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