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Vol. 20, Art. 32 (pp. 385-403)    |    2019       

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Congenital hyperinsulinism: etiopathogenesis and treatment (review)

Perminova A.A.

Federal State Budgetary Institution "Almazov National Medical Research Centre" of the Ministry of Health of the Russian Federation

Brief summary

Congenital hyperinsulinism is one of the most common causes of hypoglycemia in infants. Because of absence of adequate pharmacological correction of hypoglycemic attacks, the development of irreversible changes in the central nervous system with the subsequent disability of such patients is possible. Due to the ineffectiveness of conservative therapy, in many cases surgical intervention is required. The review was compiled in order to combine the available data in the English literature on congenital hyperinsulinism, which, despite their fewness, are extremely fragmented. This review presents data on the etiology, pathogenesis, and diagnostic methods of congenital hyperinsulinism, as well as the possibilities for its medical correction. In particular, various mutations and pathogenetic mechanisms, possibilities and difficulties of preoperative and intraoperative diagnostics, tactics and possibilities of drug therapy of congenital hyperinsulinism are described.


Key words

congenital hyperinsulinism, hyperinsulinism, nesidioblastosis, hyperinsulinaemic hypoglycaemia, pancreatic islets.

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