Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Persons with sickle cell anemia have several indications for transfusion of red blood cells. One of the complications of transfusion of red blood cells is iron overload. Iron overload has been associated with multiple endocrine abnormalities. We report herein three cases of hypothyroidism in adult individuals with sickle cell disease. All three patients were over the age of 45 years at the time of the diagnosis and had received multiple units of transfused red blood cells and had serum ferritin levels of greater than 6,000 ng/mL. All patients were diagnosed during times when they were critically ill. Replacement therapy was instituted in all cases; however, all three patients died shortly after the diagnosis of hypothyroidism was made. Congestive heart failure appeared to be a primary cause of death in all three patients. In the one patient in whom a postmortem examination was done, there was evident extensive fibrosis of the thyroid gland as well as extensive deposition of iron in the cells lining the thyroid follicles. We believe that this represents the first report of clinical hypothyroidism in patients with sickle cell anemia who have received multiple transfusions. Awareness of this condition is especially important given that congestive heart failure is common in sickle cell disease.
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PMID:Hypothyroidism in adults with sickle cell anemia. 158 Mar 5

Anaerobic threshold (AT) has been advocated as an objective method of evaluating exercise capacity in patients with chronic congestive heart failure. The factors that determine AT, however, remain still unclear. To assess the influence of oxygen transport capacity on AT, patients with iron deficiency anemia were studied before and after treatment with iron. Twenty-nine female subjects were studied. They were divided into the following 3 groups: 1) iron deficiency anemia (group IDA: Hgb less than 11 g/dl and ferritin less than 10 ng/ml) consisting of 4 athletes and 6 non-athletes, 2) latent iron deficiency (group Lat-ID: Hgb greater than or equal to 11 g/dl and ferritin less than 10 ng/ml) consisting of 4 athletes, and normal (group Nor: Hgb greater than or equal to 11 g/dl and ferritin greater than or equal to 10 ng/ml) consisting of 15 athletes and 6 non-athletes. By bicycle ergometer using ramp protocol, peak oxygen uptake (peak VO2) and AT were measured in each group. Following the 1st exercise testing, groups IDA and Lat-ID were treated by oral iron for 1-1.5 months. The 2nd exercise testing was then performed. Furthermore, to investigate whether muscle cell energy metabolism itself is altered by iron deficiency, P magnetic resonance spectroscopy (MRS) was performed in 2 relatively severe anemic patients during forearm exercise while assessing the changes in phosphocreatine and inorganic phosphate. Peak VO2 and AT in non-athletes were significantly lower in IDA group than Nor group (peak VO2 (ml/min/kg): 23.7 +/- 5.1 vs 33.3 +/- 3.8, p less than 0.01, AT (ml/min/kg): 15.9 +/- 3.3 vs 21.3 +/- 1.3, p less than 0.01). After iron administration, Hgb was increased significantly in IDA group (from 9.0 +/- 1.8 to 12.1 +/- 0.8 g/dl, p less than 0.01) accompanied by an improvement in peak VO2 and AT (peak VO2 (ml/min/kg): from 34.2 +/- 12.4 to 40.0 + 13.0, p less than 0.001, AT (ml/min/kg): from 20.9 +/- 6.3 to 25.0 +/- 8.0, p less than 0.001). Lat-ID and Nor groups showed no changes. MRS indices of cell energy metabolism of the 2 severely anemic patients did not differ from those of normal controls, and no changes were observed after iron treatment. It is concluded from these results in iron deficiency anemia that oxygen transport is a determinant of anaerobic threshold.
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PMID:[Effect of blood hemoglobin concentration on anaerobic threshold]. 191 24

Cardiac dysfunction is the most common cause of death in patients with homozygous beta-thalassemia. We studied a group of 10 older patients (mean age 17.5 years) with and without preexisting cardiac dysfunction who had begun chelation therapy on the average of 10 years after regular transfusions were initiated. Over the 4-year study period, two patients were noncompliant with deferoxamine therapy. Their clinical status and cardiac function deteriorated, and both died with evidence of arrhythmia and congestive heart failure. The remaining eight patients were compliant. Despite a drop in mean serum ferritin from 3,814 +/- 577 (SE) ng/ml to 1,056 +/- 146 ng/ml (p less than 0.01), two patients with preexisting cardiac problems and one patient without preexisting heart disease developed further abnormalities. Of the three patients whose status declined, one ultimately improved with alternative chelation therapy. These data suggest that for a few older patients, improvement or stabilization of cardiac status may not be achieved with improved compliance and reduced serum ferritin levels. For these patients, new approaches appear to be warranted. On the other hand, we have demonstrated that in most cases, older patients who began chelation therapy years after transfusions began have benefited from compliance with standard subcutaneous deferoxamine regimens.
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PMID:Chelation therapy and cardiac status in older patients with thalassemia major. 230 80

A 41-year-old hemodialyzed woman developed ascites and was found to have secondary iron overload. The dose of administered iron was approximately 11-12 g, and her serum ferritin level was 15,000 ng/ml (15,000 micrograms/l). There were no signs of congestive heart failure, fluid overload, or liver cirrhosis. A program of weekly phlebotomy combined with recombinant human erythropoietin (rhEPO) therapy was tried to eliminate the iron congestion. After 9 months of this therapy, about 5 g of iron had been removed. The ascites completely disappeared, and her serum ferritin level fell to 5,800 ng/ml (5,800 micrograms/l). This suggests that such combined therapy would be useful when iron overload must be corrected rapidly. Before therapy, the sterile ascitic fluid showed exudative characteristics with 3.7 g/dl (37 g/l) of total protein. The serum-ascites albumin difference was 0.6 g/dl (6 g/l), and the fluid contained 1,400 inflammatory cells/mm3 (1.4 X 10(9)/l). Notably, the serum-ascites albumin difference increased in parallel with iron elimination. These findings suggested that iron deposition may have played a role in changing the permeability of the peritoneum, or in impairing lymphatic drainage, both of which are presumed to be pathogenetic factors of nephrogenic ascites.
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PMID:Treatment of a patient with end-stage renal disease, severe iron overload and ascites by weekly phlebotomy combined with recombinant human erythropoietin. 236 36

Protein analyses were performed in 15 cyst fluids (CF) from mature teratoma (TD) and in 15 corresponding sera from 9 nonseminomatous germ cell tumor patients. Qualitatively, many similarities between the protein compositions of CF and corresponding sera were seen. Quantitative comparisons suggested free diffusion of plasma proteins into the cyst lumen in nine cases, whereas in five CF a decreased size selectivity of the blood-TD barrier was observed. From the quantitative data it was concluded that the significantly increased CCF/Cserum concentration ratios for the tumor markers alpha-fetoprotein (8/14), human chorionic gonadotropin (3/14), and carcinoembryonic antigen (13/13) as well as for lysozyme (12/13), ferritin (12/13), and fibronectin (3/6) were either due to local synthesis or to concentrating properties of the TD cells. The results of the current study encourage further research for new tumor-associated proteins in cyst fluids.
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PMID:Protein composition of cyst fluids from mature teratoma in patients with nonseminomatous germ cell tumors of the testis. 241 85

We investigated the value of high-dose intravenous iron chelation therapy with deferoxamine as an alternative to conventional subcutaneous therapy in eight patients receiving regular transfusions who had massive iron stores, including two with clinical heart disease. Six to twelve grams of deferoxamine was infused daily for 12 hours over 12 to 25 months through externalized central venous catheters or implanted reservoirs. Serum ferritin levels decreased by 56% to 99%. Liver iron concentrations, measured by magnetic susceptibility in two patients, were 1234 and 2438 micrograms/gm wet weight (22.1 and 43.6 mumol/gm wet weight) after treatment for 17 and 25 months, respectively. A patient with congestive heart failure and a patient with severe ventricular dysrhythmias no longer required cardiac medication after 12 to 24 months of chelation therapy. Three episodes of bacteremia and three episodes of cellulitis accounted for a catheter-related infection rate of 0.14 per 100 patient-days. The catheter removal rate was 0.20 per 100 patient-days. No patient experienced serious visual, auditory, or other toxicities. We conclude that in some patients receiving regular erythrocyte transfusions, high-dose intravenous chelation therapy with deferoxamine is superior to conventional subcutaneous treatment.
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PMID:Rapid removal of excessive iron with daily, high-dose intravenous chelation therapy. 233 79

Cardiac involvement in haemochromatosis includes development of congestive heart failure and/or cardiac arrhythmias. To elucidate the importance of this disorder among patients with severe cardiac bradyarrhythmias necessitating treatment with a permanent pacemaker, such patients were screened for evidence of iron overload. Serum ferritin was determined in 232 men treated with a permanent pacemaker. In six patients (2.6%) the serum ferritin values were twice the upper normal limit. In three of these, causes other than iron overload were found (liver cancer, myeloma and amiodarone treatment), while in three (1.3%) iron overload was suspected. All the latter three had atrioventricular block of second to third degree. In these patients percutaneous liver biopsy was performed. The specimens were examined by light and electron microscopy and the iron content was determined by atomic absorption spectrophotometry. The results of these investigations showed iron overload in the liver. The prevalence of iron overload (2.0%) among this male pacemaker-treated population with AV block II-III is significantly higher than the rates previously found in urban populations in Sweden. The present data indicate that screening for haemochromatosis is of importance among males with second and third degree atrioventricular-block of unknown etiology.
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PMID:Prevalence of a haemochromatosis among men with clinically significant bradyarrhythmias. 139 45

Ferritin was analysed with an immunoradiometric assay using anti-spleen ferritin antibodies, in pleural effusions (Pl) from 28 patients with malignant effusions (18 carcinoma, 10 mesothelioma), 15 patients with non-malignant exudative effusions of unknown aetiology, and from 12 patients with transudative effusions due to congestive cardiac failure. Geometric mean Pl-ferritin was 617 micrograms/l in carcinoma, 1301 micrograms/l in mesothelioma (p less than 0.01 against carcinoma), 931 micrograms/l in non-malignant exudates, and 178 micrograms/l in transudates (p less than 0.0001 against malignant and non-malignant exudates). There was no correlation between Pl-ferritin and Pl-protein, Pl-albumin or Pl-cell count. P1-ferritin displayed a wide overlap between the various groups, and was of no value in the discrimination between malignant and non-malignant exudates. In the differentiation between exudates and transudates, the diagnostic accuracy of Pl-ferritin was only slightly lower compared to Pl-protein and Pl-albumin. With the present method, analysis of Pl-ferritin appears to be of limited value in the routine diagnostic evaluation of pleural effusions.
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PMID:Diagnostic value of ferritin analysis in pleural effusions. 374 85

We examined the efficacy of long-term subcutaneous deferoxamine therapy in the prevention of iron-related cardiac disease in patients with thalassemia major who began treatment after the age of 10 years. Of 36 such patients without preexisting cardiac disease, 19 did not comply with the program of chelation therapy. Over the course of treatment (1977 to 1983) serum ferritin and aspartate aminotransferase levels fell in the compliant group, from mean values (+/- S.D.) of 4765 +/- 2610 to 2950 +/- 1850 ng per milliliter and 58.1 +/- 22 IU to 30 +/- 20 IU per liter, respectively (P less than 0.05), but rose in the noncompliant group, from 5000 +/- 2316 to 6040 +/- 2550 ng per milliliter and 56.6 +/- 20 to 90 +/- 35 IU per liter, respectively. Only one patient in the compliant group acquired cardiac disease and died of fulminant congestive heart failure. In contrast, 12 noncompliant patients acquired cardiac disease, and 7 died. In addition, the mean age of the compliant population (18.9 +/- 4.5 years) now approaches the mean age of acquisition of cardiac disease in the noncompliant group (19 +/- 4.3). These data demonstrate that compliance with treatment with deferoxamine may protect patients from cardiac disease induced by iron overload.
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PMID:Prevention of cardiac disease by subcutaneous deferoxamine in patients with thalassemia major. 400 Jan 98

A 47-year-old man with sickle cell anemia, chronic cor pulmonale, and congestive heart failure died following a short illness. A diagnosis of iron deficiency was established during life by usually accepted criteria including a low serum ferritin concentration. Autopsy showed no stainable iron in the bone marrow, liver, and the heart. Marked deposits of iron were seen in the kidneys and the atrophic spleen. These findings suggest that the serum ferritin concentration may not reflect the metabolically sequestered stores of iron in the spleen and the kidneys.
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PMID:Serum ferritin and sequestered stores of body iron. 619 14


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