Selective lack of growth hormone (GH) response to the GH-releasing peptide hexarelin in patients with GH-releasing hormone receptor deficiency.

2.50
Hdl Handle:
http://hdl.handle.net/10541/87876
Title:
Selective lack of growth hormone (GH) response to the GH-releasing peptide hexarelin in patients with GH-releasing hormone receptor deficiency.
Authors:
Maheshwari, Hiralal G; Rahim, Asad; Shalet, Stephen M; Baumann, Gerhard
Abstract:
The mechanism of the synergistic relationship between GH-releasing peptide (GHRP) and GHRH with respect to GH secretion is poorly understood. We report the response to hexarelin, a potent GHRP, in patients affected with a homozygous mutation in the GHRH receptor gene, with consequent GHRH resistance and GH-deficient dwarfism. This newly described syndrome is the human homolog of the little (lit/lit) mouse. Intravenous administration of hexarelin (2 microg/kg) to four male adult patients (dwarfs of Sindh) resulted in a complete lack of elevation in plasma GH levels (< 1 ng/mL), an at least 50- to 100-fold deviation from the normal response. In contrast, plasma PRL, ACTH, and cortisol levels rose in a normal manner in response to hexarelin. We conclude that an intact GHRH signaling system is critical for GHRPs to exert their effect on GH release, but that the GHRH system is not necessary for the effect of GHRP on PRL and ACTH secretion. Hexarelin (and probably other GHRPs) are not effective agents for the treatment of patients with GHRH resistance due to GHRH receptor deficiency.
Affiliation:
Center for Endocrinology, Metabolism, and Molecular Medicine, Department of Medicine, Northwestern University Medical School, Chicago, Illinois 60611, USA.
Citation:
Selective lack of growth hormone (GH) response to the GH-releasing peptide hexarelin in patients with GH-releasing hormone receptor deficiency. 1999, 84 (3):956-9 J. Clin. Endocrinol. Metab.
Journal:
The Journal of Clinical Endocrinology and Metabolism
Issue Date:
Mar-1999
URI:
http://hdl.handle.net/10541/87876
DOI:
10.1210/jc.84.3.956
PubMed ID:
10084578
Type:
Article
Language:
en
ISSN:
0021-972X
Appears in Collections:
All Christie Publications

Full metadata record

DC FieldValue Language
dc.contributor.authorMaheshwari, Hiralal Gen
dc.contributor.authorRahim, Asaden
dc.contributor.authorShalet, Stephen Men
dc.contributor.authorBaumann, Gerharden
dc.date.accessioned2009-12-14T13:26:01Z-
dc.date.available2009-12-14T13:26:01Z-
dc.date.issued1999-03-
dc.identifier.citationSelective lack of growth hormone (GH) response to the GH-releasing peptide hexarelin in patients with GH-releasing hormone receptor deficiency. 1999, 84 (3):956-9 J. Clin. Endocrinol. Metab.en
dc.identifier.issn0021-972X-
dc.identifier.pmid10084578-
dc.identifier.doi10.1210/jc.84.3.956-
dc.identifier.urihttp://hdl.handle.net/10541/87876-
dc.description.abstractThe mechanism of the synergistic relationship between GH-releasing peptide (GHRP) and GHRH with respect to GH secretion is poorly understood. We report the response to hexarelin, a potent GHRP, in patients affected with a homozygous mutation in the GHRH receptor gene, with consequent GHRH resistance and GH-deficient dwarfism. This newly described syndrome is the human homolog of the little (lit/lit) mouse. Intravenous administration of hexarelin (2 microg/kg) to four male adult patients (dwarfs of Sindh) resulted in a complete lack of elevation in plasma GH levels (< 1 ng/mL), an at least 50- to 100-fold deviation from the normal response. In contrast, plasma PRL, ACTH, and cortisol levels rose in a normal manner in response to hexarelin. We conclude that an intact GHRH signaling system is critical for GHRPs to exert their effect on GH release, but that the GHRH system is not necessary for the effect of GHRP on PRL and ACTH secretion. Hexarelin (and probably other GHRPs) are not effective agents for the treatment of patients with GHRH resistance due to GHRH receptor deficiency.en
dc.language.isoenen
dc.subject.meshAdrenocorticotropic Hormone-
dc.subject.meshAdult-
dc.subject.meshHuman Growth Hormone-
dc.subject.meshHumans-
dc.subject.meshHydrocortisone-
dc.subject.meshMale-
dc.subject.meshOligopeptides-
dc.subject.meshProlactin-
dc.subject.meshReceptors, Neuropeptide-
dc.subject.meshReceptors, Pituitary Hormone-Regulating Hormone-
dc.subject.meshReference Values-
dc.titleSelective lack of growth hormone (GH) response to the GH-releasing peptide hexarelin in patients with GH-releasing hormone receptor deficiency.en
dc.typeArticleen
dc.contributor.departmentCenter for Endocrinology, Metabolism, and Molecular Medicine, Department of Medicine, Northwestern University Medical School, Chicago, Illinois 60611, USA.en
dc.identifier.journalThe Journal of Clinical Endocrinology and Metabolismen

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