Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic pulmonary hemosiderosis gives rise to anemia, due to repeated intra-alveolar hemorrhage, the reabsorption of which leads to hemosiderin deposits in the lung parenchyma. The authors report a case in a young woman aged 24 years whose illness started with anemia, then two months later, with hemoptysis and a broncho-pulmonary syndrome with a low grade fever. On the 6th month, there occurred a hazy infiltrate of both lung bases which was fleeting, mobile and recurrent. In the light of this triad of anemia, hemoptysis and infiltrates, the diagnosis of idiopathic pulmonary hemosiderosis was made and confirmed by three examinations:--Lung biopsy: siderophages were found in the sub-mucosa,--Radio-isotope examination, using Fe 59 which revealed iron deposits in the lung,--A surgical lung biopsy which showed a congestive area and a fibrous area. The congestive area was the site of recent hemorrhage, the alveolar limits were filled with siderophages. The fibrous area was the site of chronic repair of older hemorrhage. It was mutilating. The course was complicated by massive bleeding which led to acute recovering respiratory failure. The patient is at present stabilised by corticosteroids. Three hundred cases of idiopathic pulmonary hemosiderosis have been reported in the world literature. Although the main characteristic is intra-alveolar hemorrhage, its course has not yet been determined. It seems however, to be due to an immunologic process as shown by the relationship between this curious disease and Goodpasture's syndrome.
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PMID:[Idiopathic pulmonary hemosiderosis. Apropos of 1 case]. 18 18

Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.
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PMID:Familial idiopathic pulmonary hemosiderosis. 37 18

A male forestry worker presented with chest pain followed by severe continuing haemoptysis and an extensive bilateral nodular pulmonary infiltrate. A needle biopsy of lung demonstrated micronodular deposits of malignant tissue. The patient died from respiratory failure. Necropsy showed a disseminated haemangiosarcoma arising in the right atrium. Haemoglobin and serum iron levels were normal. Electron microscopy of the lung biopsy showed a close relationship between tumour cells and basement membrane and suggested that haemorrhage occurred directly from the tumour nodules. The ultrastructure of alveoli adjacent to tumour deposits was normal. This case provides further indirect evidence that the clinical and histological features of idiopathic pulmonary haemosiderosis cannot be explained by the mere occurrence of alveolar haemorrhage.
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PMID:Pulmonary haemorrhage in disseminated cardiac haemangiosarcoma. 55 87

A 35-year old man with cough, hemoptysis, and dyspnea was found to have diffuse pulmonary infiltrates and iron-laden macrophages in the sputum. Pulmonary siderosis was confirmed by transbronchial biopsy. An associated hypochromic anemia required frequent transfusion. Though marrow iron stores were absent, reticulocytosis was maintained. Corticosteroid therapy resulted in cessation of hemoptysis, clearing of the pulmonary infiltration, and a substantial reduction in transfusion requirement. Splenectomy was of no benefit. The patient developed cerebral symptoms with seizures, and rapid deterioration led to cerebral symptoms with seizures, and rapid deterioration led to death. Disseminated hemangiosarcoma was found at autopsy. Steroid responsiveness of the associated pulmonary siderosis suggests that it had an immune basis.
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PMID:Angiosarcoma with pulmonary siderosis and persistent reticulocytosis. Steroid responsiveness suggests an immune basis. 56 58

The paper is concerned with observations over 3 patients in whom unusual vasculitis lay at the basis of the clinicopathological manifestations. All the patients were men of the young age. The disease debut was marked by fever, weakness, dyspnea, palpitation, cough, hemoptysis, the articulation syndrome. In two cases, there was hemorrhagic rash on the leg skin. All the patients manifested liver and spleen enlargement, two patients had lymphoadenopathy. The leading clinical symptoms included dilated cardiomyopathy, complete blockade of the inferior peduncle of His bundle and reduction of myocardial contractility. Anemia belonged to iron deficient one. The clinical examples provided indicate that immunocomplex vasculitis with evident lesions of the lungs and myocardium, not going into criteria for the known diseases, is not likely to be a casuistic rarity. Those syndromes may be associated with more or less pronounced hemosiderosis of the lungs (and, probably, of the lymph nodes, spleen and liver), with transitory or steady derangements of myocardial conduction, which attests to diffuse lesions of the myocardium possibly with both immune complexes and hemosiderin. The pathology requires further studies.
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PMID:[Generalized immune-complex vasculitis combined with pulmonary hemosiderosis and dilated cardiomyopathy]. 214 20

We have reviewed the alveolar hemorrhage (AH) syndromes, defined as immune or idiopathic disorders associated with diffuse microvascular hemorrhage into the acinar portion of the lung. The disorders that are most often associated with AH include antibasement membrane antibodies (ABMA) disease, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus, systemic vasculitides, and idiopathic rapidly progressive glomerulonephritis. An approach to the recognition, diagnosis, and treatment of the AH syndromes has been outlined and several illustrative case studies have been presented. Recognition of AH is not usually difficult, but does require a high index of suspicion, since many disease processes may give rise to hemoptysis with infiltrates on chest roentgenogram. Recognition of AH is aided by careful clinical and laboratory assessment for evidence of extrapulmonary disease; simple hematologic studies such as sequential hemoglobins and iron studies; and measurement of carbon monoxide uptake by the lungs. Early recognition of AH may decrease the likelihood of respiratory failure and end-stage renal disease. The specific etiology of AH is usually determined by clinical examination, serologic assay for ABMA, and percutaneous renal biopsy by immunofluorescence. Open-lung biopsy is required in a minority of cases. High-dose pulse methylprednisolone appears to effectively control AH of diverse etiology. Combined plasma exchange and immunosuppression controls AH in ABMA disease and is the treatment of choice in this disorder. Cyclophosphamide is used for Wegener's granulomatosis, and sometimes in systemic necrotizing vasculitis, in an attempt to prevent irreversible damage to the kidneys.
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PMID:Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders. 639 80

During the 3 decades 1950-1979 onset of idiopathic pulmonary haemosiderosis occurred in 10 Swedish children. Complete records were available from the eight children with onset during 1960-1979, which indicates that the yearly risk of onset is 0.24 case per million children. The first symptoms started at the mean age of 5.8 years (range: 10 months-11 years). From the beginning all children had a severe microcytic, hypochromic, sideropenic anaemia. Pulmonary symptoms were present from the beginning in four children (but haemoptysis in only one) and developed in the remaining children after 1 1/2-2 1/2 years. Various therapeutical regimens were tried. Iron therapy seemed of temporary beneficial effect and most children seemed to benefit from prednisone therapy during disease bouts, although the effect of the therapy in the long run could not be determined. The four children with onsets during the 1960s died of their disease after 2-13 years. The four children with onset during the 1970s are still alive. One of them--a 20-year-old female, has for two years complete clinical remission and has normalized haematological, X-ray and pulmonary function data.
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PMID:Idiopathic pulmonary haemosiderosis in Swedish children. 648 74

The aim of this study was to assess quantitatively the relationship between the number of iron-laden macrophages, extent of hemosiderin content and amount of dark-stained hemosiderin clumps in bronchoalveolar lavage (BAL) fluids. The series included specimens from 20 subjects whose BAL had yielded iron-laden macrophages. Six had developed hemoptysis 3-15 days prior to BAL. Quantitative studies were performed on smears stained by the ferrocyanide reaction, using true color image analysis for image segmentation. A top-hat function based on mathematical morphologic concepts was used for extraction of dark clumps of hemosiderin. The results demonstrated the presence of iron-laden macrophages three days after hemoptysis. There was a moderate correlation between the percentage of macrophages and amount of hemosiderin content, but the extent of dark-stained clumps did not increase in parallel to the increase in hemosiderin content and probably represented an advanced stage in the phagocytic processing of hemosiderin formed from engulfed red blood cells. The main difference between subjects with and without previous hemoptysis was higher values for dark clumps of hemosiderin in the former.
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PMID:Image analysis of hemosiderin-laden macrophages in bronchoalveolar lavage fluid. 801 Oct 36

For 6 weeks a 52-year-old woman had complained of increasing fatigue, blood-streaked vomitus, retrosternal burning and pain between the shoulder blades. Physical examination showed no abnormalities. Blood sedimentation rate was increased to 15/40 mm and the iron concentration was slightly reduced. Computed tomography demonstrated densities in the left upper lobe of the lung and both lower lobes. Scintigraphy revealed a perfusion defect in the left apex of the lung while bronchoscopy demonstrated acute bronchitis in the left upper lobe. Further haemoptysis occurred 3 months later, but several bronchoscopies failed to elucidate their cause. Three days later another haemoptysis caused respiratory arrest. After resuscitation the bleeding was localized to the right main bronchus, and the right upper and middle lobes were resected. The patient died the next day from a massive haemoptysis. Post-mortem examination showed angiodysplasia in all lobes of the lung. The branches of the pulmonary artery were dilated, their wall was irregular and the muscular tunica media reduced. The elastic lamellae were fragmented and there were cell-rich intimal pads. These changes most closely resembled fibromuscular dysplasia.
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PMID:[Pulmonary angiodysplasia with fatal pulmonary hemorrhage]. 851 18

Idiopathic pulmonary haemosiderosis (IPH) is a disorder characterised by the triad of haemoptysis, diffuse parenchymal infiltrates on chest roentgenogram and iron-deficiency anaemia. It is a diagnosis of exclusion and the prognosis is bleak despite the varied management options. We report a case of IPH occurring in a child who presented at four months of age with cough, wheeze, haemoptysis and pallor and whose symptoms are currently controlled with high-dose inhaled budesonide and low-dose oral prednisolone.
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PMID:Idiopathic pulmonary haemosiderosis--a case report. 971 27


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