title: Response to growth hormone-releasing hormone as evidence of hypothalamic defect in optic nerve hypoplasia.
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.affiliation: Department of Child Health, St Bartholomew's Hospital, London, UK.
authors.forename: A A
authors.initials: AA
authors.lastname: Leaf
authors.forename: R J
authors.initials: RJ
authors.lastname: Ross
authors.forename: R B
authors.initials: RB
authors.lastname: Jones
authors.forename: G M
authors.initials: GM
authors.lastname: Besser
authors.forename: M O
authors.initials: MO
authors.lastname: Savage
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005934:Glucagon / Q000494:pharmacology; D006130:Growth Disorders / Q000503:physiopathology*; D013006:Growth Hormone / Q000172:deficiency; D013007:Growth Hormone-Releasing Hormone / Q000031:analogs & derivatives* / Q000494:pharmacology; D006801:Humans; D007030:Hypothalamo-Hypophyseal System / Q000187:drug effects* / Q000503:physiopathology; D007328:Insulin / Q000494:pharmacology; D008297:Male; D009900:Optic Nerve / Q000002:abnormalities*; D010446:Peptide Fragments / Q000494:pharmacology*; D010908:Pituitary Hormones, Anterior / Q000172:deficiency; D017337:Sermorelin
chemical_list: D007328:Insulin; D010446:Peptide Fragments; D010908:Pituitary Hormones, Anterior; D017337:Sermorelin; D013006:Growth Hormone; D005934:Glucagon; D013007:Growth Hormone-Releasing Hormone