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A case is reported of an infant with features of Noonan Syndrome and congenital pulmonary lymphangiectasis. Soon after birth, persistent respiratory distress developed, and, at 4 months of ge, a chylothorax was noted. Medium chain triglyceride therapy by the enteral route was tried, but parenteral alimentation and chest-tube drainage were required. Diagnosis of pulmonary lymphangiectasis was confirmed by biopsy.
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