Document Detail


Autosomal dominant aplasia cutis in three generations and one case with preaxial polydactyly in the last generation.
MedLine Citation:
PMID:  21614989     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Aplasia Cutis Congenita (ACC), characterized by the focal absence of the skin and skin adnexia resulting from a developmental failure, may occur as part of Adams-Oliver Syndrome (AOS) which can be defined as a congenital inherited disorder, consisting of terminal transverse limb defects and vascular anomalies in addition to ACC. Coexistence of isolated preaxial polydactyly without terminal extremity defect and ACC is extremely rare. Furthermore, ACC and preaxial polydactyly has not been reported previously. Here we report a three-generation family with autosomal dominant aplasia cutis congenita and preaxial polydactyly in the last generation and discuss whether it is a coincidence or not.
Authors:
B Yağci-Küpeli; K Qağlar; S Büyük; S Balci
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Genetic counseling (Geneva, Switzerland)     Volume:  22     ISSN:  1015-8146     ISO Abbreviation:  Genet. Couns.     Publication Date:  2011  
Date Detail:
Created Date:  2011-05-27     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9015261     Medline TA:  Genet Couns     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  55-61     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ihsan Doğramaci Children Hospital 06100, Ankara,Turkey.
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