Long Term Medical Treatment of Congenital Hyperinsulinemic Hypoglycemia (original) (raw)
2014, Journal of Endocrinology and Diabetes
Congenital hyperinsulinism (CH) is the most common cause of transient and permanent Hypoglycemia of infancy [1].Congenital Hyperinsulinemic Hypoglycemia is a consequence of dysregulated and inappropriate secretion of insulin due to dysfunctional K ATP channels. Prevention of hypoglycaemic episodes is the key to avoid long term neurological sequelae associated with congenital hyperinsulinism (CH) [2-4]. Inadequate suppression of insulin causes increase uptake of glucose by insulin sensitive tissues and inhibition of glycogenolysis, gluconeogenesis, lipolysis and ketogenesis [5]. All these process make developing brain vulnerable to irreversible brain injury. The estimated incidence of CH in general population is 1/50,000 live births, but it is reported higher in parts of Finland and Saudi Arabia (1/2675 with high consanguineous marriages) [6-8]. The aim of management in infants with CH is to avoid recurrent episodes of Hypoglycemia and to maintain blood glucose level above 70 mg/dl (3.9 mmol/l). This is critical to protect brain and prevent seizures associated with Hypoglycemia. Our understanding of genetics, histology and pathogenesis is significantly improved but paediatricians are still struggling to find satisfactory treatment option. High carbohydrate content feed is necessary to maintain normoglycaemia. Addition of raw corn starch and polycose in feed can improve the carbohydrate content [9]. Intensive feeding plan may be achieved by nasogastric or gastrostomy tube [10]. A number of pharmacological agents are being used to achieve normoglycemia in CH patients. There must be a rationale approach in introduction, increase in dose and change of drugs. Diazoxide is the first line drug introduced in 1965 for treatment of CH [11]. Diazoxide is given orally and it is always reasonable to proof that child is not responsive to oral therapy. Diazoxide may cause fluid retention that can precipitate cardiac failure. The most common side effect is the stimulation of generalised
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