Document Detail


Orofaciodigital syndrome.
MedLine Citation:
PMID:  22278878     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Orofaciodigital syndrome is a very rare entity with X-linked dominant inheritance characterized by oral, facial, and digital anomalies. Thirteen different types have been described in the literature to date. Of these, orofaciodigital syndrome type I has the highest incidence. Renal and central nervous system malformations may accompany the oral, facial, and digital anomalies. We report a case of orofaciodigital syndrome type I in a 9-year-old girl. The patient was admitted with a complaint unrelated to the syndrome. The coexistence of an oral anomaly with a digital anomaly in this patient led us to search for other possible anomalies. Ultrasonography revealed a diagnosis of polycystic kidneys. Physicians must be mindful of the external appearance of patients with this syndrome and be aware of life-threatening anomalies possibly associated with it.
Authors:
Baris Naiboglu; Cagatay Oysu; Tanju Gokceer
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ear, nose, & throat journal     Volume:  91     ISSN:  1942-7522     ISO Abbreviation:  Ear Nose Throat J     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-01-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7701817     Medline TA:  Ear Nose Throat J     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E8-9     Citation Subset:  IM    
Affiliation:
Department of Otorhinolaryngology Head and Neck Surgery, Haydarpasa Numune Education and Research Hospital, Acıbadem Necipbey sokak No 28, C/4 Kadikoy, İstanbul, Turkey. drbnaib@yahoo.com.
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