Anodontia as the sole clinical sign of the ectrodactyly-ectodermal dysplasia-cleft lip (EEC) syndrome |
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Authors: | K H Chranowska M Krajewska-Walasek Z Rump L Wisniewski J P Fryns |
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Affiliation: | Department of Human Genetics, Child Health-Centre Memorial Hospital, Warsaw. |
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Abstract: | In this report we present another family with oligosymptomatic expression of the EEC syndrome. A mother with complete absence of the permanent teeth had two children with split hand/split foot deformity, as typically seen in the EEC syndrome. Cleft lip/cleft palate was also present in one of them. The great variability in expression of this autosomal dominant syndrome is discussed and the difficulties in genetic counseling are emphasized. |
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