Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 31-year-old woman was admitted to our hospital because of dyspnea on exertion. She had had the same symptom a few years earlier. A chest radiograph revealed ground-glass-like shadows on both sides, and the arterial PaO2 was 54 Torr when the patient was breathing room air. We first suspected hypersensitivity pneumonitis, but the dyspnea and hypoxemia did not resolve during treatment in the hospital, and returned home. She felt no obvious changes in symptoms while at home. We found no antibodies to Tricosporon cutaneum in her serum. Computed tomography of the chest revealed diffuse pan-lobular areas of high density. Bronchoalveolar lavage was done and the total cell count was 9.6 X 10(7). About three quarters (73%) of the recovered cells were lymphocytes. Examination of a transbronchial biopsy specimen showed alveolar thickening with infiltration of small mononuclear cells. No granulomas or Massons bodies were seen, and no antibodies specific to collagen-vascular disease were detected. We therefore excluded the diagnosis of hypersensitivity pneumonitis, idiopathic interstitial pneumonia and collagen-associated pneumonitis. There was no evidence of drug-induced pneumonitis. She was seropositive for HTLV-I. We suspected that this was a case of HTLV-1-associated pneumonitis.
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PMID:[Interstitial pneumonia in an HTLV-I carrier]. 910 56

A 22-year-old first man came to our hospital because of dyspnea on exertion in February 1993, and was admitted in September 1994 because of progression of dyspnea. A chest roentgenogram showed diffuse ground-glass-opacities in the middle and lower lung fields, and an elevated diaphragm. Pulmonary-function testing revealed a low %VC and a low diffusing capacity. Examination of a specimen obtained by thoracoscopic lung biopsy revealed usual interstitial pneumonia. Immunohistochemical examinations showed the expression of intercellular adhesion molecule-1 on vascular endothelial cells and on alveolar epithelial cells. Dust inhalation and collagen vascular disease were ruled out and the diagnosis was idiopathic interstitial pneumonia. This condition develops only rarely in patients under 60 years old.
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PMID:[Juvenile onset of idiopathic interstitial pneumonia]. 916 50

Primary pulmonary hypertension (PPH) is a pulmonary vascular disease characterized by an elevation in mean pulmonary artery pressure and pulmonary vascular resistance. Recently, PPH gained national attention because of its association with appetite suppressants. PPH may also be associated with pregnancy, hypothyroidism, autoimmune disorders, human immunodeficiency virus infection, and the use of drugs such as oral contraceptives and cocaine. Patients with PPH may report dyspnea on exertion and fatigue. Early diagnosis is crucial. New therapeutic regimens have dramatically reduced mortality rates and improved quality of life by halting the progression of pulmonary vascular remodeling and averting right-sided heart failure. These therapies include high-dose calcium channel antagonists, anticoagulants, and continuous intravenous prostacyclin. Lung or heart-lung transplantation remains a viable therapeutic option for patients who are treated late in the disease process, who are not responsive to medical management, or who remain symptomatic and continue to deteriorate.
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PMID:Diagnosing and treating primary pulmonary hypertension. 1050 69

Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon disease with a severe prognosis. Initially considered as a constantly fatal disease, it can be cured since the advent in the late 80's of thromboendarterectomy. CTEPH occurs in approximately 0.1% of patients who survive acute pulmonary embolism. Such outcome is due to the failure of the normal thrombi resolution in the pulmonary circulation. This disease is observed in both sexes and occasionally as early as in the third decade. Many patients have no history of acute venous thromboembolism which is responsible for the frequent diagnosis delay. Therefore, it is of paramount importance to search for pulmonary vascular disease when patients complain solely of dyspnea on exertion. These patients should undergo echocardiography and, if necessary, right heart catheterization. Once the diagnosis of pulmonary hypertension is established the next step is to find the cause. Ventilation-perfusion scanning is probably the most sensitive non-invasive test to provide evidence that pulmonary hypertension is related to chronic thromboembolism. Angiography and helical computed tomography allow to confirm the diagnosis of CTEPH and to determine whether it is accessible or not to thromboendarterectomy. Most patients who undergo thromboendarterectomy improve clinically and in terms of gas exchange and pulmonary hemodynamics. When thrombi are inaccessible surgically, patients should be placed on the list for lung transplantation if they fulfill the criteria established for primary pulmonary hypertension. When all surgical procedures are contraindicated, anticoagulant and oxygen therapy remain the sole possibility of treatment.
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PMID:[Chronic thromboembolic pulmonary arterial hypertension]. 1090 51

A 53-year-old woman was admitted to our hospital in May 1999, because of progressive dyspnea and liver dysfunction. She had been receiving the replacement therapy of thyroid hormone for thirteen years and suffering from Raynaud's phenomenon for 9 years. She experienced exertional dyspnea and sicca symptom for 3 years, and had an episode of syncope 4 months before admission. An echocardiogram showed dilation of the right ventricle, tricuspid regurgitation and the estimated mean pressure of the pulmonary artery was higher than 120 mmHg. She was diagnosed as having severe pulmonary hypertension (PH) complicated with primary Sjogren's syndrome and primary biliary cirrhosis without portal hypertension She was treated with anticoagulant (warfarin) and oral prostagrandin I2 (prostacyclin). However, right heart failure and jaundice gradually progressed and she suddenly died in December 1999. At autopsy, the heart was enlarged with right ventricular hypertrophy. Small arteries and arterioles in the lung showed concentric intimal proliferation and severe plexogenic vascular disease. Deposition of immunoglobulin was not observed in the pulmonary arteries. Since the prognosis of PH is poor, it is important to analyze the etiology of the disease for the development of the treatment.
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PMID:[Pulmonary hypertension in a patient with primary Sjogren's syndrome, Hashimoto's disease, and primary biliary cirrhosis]. 1115 16

The cardiopulmonary exercise test (CPET) is frequently used for the quantitative evaluation of exercise tolerance and for the qualitative assessment of the origin of dyspnea. Although reasonably safe, CPET is contraindicated in acute clinical situations and relatively contraindicated in severe pulmonary hypertension. Pulmonary emboli are usually present during the acute onset of pulmonary hypertension; however, in situations where pulmonary vascular disease is more unsuspected, CPET may help with the diagnosis. We present a report of a 47-year-old Caucasian female with a 2-month history of exertional dyspnea. She had a normal pulmonary function test, arterial blood gas analysis and CT scan of the chest. On CPET she was found to have a higher than normal physiological dead space to tidal volume ratio, increased ventilatory equivalent for the elimination of CO(2) (V(E)/VCO(2)), persistently positive arterial-end-tidal PCO(2 )difference at peak exercise, and a low anaerobic threshold suggestive of pulmonary vascular disease. She was subsequently diagnosed with pulmonary embolic disease by her ventilation/perfusion scan and pulmonary arteriogram. This report highlights the importance of recognizing the hallmarks of unsuspected pulmonary vascular disease on CPET.
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PMID:Abnormalities on cardiopulmonary exercise test in a dyspneic patient. A case report of unsuspected pulmonary embolism. 1245 9

A 63 year-old female presented with dyspnea on exertion. Her chest X-ray showed cardiomegaly, and right ventricular overload and tricuspid regurgitation were detected. Her pulmonary ventilation and blood flow scintigraphy findings were suspicious of pulmonary vascular disease; the diagnosis was pulmonary hypertension and bilateral branch pulmonary artery stenosis. After the inflammation settled, the stenotic bilateral branch pulmonary artery was reconstructed with a prosthetic vessel and the pulmonary pressure normalized immediately. A resected specimen revealed that the stenotic changes were from Takayasu's disease. The patient's postoperative course was uneventful, and pulmonary ventilation and blood scintigraphy returned to an almost normal range. At follow-up 5 years and 6 months after the operation, there was no evidence of pulmonary artery disease (eg, stenosis and/or ischemia) or of any change in the central vessels of the retina, the so-called Takayasu's retinopathy.
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PMID:Reconstruction of bilateral branch pulmonary artery stenosis caused by Takayasu's aortitis. 1527 40

A 70-year-old man with a past history of lung resection for early stage lung cancer was admitted to our hospital because of worsening exertional dyspnea. Right heart catheterization revealed severe pulmonary arterial hypertension (PAH) with pulmonary vascular resistance of 1671.64 dyne.sec.cm(-5). The patient was treated with sildenafil added to an oral prostacyclin analog, beraprost, and long term oxygen therapy. His exertional dyspnea continued to improve until his sudden death following nasal bleeding. Autopsy revealed marked thickening of pulmonary arteriolar walls, but no recurrence of lung cancer, significant pulmonary embolism or pulmonary parenchymal disease. His PAH could not be explained by the mild airway obstruction or sleep apnea syndrome, and unrelated pulmonary vascular disease was suspected.
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PMID:[Unexplained pulmonary arterial hypertension in a patient with lung resection, treated with sildenafil and beraprost]. 1714 79

Hereditary hemorrhagic telangiectasia (HHT) is a hereditary vascular disease with multiple arterial venous malformations. Pulmonary arterial hypertension is occasionally associated with HHT; however, there is no evidence of targeted therapy for this subgroup of patients. This report describes a 37-year-old woman who presented with dyspnea on exertion. She was finally diagnosed with pulmonary arterial hypertension associated with HHT (HHT2, MIM 600376), and had multiple arterial venous malformations in the lung, liver and pancreas. An oral dual endothelin receptor (ET(A)/ET(B)) antagonist, bosentan, was prescribed, and her symptoms, exercise capacity and laboratory findings improved greatly after treatment. Pulmonary arterial pressure also decreased. Endothelin receptor antagonist is a possible treatment option for pulmonary arterial hypertension associated with HHT.
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PMID:Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia. 2113 5

Pulmonary vascular diseases encompass a large and diverse group of underlying pathologies ranging from venous thromboembolism to congenital malformations to inflammatory vasculitides. As a result, patients can present either acutely with dyspnea and chest pain or chronically with dyspnea on exertion, hypoxia, and right heart failure. Imaging, particularly with multidetector CT, plays a key role in the evaluation and management of patients with suspected pulmonary vascular disease and, given the widespread routine use of high-quality CT pulmonary angiography, it is imperative that radiologists be familiar these pathologies.
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PMID:Pulmonary vascular diseases. 2602 2


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