Introduction: Standard dose for GH deficient children is 0.025 mg/kg per day given subcutaneously once daily. Inborn panhypopituitarism is a special subset of GH deficiency. Its management is difficult because several hormones need to be replaced. We present three patients with perinatal onset panhypopituitarism.
Case reports: Case 1 is a 7-year-old boy who had hypoglycaemic seizure with reanimation due to circulatory arrest shortly after birth. Panhypopituitarism was diagnosed as hypoglycemia was ongoing during intensive care treatment. After initiation of treatment with GH and cortisol hypoglycemia ceased. GH-therapy was initiated and was increased up to a dose of 0.036 mg/kg per day to achieve good growth velocity and IGF1 in the upper normal range. Since the age of 4, dose/kg was slowly reduced to 0.026 mg/kg per day maintaining good growth velocity and good IGF1 levels. The patients prospective length is normal. Case 2 is a 4-year-old girl who was born after fetal hydrops and emergency C-section. In the first few weeks of life hypothyroidism and hypocortisolism was diagnosed and treatment initiated. MRI showed aplastic hypophysis, hormonal testing showed panhypopituitarism. Treatment with GH (0.025 mg/kg per day) was started, but no catch-up growth was reached and IGF1 remained low. GH-dose was increased in several steps to a dose of 0.052 mg/kg per day to achieve good growth velocity and IGF1 levels in the normal range. Case 3 is a 20-year-old man with hypoplastic pituitary gland of unknown cause. Treatment for panhypopituitarism was initiated at the age of 11. He was treated with GH in a dose of 0.025 mg/kg per day, but catch up-growth and normal IGF1 levels were only achieved with a dose of 0.035 mg/kg per day. The patient is still growing due to constitutional delay of growth and will reach his genetic length.
Conclusion: Our cases show that in patients with panhypopituitarism much higher doses may be (initially) necessary to achieve appropriate growth velocity, normal IGF1 levels and normal length. We conclude that these patients need to be closely monitored and GH-dose must be increased if growth velocity and IGF1 levels are not within normal limits. Further studies are necessary to evaluate the reason for these findings.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology