogenase, thalassemia, genetic microspherocytocis. Metabolism research was carried out based on electrocardiographic, echocardiographic, and cytochemical methods. Results: It has been established that 96.3% of children with HHA had the signs of myocardial dystrophy. Analysis of echocardiogram shows heart cavity dilation (primarily left heart) and lower ejection fraction (55.2±1.6%). Analysis of cytochemical status of peripheral blood lymphocytes shows arrest of activity of energetic metabolism enzymes – succinate dehydrogenase (up to 11.54±0.25) and alpha-glycerophosphatedehydrogenase (up to 5.37±0.21). Conclusions: Results of performed laboratory and instrumental research methods show crude metabolic disturbances in children with HHA explained by the chronic hemic hypoxia, hemosyderosis of internals, and reduced activity of intracellular dehydrogenase. Keywords: Hereditary hemolytic anemia, hypoxia, myocardial dystrophy, metabolic disorders.
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