- Case Report
- A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome
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Dong Hee Lee, Won Bae Kim, Jung Hye Choi, Su Nam Lee
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Clin Exp Pediatr. 1997;40(6):877-882. Published online June 15, 1997
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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... |
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