Craniofacial and dental characteristics of Silver-Russell syndrome.
We found significant differences in a craniometric, cephalometric, and dental study of 19 Silver-Russell syndrome patients (13 without growth hormone treatment) with appropriate controls. Although head circumference was normal for age, head length was increased, while cranial and facial widths and facial heights were reduced. Posterior facial height, posterior cranial base length, cranial base height, and mandibular body size were significantly smaller than in healthy children of the same height. Articulatory speech disorders were common. Enamel defects pointed to an early prenatal insult. Delayed dental age and small mandibular and cranial base dimensions support the possibility of physiological growth hormone deficiency in many Silver-Russell syndrome children; however, facial soft tissue structures were strikingly different from those observed in classical growth hormone deficiency.
Kotilainen
J
J
Department of Pedodontics and Orthodontics, University of Helsinki, Finland.
Hölttä
P
P
Mikkonen
T
T
Arte
S
S
Sipilä
I
I
Pirinen
S
S
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D001184:Articulation Disorders / Q000235:genetics / Q000473:pathology; D001827:Body Height / Q000235:genetics; D016022:Case-Control Studies; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D002886:Chromosomes, Human, Pair 17; D003744:Dental Enamel Hypoplasia / Q000235:genetics / Q000473:pathology; D005145:Face / Q000002:abnormalities / Q000473:pathology; D005260:Female; D005799:Genes, Dominant; D013006:Growth Hormone / Q000172:deficiency; D006801:Humans; D008297:Male; D009154:Mutation; D012886:Skull / Q000002:abnormalities / Q000473:pathology; D013577:Syndrome; D014070:Tooth / Q000254:growth & development / Q000473:pathology
D013006:Growth Hormone