ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 16 Issue: 1 Year: 2024
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Turkish Society for Pediatric Endocrinology and Diabetes
Syndromic Disorders with Short Stature [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. 2014; 6(1): 1-8 | DOI: 10.4274/Jcrpe.1149

Syndromic Disorders with Short Stature

Zeynep Şıklar1, Merih Berberoğlu1
Ankara University School Of Medicine, Department Of Pediatric Endocrinology, Ankara, Turkey

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.

Keywords: short stature,Noonan syndrome,Prader-Willi syndrome,Aarskog syndrome,Silver-Russell syndrome

Zeynep Şıklar, Merih Berberoğlu. Syndromic Disorders with Short Stature. J Clin Res Pediatr Endocrinol. 2014; 6(1): 1-8
Manuscript Language: English
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