Archive | 2021
Врожденный гиперинсулинизм у новорожденных и детей раннего возраста: состояние проблемы и результаты хирургического лечения
Abstract
Congenital hyperinsulinism causes irreversible damage to the\xa0cerebral cortex with subsequent disability in\xa0children. The\xa0article presents the\xa0features of\xa0etiopathogenesis, clinical picture of\xa0the\xa0disease. The\xa0histological variants of\xa0pancreatic lesions are analyzed in\xa0detail. The\xa0principles of\xa0correct diagnosis are formulated. A\xa0new in\xa0Russia\xa0method for\xa0the\xa0preoperative determination of\xa0the\xa0histological form of\xa0the\xa0disease, which is carried out at the\xa0y, Almazov National Medical Research Centre since 2017, – PET/\u200aCT with 18F-DOPA, explained the\xa0biochemical basis of\xa0its clinical application and the\xa0examination technique. The\xa0principles of\xa0the\xa0selection of\xa0drug therapy with possible complications, the\xa0need for\xa0an adequate assessment of\xa0its effectiveness are described. If it is impossible to achieve a\xa0stable target euglycemia\xa0without the\xa0need for\xa0intravenous glucose infusion, surgical correction of\xa0the\xa0disease is indicated. In\xa0schematic drawings and intraoperative photographs, approaches to surgical treatment are described, the\xa0stages of\xa0operations and possible complications are clearly disassembled. The\xa0results of\xa0surgical interventions at the\xa0N.N. V.A. Almazov for\xa001.2017–02.2021, where 39\xa0children with congenital hyperinsulinism were operated on. According to PET/CT with 18F-DOPA, 15\xa0diffuse and 24\xa0focal forms were diagnosed. After surgery, in\xa012\xa0(31%) patients, a\xa0diffuse lesion of\xa0the\xa0gland was confirmed, in\xa023\xa0(59%)\xa0– a\xa0focal nature of\xa0the\xa0lesion, in\xa04\xa0(10%)\xa0– an atypical form was diagnosed intraoperatively. Of\xa039\xa0children, 36\xa0(92%) have complete relief of\xa0hyperinsulinism, a\xa0significant improvement in\xa0psychomotor development, of\xa0which 9\xa0(23%) need insulin\xa0replacement therapy with minimal dosages, these are 8\xa0children with a\xa0diffuse form of\xa0the\xa0disease and 1\xa0child with an atypical one. Intraand postoperative complications were not observed. Thus, partial pancreatectomy for\xa0focal forms, subtotal for\xa0atypical and near total for\xa0diffuse forms, can cope with hypoglycemia\xa0due to congenital hyperinsulinism and prevent damage to the\xa0central nervous system of\xa0newborns and infants.