[Clinical practice guidelines "Congenital hyperinsulinism"].

Congenital hyperinsulinism (CHI) is a rare, genetically heterogeneous disorder characterized by inappropriate hypersecre tion of insulin by pancreatic beta cells and is the most common cause of persistent hypoglycemia in young children. The in cidence of CHI in the general population ranges from 1:30,000 to 1:50,000 live births. Timely diagnosis and treatment of CHI are critical, as persistent hypoglycemia can lead to irreversible damage to the central nervous system. This review summa rizes current understanding of the etiology and pathogenesis of CHI and presents an up-to-date classification of CHI based on duration, etiology, histological form, and response to diazoxide therapy. A detailed diagnostic algorithm is provided, including provocative tests, molecular genetic testing, and imaging studies. The stages of conservative treatment and indi cations for surgical treatment are discussed. The review is based on updated clinical guidelines and is intended for practicing physicians - pediatricians, endocrinologists, and neonatologists - who care for children with hypoglycemic conditions.