Syndromic disorders with short stature.

Abstract

Short stature is one of the major components of many dysmorphic syndromes. Growth failure may be due to a wide variety of mechanisms, either related to the growth hormone (GH)/insulin-like growth factor axis or to underlying unknown pathologies. In this review, the relatively more frequently seen syndromes with short stature (Noonan syndrome, Prader-Willi syndrome, Silver-Russell syndrome and Aarskog-Scott syndrome) were discussed. These disorders are associated with a number of endocrinopathies, as well as with developmental, systemic and behavioral issues. At present, GH therapy is used in most syndromic disorders, although long-term studies evaluating this treatment are insufficient and some controversies exist with regard to GH dose, optimal age to begin therapy and adverse effects. Before starting GH treatment, patients with syndromic disorders should be evaluated extensively.

• Authors: Zeynep Şıklar, Merih Berberoğlu
• Journal: Journal of clinical research in pediatric endocrinology (J Clin Res Pediatr Endocrinol) Vol. 6 Issue 1 Pg. 1-8 ( 2014) ISSN: 1308-5735 [Electronic] Turkey
• PMID: 24637303 (Publication Type: Journal Article, Review)

Chemical References

• Human Growth Hormone

Topics

• Dwarfism (drug therapy, pathology)
• Growth Disorders (drug therapy, pathology)
• Human Growth Hormone (therapeutic use)
• Humans
• Syndrome