Background: A case of hypopituitarism usually related to hypothyroidism and hypoadrenalism. The unexpected effect of somatropin treatment is reported presented with clinical signs.
Method and results: A 4-year-old adynamic boy in a wheel chair with normal height (105 cm, −0.7 SDS) but low weight (13 kg) and reduced TSH and thyroid hormones was transferred for further endocrine evaluation. Born after twin pregnancy in 35 weeks (2780 g, 49 cm, and 34 cm) together with a healthy brother, he sucked slightly more weakly than his brother. Walking was weak and late (20 months). He could neither climb nor run. He received physiotherapy up from 6 months and further examinations for neuromuscular diseases were started without results. His health deteriorated. With 4 years, he was adynamic, could hardly walk and used a wheel chair. Hypomimic, he seldom reacted on questions and slept 18 h/day. There were no signs for paresis and the tendon reflexes were of low normal activity. He was obstipated since infancy. Basal TSH and the surge after TRH were reduced and no circadian TSH rhythm could be detected. Thyroxine treatment was introduced with only little improvement in 4 months. Pituitary examination revealed a partial hypoadrenalism with high total cortisol but reduced free saliva and urine cortisol due to high CBG. There was a complete somatropin deficiency. 5 mg/day Cortisol was introduced without further improvement in the following 2 months. Then, somatropin treatment was started. After 2 months the symptoms almost resolved and after 6 months the boy was clinically totally normal.
Conclusion: Complete somatropin deficiency may present without growth retardation. Hypoadrenalism combined with severe hypothyroidism in panhypopituitarism requires determination of free cortisol for diagnosis due to increased CBG. Treatment with thyroxine and cortisol alone in panhypopituitarism may not resolve all typical symptoms but only in addition with somatropin. The metabolic effects of somatropin in children need more attention.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology