CRANIOFACIAL AND DENTAL CHARACTERISTICS OF SILVER-RUSSELL SYNDROME

Citation
J. Kotilainen et al., CRANIOFACIAL AND DENTAL CHARACTERISTICS OF SILVER-RUSSELL SYNDROME, American journal of medical genetics, 56(2), 1995, pp. 229-236
Citations number
38
Categorie Soggetti
Genetics & Heredity
ISSN journal
01487299
Volume
56
Issue
2
Year of publication
1995
Pages
229 - 236
Database
ISI
SICI code
0148-7299(1995)56:2<229:CADCOS>2.0.ZU;2-H
Abstract
We found significant differences in a craniometric, cephalometric, and dental study of 19 Silver-Russell syndrome patients (13 without growt h hormone treatment) with appropriate controls, Although head circumfe rence was normal for age, head length was increased, while cranial and facial widths and facial heights were reduced, Posterior facial heigh t, posterior cranial base length, cranial base height, and mandibular body size were significantly smaller than in healthy children of the s ame height, Articulatory speech disorders were common, Enamel defects pointed to an early prenatal insult, Delayed dental age and small mand ibular and cranial base dimensions support the possibility of physiolo gical growth hormone deficiency in many Silver-Russell syndrome childr en; however, facial soft tissue structures were strikingly different f rom those observed in classical growth hormone deficiency. (C) 1995 Wi ley-Liss, Inc.