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Results for “HEMOSIDEROSIS”

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At least 1,531 records · Page 85Linked to original sources
PubMed1984

Significance of erythrocyte lipid peroxidation and superoxide dismutase activity in a patient with idiopathic pulmonary haemosiderosis.

In a patient with idiopathic pulmonary haemosiderosis, lipid peroxidation of erythrocytes and their superoxide dismutase activity were determined. The latter was found to be decreased, and the erythrocytes showed easy peroxidisability, which was shown by vulnerability to hydrogen peroxide and a marked elevation of iron-catalysed lipid peroxidation in addition to a higher basal malondialdehyde level, an end product of lipid peroxidation. These findings were correlated with haemolysis, iron-deficiency anaemia and a shortened survival of erythrocytes. Although the aetiological significance of our observations still remains to be clarified, it appears that antioxidant enzymes and the peroxidative reactivity of erythrocytes should be carefully evaluated in other patients with idiopathic pulmonary haemosiderosis.

Child↗
PubMed1981

Intravenous and subcutaneous desferrioxamine therapy in children with severe iron overload.

Ten children with transfusion dependent anemias (thalassemia, sideroblastic anemia, congenital pure red cell aplasia) received either intravenous desferrioxamine (DF) in increasing doses up to 450 mg/kg at the time of transfusion or daily subcutaneous DF up to 110 mg/kg on an outpatient basis. No patient on intravenous DF reached a negative iron balance. All children with a subcutaneous DF dose of more than 60 mg/kg obtained a negative iron balance with a net iron excretion (transfusion iron already substracted) between 206 to 810 mg (mean 496 mg) monthly. The effectiveness of regular subcutaneous DF on liver storage iron could be confirmed in 4 patients by liver biopsy, showing a decrease between 40-60% iron after 12-14 months of chelation therapy. So far the daily iron excretion has remained constant with a given dose of DF over a period up to 15 months. Even if poor compliance in some patients is taken into account, it is possible with this method of treatment to prevent further accumulation of iron in chronically transfused children.

Adolescent↗
PubMed1978

Ultrastructure and x-ray microanalysis of siderosome.

Comparative ultrastructural examination and energy-dispersive electron probe X-ray microanalysis were performed on a long standing skin lesion of hemosiderotic histiocytoma. Iron-containing fine particles present in siderosome were the main element of interest. Qualitative study of spectra over the siderosomes clearly demonstrated the characteristic X-ray energy emitted by iron, whereas spectra obtained from adjacent cytoplasm revealed minimal iron peak. On quantitative evaluation of the spectra yielded from various siderosomes, iron counts intensity was found to be proportionally increased with increment in amount and electron opacity of the siderosomal inclusion. Accounts on chemical nature of the siderosomal inclusion and on the presence of lipid residue in cytoplasms were noted.

Adult↗
PubMed1988

Diagnosis and treatment of iron overload in paediatric patients on chronic haemodialysis.

In this study the incidence and contributing factors of iron overload in paediatric patients treated with intermittent haemodialysis were evaluated. Particular attention was given to the diagnostic value of serum ferritin in the assessment of body iron stores in patients with hepatocellular damage. The results of treatment of secondary haemosiderosis with desferrioxamine (DFO) are reported. Serum ferritin levels were measured in 18 children and adolescents undergoing long-term haemodialysis; 8 of these had biochemical evidence of hepatocellular damage. In all patients a good correlation was found between serum ferritin levels and the amount of iron stored in the reticuloendothelial system. Six patients developed iron overload. Patients with secondary haemosiderosis were younger at the start of haemodialysis and received significantly more blood. Although not significant, more patients with haemochromatosis-associated alleles and bilateral nephrectomy had iron overload, and the duration of dialysis was obviously longer for overload patients (40 months versus 26 months). The patients with iron overload were treated with DFO. The data from all patients showed that DFO was ineffective when administered at a dose of 25 mg/kg during dialysis and that in individual patients changes in serum ferritin correlated with changes in the amount of blood transfusions administered.

Adolescent↗
PubMed1996

Seizure control following surgery in supratentorial cavernous malformations: a retrospective study in 77 patients.

Of 168 patients operated on consecutively for a supratentorial cavernous malformation, 77 had seizures as the initial symptom. The effectiveness of surgery in controlling seizures and the risk of surgery were evaluated by retrospective review of the patients' charts. The follow-up period was 1 to 9 years (mean 39 months) and the review period totalled 284 lesion-years. Only two patients showed postoperative deterioration in neurological status (morbidity risk: 2.6%), no patient died (mortality: 0%). Sixty-eight (88.3%) patients were seizure-free after operation and five (6.5%) showed a marked reduction in the frequency of their seizures. This corresponds to an overall positive effect of surgery of 94.8% of the patients. There was no substantial evidence that excision of the haemosiderin-stained tissue around the cavernoma along with the lesion itself provided better results than resection of only the cavernoma. Better results with regard to seizure control, however, were associated with shorter duration of symptoms before surgery.

Adolescent↗
PubMed1995

Cardiac iron overload in thalassemic patients: an endomyocardial biopsy study.

Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade. Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis. It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.

Adult↗
PubMed1991

MRI of hepatic iron deposition in patients with renal transplant.

Excess hepatic iron deposition was found in five of 15 (33%) renal transplant patients undergoing magnetic resonance (MR) screening for avascular necrosis of the femoral heads. Only one of these patients had overt liver disease. The number of prior blood transfusions was a significant factor for this deposition, whereas the age and sex of the patients, number and type of transplants, histocompatibility alleles (HLA), and years of hemodialysis and of chronic renal failure were not significant etiological factors. Liver/fat intensity ratios of less than 0.29 on T1-weighted images and ratios of less than 0.21 on T2-weighted images and a calculated T2 value of less than 35 ms were the best indicators of iron overload. Renal transplant patients are at great risk for excess hepatic iron deposition and MR imaging is a promising tool for the diagnosis of iron overload in this patient population.

Adult↗
PubMed1989

"Porcelain liver" appearance due to Glisson's capsule phleboliths.

A case of multiple Glisson's capsule phleboliths is reported in a 29-year-old-woman who had portal vein obstruction with spontaneous shunting resulting in hepatic siderosis and cirrhosis. The phleboliths were visible as multiple linear calcifications on plain film and computed tomography. Diagnosis was established by surgical liver biopsy. The potential mechanisms of this unique entity is discussed.

Adult↗
PubMed1976

Lipoperoxide formation in the retina in ocular siderosis.

Insertion of iron nail into the vitreous cavity provoke the formation of lipoperoxide in the retina. In acord with the increase in lipoperoxide in the retina, ERG began to decrease. In vitro experiment using isolated retina, lipoperoxide was found to be increased in the presence of ferric or ferrous ions, while it was inhibited by adding antioxidants or ethylenediamine tetraacetate. From these results, direct cause of retinal degeneration in siderosis could be ascribed to the formation of lipoperosice by iron-ions liberated from the piece of iron, resulting into the degeneration of the visual cell layers of the retina.

Animals↗