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[A case of idiopathic pulmonary hemosiderosis of adult onset].

A 51-year-old man presenting with hemoptysis was admitted to our hospital. Chest radiography revealed air space consolidation in the right lung field. Laboratory data showed anemia, hypoxemia, and no evidence of inflammatory signs, bleeding tendency, renal dysfunction, or collagen vascular diseases. Tests of anti-GBM antibody, P-ANCA, and C-ANCA were negative. Microscopic examination of the lung tissue specimens obtained by video assisted thoracic surgery revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli. No deposition of immunoglobulin and vasculitis were seen. These findings were consistent with a diagnosis of idiopathic pulmonary hemosiderosis. Steroid therapy had a limited effect, and the patient died. Idiopathic pulmonary hemosiderosis of adult onset is rare in Japan.

Hemosiderosis↗

[Liver hemosiderosis in chronic hepatitis].

Liver hemosiderosis (LH) was studied in chronic hepatitis (CH) B and C and CH of other and alcoholic (AL) etiology. LH was present in 122 patients (a study group) and was absent in 188 patients (a comparison group). LH is considered to be a marker of organ iron overload. The total incidence of LH was 39.4 +/- 2.7%, including 33.3 +/- 5.6% in LH-B, 49.3 +/- 5.8% in LH-C, and 48.7 +/- 8.0% in AL. The association with LH was characterized by a longer duration of the disease, by a higher activity and degree of hepatic cirrhosis. The efficiency of reaferon therapy was lower in hepatitis B and C in the presence of LH. These forms of the disease are considered to be secondary hemosiderosis.

Hemosiderin↗

[Inhaled corticosteroids in idiopathic pulmonary hemosiderosis: 2 cases].

Idiopathic pulmonary hemosiderosis, a rare clinical entity, is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. It is usually treated with systemic corticosteriod during acute bleeding episodes and with prolonged courses of oral corticosteroids and/or other immunosuppressant drugs for maintenance therapy. Because prolonged treatment with these agents is frequently associated with significant side effects, favorable response to inhaled corticosteroids would be ideal in this condition. The authors reports 2 cases of idiopathic pulmonary hemosiderosis diagnoses in the pediatric department of Sfax: a 20 month old boy and an 8 year old girl successfully treated with inhaled beclomethazone dipropionate using doses of 500_g/day and low dose oral prednisolone (0.5 mg/kg one day/2).

Administration, Inhalation↗

[Progressive bilateral hearing loss with superficial hemosiderosis of the central nervous system: contribution of cochlear implantation].

Two cases of progressive bilateral hearing loss associated with superficial hemosiderosis of the central nervous system are reported. This is a rare disease caused by repetitive hemorrhage in subarachnoid spaces with hemosiderin deposits on the brain surface and cranial nerves. MRI provided the etiologic diagnosis in both cases based on typical low-density signals from the brain, the brainstem, and the cerebellar surfaces on T1 and T2 sequences. In one case a fourth ventricle ependymoma, which was the probable cause of hemosiderosis, was also discovered. Based on a literature review, we discuss the pathophysiological hypotheses, the modalities for treating hearing loss including cochlear implantation despite retrocochlear sensorineural hearing loss.

Adult↗

[Two cases of idiopathic pulmonary hemosiderosis: analysis of chest CT findings].

Chest CT findings are reported in two cases of idiopathic pulmonary hemosiderosis. In both cases, CT was performed after remission of an acute exacerbation following corticosteroid therapy. Case 1 was a 17-year-old woman with Down's syndrome. Chest radiograph showed diffuse ground-glass like and reticulonodular shadows, which were predominant in the bilateral lower lung fields. Chest CT showed a diffuse increase of lung filed density, especially in the dorsal zone of both lower lobes. Open lung biopsy revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli, and in addition, marked fibrous thickening of the alveolar septa. Case 2 was a 7-year-old girl. Chest radiograph showed diffuse micronodular shadows in both lungs. Chest CT showed diffuse poorly-circumscribed micronodular lesions with uniform distribution, and lung field density was normal except for the right upper lobe with patchy infiltrates. Although lung biopsy was not performed, fibrous thickening of the alveolar septa was presumed to be mild even if present, since pulmonary function and blood gas analysis were within normal limits. In these two cases, lung field density of CT seemed to reflect the degree of diffuse fibrous thickening of the alveolar septa, and it is suggested that CT is valuable in the evaluation of fibrous thickening of the alveolar septa secondary to recurrent pulmonary hemorrhages. Comprehensive review of CT findings of idiopathic pulmonary hemosiderosis was also performed.

Adolescent↗

[The association of pulmonary hemosiderosis and celiac disease. Apropos of a new case in a child].

A new case of pulmonary hemosiderosis with coeliac disease is reported. This is an extremely rare combination of which only nine instances have been published over the last 20 years. Three of the reported cases occurred in children. Apart from a marked predominance of males, the combination has no specific features. Firm evidence of a causal relationship between the two diseases is lacking but treatment with a gluten-free diet alone apparently had beneficial effects on the lung disease in two patients. Three pathogenic hypotheses are discussed herein: deposition of circulating immune complexes involving food allergens on the basement membrane of alveolar capillaries; reaction between antireticulin antibodies and an alveolar basement membrane antigen; or effect of adenovirus 12, a potential causative factor for celiac disease. Patients with idiopathic pulmonary hemosiderosis should routinely have tests for gluten intolerance, for instance a lactulose-mannitol intestinal permeability test. Lastly, other concomitant food allergies are reported.

Celiac Disease↗

Life threatening exacerbation in idiopathic pulmonary hemosiderosis salvaged by cyclophosphamide infusion.

A seven-year-old girl presented with frequent fever, cough and shortness of breath of three months duration. On the basis of her clinical features, peripheral blood and sputum findings, she was diagnosed as a case of idiopathic pulmonary hemosiderosis. After initial stabilisation with steroids and chloroquine, she presented four years later with massive pulmonary hemorrhage and respiratory failure, which responded dramatically to cyclophosphamide infusion. The rare occurrence of pulmonary hemosiderosis and different treatment regimens is discussed.

Child↗

[Pulmonary hemosiderosis. Hemodynamic or idiopathic origin? Diagnostic contribution of imaging and review of the literature].

We report two cases of Pulmonary Hemosiderosis. The first patient (adult male) had an angiosarcoma of the left auricle and developed pulmonary arterial hypertension, was treated by surgery and chemotherapy. The other patient (adult female) suffered from Idiopathic Pulmonary Hemosiderosis (IPH) and had been treated by corticosteroids for fifteen years, with one acute episode during this period. We discuss the etiological diagnosis and the contribution of chest radiographs, CT-scan and MR Imaging in this rare illness according to literature. IPH occurs mostly in childhood with acute and fatal issue, whereas presents as a chronic form in adults. Assessment of IPH requires large investigations to eliminate hemodynamic, hemato-, immuno-, carcinological disorders, and infectious agents as well. Review.

Adult↗

Quantitative evaluation of chlortoluran-induced splenic hemosiderosis by means of an image analyzer.

This study investigated a technique for the quantification of hemosiderin layed down in the spleen. The model used was the rat to which the urea-based herbicide chlortoluran was fed for 90 days. There was good correlation between hematological, biochemical, histological and image analytical techniques when splenic hemosiderosis was of a moderate to severe degree. Where hemosiderosis was of a slight nature, only the technique of image analysis was able to detect the on-going changes.

Animals↗

[Anatomoclinical approach in idiopathic pulmonary hemosiderosis. Apropos of 12 cases].

Idiopathic pulmonary hemosiderosis (IPH) is an infrequent condition whose severe and unpredictable prognosis justifies extensive etiologic investigations. We have assembled 12 cases of IPH (nine children), three adults). In seven patients, pulmonary bleeding was demonstrated upon bronchoalveolar bleeding was demonstrated upon bronchoalveolar lavage (BAL) that recovered numerous hemosiderin-laden alveolar macrophages (Golde index: 247 +/- 53). All 12 patients underwent a surgical lung biopsy. Light microscopy studies showed hemosiderosis, often with lymphoid hyperplasia (n = 11), and occasionally with large germinal centers (n = 4), interstitial mastocytes (n = 7), ferric tattoo of the elastic network (n = 4), and a variable degree of interstitial necrosis (n = 4). Ultrastructural studies were performed in six cases and showed swelling of capillary endothelial cells (n = 5), interruptions in the endothelium (n = 3), tattoo of basement membranes (BMs) and elastic tissue (n = 3), intracapillary platelet aggregates (n = 2), and focal thickening of capillary BMs (n = 2). Four biopsies were studied using immunofluorescence (IF): no deposits of immune complexes were found. Indirect IF assays for antiglomerular and alveolar BMs was performed in two patients and was negative in both.

Adolescent↗

Fatal Yersinia enterocolitica septicemia complicated by unique hemosiderosis. A case report.

A fatal case of Yersinia enterocolitica septicemia which was complicated by unique hemosiderosis is reported. On admission, the patient had diabetes and showed unusual hyperferritinemia. Postmortem examination revealed that the liver was studied with abscesses, and Yersinia antigen was expressed in foamy macrophages within these abscesses. Moreover, the cadaver showed generalized hemosiderin deposition, which was mainly observed in the liver and, to lesser degrees, in the pancreas, spleen, lymph nodes, brain, thyroid and kidneys. Since there was no apparent cause of the hyperferritinemia and generalized hemosiderosis, consideration was given to possible primary hemochromatosis. However, no liver or pancreatic fibrosis was demonstrated. Kupffer cells were also loaded with hemosiderin, and therefore it was considered that these cells had lost their ability to phagocytize the microorganism adequately, leading to major liver involvement. Interestingly, hemosiderin deposits in the kidneys were mostly present in glomerular epithelial cells. To our knowledge, this unique presentation has not been previously described in humans.

Adult↗

[Pulmonary hemosiderosis in children. Immunologic study].

Circulating anti alveolar basement membrane antibodies have been detected by indirect immunofluorescence on lung rat in two out of five children presenting with pulmonary hemosiderosis. The two cases are reported and seem to be consistent with an auto-immune disease due to anti-basement membrane antibodies. Authors outline the presence of anti-reticulin antibodies in the two cases reported and the high level of IgE which seems a frequent finding in pulmonary hemosiderosis.

Animals↗

[Frequent occurrence of Yersinia infection in hemosiderosis].

Antibodies against Yersinia were found in 12 of 50 patients with hemosiderosis, in 11 of 47 patients with thalassemia major and in one of three patients with Blackfan-Diamond anemia. All patients were treated with subcutaneous continuous deferoxamin-infusions. A systemic yersiniosis occurred in seven patients, all with homozygous beta-thalassemia, in five during and in two before treatment with deferoxamin. Hemosiderosis and infusions with deferoxamin seem to increase the risk of yersinia septicemia.

Adolescent↗

[Idiopathic pulmonary hemosiderosis in children. A report on 7 cases (author's transl)].

The authors describe 7 cases of idiopathic pulmonary hemosiderosis occurring in children, and emphasize the diagnostic value of the association of pulmonary and anemic manifestations, one of which could appear to be an isolated finding and thus delay diagnosis. Complementary investigations are discussed and the relative inocuity of a lung biopsy with a Gerbeaux needle, and the greater value of bronchoscopic samples over those obtained by gastric intubation for evaluating the presence of siderophagic activity emphasized. Functional pulmonary tests, especially for blood gases and ventilatory function, were also conducted. The difficulties in classifying the various types of pulmonary hemosiderosis, especially the "idiopathic" form in children, are outlined. Immunological disorders must be systematically searched for in order to choose the most effective therapy: corticoids or immunodepressants.

Child↗

Increased IgD milk antibody responses in a patient with Down's syndrome, pulmonary hemosiderosis and cor pulmonale.

IgD antibody responses to cow's milk were investigated in a two-year-old black boy with evidence of pulmonary hemosiderosis and pulmonary hypertension. Initially a broad spectrum of immunologic responses to cow's milk were observed including IgD, IgE, and precipitin antibodies. Specific IgD antibody responses to cow's milk could be modulated in terms of challenge or elimination and correlated with the clinical course. It is possible that IgD antibodies may be important in milk-related pulmonary hemosiderosis.

Animals↗

[Idiopathic pulmonary hemosiderosis (case report)].

A case is presented, of idiopathic pulmonary hemosiderosis in a female aged 19 years. The clinical onset occurred at the age of 3 years and the evolution was characterized by subfever, dispnoea, coughing and cyanosis. Radiological examination revealed reticulation and uniform distribution of nodules in both lungs. Other laboratory investigations have revealed hypochromic anaemia, restrictive syndrome and reduction of the maximal ventilation rate with marked arterial hypoxemia. Pulmonary bioptic puncture was performed and the patient died three days after puncture. Histopathologic examination of the bioptic sample and of necroptic specimens revealed fibrotic interstitial nodules filled with siderophages. The small and the median arteries displayed fragmentation of the elastic fibers, and hyperelastosis. The changes oberved allowed to make an exact diagnosis of idiopathic pulmonary hemosiderosis.

Adult↗

Hemosiderosis in a dialysis patient: treatment with hemofiltration and deferoxamine chelation therapy.

Although the highly permeable membranes utilized in hemofiltration are theoretically more permeable to deferoxamine-chelated iron than the standard cuprophan membranes used in conventional hemodialysis, no clinical data support this contention. Ours are the first published results of a preliminary short-term trial of combined therapy with deferoxamine and hemofiltration in a dialysis patient with hemosiderosis. An average of 15.3 mg of iron was mobilized with a 19.5-liter exchange over only 4 1/2 hours of postdilution hemofiltration. This compares favorable with previous reports in which 8 to 12 hours of dialysis were performed with Kiil dialyzers, and also with the 24-hour urinary excretion of chelated iron in iron-overloaded patients with normal renal function. We conclude that combined therapy with deferoxamine and hemofiltration offers promises as an effective means of iron mobilization in dialysis patients with hemosiderosis.

Aged↗