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A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome

Journal of the Korean Pediatric Society 1997;40(6):877-882.
Published online June 15, 1997.
A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome
Dong Hee Lee1, Won Bae Kim1, Jung Hye Choi1, Su Nam Lee2
1Department of Pediatrics, Inchon Christian Hospital, Inchon, Korea
2Department of Pathology, Inchon Christian Hospital, Inchon, Korea
Noonan 증후군 환자에서 발생한 선천성 폐림프관 확장증 1례
이동희1, 김원배1, 최정혜1, 이수남2
1인천기독병원 해부병리과
2인천기독병원 해부병리과
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.
Key Words: Congenital pulmonary lymphangiectasia, Noonan syndrome


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