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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Iron-chelating treatment is indicated in all children on prolonged transfusion therapy (i.e., chiefly patients with thalassemia and Blackfan-Diamond anemia). The purpose of iron-chelating treatment is to prevent the development of manifestations of iron overload including cardiac hemosiderosis and insulin-dependent diabetes mellitus (which are two potentially fatal complications), hepatic cirrhosis, hypoparathyroidism, hypothyroidism, and delayed puberty. Deferoxamine is the only effective iron-chelating agent and should be given in a daily dose of 40 mg/kg at initiation of the transfusion program. Administration is by subcutaneous infusions from 8 to 10 hours per day. The goal of iron-chelating treatment is to maintain serum ferritin levels between 500 and 1,000 ng/ml. This long-term treatment is a significant burden for patients and it can be hoped that non-toxic iron-chelating agents, active by mouth, will become available.
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PMID:[Iron chelation in children]. 268 51

Iron chelation therapy must be associated with the regular blood transfusions required for thalassaemia and other chronic anemias. We report here a study concerning 4 groups of patients, aged 6 to 28, regularly transfused at Necker Enfants-Malades hospital: a) 20 with thalassaemia major; b) 6 with thalassaemia intermedia; c) 2 with sickle cell disease and d) 2 with Blackfan-Diamond syndrome. The transfusion regimen consisting of monthly or quarterly transfusions varied as a function of the groups. Desferal was used in all patients. The dosage and the route of administration (IV, IM, SC) were adapted to the amount of iron transfused and to the nature of the disease. The serum ferritin level was considered as the indicator of the iron overload. Comparisons were established between the quantities of iron transfused, ferritin levels, and parameters such as dosage, route of administration and compliance to Desferal. During the period of study 3 patients died from cardiac failure due to transfusional hemosiderosis. Endocrine complications (diabetes 2 cases, hypocalcemia 3 cases, hypothyroidism 1 case and delayed puberty 7 cases) were observed. This high incidence of complications induced by post-transfusional iron overload has recently prompted us to improve the quality of chelation therapy through the use of the services of a specialized center where patients as well as their families can be trained more adequately in home care and self-treatment.
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PMID:[Treatment of post-transfusion iron overload by deferoxamine]. 273 4

Refractory macrocytic anemia with hypolobulated megakaryocytic nuclei and partial deletion of the long arm of chromosome 5 has been termed the 5q- syndrome. Although long survival has been reported in a few cases of 5q- refractory anemia, accumulating evidence suggests that this syndrome is a preleukemic state with risk of transformation to acute nonlymphocytic leukemia as well as complications of bone marrow failure. This report describes the first apparently successful therapy for this disorder in a young man who originally presented with a clinical picture consistent with pure red cell aplasia and normal marrow chromosomes but with hypolobulated megakaryocytic nuclei. He was treated with vitamins, androgens, and sequential trials of immunosuppressive therapy, all without response. Two years after diagnosis, repeated marrow cytogenetic studies showed a 5q- abnormality in 70 percent and later in 100 percent of marrow metaphases. Because of transfusion-induced hemosiderosis and the availability of a cytogenetically normal monozygotic twin, bone marrow transplantation was undertaken. In light of the clonal (and suspected preleukemic) nature of the 5q- syndrome, the patient's marrow was ablated with a busulfan plus cyclophosphamide regimen used for patients with nonlymphocytic leukemia. Sustained engraftment of cytogenetically normal marrow ensued. Two years after transplantation, and following six months of regular phlebotomy, the patient was hematologically normal with a normal serum ferritin level.
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PMID:Hematologic and cytogenetic remission of 5q-refractory anemia after syngeneic bone marrow transplantation. 308

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.
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PMID:Diagnosis and treatment of iron overload in paediatric patients on chronic haemodialysis. 315 30

There have been some reports on the risk of developing hemosiderosis in hemodialysis patients when heavily transfused and simultaneously possessing hemochromatosis alleles (HA). We evaluated 99 patients on chronic hemodialysis estimating their serum ferritin (SF) levels, transfusion rate, and prevalence of HLA A3, B7 and B14 alleles, which are considered to be more frequent in idiopathic hemochromatosis. We analyzed the patients as a whole group and also separately as low or high transfusion groups. There was no correlation between the number of HA and the mean SF levels. The presence of HA is not a risk factor for the development of hemosiderosis when excessive transfusions and parenteral iron administration are avoided.
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PMID:Serum ferritin and hemochromatosis alleles in chronic hemodialysis patients. 322 55

Acquired hemosiderosis resulting from massive iron deposits in various organs, including heart, liver, and pancreas, may lead to architectural and functional disturbances of these organs. Even though iron overload can occur in nonuremic as well as in uremic individuals, the dialysis patient is at particular risk for developing hemosiderosis. Many dialysis patients receive exogenous iron from either oral iron therapy or blood transfusions. In addition, these patients seem to be at high risk for retaining iron. A diagnosis of excess iron deposition should be considered if the patient has unexplained cardiomyopathy, hepatic cirrhosis, proximal myopathy, diabetes mellitus, arthropathy, or immune dysfunction such as listeriosis. Several techniques are available for determining iron overload. Diagnostic tests include measuring serum ferritin levels, staining bone marrow preparations for excess iron, measuring tissue hemosiderin concentrations, magnetic resonance imaging, and the deferoxamine (DFO; Desferal) "challenge test." The simplest treatment for iron overload in nonuremic patients is removal of iron by venesection. However, in patients in whom venesection is not feasible, the chelating agent DFO can effectively remove excess iron. In the dialysis patient, DFO therapy can be combined with either dialysis or hemoperfusion to remove the iron-DFO complex that would otherwise be removed by the kidney. DFO therapy in the nondialyzed individual has proven to be successful, but before treatment, the benefits of the treatment must be weighed against possible adverse side effects such as cataracts, changes in color vision, and anaphylaxis. In the dialysis patient, indications for iron removal are less clearly defined.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Management of iron overload in dialysis patients. 329 89

Diabetes mellitus was observed in 29 of 448 patients with thalassaemia major attending seven Italian centres. Twelve patients, at onset of clinical diabetes, presented with an asymptomatic glycosuria, 13 with ketosis, and four with ketoacidosis. All were diagnosed after 1979, at a mean age of 17 years. Mean age at diagnosis of diabetes was lower in patients born in the last two decades. In these patients transfusions were started at a younger age and pre-transfusion haemoglobin concentration, serum ferritin concentration, incidence of liver disease, and the presence of a family history of diabetes were higher than in patients born previously. Although 27 (93%) cases had iron chelating treatment the mean serum ferritin concentration was 5600 micrograms/l; 25 (92%) of these patients had signs of liver impairment. The determination of C peptide in 10 patients showed a wide variation in pancreatic beta cell function, and insulin requirements ranged between 0.15 and 1.72 U/kg body weight. Metabolic control was generally poor. The onset of diabetes mellitus was followed in most patients by the appearance of other endocrine or cardiac complications, or both. Fourteen patients died within three years of presenting with overt diabetes. Haemosiderosis, liver infections, and genetic factors seemed to be crucial in diabetes development. Thalassaemic patients developing clinical diabetes mellitus are at high risk for other complications and should be strictly monitored, especially for thyroid impairment.
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PMID:Insulin dependent diabetes in thalassaemia. 334 50

This study describes the ultrastructural features of hemosiderosis occurring in five transplant recipients. Iron deposits in the form of homogeneous and heterogeneous siderosomes (iron-laden lysosomes) were found within the hepatocytes and macrophages in areas of fibrosis, and to a lesser degree within Kupffer cells and bile duct epithelium. Disruption of siderosomal membranes with extrusion of ferritin particles into the cytosol was demonstrated in these specimens and may be significant in the pathogenesis of hepatocellular injury.
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PMID:Hepatic iron overload in renal transplant recipients: ultrastructural observations. 352 Oct 19

Hemorrhage values and the amount of iron entering the body with drugs and blood transfusions were determined in 107 patients with the terminal CRF stage. Of them 59 received regular hemodialyses. The level of serum iron and ferritin as well as iron reserves in the body were investigated at the start and end of the study. In the end a histochemical study of the content of hemosiderin in the bone marrow, liver and spleen was performed. A close interrelationship of iron reserves determined with a modified desferal test and the level of serum ferritin (r = 0.94) was established. The highest iron reserves were revealed in the patients receiving blood transfusions and parenteral iron drugs. Criteria for the assessment of iron reserves in patients with renal failure were determined by means of the modified desferal test and investigation of serum ferritin. Normal ferritin reserves in such patients corresponded to serum ferritin values within the range of 50-400 micrograms/l and indices of the modified desferal test ranging from 0.4 to 2.0/0.5 g of desferal. Of a degree of hemosiderosis one could judge on the basis of a histochemical investigation of tissue hemosiderin only. Iron drugs per os were proposed for the prevention of disorders of iron balance in patients with renal failure.
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PMID:[Iron reserves in patients with chronic renal insufficiency]. 368 52

A case of nontransfusion-dependent sideroachrestic anemia (SA) with hemosiderosis is described that showed significant improvement of hemosiderosis and fibrosis of the liver following treatment with desferrioxamine. The anemia, although not transfusion dependent, did not allow continued therapy with phlebotomies. Following the removal of about 16 g iron over 4.5 years, normalization of serum ferritin and reversal of fibrosis of the liver were observed. Management problems and the prognostic implications of desferrioxamine therapy are discussed.
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PMID:Sideroachrestic anemia with iron loading: treatment with desferrioxamine. 371 11


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