Abstract
Hypoglycemia in children carries high risk of CNS lesions. Early recognition and adequate therapy are mandatory. We report our experience with bGH and PD in 3 children with severe HH due to ICD. Case 1, a 13 months-old white boy with severe hypoglicemic sindrome since the age of 11 months, presented neuroglycopenic signs, marked muscular hypotonia and 2 episodes of loss of conscience. Full description of the case has already been published (Acta Pediatr Suppl 388:121–5,1993).Case 2, a 70 days white girl, had a seizure at 50 days of life; physical findings were unremarkable. Patient 3 was a white boy with one day age, who was in hypoglycemic coma from 12 to 17 hours of age. HH was documented in each patient. GH, cortisol, T3,T4, TSH, blood chemistry and immageologic studies were normal. EEG was deeply altered in case 3. Case 1, although having persistent neuroglycopenic symptoms had normal EEG. Patients received bGH (0.16-0.4 IU/Kg) and PD(10 mg/m2) on a daily basis. Patient 3 required the highest dose of GH but recovered neither consciousness nor normal glycemia. He died at the age of 47 days during status epilepticus. Cases 1 and 2 have been on therapy for 37 and 12 months respectively with normalization of blood glucose; however serum insulin has kept persistently high. Patient 1 has had normalization of neurological disturbances. Surgery and diazoxide have been the commonest therapeutical approach in children with HH.bGH and PD open a new perspective in handing these cases.
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Simoni, G., Demeterco, C., Nesi, S. et al. 13 BISOYNTHETIC HUMAN GROWTH HORMONE (bGH) AND PREDINISONE (PD) COMBINED THERAPY FOR HYPERNINSULIMEEIC HYPOGLICEMIA (HH) DUE TO ISLET CELL DISMATURITY (ICD). Pediatr Res 36, 680 (1994). https://doi.org/10.1203/00006450-199411000-00071
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DOI: https://doi.org/10.1203/00006450-199411000-00071