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dc.contributor.authorMaheshwari, Hiralal G
dc.contributor.authorRahim, Asad
dc.contributor.authorShalet, Stephen M
dc.contributor.authorBaumann, Gerhard
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.
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
html.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.


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