Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0020538 (hypertension)
170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 58-year-old woman with Goodpasture syndrome and active ulcerative colitis is described. On admission, the patient had exertional dyspnea, hemoptysis, severe hypertension, and peripheral edema. Her serum levels of urea nitrogen and creatinine were increased, and her hemoglobin concentration was reduced. The patient had a rapidly progressive glomerulonephritis with acute renal failure. She was treated with methylprednisolone, cyclophosphamide, and plasmapheresis but failed to regain renal function. Circulating anti-glomerular basement membrane (anti-GBM) antibody was positive; however, serum antinuclear antibody, proteinase-3-antineutrophil cytoplasm antibody and myeloperoxidase-antineutrophil cytoplasm antibody were negative. Nineteen months after initial presentation, she developed abdominal pain and severe diarrhea. These symptoms did not improve with conventional treatment. Colonoscopy performed after 3 months showed multiple ulcers in the colon. She was diagnosed with ulcerative colitis. She underwent granulocyte and monocyte adsorption apheresis once per week for 5 weeks. At 8 weeks, her symptoms had improved; her stool number was markedly decreased, and the bloody stools and abdominal pain disappeared. These results suggest that granulocyte and monocyte apheresis may be of benefit in the therapy of a patient with ulcerative colitis who previously had Goodpasture syndrome
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PMID:Granulocyte and monocyte adsorption apheresis in a patient with antiglomerular basement membrane glomerulonephritis and active ulcerative colitis. 1279 51

Primary pulmonary hypertension is a rare disease of the pulmonary vasculature manifested by dyspnea on exertion, syncope, and signs and symptoms of right heart failure. In the absence of adequate treatment, primary pulmonary hypertension has a grave prognosis, with a median survival of 2.8 years. Pulmonary arterial hypertension develops in association with known risk factors and predisposing clinical conditions, and shares many clinical, pathological and therapeutic characteristics with primary pulmonary hypertension. Therapeutic choices in pulmonary arterial hypertension depend on the etiology of the disease, severity of functional impairment and hemodynamic response following acute vasodilator administration during right heart catheterization. Agents currently approved for the specific treatment of pulmonary arterial hypertension are continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan. A small group of patients who demonstrate true acute vasoreactivity at right heart catheterization may be chronically treated with oral calcium channel blockers. In addition, most patients with pulmonary hypertension receive conventional treatment, represented by anticoagulants, diuretics, inotropic medication or oxygen supplementation. Treatment of pulmonary arterial hypertension has significantly altered the natural course of the disease, with pronounced symptomatic, functional and survival benefit. Current clinical research focuses on the discovery of new targets of therapy and the use of a combination treatment approach, which will offer hope and valuable insight into the pathogenetic basis of this devastating illness.
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PMID:Current medical treatment of pulmonary arterial hypertension. 1502 2

A 25-year-old male asylum-seeker presented with chest pain, exertional dyspnea, and orthopnea 20 years after the surgical repair of a pentalogy of Fallot. An extracardiac mass compressing the right ventricle was subsequently detected and surgical decompression was performed to relieve the resulting right intraventricular hypertension. At operation, the mass proved to be a coagulase-negative, staphylococcal abscess. In addition, the removal of the mass unmasked a previously nonrecognized pulmonary outflow stenosis that required balloon dilatation and beta-blocker therapy. While infections are known to occur after sternotomy, the formation of an abscess in the anterior mediastinum several years after the intervention appears to be exceptional; this diagnosis came to mind only after the more common complications had been considered, e.g., pseudoaneurysm or pericardial hematoma. To our knowledge, this is the first report of an abscess in the anterior mediastinum that had probably formed over many years following a sternotomy, compressed the right ventricle and masked a pulmonary stenosis.
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PMID:Precordial abscess inducing chest pain 20 years after surgical repair of a pentalogy of fallot. 1529 94

Idiopathic fibrosing mediastinitis is a rare entity involving more severely the more compliant structures within the mediastinum. In this report a rare case of simultaneous involvement of both the superior vena cava (SVC) and pulmonary veins is described in a 16--year old male with progressive dyspnea on exertion, cough and a three months' history of blood--tinged sputum. Physical examination and imaging studies revealed signs of pulmonary venous hypertension (PVH) and SVC stenosis. Fibrosing mediastinitis was confirmed by multiple biopsy samples.
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PMID:Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male. 1551 Jul 14

Chronic thromboembolic pulmonary hypertension (CTEPH), a disease associated with considerable morbidity and mortality, is the consequence of unresolved thromboembolic occlusion in pulmonary vasculature. CTEPH was considered a rare disease occurring in 0.1-0.5% of patients with pulmonary emboli who survive. Recently, a much higher incidence was reported and some risk factors such as a previous pulmonary embolism (PE), an idiopathic form of PE and the severity of perfusion defect at the time of diagnosis have been identified. Exertional dyspnea is the main symptom at the beginning of the disease while later on patients may suffer from syncope related to low cardiac output or hemoptysis as a consequence of high pulmonary artery pressure. In suspected patients, a confirmation of pulmonary arterial hypertension should be ascertained at transthoracic echocardiography. Then the obstructive nature of the disease may be revealed by ventilation-perfusion lung scan but is better described at pulmonary angiography. Computed tomography scan may be useful to rule out confounding disorders. To prevent recurrences, long-term oral anticoagulants to maintain an INR between 2.5 and 3.5 (target 3.0) are indicated. Treatment of severe CTEPH is essentially surgical (thromboendarterectomy). This procedure may be difficult when distal branches of pulmonary vascular tree are involved. In selected cases, alternative therapies may be the arterial pulmonary vessel angioplasty and lung transplantation.
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PMID:From acute pulmonary embolism to chronic thromboembolic pulmonary hypertension. 1627 Apr 75

The authors report the case of a 57-year-old patient, diagnosed with systemic sclerosis 16 years ago. She had been followed in the rheumatology outpatient clinic since 2003 and complained of marked fatigue and dyspnea on exertion. In addition to full medical evaluation, she also underwent standard Doppler echocardiography at rest, which was unremarkable, and exercise stress echocardiography. In the latter examination, she was diagnosed with pulmonary arterial hypertension, later confirmed during right heart catheterization. The authors discuss the potential value of this methodology for the study of patients with systemic sclerosis.
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PMID:Exercise stress echocardiography for detection of pulmonary arterial hypertension in a patient with systemic sclerosis. 1667 50

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

The aim of this paper was to study the spectrum of juvenile scleroderma (JSSc) seen at a tertiary care referral center in Asia. Retrospective analysis of case records of patients with systemic sclerosis, having age of onset less than 16 years and seen at our hospital from 1988 to 2004, was done. Patients with linear scleroderma and morphea were excluded. There were 23 patients (19 girls, 4 boys) with median age of onset of 12 years (range 5-16 years). The median age at presentation was 17 years (range 10-34 years). The median time from first symptoms to presentation was 4 years (range 0.2-26 years). Among these, 14 had diffuse systemic sclerosis (DSSc), while 9 had limited scleroderma (LSSc). The clinical features seen at presentation in patients were: Raynaud's phenomenon in 19, digital ulcers in 14, loss of finger tip pulp in 12, reflux in 8, dysphagia in 7, arthritis in 8, digital gangrene in 2, and pulmonary artery hypertension in 1. Antinuclear antibody was positive in 15 out of 18 patients tested. Interstitial lung disease was seen in 15 patients, 6 of whom had diffuse disease. The median skin score was 22 (range 7-48) . One patient died of primary pulmonary hypertension within 1 year of onset of symptoms. At a mean follow-up of 34 months, 14 patients were stable or had improvement in skin score or dyspnea on exertion. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good.
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PMID:Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. 1718 Feb 99

Pulmonary arterial hypertension is a group of diseases which forms a small subset of those with elevated pulmonary artery pressure (pulmonary hypertension). The recent development of selective pulmonary vasodilator has lead to a substantial resurgence of interest in what have been previously regarded as rare and incurable diseases. This review aims to describe the spectrum of pulmonary vascular diseases, the evolving understanding as to pathogenesis, the evolving evidence of efficacy for drug therapies, trying to put this into a contemporary Australian context. Several key pathogenic pathways may be involved: prostacycline, Nitric Oxide-cGMP-phosphodiesterase 5 and endothelin- all of which are exploited for therapeutic benefit by newly available drug therapies. A recently modified classification system reasserts the importance of precise diagnosis. The cardinal symptom of exertional dyspnea warrants careful evaluation in an attempt to prevent (frequently occurring) substantial delay in diagnosis. Echocardiogram is the cornerstone of screening for pulmonary arterial hypertension; however, a detailed evaluation including a carefully performed right heart catheterisation with sufficient data to allow calculation of pulmonary vascular resistance is key to accurate diagnosis. These new approaches to therapy are already substantially improving quality of life and prognosis.
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PMID:Assessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006. 1719 43

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malformations (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15-33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10-19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5-19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.
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PMID:Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). 1764 82


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