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.
Format:	Artikel
Sprache:	eng
Schlagworte:	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
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Zusammenfassung:	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.
ISSN:	8755-6863 1099-0496
DOI:	10.1002/ppul.1950140211