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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An autopsy case of mixed connective tissue disease (MCTD) with pulmonary hypertension is presented. A 34-year-old woman suffering from arthralgia, Raynaud's phenomenon, and dyspnea of 6-years duration was diagnosed as having MCTD on the basis of a high titer (1:160,000) of serum antibody to the ribonuclease-sensitive component of extractable nuclear antigen. Examination of cardiac function revealed the complication of pulmonary hypertension. Autopsy revealed concentric intimal cellular proliferation of the small arteries and arterioles of both lungs. Typical plexiform lesions of these vessels were also observed. These findings coincide with those of plexogenic pulmonary angiopathy of primary pulmonary hypertension (PPH). This is the second autopsy case of MCTD with fatal pulmonary hypertension reported and our observations suggest that some cases with PPH who had immunological abnormalities but could not be classified as cases of classical collagen disease, may have been induced by MCTD.
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PMID:Mixed connective tissue disease with fatal pulmonary hypertension. 715 44

Wasted ventilation fraction (Vd/Vt) normally declines substantially during exercise in persons without lung disease. Failure of Vd/Vt to decrease during exercise has been reported to be one of the earliest abnormalities in patients with dyspnea caused by pulmonary vaso-occlusive disease, suggesting that measurement of Vd/Vt at rest and during exercise are useful in the diagnosis of pulmonary vascular disorders. We studied pulmonary hemodynamic and Vd/Vt responses to exercise in 11 patients in the supine position with suspected pulmonary vascular involvement caused by progressive systemic sclerosis, systemic lupus erythematosus, or recurrent pulmonary emboli, 10 of whom had dyspnea at rest and/or on exertion. In contrast to previous reports of no change or an increase in Vd/Vt during exercise in patients with pulmonary vascular disease, we found Vd/Vt to decrease significantly during exercise in 8 of 9 patients in whom mean pulmonary artery pressures were abnormally elevated at rest and/or during exercise. Our findings suggest that normal responses of Vd/Vt to exercise do not exclude hemodynamically significant pulmonary vaso-occlusive disease.
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PMID:Lack of sensitivity of measurements of Vd/Vt at rest and during exercise in detection of hemodynamically significant pulmonary vascular abnormalities in collagen vascular disease. 723 73

A clinico-pathological study was done to elucidate characteristic features of subacute interstitial pneumonia. The patients were four men and mine women, with a mean age of 60 years. In ten patients, the disease was idiopathic, three had collagen vascular disease, (and one was undergoing gold therapy for rheumatoid arthritis). The time interval between onset of symptoms and open lung biopsy was 80 +/- 40 days. Eleven patients had progressive dyspnea, seven had coughing, and only one complained of fever. Fine crakles were heard in ten patients. Mild increases in CRP were observed in all cases. Mild increases in total serum IgG concentration were observed in five of eight cases. Multiple patchy infiltration or diffuse interstitial shadows, located predominantly in the lower fields of both lungs were the characteristic chest roentgenographic findings. The average %VC was 62.7 +/- 17% and the average PaO2 was 68.3 +/- 10 Torr. Bronchoalveolar lavage was done in nine patients, and the mean total cell count was 16.5 +/- 10.2 x 10(4)/ml. A moderate increase in lymphocytes (30.8 +/- 18.6%) with a low CD4/8 ratio (0.48 +/- 0.57), a mild increase in neutrophils (6.2 +/- 9.1%), and a mild increase in eosinophils (2.3 +/- 3.7%) were observed. Pathologically, interstitial cellulo-fibrous changes associated with alveolar space closure due to organization of exudate were the main features. Patients were given steroid pulse therapy or oral steroids. The results were mild to marked improvements in chest roentgenographic findings and lung function.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical features of subacute interstitial pneumonia--clinico-pathological study based on open lung biopsy findings]. 756 95

The pattern of change in arterial oxyhemoglobin saturation (SpO2) measured by pulse oximetry (PO) during exercise, including daily activities, was studied in 23 patients with interstitial lung disease (ILD), 19 of whom had idiopathic pulmonary fibrosis, 3 collagen vascular disease, and 1 BOOP. Hypoxemia, as detected by PO appeared without dyspnea at the beginning of exertion or at a mild workload. Exercise usually induced significant changes in the same fashion first in heart rate, then in SpO2, and then in dyspnea, but stair climbing had a different pattern. Workload at the lactate threshold (LT) and symptom-limited maximal exercise (SL) in a bicyclergometer incremental exercise test correlated well with distance walked in a 10-minute walking test (10-eMD) (p < 0.01). Similarly, the degree in desaturation in each exercise test was closely correlated (p < 0.01). Exertional hypoxemia was more prominent in patients with ILD than in those with chronic pulmonary emphysema. Nevertheless, dyspne changed less per change in SpO2 in ILD patients. We conclude that prolonged monitoring of SpO2 by PO will disclose the presence of exertional desaturation in ILD patients.
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PMID:[Exertional hypoxemia evaluated by pulse oximetry in patients with interstitial lung diseases]. 760 20

One hundred and seventy nine children reporting to the paediatric cardiac clinic were studied consecutively over a 2 year period (1991-1993) using medical history, physical examination, chest x-ray, electrocardiography, echocardiography and complete haemogram. 100 (56%) of them had congenital heart disease, 70(39%) had rheumatic heart disease, four had cardiomyopathy and in five children no abnormality was detected. 58 of patients with congenital heart disease were females and 42 were males. Their age ranged between two weeks and 15 years and four months. Symptoms started in infancy in 89% of 94 symptomatic patients. The commonest symptoms were breathlessness, failure to thrive, repeated chest infections and cynosis. The other six patients were detected incidentally. Almost all types of defects were represented, the commonest being ventricular septal defect (VSD), Fallot's tetralogy, pulmonary stenosis, patent ductus arteriosus, atrioventricular septal defect and secondum atrial septal defect. Five patients with VSD were preterms. 91% of patients with VSD were symptomatic. VSD was small in one-third of patients. six patients with VSD aged 5-11 years had evidence of pulmonary hypertension, one of whom had Eisenmenger with cynosis and one found inoperable because of pulmonary vascular disease. Twenty patients had extracardiac malformations in 65% of whom more than one system was involved. 53% patients were anaemic. 47% of patients were underweight and 33% were marasmic while only 14% of controls were underweight and none of them was marasmic.
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PMID:Pattern of congenital heart disease in Sudanese children. 787 91

A 26-year-old woman with systemic lupus erythematosus (SLE) developed dyspnea and hypoxemia on exertion. She died from rapidly progressive respiratory failure. Autopsy revealed right ventricular hypertrophy and occlusion of the pulmonary veins compatible with pulmonary venoocclusive disease (PVOD). Although PVOD has been reported in patients with suspected collagen vascular disease, this appears to be the first reported case of PVOD occurring in a patient with SLE.
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PMID:Pulmonary venoocclusive disease in a patient with systemic lupus erythematosus. 801 50

A 16-year-old girl developed dyspnea 9 years after surgery for patent ductus arteriosus with pulmonary hypertension (pulmonary to systemic peak systolic pressure ratio; Pp/Ps 1.0). The postoperative course had been uneventful with a decreased Pp/Ps. It was revealed this time, however, that the pulmonary arterial pressure again elevated to be 186/133 mmHg (Pp/Ps 1.90). Postoperative progression of pulmonary angiopathy was first suspected. Whereas, the findings with pulmonary perfusion scintigraphy and arteriography were remarkably different between the right and left lungs, suggesting that the progression of pulmonary hypertension was not caused by that of pulmonary angiopathy but by the association of thromboembolism in small pulmonary arteries. The patient succumbed to intractable cardiopulmonary failure, strongly appealing for the necessity of organ-transplantation therapy in Japan. This case also indicated the particular importance of a close follow-up of the cases with pulmonary hypertension remaining after corrective surgery.
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PMID:[Late progression of pulmonary hypertension in a patient operated for patent ductus arteriosus: pulmonary angiopathy or thromboembolism?]. 803 83

The safety of autologous blood donation by "high-risk" patients (those with some preexisting medical conditions) has been questioned. The authors reviewed 1393 consecutive blood donation records (207 high-risk autologous [HRA], 665 non-high-risk autologous [NHRA], and 521 directed donors [DD]) to determine the safety and outcome of blood donation by HRA patients as compared with other donors at their center. The HRA group included patients with a history of significant coronary artery on cerebral vascular disease, recent seizures, cardiac arrhythmia, chronic heart failure, valvular or congenital heart disease, symptomatic dyspnea, insulin-dependent diabetes and/or current therapy with two or more antihypertensive medications. Those designated NHRA were all other autologous donors; DD met all criteria for homologous donation. Donor characteristics including predonation hematocrit, pre- and postdonation mean arterial pressure and heart rates were similar in all groups. Eight HRA donors (3.9%) had reactions, compared with 21 NHRA (3.2%) and 23 DD (4.4%), a difference that was without statistical significance. The reaction rate in all autologous donors (HRA and NHRA) was 3.4%. No differences in symptoms reported, hemodynamics or reaction severity were observed among the three groups (P > .05). A multiple logistic regression was performed within and among the groups with the risk factor categories listed above and medication classes including beta blockers, cardiac glycosides, calcium-channel blockers, antihypertensive agents, nitrates, and antiarrhythmic agents (chi 2 = 14.9; P = .0006). Only first-time donation (P = .0001) and cardiac glycoside usage (P = .04) were positively associated with an untoward reaction. The authors conclude that donation by HRA donors is at least as safe as that by donors who meet homologous donation criteria in their population and setting.
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PMID:Comparable safety of blood collection in "high-risk" autologous donors versus non-high-risk autologous and directed donors in a hospital setting. 787 63

We review current concepts about the clinical manifestations, diagnosis and treatment of patients with bronchiolitis obliterans (BO) with emphasis on clinical/pathological correlations and recent developments. BO is a relatively rare disease, but its incidence is probably higher than generally believed and is continuously rising, partly because of better recognition, but also because of increased exposure to industrial fumes, and its occurrence in lung transplantation. BO is characterized histologically by varying degrees of obliteration of the lumen of the respiratory bronchioles by organizing connective tissue often extending into the alveoli ('proliferative' BO with organizing pneumonia--BOOP) or by more extensive fibrosis and scarring of the more proximal, conductive bronchioles ('constrictive' BO). Diverse clinical conditions have been associated with the development of BO, notably viral and mycoplasma infection, toxic fume exposure and immune reactions in the setting of a collagen vascular disease, drug reaction or organ transplantation. The clinical course and features of BO may vary considerably according to the aetiology, histological pattern and stage of the disease. The most common presentation is that of a progressive dry cough and dyspnea, associated with diffuse patchy interstitial lung infiltrates on chest X-ray. In the more advanced cases, lung function tests show either restrictive or obstructive defects, depending on the extent of alveolar involvement, and hypoxemia without CO2 retention. The diagnosis is often possible on clinical grounds, however, in a seriously ill patient uncertainty should be resolved by tissue diagnosis, preferably by open lung biopsy. Treatment is based on symptomatic therapy. The use of corticosteroids is controversial, but common. Patients with BOOP are exceptional, in that there may be no underlying condition ('idiopathic' BOOP or cryptogenic organizing pneumonia--COP), a restrictive ventilatory defect is usual and the response to corticosteroids often remarkable.
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PMID:Bronchiolitis obliterans--current concepts. 814 Feb 11

Neurological and vascular complications of Arnold-Chiari malformation treated with ventriculoatrial shunting may result in sudden or unexpected death. Two patients with Arnold-Chiari malformation and ventriculoatrial shunting had variable clinical manifestations and diagnostic difficulties. A 3-year-old girl with a 1-day history of right-sided heart failure died unexpectedly soon after cardiac catheterization. At autopsy examination an adherent thrombus around the ventriculoatrial catheter tip, pulmonary infarction, and embolic pulmonary arterial hypertensive changes were found. In the second case, a 21-year-old man died suddenly after a brief episode of dyspnea. He had a 1-year history of "asthma" before death. Autopsy examination confirmed pulmonary infarction and embolic pulmonary arterial hypertensive changes. There was no histological evidence of asthma. Deaths in both cases were due to pulmonary infarction stemming from thromboemboli derived from ventriculoatrial catheterization. Both patients had evidence of long-standing clinically unsuspected vascular disease, which may have contributed to death. Cardiac catheterization may also have precipitated death in the first patient. Other possible problems leading or contributing to sudden death in such patients include pulmonary hypertension with chronic cor pulmonale, airway obstruction from recurrent laryngeal nerve paralysis, and shunt blockage with acute hydrocephalus. Lethal brainstem compression may also accompany relatively minor trauma associated with chronic cerebellar tonsillar herniation in these patients.
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PMID:Mechanisms of sudden death and autopsy findings in patients with Arnold-Chiari malformation and ventriculoatrial catheters. 887 Aug 79


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