Abstract
Congenital hyperinsulinism (HI) comprises conditions of various initial severities, which might improve with time. Short-term management, to normalize glycemia and avoid brain damage, frequently includes the use of glucagon and intravenous and/or enteral dextrose. A long-term medical management plan is considered when the condition does not resolve over a few days or is not curable by a selective and partial pancreatectomy. The long-term management plan must be tailored for each patient, weighting the limitations, side effects, and contraindications of each drug. When the patient is not responsive to diazoxide, the first-line oral treatment, treatment with a somatostatin analogue is usually considered. If the efficacy of somatostatin analogues is not sufficient to prevent hypoglycemia, alternative measures may include carbohydrate-enriched diets, sometimes through an enteral tube (nasogastric or gastric) and/or continuous enteral dextrose.
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