Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphangiomas are regarded as malformations arising from sequestration of lymphatic tissue that fail to communicate with the lymphatic system. Lymphangiomatosis is defined as a pathological condition where either multiple lymphangiomas are present or multiple organ systems are involved. We report a 30-year-old male who presented with cough, hemoptysis and severe dyspnea as the result of progressive chylothorax and chylopericardium. Despite three months of treatment with total parenteral fat-free nutrition, octreotide, repeated pleurocentesis, and pleuroperitoneal shunting, a life-threatening deterioration of his disease developed. Radiologic findings included multiple bilateral cystic lesions in the mediastinum, hili, pleura and pericardium, along the lymphatic vessels. Although general experience with surgical treatment of lymphangiomatosis is limited, our patient nevertheless made an almost complete functional recovery after pulmonary lymphangiectomy. Thus, preoperative risk assessment based primarily on radiologic findings may be misleading. To our knowledge this is the first report in the literature to indicate that surgery should be an option in patients with multiorgan lymphangiomatosis.
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PMID:Successful surgical treatment of thoracic multiorgan lymphangiomatosis. 1992 Nov 32

Lymphangiomatosis (eg, generalized lymphatic anomaly) is an abnormal proliferation of lymphatic endothelial cells. It is often a childhood disease, but it may present in adulthood by infiltrating organs and cause obstruction, bleeding, or disruption of lymphatic flow. Pulmonary involvement may be mild or cause diffuse interstitial lung disease, airway obstruction, hemoptysis, chylothorax, chylopericardium, and culminate in respiratory failure. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy. This report presents a patient with lymphangiomatosis and life-threatening hemoptysis in whom positive immunostaining forc-KITsuggested upregulation of tyrosine kinase and whose disease was controlled with imatinib.
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PMID:Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly). 2705 87