Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chylothorax in the absence of tumor or trauma is uncommon. Lymphangiomatosis of the bone, although extremely rare, has been associated with chylothorax. The authors describe the case of a 12-year-old boy who presented with a symptomatic left chylothorax associated with lymphangiomatosis of the ribs, scapula, and clavicle. Despite tube thoracostomies and the initiation of total parenteral nutrition, massive losses of chyle persisted, resulting in hypoproteinemia and severe lymphopenia. Control of the chylothorax was achieved by a parietal pleurectomy and application of fibrin glue (Tisseel). In the literature there are 16 cases of chylothorax associated with lymphangiomatosis of the bone. Their presentation, treatment, and outcome are reviewed. Conservative treatments such as dietary manipulations or thoracenteses were rarely successful. Thoracotomy with parietal pleurectomy on the side of the effusion is usually effective in controlling the chylothorax. Lymphangiomatosis should be considered a diagnostic possibility for any child who presents with a chylothorax.
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PMID:Massive chylothorax associated with lymphangiomatosis of the bone. 780 41