Response to growth hormone-releasing hormone as evidence of hypothalamic defect in optic nerve hypoplasia.

Acta paediatrica Scandinavica1989PMID: 2545075

Plain Language Summary

Four children with optic nerve hypoplasia were found to be growth hormone deficient when tested with standard stimulation tests, but all responded to GHRH injection, indicating that their deficiency originates in the hypothalamus rather than the pituitary. One child showed an excellent growth response to subcutaneous GHRH treatment, which is the most physiologically appropriate therapy for this type of hypothalamic defect. These cases highlight the importance of GHRH testing in children with optic nerve hypoplasia to identify those who could benefit from GHRH-based treatment.

Abstract

Hypothalamic-pituitary function was studied in four children with Optic Nerve Hypoplasia (ONH). All were found to be growth hormone deficient when provoked with glucagon or insulin induced hypoglycaemia (ITT), but did respond to bolus injection of GHRH. This indicates a primary hypothalamic defect. Virtual absence of pituitary tissue on high resolution CT scan explained the poor response of one child. One child has shown an excellent response to treatment with subcutaneous GHRH, which is physiologically the most appropriate treatment for this condition.

Authors

Leaf, A A; Ross, R J; Jones, R B; Besser, G M; Savage, M O