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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension characterized by thin-walled microvessels infiltrating the peribronchial and perivascular interstitium, the lung parenchyma, and the pleura. These proliferating microvessels are prone to bleeding, resulting in accumulation of hemosiderin-laden macrophages in alveolar spaces. Here we report 2 cases of PCH with pulmonary hypertension, 1 of them associated with mechanical intravascular hemolysis, a feature previously reported in other hemangiomatous diseases, but not in PCH. Case 2 was diagnosed by pulmonary biopsy; to our knowledge the patient is the second adult to be treated with interferon alpha-2a. Review of the literature identified 35 patients with PCH and pulmonary hypertension. The prognosis is poor and median survival was 3 years from the first clinical manifestation. Dyspnea and right heart failure are the most common findings of the disease. Hemoptysis, pleural effusion, acropachy, and signs of pulmonary capillary hypertension are less common. Chest X-ray or computed tomography scan usually shows evidence of interstitial infiltrates, pulmonary nodules, or pleural effusion. Hemodynamic features include normal wedge pressures. Radiologic and hemodynamic findings are undifferentiated from those of pulmonary veno-occlusive disease but differ from other causes of primary pulmonary hypertension. Epoprostenol therapy, considered the treatment of choice in patients with primary pulmonary hypertension, may produce pulmonary edema and is contraindicated in patients with PCH. Regression of lesions was reported in 1 patient treated with interferon therapy and 2 other patients stabilized, including our second patient. PCH was treated successfully by lung transplantation in 5 cases. Early recognition of PCH in patients with suspected primary pulmonary hypertension is possible based on clinical and radiologic characteristics. Diagnosis by pulmonary biopsy is essential for allowing appropriate treatment.
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PMID:Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature. 1244 98

A 12-year-old boy with polysplenia and single ventricle experienced recurrent episodes of pneumonia, hemoptysis, and pulmonary hypertension. Unilateral pulmonary vein obstruction was diagnosed, and a left pneumonectomy was performed. Microscopy of the resected specimen revealed pulmonary veno-occlusive disease in the small pulmonary venules, and old arteritis in the small pulmonary arteries.
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PMID:Severe unilateral pulmonary vascular changes in child with polysplenia. 1253 90

OBJECTIVE: To report a fatal outcome from pulmonary hemosiderosis in an infant with scimitar syndrome after prolonged pulmonary vasodilator therapy. DESIGN: Case report. SETTING: A tertiary care pediatric intensive care unit. SUBJECT: An infant with scimitar syndrome. INTERVENTIONS: Treatment included redirection of anomalous right pulmonary venous drainage and closure of atrial septal defect, assisted ventilation via tracheostomy, and protracted nitric oxide and prostacyclin therapy until his death at 1 yr of age. RESULTS: Inhaled nitric oxide (iNO) and/or prostacyclin (PGI(2)) were administered for 6.5 months. Numerous echocardiograms demonstrated good control of pulmonary pressures and no evidence of pulmonary venous obstruction. Repeated attempts to slowly wean from the pulmonary vasodilators resulted in return of pulmonary pressures to systemic levels. Although there was no clinically apparent hemoptysis, pulmonary infiltrates worsened, prompting an open-lung biopsy that revealed pulmonary hemosiderosis. During the last 4 days of the patient's life, the pulmonary hypertensive crises with suprasystemic pressures and pulmonary infiltrates worsened regardless of aggressive vasodilator therapy with iNO, PGI(2), alkalinization, and isoproterenol. Vasodilator therapy was withdrawn and the patient rapidly died. CONCLUSION: We achieved long-term control of pulmonary hypertension with iNO and/or PGI(2) without apparent tachyphylaxis or other major reported side effects. Although pulmonary hypertension was successfully controlled with prolonged iNO and intravenous PGI(2) administration in this patient with scimitar syndrome, the patient died of hypoxemic respiratory failure from pulmonary hemosiderosis. Early evaluation of roentgenographic infiltrates for hemosiderosis and potential lung transplantation in similar patients may be warranted.
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PMID:Pulmonary hemosiderosis in scimitar syndrome after prolonged management of pulmonary hypertension. 1279 55

A 69-year-old man presented with progressive dyspnea, hemoptysis and fatigue for the previous 4 weeks. Chest radiograph and CT-scan were suggestive of lung cancer. The patient's condition worsened and within in few days he developed respiratory failure and acute cor pulmonale. Despite intensive care measures directed at suspected lung thromboembolism, the patient died on day 12. Autopsy revealed disseminated obstruction of small pulmonary arteries by microscopic tumor emboli. No signs of venous thromboembolism were found. Pulmonary tumor microembolism should be considered whenever a patient with malignancy presents with unexplained progressive dyspnea or pulmonary hypertension.
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PMID:[Right heart failure due to pulmonary tumor microembolism -- a rare differential diagnosis]. 1521 32

Unilateral primitive agenesia of pulmonary artery is a rare congenital disorder. Experience of three hospital-based pulmonology pediatric units including eight children (three boys and five girls) with such anomaly is reported (11 years median follow-up, range 6 months to 10 years). Median age at diagnosis was 4 years. Clinical features included recurrent respiratory tract infections (75%), effort dyspnea (50%) and, curiously, asthmatic symptoms (75%). Diagnosis was evoked on chest X rays and confirmed by angiography in all cases. Agenesis of the right pulmonary artery was more frequent (five cases). Abnormal vascular ring was associated in two cases of agenesis of the left pulmonary artery. Pulmonary function tests were performed in six children, with bronchial obstruction in two. Regular clinical follow-up, up to adult age in four cases, showed no complication, such as hemoptysis or pulmonary hypertension in our patients.
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PMID:[Isolated agenesia of pulmonary artery]. 1535 Sep 99

Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion. The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.
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PMID:Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and a review of the literature. 1536 25

Severe thoracic sarcoidosis includes manifestations with significant clinical and functional impairment and a risk of mortality. Severe thoracic sarcoidosis can take on various clinical presentations and is associated with increased morbidity. The purpose of this article was to describe the CT findings in severe thoracic sarcoidosis and to explain some of their mechanisms. Subacute respiratory insufficiency is a rare and early complication due to a high profusion of pulmonary lesions. Chronic respiratory insufficiency due to pulmonary fibrosis is a frequent and late complication. Three main CT patterns are identified: bronchial distortion, honeycombing and linear opacities. CT can be helpful in diagnosing some mechanisms of central airway obstruction such as bronchial distortion due to pulmonary fibrosis or an extrinsic bronchial compression by enlarged lymph nodes. An intrinsic narrowing of the bronchial wall by endobronchial granulomatous lesions may be suggested by CT when it shows evidence of bronchial mural thickening. Pulmonary hypertension usually occurs in patients with end-stage pulmonary disease and is related to fibrotic destruction of the distal capillary bed and to the resultant chronic hypoxemia. Several other mechanisms may contribute to the development of pulmonary hypertension including extrinsic compression of major pulmonary arteries by enlarged lymph nodes and secondary pulmonary veno-occlusive disease. Aspergilloma colonization of a cavity is the main cause of hemoptysis in sarcoidosis. Other rare causes are bronchiesctasis, necrotizing bronchial aspergillosis, semi-invasive pulmonary aspergillosis, erosion of a pulmonary artery due to a necrotic sarcoidosis lesion, necrosis of parenchymal sarcoidosis lesions and specific endobronchial macroscopic lesions.
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PMID:CT findings in severe thoracic sarcoidosis. 1544 10

The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion with a diverse clinical presentation. If the connecting ductus arteriosus closes after birth, the ipsilateral pulmonary artery will lose its source of blood supply, resulting in hypoplasia or obliteration of intrapulmonary vessels. Despite a seemingly benign early clinical course, a significant number of untreated patients will develop pulmonary hypertension, hemoptysis, and recurrent respiratory infections. Early detection and surgical repair provides restoration of physiologic pulmonary circulation, regression of pulmonary hypertension, and the potential for normal distal pulmonary vascular development.
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PMID:Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. 1579 92

There is an increased incidence of pulmonary hemorrhage and hemoptysis among patients with congenital heart disease (CHD). The pathophysiology of pulmonary hemorrhage in CHD includes pulmonary hypertension, pulmonary venous congestion, aorto-pulmonary collaterals, pulmonary arteriovenous malformations, and dilated bronchial arteries. We present the case of a 6-year old boy who required treatment for massive hemoptysis after staged palliation for hypoplastic left heart syndrome (HLHS). Effective management of this life threatening entity is described as well as the anesthetic implications of performing rigid bronchoscopy in a patient with the Fontan circulation and massive hemoptysis.
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PMID:Severe hemoptysis in a child after the Fontan procedure. 1591 Mar 55

This report describes a case of cardiac right auricle rupture (RAR) in a flock of 11,500 broilers that were 14 days old. The birds were housed at an altitude of 300 m, with an external temperature of -10 degrees C and an internal temperature of 15 degrees C. There was 3.6% mortality, due to sudden deaths, from 10 to 14 days of age. All necropsied birds had haemopericardium due to RAR at the point of the junction with the vena cava, and 85% of them had blood in the oral cavity and external acoustic meatus. The vena cava and its caudal branches, the intestinal vessels, and the sinus durae matris and sinus saggitalis were distended. Histological examination showed haemorrhages into the myocardium, degeneration of the cardiac muscle fibres, as well as oedema of the lungs and hypertrophy of the smooth muscle bundles of the parabronchial walls. Blood in the mouth of the broilers may have been due to haemoptysis, which in humans is caused mainly by mitral stenosis. In broilers, mitral stenosis and/or insufficiency, and left ventricular failure with consequent pulmonary hypertension (PH) were considered as possible triggers for right ventricular failure. The alarm reaction in hypoxaemia, due to secondary factors such as cold, caused tachycardia and tachypnoea, may have induced further elevation of PH, and acute myocardial infarction causing cardiac rupture and haemopericardium in this case. Hypertension and PH, due to possible mitral stenosis/insufficiency in association with acute myocardial ischaemia, were probably the determinant factors causing this acute episode. This opens the possibility that the RAR may be cardiogenic.
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PMID:Rupture of the right auricle in broiler chickens. 1623 69


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