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Familial Occurrence of Congenital Pulmonary LymphangiectasisGenetic Implications
Ajovi B. Scott-Emuakpor, MD, PhD;
Stephen T. Warren, PhD;
Saroj Kapur, MD;
Ernesto B. Quiachon, MD;
James V. Higgins, PhD
Am J Dis Child. 1981;135(6):532-534.
Abstract
Congenital pulmonary lymphangiectasis (CPL) is a rare, generalized disease of the lung, consisting of lymphatic cysts in the subpleural and interlobular connective tissue. This disorder typically manifests a clinical picture of acute respiratory distress with cyanosis shortly after birth, with death occurring in the neonatal period. Several cases of this disorder have been described in the literature, but there has been no family with more than one affected child. We report the first instance, to our knowledge, of familial cases of CPL, which raises an important question regarding a possible genetic component in this disorder. The implications of this are discussed.
(Am J Dis Child 1981;135:532-534)
Author Affiliations
From the Department of Pediatrics and Human Development, College of Human Medicine, Michigan State University, East Lansing (Drs Scott-Emuakpor, Warren, Kapur, and Higgins); and the Department of Pathology, Ingham Medical Center, Lansing, Mich (Dr Quiachon).
Footnotes
Reprint requests to Department of Pediatrics and Human Development, B240 Life Sciences, Michigan State University, East Lansing, MI 48824 (Dr Scott-Emuakpor).
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