36 TREATMENT OF HYPERINSULINEMIC HYPOGLYCEMIA |[lpar]|HH|[rpar]| DUE TO ISLET CELL DISMATURITY |[lpar]|ICD|[rpar]| WITH RECOMBINANT HUMAN GROWTH HORMONE |[lpar]|rhGH|[rpar]| AND PREDNISONE |[lpar]|PD|[rpar]|

ICD (included Nesidioblastosis) is not an infrequent cause of HH in infancy. Persistence and/or recurrence of prolonged hypoglyoemic states may damage the CNS permanently. A prompt investigation and an agressive therapy are mandatory in most cases. The authors report a case of a 13 months old, white, male boy, with severe hypoglyoemic syndrome since the age of 11 months. Clinical manifestations were predominantly neuroglycopenic with two episodes of loss of conscience. Physical examination disclosed a generalized muscle hypotonia, marked psychomotor regression and paleness. Height and weigtit were in the 10th and 3rd percentiles respectively. Blood chemistry (including pH and gases), T4, TSH, GH, Cortisol, EEG, abdominal ultrasound and computarized tonography were normal. Insulin and C-peptide were elevated and insulin/glucose ratios were consistently above 3. Up to the age of 23 months the patient has been hospitalized 10 times for periods of 48 hours, with intervals of 15 to 30 days between each hospitalization. During each hospitalization blood samples have been drawn every 4 hours to measure glucose, insulin, C-peptide, cortisol and GH; the dosis of the drugs adjusted accordingly. While on 0.2 IU/Kg/day, sc, 2 injections (8 AM, 8 PM) of rhGH and 10 mg/m2/day (10 PM) of PD blood glucose has been always normal. Insulin levels have shown a tendency to higher values. Clinical improvement was significant and no episode of loss of conscience has been reported. Height and weight are in the 25th an 50th percentiles respectively. No cushingoid features are present.