J Korean Pediatr Soc.
1997 Jun;40(6):877-882.
A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome
- Affiliations
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- 1Department of Pediatrics, Inchon Christian Hospital, Inchon, Korea.
- 2Department of Pathology, Inchon Christian Hospital, Inchon, Korea.
Abstract
- Congenital pulmonary lymphangiectasia is a rare and perphaps underestimated cause of fatal respiratory distress in the neonate or infant. Pathologically, this condition is characterized by wide lymphatic channels in the subpleural and peribronchovascular spaces and the interlobular septa. Noonan syndrome is characterized by a phenotype similar to Turner syndrome but with a normal karyotype. Both pulmonary and intestinal lymphangiectasia have been reported in patients with Noonan syndrome. We have experienced a case of congenital pulmonary lymphangiectasia in Noonan syndrome, who presented at birth with cyanosis and persistent respiratory distress, and died on 93 postnatal days. We report this case, which was diagnosed by open lung biopsy and autopsy, with brief review of the related literature.
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