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Agenesis

The Experts below are selected from a list of 297 Experts worldwide ranked by ideXlab platform

Nancy Mann – 1st expert on this subject based on the ideXlab platform

  • pulmonary Agenesis a predictor of ipsilateral malformations
    American Journal of Medical Genetics, 1997
    Co-Authors: Michael L Cunningham, Nancy Mann

    Abstract:

    Pulmonary Agenesis is a rare malformation that can be isolated or associated with other anomalies. We became interested in pulmonary Agenesis after evaluation of a child with right pulmonary Agenesis, an unlobed left lung, bilateral cleft lip and palate, maxillary and mandibular hypoplasia, bilateral microtia, bilateral radial ray hypoplasia, horseshoe kidney, and complex congenital heart disease. A review of the occurrence of pulmonary Agenesis with other congenital anomalies uncovered a striking association with ipsilateral radial ray defects and/or hemifacial microsomia. The presence of bilateral facial or radial ray anomalies was indicative of bilateral pulmonary involvement. A review of the cases of pulmonary Agenesis and associated anomalies at the Children’s Hospital and Medical Center confirmed the association of pulmonary Agenesis and ipsilateral involvement of face and/or radial ray. The association of pulmonary Agenesis and ipsilateral malformations may shed light on its pathogenesis. Although the cause of these associated anomalies remains unclear, abnormalities in the development of the aortic arches during embryogenesis is an attractive hypothesis. Am. J. Med. Genet. 70:391–398, 1997. © 1997 Wiley-Liss, Inc.

Michael L Cunningham – 2nd expert on this subject based on the ideXlab platform

  • pulmonary Agenesis a predictor of ipsilateral malformations
    American Journal of Medical Genetics, 1997
    Co-Authors: Michael L Cunningham, Nancy Mann

    Abstract:

    Pulmonary Agenesis is a rare malformation that can be isolated or associated with other anomalies. We became interested in pulmonary Agenesis after evaluation of a child with right pulmonary Agenesis, an unlobed left lung, bilateral cleft lip and palate, maxillary and mandibular hypoplasia, bilateral microtia, bilateral radial ray hypoplasia, horseshoe kidney, and complex congenital heart disease. A review of the occurrence of pulmonary Agenesis with other congenital anomalies uncovered a striking association with ipsilateral radial ray defects and/or hemifacial microsomia. The presence of bilateral facial or radial ray anomalies was indicative of bilateral pulmonary involvement. A review of the cases of pulmonary Agenesis and associated anomalies at the Children’s Hospital and Medical Center confirmed the association of pulmonary Agenesis and ipsilateral involvement of face and/or radial ray. The association of pulmonary Agenesis and ipsilateral malformations may shed light on its pathogenesis. Although the cause of these associated anomalies remains unclear, abnormalities in the development of the aortic arches during embryogenesis is an attractive hypothesis. Am. J. Med. Genet. 70:391–398, 1997. © 1997 Wiley-Liss, Inc.

Enting Wu – 3rd expert on this subject based on the ideXlab platform

  • unilateral lung Agenesis detrimental roles of surrounding vessels
    Pediatric Pulmonology, 2007
    Co-Authors: Ankou Chou, Shuchien Huang, Shyhjye Chen, Peiming Huang, Joukou Wang, Meihwan Wu, Yihsharng Chen, Chungi Chang, Ingsh Chiu, Enting Wu

    Abstract:

    Summary. Unilateral lung Agenesis is a rare congenital defect and could be associated with multiple abnormalities. The patients usually have poor long-term outcomes especially in those with right lung Agenesis. We reviewed the 10-year experience in our hospital to describe special clinical features and try to delineate the causes of poor outcomes. From 1995 to 2005, 14 patients less than 18 years of age with unilateral lung Agenesis (4 with left Agenesis, 10 with right Agenesis) were enrolled. Medical records reviewed included diagnosis, presentation, chromosome anomalies, cardiovascular anomalies and interventions, outcomes. We found that the mechanisms of severe airway disease in right lung Agenesis included (1) trachea compression by the aortic arch, (2) the presence of ‘‘pseudo–ring-sling complex,’’ (3) distended pulmonary artery due to left to right shunt which impinged the only bronchus, and finally (4) the persistent LSVC that restricts the growth of trachea. The etiologies of airway complication in left lung Agenesis included anomalous aortic arch compression on trachea and the coexisting heart disease with significant left to right shunt, which impinged on the bronchus. In conclusion, unilateral lung Agenesis has frequently associated airway problems due to its surrounding vessels. Satisfactory airway intervention remains challenging. This disease still requires great effort to improve patient outcomes. Pediatr Pulmonol.