Педиатрическая фармакология (Jul 2013)
RESULTS OF LIFE QUALITY EVALUATION IN CHILDREN WITH HEPATIC VARIANT OF GLYCOGEN DISEASE
Abstract
Glycogen storage disease (glycogenosis) is a common name for a group of hereditary diseases characterized by excessive accumulation of glycogen with normal or altered structure in various organs and tissues, most often – in liver and muscles. This pathology is still diagnosed late and not completely; this considerably aggravates the disease’s prognosis. The modern stage of glycogenoses’ study is characterized by many unsolved issues; in particular, there are no clear examination and complex rehabilitation algorithms for such patients. Moreover, the authors have not revealed any literary data on the study of life quality of children with glycogen storage disease. The article presents results of the authors’ studies of life quality and its connection with clinical-paraclinic parameters in patients with this pathology.
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