fibrosing mediastinitis |
Disease ID | 1792 |
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Disease | fibrosing mediastinitis |
Definition | Typically, people with fibrosing mediastinitis were originally infected with H. capsulatum as children but the symptoms begin in most patients between the ages of 21 and 40 years of age. There is no evidence that there is a preferred ethnic origin or gender disparity.Patients with histoplasmosis-related fibrosing mediastinitis present with signs of fatigue, shortness of breath (dyspnea), cough with blood (hemoptysis) or without, chronic chest (pleuritic) pain and recurrent pulmonary infection. Typically these symptoms occur because there is occlusion of one of the main vessels in the body such as the superior vena cava (the vein that returns blood from the head and neck, and upper limbs to the right atrium, formed in the superior mediastinum by union of two brachiocephalic veins), the central airways, or pulmonary arteries and/or veins. Superior vena cava syndrome, the swelling that develops in some patients due to obstruction of the vena cava, is a common symptom. Some patients do not have the syndrome, despite having obstruction of the SVC, if collateral alternative veins (sometimes visible on the anterior chest) enlarge sufficiently to return blood to the heart. Cough and shortness of breath are the most common symptoms when obstruction of the central airways occurs. Pulmonary venous obstruction usually presents with shortness of breath and coughing blood (hemoptysis). Symptoms can be present for years before diagnosis.Patients with idiopathic fibrosing mediastinitis may present symptoms of fever, chills, sweats, shortness of breath, cough and chest pain. They may also have fibrosis elsewhere in the body which may cause symptoms at those other sites. - NORD Reference: NORD |
Synonym | fibrosis mediastinal fibrosis of mediastinum fibrosis of mediastinum (disorder) fibrous mediastinitis fibrous mediastinitis (disorder) fibrous mediastinitis [ambiguous] mediastinal fibrosis mediastinitis sclerosing mediastinitis, sclerosing mediastinum fibrosis sclerosing mediastinitis sclerosing mediastinitis (disorder) |
Orphanet | |
UMLS | C0264573 |
SNOMED-CT | |
Comorbidity | UMLS | Disease | Sentences' Count(Total Sentences:8) C0020542 | pulmonary hypertension | 3 C0020538 | hypertension | 3 C0019655 | histoplasmosis | 2 C0036202 | sarcoidosis | 1 C0041296 | tuberculosis | 1 C0085278 | antiphospholipid antibody syndrome | 1 C0043092 | wegener's granulomatosis | 1 C0040053 | thrombosis | 1 |
Curated Gene | (Waiting for update.) |
Inferring Gene | (Waiting for update.) |
Text Mined Gene | (Waiting for update.) |
Locus | (Waiting for update.) |
Disease ID | 1792 |
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Disease | fibrosing mediastinitis |
Integrated Phenotype | (Waiting for update.) |
Text Mined Phenotype | HPO | Name | Sentences' Count(Total Phenotypes:12) HP:0000822 | Hypertension | 3 HP:0002092 | Pulmonary artery hypertension | 3 HP:0004415 | Pulmonary artery stenosis | 1 HP:0002239 | Gastrointestinal hemorrhage | 1 HP:0004936 | Blood clot in vein | 1 HP:0002584 | Intestinal hemorrhage | 1 HP:0010310 | Chylothorax | 1 HP:0002955 | Granulomatosis | 1 HP:0002617 | Aneurysmal dilatation | 1 HP:0001907 | Thromboembolic disease | 1 HP:0005200 | Retroperitoneal fibrosis | 1 HP:0003613 | Antiphospholipid antibodies | 1 |
Disease ID | 1792 |
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Disease | fibrosing mediastinitis |
Manually Symptom | UMLS | Name(Total Manually Symptoms:9) |
Text Mined Symptom | UMLS | Name | Sentences' Count(Total Symptoms:4) |
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Disease ID | 1792 |
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Disease | fibrosing mediastinitis |
Case | (Waiting for update.) |