Generalized lymphangiomatosis and chylothorax in the pediatric age group

Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Trea...

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Veröffentlicht in:Pediatric pulmonology 1992-10, Vol.14 (2), p.126-130
Hauptverfasser: Shah, A. R., Dinwiddie, R., Woolf, D., Ramani, R., Higgins, J. N. P., Matthew, D. J.
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container_end_page 130
container_issue 2
container_start_page 126
container_title Pediatric pulmonology
container_volume 14
creator Shah, A. R.
Dinwiddie, R.
Woolf, D.
Ramani, R.
Higgins, J. N. P.
Matthew, D. J.
description Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation. © 1992 Wiley‐Liss, Inc.
doi_str_mv 10.1002/ppul.1950140211
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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Biological and medical sciences
Blood and lymphatic vessels
Bone
Bone Diseases - diagnostic imaging
Bone Diseases - etiology
Cardiology. Vascular system
Child
Chylothorax - etiology
Humans
immunohistochemistry
Infant
Lung - diagnostic imaging
Lung - pathology
Lymphangiectasis - complications
Lymphangiectasis - diagnosis
Lymphangiectasis - pathology
Male
Medical sciences
palliative treatment
pericardial involvement
spleen
Splenic Diseases - diagnostic imaging
Splenic Diseases - etiology
Tomography, X-Ray Computed
title Generalized lymphangiomatosis and chylothorax in the pediatric age group
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