[Case of diagnostics of a rare form of glycogen disease].

Differential diagnosis of hypoglycemic syndrome remains an urgent problem in Pediatrics. In this article, a case of glycogen storage disease (BNG) type 0 is described in the boy, which is undoubtedly a rare pathology, which makes it difficult to diagnose this form of glycogenosis. In this description, the case of type 0 BNG is caused by a mutation in the GYS2 gene encoding the hepatic isoform of glycogen synthase. This form of the disease is usually asymptomatic in infancy. However, it can be suspected in the case when the refusal of night feeding causes certain difficulties due to the hungry ketotic hypoglycemia that occurs in the child. The first clinical symptoms appear in early childhood with the inclusion of a more varied diet in the child, increasing the intervals between meals. Symptoms of the disease are primarily caused by hypoglycemia. It should be noted that, despite the low levels of glycemia, most children do not have any mental development disorders, since the increase in the level of ketone bodies in the blood plasma provides the brain with an alternative source of energy.

[Use of ospen (a drug form of phenoxymethylpenicillin) in pediatric practice]. / Primenenie ospena (lekarstvennoi formy fenoksimetilpenitsillina) v pediatricheskoi praktike.

Ospen is a dosage form of phenoxymethylpenicilin. As a therapeutic and prophylactic agent it was shown efficient in treatment of patients with diseases of the otorhinolaryngological organs due to primary and secondary coccal infections. High antiinflammatory activity of ospen was observed in acute and aggravated chronic tonsillitis, otitis, sinusitis and acute respiratory virus infections as well as in prevention of complications after adenotonsillectomy and various manipulations connected with penetration into the inner cavities. Ospen was highly active as a prophylactic agent against hospital respiratory infections. It prevented its distribution and aggravation of the main diseases. Adverse reactions to the use of ospen were minimal and no toxic manifestations were recorded.