[Serum lipoproteins in the generalized form of type III glycogenosis].
Distinct accumulation of glycogen, anomalous in structure, and absence of amylo-1,6-glucosidase activity were observed in studies of material obtained by biopsy from liver and muscle tissues of a patient with generalized form of glycogenosis type III. Anamalous glycogen (limitdextrin) was also found in erythrocytes. Concentration of lipoproteins, especially of low density lipoproteins 12.20 S and 0-12 S, was increased in blood serum. Spectrum of lipoproteins acquired a tendency to normalization simultaneously with clinical improvement after intravenous administration of glucose and treatment with cholesterolamine per os.
D002648:Child; D002792:Cholestyramine Resin / Q000627:therapeutic use; D005947:Glucose / Q000627:therapeutic use; D006003:Glycogen / Q000032:analysis; D006008:Glycogen Storage Disease / Q000378:metabolism*; D006010:Glycogen Storage Disease Type III / Q000188:drug therapy / Q000378:metabolism*; D006801:Humans; D008074:Lipoproteins / Q000097:blood*; D008077:Lipoproteins, LDL / Q000097:blood; D008079:Lipoproteins, VLDL / Q000097:blood; D008099:Liver / Q000378:metabolism*; D008297:Male; D009132:Muscles / Q000378:metabolism*
D008074:Lipoproteins; D008077:Lipoproteins, LDL; D008079:Lipoproteins, VLDL; D002792:Cholestyramine Resin; D006003:Glycogen; D005947:Glucose
Lipoproteidy syvorotki krovi pri generalizovannoĭ forme glikogenoza III tipa.