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

A 17-year-old boy with pulmonary veno-occlusive disease underwent Swan--Ganz catheterization. A normal pulmonary capillary wedge pressure was recorded in the presence of severe pulmonary arterial hypertension and roentgenographic evidence of pulmonary venous congestion. This triad of findings permitted an unequivocal diagnosis of pulmonary veno-occlusive disease, which was later confirmed at autopsy. The hemodynamics of this condition and of others included in the differential diagnosis are presented schematically.
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PMID:Pulmonary veno-occlusive disease: antemortem diagnosis from roentgenographic and hemodynamic findings. 45 8

Pulmonary veno-occlusive disease has recently been recognized as a distinct pathological entity and a cause of pulmonary arterial hypertension. Twenty previously reported cases and a new patient are here reviewed. The majority presented with breathlessness and in the early stages of the disease, when the abnormal signs were not striking, some patients were wrongly diagnosed as suffering from an anxiety state. The condition usually has an insidious onset but is remorselessly progressive and since no effective treatment is available at present, invariably fatal and the majority of patients have died within two years. The fully developed clinical picture is dominated by symptoms and signs of pulmonary arterial hypertension, similar to those found with other diseases causing a raised pulmonary arterial blood pressure. However, some patients with pulmonary veno-occlusive disease show, in addition, signs of pulmonary venous and capillary hypertension, which can lead to its clinical recognition when associated with a normal left atrial blood pressure. In this condition the pulmonary wedge pressure would appear to be unreliable as a record of the left atrial blood pressure. Pulmonary angiography and lung scanning will differentiate pulmonary veno-occlusive disease from massive thromboembolic pulmonary arterial hypertension but not from primary pulmonary arterial hypertension or micro thromboembolism. Although in some patients it should now be possible to recognise pulmonary veno-occlusive disease in life, there will be others where, even after full investigation, it will still be impossible to differentiate the condition from primary pulmonary arterial hypertension or micro thromboembolism and in these the diagnosis will only be made when the distinctive histological pattern of the disease is demonstrated. In pulmonary veno-occlusive disease there is a widespread occlusion of the pulmonary veins and venules by a loose intimal fibrosis which is often basophilic. Recanalization of the occluded veins is common and in some cases may be very striking. These occlusive lesions in the pulmonary veins lead to an elevation of pulmonary arterial pressure with associated disease of these vessels, and are also responsible for chronic oedema of the elveolar walls with subsequent development of interstitial pulmonary fibrosis. In the present case organised thrombi were present in the pulmonary arteries in addition to the pulmonary venous lesions.
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PMID:Pulmonary veno-occlusive disease. 115 89

In order to determine the incidence and causes of death during the first 100 days after BMT (early deaths) in a pediatric population we have examined data reported in the AIEOP BMT Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306) BMT were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic HLA matched and 38% for autologous transplants at 5 years, 24% for HLA mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after BMT: 33/306 (11%) after autologous BMT, 24/150 (16%) after allogeneic matched BMT and 13/30 (43%) after mismatched BMT. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic BMT); infection: 12 children (five after autologous and seven after allogeneic BMT); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic BMT); cardiac failure: five children (four after autologous BMT); veno-occlusive disease: eight children (three after autologous, five after allogeneic BMT); acute renal failure: three children (one after autologous and two after allogeneic BMT); multiple organ failure: two cases (one after autologous BMT); cerebral hemorrhage: three children (one after autologous BMT); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic BMT).
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PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3

Pyrrolizidine alkaloids such as monocrotaline are bioactivated in the liver, resulting in veno-occlusive disease of the liver, pulmonary arterial hypertension, and right ventricular hypertrophy. We have searched for the formation of a reactive, alkylating pyrrole intermediate in the metabolism of monocrotaline by isolated rat liver microsomes, using the sulfhydryl-containing resin, thiopropyl sepharose 6B, as a trapping agent. Control experiments show that a toxic, chemically reactive, alkylating pyrrole such as dehydromonocrotaline binds covalently to the resin via a thioether bond, but that a less toxic, poorly alkylating pyrrole, such as dehydroretronecine, does not. Isolated hepatic microsomes metabolize monocrotaline to produce a pyrrole that binds to the resin, and that can be detected by means of the Ehrlich color reagent (p-dimethylaminobenzaldehyde). The pyrrole is releasable by silver nitrate treatment, thereby establishing it to be bound via a thioether linkage. In buffered ethanolic silver nitrate the major product is 7-ethoxy-1-hydroxymethyl-6,7-dihydro-5H-pyrrolizine (O7-ethyldehydroretronecine). This establishes that the thioether linkage is at the 7-position. The same product is obtained on release of the resin-bound pyrrole formed from the reaction of dehydromonocrotaline with the resin, thereby establishing the intermediacy of dehydromonocrotaline in the metabolism of monocrotaline.
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PMID:Detection of a reactive pyrrole in the hepatic metabolism of the pyrrolizidine alkaloid, monocrotaline. 164 51

Qualitative and quantitative studies were performed on pulmonary blood vessels in lung tissue obtained by biopsy, pneumonectomy, or autopsy from 58 patients in the Registry of Primary Pulmonary Hypertension sponsored by the Heart, Lung, and Blood Institute of the National Institutes of Health. In 49 patients (84%), the hypertensive vascular disease involved predominantly or exclusively muscular pulmonary arteries and arterioles. In each of these 49 patients, pulmonary artery medial hypertrophy was observed, and in 48 patients, it was also associated with intimal or luminal lesions. On the basis of the predominant histopathologic features, 25 of the 48 patients were classified as having pulmonary arteriopathy with plexiform lesions characterized by a combination of concentric laminar intimal fibrosis, eccentric intimal fibrosis, and plexiform lesions; in nine of these 25, recanalized thrombi were also present. Pulmonary arteriopathy with thrombotic lesions, defined by the presence of both eccentric intimal fibrosis and recanalized thrombi but without plexiform lesions, was observed in 19 patients. Intimal fibrosis, either concentric or eccentric, without plexiform or thrombotic lesions was found in four patients. Among the remaining nine patients in the Registry, pulmonary veno-occlusive disease was present in seven and chronic pulmonary venous hypertension in one. Pulmonary blood vessels were microscopically normal in a lung biopsy specimen from another patient. In general, patients with plexiform lesions and those with veno-occlusive disease had a much poorer prognosis than patients with thrombotic lesions. The present study shows the existence of several distinct histopathologic patterns of pulmonary vascular disease in individuals with primary pulmonary hypertension diagnosed by standardized clinical and laboratory criteria.
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PMID:Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. 280 80

This paper is a review of current data on a rare pathology which causes primary pulmonary arterial hypertension: pulmonary veno-occlusive disease. The manifestations of the disease consist of dyspnoea of progressive onset, crepitations in the lower pulmonary lobes and diffuse interstitial syndrome with Kerley's B lines. These signs are associated with severe hypoxia and severe pulmonary arterial hypertension with paradoxically normal wedge pressure. Pathological specimens must be obtained to confirm the diagnosis. They show thrombosis of pulmonary veins less than 2 mm in diameter and sometimes of arterioles, presence of connective tissue with few cells and images of recanalization, muscularization of pulmonary arterioles, lesions of interstitial nodular fibrosis and presence of haemosiderin-rich macrophages. The disease is frequently associated with other pathologies, including heart disease, blood disease and pulmonary capillary haemangiomatosis. In recent years, veno-occlusive disease has been found to occur immediately after chemotherapy for cancer and bone marrow or kidney transplantation. Three physiopathological hypotheses have been put forward to explain the disease: infection, autoimmune reaction and toxic reaction.
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PMID:[Veno-occlusive disease of the lung]. 331 49

A 25-year-old patient had unilateral absence of the right pulmonary artery (UARPA) and severe left pulmonary artery hypertension. After death from congestive right heart failure, autopsy revealed histologic signs of pulmonary veno-occlusive disease (PVOD) and pulmonary hypertension (PH). An accessory arterial vessel that was thrombotically occluded was found connecting the ascending aorta and the right pulmonary hilum. There was also histologic evidence of arterial thrombi within the right lung arterial vascular bed. The PH in UARPA usually occurs very early during the course of disease. From histologic findings and medical history, it is likely that in this case, late-onset elevation of pulmonary pressures was triggered by the occurrence of PVOD. This is the first case of UARPA and PVOD--a congenital unilateral arterial malformation in the presence of bilateral involvement in a possibly acquired venous obliterative disease.
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PMID:Pulmonary veno-occlusive disease in a patient with unilateral absence of right pulmonary artery. 337 Nov 12

Four cases of pulmonary veno-occlusive disease are described. Two patients, who were brothers, had respiratory tract infections. The third patient had chronic active hepatitis and coeliac disease suggesting an abnormality of the immune system; the fourth patient had no obvious cause but presented initially with systemic hypertension. Three of the cases had been diagnosed initially as primary pulmonary hypertension either on open lung biopsy or clinically. In all cases the pulmonary arteries were abnormal with medial hypertrophy, intimal fibrosis and, in some cases, thrombosis in elastic pulmonary arteries. These findings suggest that pulmonary veno-occlusive disease is not confined to veins and should be considered as a widespread pulmonary vascular disease. The range of aetiological factors indicate that it should not be considered as a single disease entity.
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PMID:Pulmonary veno-occlusive disease. A report of four cases. 378 91

Among 80 cases with a clinical diagnosis of primary (unexplained) pulmonary hypertension, 45 (56%) had thromboembolic disease and 22 (28%) had plexogenic arteriopathy; the remaining 13 (16%) had pulmonary veno-occlusive disease, primary medial hypertrophy, primary pulmonary arteritis, or changes consistent with pulmonary venous hypertension. The mean age was 16 years for primary pulmonary arteritis, 21 to 34 years for plexogenic pulmonary arteriopathy, primary medial hypertrophy, and pulmonary veno-occlusive disease, and 41 and 45 years for thromboembolic disease and pulmonary venous hypertension, respectively. In all forms except pulmonary veno-occlusive disease and apparent pulmonary venous hypertension, female patients were involved twice as often as male patients. With the exception of apparent pulmonary venous hypertension, patients with plexogenic pulmonary arteriopathy had the longest survival (63 months). Sudden death, however, occurred most frequently in patients with plexogenic disease (45%) and occurred 2.5 times as often in this group as in patients with thromboembolic disease. Among our 80 cases, the most frequent histopathologic lesions were medial hypertrophy, intimal proliferation and fibrosis, fibrinoid degeneration and necrosis, and thrombosis. Thrombi were commonly observed and may have developed in situ or by embolization; they were often rich in platelets when they occurred in small pulmonary vessels. These histologic features may form the morphologic substrate for elevated pulmonary vascular resistance, and their recognition may provide the rationale for possible intervention with pulmonary vasodilators, anticoagulants, or platelet inhibitors.
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PMID:Primary pulmonary hypertension: a histopathologic study of 80 cases. 396 21

We report the case of a patient with transient hypertension in relation to acute veno-occlusive disease of the liver. Ascites and esophageal varices, both transient, occurred in this patient and a transient elevation of the gradient between wedged and free hepatic venous pressures was observed.
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PMID:Transient intrahepatic portal hypertension. A case report. 721 16


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