Document Detail


Height gain with combined growth hormone and gonadotropin-releasing hormone analog therapy in two pubertal siblings with a growth hormone-releasing hormone receptor mutation.
MedLine Citation:
PMID:  17925337     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
CONTEXT: Patients with GHRH receptor (GHRH-R) mutations present with familial isolated GH deficiency, which untreated leads to a severely compromised adult height. Few data are available about the efficacy of treatment with GH in combination with a GnRH analog (GnRHa) in adolescence. OBJECTIVE: The objective of the study was to describe the evolution of growth and skeletal age of a brother and sister of Moroccan descent with a homozygous GHRH-R mutation who presented at an advanced age (16 and 14.9 yr, respectively) and pubertal stage (Tanner stage G4 and B3, respectively) with a height of -5.1 sd score and -7.3 sd score on treatment with a combination of GH and GnRHa for 2.5 and 3 yr followed by GH alone. METHODS: GH was given in a dosage of 0.7 mg/m2.d (25 microg/kg.d) sc and triptorelin in a dosage of 3.75 mg per 4 wk im. Height and pubertal stage were measured three-monthly, bone age yearly. RESULTS: Combined GH and GnRHa treatment resulted in a height gain of 24 and 28.2 cm, respectively, compared with the initial predicted adult height by the method of Bayley and Pinneau. Adult height was within the population range and well within the target range. CONCLUSIONS: Our patients demonstrate that, in case of isolated GH deficiency caused by a GHRH-R mutation, combined treatment of GH and GnRHa can be very effective in increasing final height, even at an advanced bone age and pubertal stage.
Authors:
Marie J E Walenkamp; Alberto M Pereira; Wilma Oostdijk; Wilhelmina H Stokvis-Brantsma; Roland W Pfaeffle; Oliver Blankenstein; Jan M Wit
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Publication Detail:
Type:  Journal Article     Date:  2007-10-09
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  93     ISSN:  0021-972X     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  2008 Jan 
Date Detail:
Created Date:  2008-01-07     Completed Date:  2008-03-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  204-7     Citation Subset:  AIM; IM    
Affiliation:
Department of Pediatrics J6-S, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The Netherlands. m.j.e.walenkamp@lumc.nl.
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MeSH Terms
Descriptor/Qualifier:
Adult
Body Height / drug effects*
Female
Growth Disorders / drug therapy*,  genetics
Human Growth Hormone / therapeutic use*
Humans
Male
Morocco / ethnology
Netherlands
Point Mutation*
Receptors, Neuropeptide / genetics*
Receptors, Pituitary Hormone-Regulating Hormone / genetics*
Triptorelin / therapeutic use*
Chemical
Reg. No./Substance:
0/Receptors, Neuropeptide; 0/Receptors, Pituitary Hormone-Regulating Hormone; 0/somatotropin releasing hormone receptor; 12629-01-5/Human Growth Hormone; 57773-63-4/Triptorelin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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