Immotile cilia syndrome including polysplenia, situs inversus, and extrahepatic biliary atresia
- 1 July 1989
- journal article
- research article
- Published by Wiley in American Journal of Medical Genetics
- Vol. 33 (3) , 390-393
- https://doi.org/10.1002/ajmg.1320330320
Abstract
Here we report on 2 sibs with immotile cilia syndrome (ICS), born to consanguineous Arab parents. Both had the Kartagener triad. In addition, one sib had polysplenia and extraheptic biliary atresia. This observation, together with other literature reports, suggests that the occurrence of ICS with polysplenia and extrahepatic biliary atresia represent the result of a single dysmorphogenetic process. It is concluded that polysplenia and extrahepatic biliary atresia are rare and unusual manifestations of ICS.Keywords
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