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Results for “HEMOSIDEROSIS”

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At least 163 records · Page 9Linked to original sources
PubMed1990

[Possibility of the development of non-transfusion hemosiderosis in beta-thalassemia, caused by association with HLA-linked hemochromatosis].

The data of HLA-haplotyping were used as an allele marker controlling hereditary hemochromatosis in 23 patients with beta-thalassemia. The results obtained have permitted a conclusion that hemosiderosis in patients with beta-thalassemia may be caused by association of beta-thalassemia gene with hereditary hemochromatosis. Early diagnosis of hyperferremia is of great prognostic importance as the adequate treatment timely conducted can prevent the development of irreversible changes in the patients.

Adolescent↗
PubMedDate not supplied

[Corneal hemosiderosis].

Two clinical cases of hematic infiltration of the cornea after severe ocular contusions are reported. Lesion of the cornea endothelium determines infiltration of the parenchyma with hemosiderin and hypofucsin, the products of hemoglobin break down. Metabolic activity of the cornea suffers, oxygen supply becomes insufficient and produces degradation of the cornea parenchyma with loss of the visual function. The affection is interpreted as a hemosiderosis process.

Adult↗
PubMedDate not supplied

Goodpasture's syndrome mimicking idiopathic pulmonary hemosiderosis.

A patient initially diagnosed by clinical findings and pulmonary biopsy as suffering from idiopathic hemosiderosis was subsequently proven by renal biopsy and serologic assay to have Goodpasture's syndrome with minimal renal alterations. The authors speculate on the relationship of these two complex disorders.

Adult↗
PubMed1989

Pulmonary hemosiderosis in a child with cystic fibrosis.

Two episodes of acute iron deficiency anemia with blood-stained sputum and symptoms of severe acute pulmonary exacerbation were observed in a child with cystic fibrosis (CF). Hemosiderin laden macrophages (siderophages) were repeatedly found in sputum and gastric juice, suggesting the coexistence of pulmonary hemosiderosis (PH). The possibility that pulmonary immune-mediated mechanisms characteristic of CF may have played a role in the development of PH is considered.

Cystic Fibrosis↗
PubMedDate not supplied

An autopsy case of idiopathic superficial hemosiderosis of the central nervous system: a microscopic and immunohistochemical study.

The brain of a patient with idiopathic superficial hemosiderosis of the central nervous system was examined by light and electron microscopy. A histopathological study revealed massive, symmetrically situated necrosis in the temporal and insular lobes, which has not been described in previous reports. In addition, immunohistochemistry revealed ovoid bodies in the astroglia. Electron microscopy showed that these bodies were composed of fine granules, 50-60 A in diameter, and electron-dense amorphous material. Based on detailed histopathological and ultrastructural findings, the mechanism of ovoid body formation is discussed.

Aged↗
PubMedDate not supplied

Deferoxamine for the treatment of hemosiderosis during CAPD.

The effects of chelation therapy by intravenous and intraperitoneal administration of deferoxamine were compared during maintenance continuous ambulatory peritoneal dialysis (CAPD) in a child with end stage renal disease and hemosiderosis. We demonstrate that intraperitoneally administered deferoxamine is safer, more practical and efficient than weekly intravenously administered deferoxamine for the treatment of iron overload in the pediatric patient undergoing CAPD.

Adolescent↗
PubMedDate not supplied

[Long-term evaluation of immunosuppressive therapy in childhood idiopathic pulmonary hemosiderosis].

Idiopathic pulmonary hemosiderosis (IPM) is a rare disease of unknown etiology, whose diagnostic, prognostic and therapeutic approach is still open to discussion. In this paper the authors report a study regarding three cases of IPH initially detected in 13, 11 and 7 year-old children. The patients were treated with cyclophosphamide and prednisone according to different cycles depending on the clinical stage in the disease. All three patients are still alive after 10, 6 and 5 years since initial diagnosis. This therapeutic protocol therefore seems to be effective in preventing the progression of IPH and in maintaining the patients in an asymptomatic condition.

Adolescent↗
PubMed1986

Integrated image and X-ray microanalysis of hepatic lysosomes in a patient with idiopathic hemosiderosis before and after treatment by phlebotomy.

Morphometrical and X-ray elemental information was extracted from Scanning Transmission Electron Microscopy (STEM) images of hepatic lysosomes of a patient with idiopathic hemosiderosis before and after treatment by phlebotomy. The elements of interest were iron, stored in pathological quantities in hepatic lysosomal structures and cerium, used as a capture ion after a cytochemical reaction to detect acid phosphatase activity in the lysosomal structures. Morphologically the lysosomal structures are heteromorph and the elements iron and cerium are heterogeneously distributed. With "reduced raster" (= reduced scanning area) analysis at 16 X 16 pixelpoints (integrating image and X-ray microanalysis), a marked difference in the area of the cross sectioned lysosomal structures before and after treatment could be demonstrated. Simultaneously the difference in the relative orientation of the elements iron and cerium before and after phlebotomy could be visualized. Chelex ion exchange beads, loaded with 11.5% w/w iron, and coembedded with the tissue blocks, were used as an internal standard. A mean iron peak to background ratio was obtained and a factor, converting ratio to absolute iron concentration, was calculated. The same calculation procedure, now per pixelpoint, was followed for the hepatic lysosomal structures. A marked difference in iron concentration in the individual lysosomal structures was observed before and after treatment by phlebotomy.

Bloodletting↗
PubMed1985

Rapid calcification of the bovine pericardial valve in adolescence. Critical aortic stenosis, hemolytic anemia, and quantitative renal hemosiderosis.

A 16-year-old boy developed marked calcific stenosis of an Ionescu-Shiley valve that was implanted in the aortic position 42 months before his sudden death. Renal hemosiderosis caused by intravascular hemolysis was quantitated by atomic absorption spectrophotometry. The accelerated calcification of the bovine pericardial valve in our patient supports the view that, in general, xenografts should not be used in adolescents. Frequent follow-up evaluations are imperative in young patients who receive these valves to avoid the irrevocable progression of valvular stenosis and hemolytic anemia.

Adolescent↗
PubMed1985

[Risk factors in the development of idiopathic pulmonary hemosiderosis].

A long-term study of six children suffering idiopathic pulmonary hemosiderosis (IPH) was realized, in order to evaluate prognostic factors and the best therapy. The treatment was initiated with prednisone 1-2 mg/kg/day which was gradually decreased until total suppression. Clinical remission was always achieved but relapses were frequently observed. All but one of the patients suffered more episodes which were controlled by the initial treatment. In three cases the additional use of immunosuppressors drugs was needed, chlorambucil 0.2 mg/kg/day during a period of 3-6 months or cyclophosphamide 2.5 mg/kg/day for four months, thereby obtaining a better control of the disease and also a decreased number of crises. Two patients died during acute exacerbations. It is suggested that the main risk factors are the male sex and onset before three years of age. The outcome was also worse when the first crisis was more severe.

Child↗
PubMedDate not supplied

[Dyserythropoietic congenital anemia type II with intensive hepatic hemosiderosis in a 1-month-old infant].

A case of dyserythropoietic congenital anemia type II (HEMPAS) in an infant 35 days of age is reported. Diagnosis was based upon morphological features of the erythroblasts, 25% of them were bi or multinucleated and a positive acidified serum test (pseudo HAM). The early and severe anemia which was present from the first days of life is emphasized. An additional and significant hemolytic factor with a relative tocopherol deficiency is suggested. A very important degree of hepatic hemosiderosis was found at necropsy. This finding reveals the very active dyserytropietic disorder in this case from the beginning of intrauterine file.

Anemia, Hemolytic, Congenital↗
PubMed1977

Renal amyloidosis with nephrotic syndrome in a child suspected of having idiopathic pulmonary hemosiderosis.

A case of renal amyloidosis with nephrotic syndrome in a 12-year-old girl suffering from a chronic pulmonary disease is reported. Data from this patient's history, laboratory examination and radiographic evaluation of the pulmonary lesions favour the diagnosis of a long-standing idiopathic pulmonary hemosiderosis. A possible etiologic relationship between the pulmonary lesions and amyloid deposition in the kidney may be assumed.

Amyloidosis↗
PubMedDate not supplied

C1q-binding immune complexes and other immunological studies in children with pulmonary hemosiderosis.

We report immunological studies performed in 19 sera from 4 children with idiopathic pulmonary hemosiderosis (IPH). Circulating C1q binding immune complexes (IC) were found in 3/4 of the patients during the acute phase. One child showed IC in 4 consecutive crises but they disappeared very rapidly (within 36 hours). Only one serum was positive after acute crisis. In one patient who had 2 episodes. IC were never detected. Precipitation with 2% polyethylene glycol did not correlate with the C1q binding assay. Pulmonary biopsy was available in just one patient and neither immunoglobulins nor complement were found by immunofluorescence. IgA, IgM, IgE, C4 or B factor abnormalities were only occasionally seen. Antinuclear, anti-reticulin and anti-alveolar basement membrane antibody test were always negative. Precipitins against cow's milk proteins were not detected. Seric IC could be primary or secondary to macrophage blockade by hemosiderin. Even in this case, it is well known that circulating IC can perform some immunological actions by interacting with cell receptors or releasing active mediators. In the future, the possibility that IC could contribute to IPH pathogenesis, or modify the treatment response must be taken into account.

Antigen-Antibody Complex↗
PubMedDate not supplied

[Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophophamide therapy].

A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.

Adolescent↗
PubMed1982

[Idiopathic pulmonary hemosiderosis: anatomoclinical study of 2 cases].

Two children with idiopathic pulmonary hemosiderosis are described. Both of them presented initially a clinical picture of recurring iron deficiency anemia, before pulmonary symptoms and signs made it possible to establish correct diagnosis. In one case diagnosis was considered only when an overwhelming pulmonary hemorrhage occurred, leading to the patient's death. At necropsy, areas of fresh alveolar hemorrhage and hemosiderin loaded macrophages were found, as well as moderate to severe interstitial fibrosis and hyperplastic alveolar cells. There was not disruption of the basal alveolocapillary membrane.

Anemia, Hypochromic↗
PubMedDate not supplied

[Idiopathic pulmonary hemosiderosis; long-term follow-up in 10 patients treated with corticoids and immunosuppressive agents].

A long-term follow-up of idiopathic pulmonary hemosiderosis in 10 patients treated with corticoids and immunosuppressors. Ten patients with I.P.H. are studied from the clinical, radiological, hematologic and respiratory functional viewpoints. The long-term evaluation (mean 7.4 years) is described together with the response to steroidal treatment (prednisone) associated with an immunosuppressor (6 +/- mercaptopurine). Only one out of the 9 patients did not return to control following 1 year of treatment. A relationship between the decrease or suppression of one or both drugs and the appearance of relapses is observed in all patients. Comments are made on the usefulness of the associated treatment and its administration is suggested for long periods giving prednisone every 48 hours. The possibility to discontinue both drugs is discussed, but not before the patient has remained asymptomatic for at least one year. Recent studies with ultramicroscopy tending out the pathogenesis of the disease are stressed.

Child↗