Document Detail

Developmental syndromes: growth hormone deficiency and treatment.
MedLine Citation:
PMID:  19293579     Owner:  NLM     Status:  MEDLINE    
Developmental syndromes are characterized by numerous phenotypical signs and malformations. In most of them such as Turner, Noonan, Prader-Willi, Silver-Russel, Williams, Kabuki, Leri-Weill syndrome and skeletal dysplasias, short stature is a common feature. Growth defect is very often related to a defect in cellular growth, but some unknown abnormality in GH action is possible. Recently, the greater availability of recombinant GH has expanded the interest towards GH secretion and therapy also in developmental syndromes. We recognize syndromes associated with GH deficiency (GHD), showing a developmental midline defect such as Pallister-Hall syndrome, septo-optic dysplasia, but many of these conditions do not have a convincing link with GHD. Moreover, some conditions, in particular the well-studied Turner syndrome, that do not have a real GHD, have proven to benefit from GH therapy at supra-physiological doses obtaining a higher final height than the expected one according to the natural history. This has expanded the indications for GH therapy. The aim of our paper is to review the literature on GH secretion, on the effects and costs-benefits of GH therapy in many dysmorphic syndromes, presenting some results of GH secretion and therapy obtained in our experience.
Laura Mazzanti; Federica Tamburrino; Rosalba Bergamaschi; Emanuela Scarano; Francesca Montanari; Michele Torella; Elisa Ballarini; Alessandro Cicognani
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Publication Detail:
Type:  Journal Article; Review     Date:  2009-02-27
Journal Detail:
Title:  Endocrine development     Volume:  14     ISSN:  1421-7082     ISO Abbreviation:  Endocr Dev     Publication Date:  2009  
Date Detail:
Created Date:  2009-03-18     Completed Date:  2009-05-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101138956     Medline TA:  Endocr Dev     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  114-34     Citation Subset:  IM    
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
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MeSH Terms
Growth Disorders / drug therapy*,  physiopathology*
Human Growth Hormone / deficiency*,  therapeutic use*
Mental Retardation / drug therapy,  physiopathology
Reg. No./Substance:
12629-01-5/Human Growth Hormone

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