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

We performed a study of magnetic resonance imaging (MRI) assessment of hemosiderosis in the heart (T2/T2*), liver (T2*), pancreas (T2*), and pituitary gland (T2/T2*/SIR) in 20 hemopoietic stem cell transplant (HSCT) recipients (median peak ferritin levels 7615 pmol/L, range 3411 to 33000 pmol/L). MRI reading was abnormal in the heart (5%), liver (85%), pancreas (40%), and pituitary gland (55%). The heart T2 correlated with peak ferritin levels (P=.024), while the liver T2* correlated with current ferritin (P=.038) values only. Pancreatic T2* values correlated with pituitary T2 and signal intensity ratio values. The ejection fraction was abnormal in 10% of cases and did not correlate with ferritin level or heart T2. The peak liver enzymes correlated with peak ferritin (P=.025), but the current liver enzymes were mostly normal. Pancreatic assessments (fasting glucose, insulin, beta cell function, insulin reserve, and C-peptide) and pituitary growth hormone axis assessments (growth hormone, insulin growth factor-1, and insulin growth factor binding protein-3) were abnormal in 40% to 70% of cases. They were unrelated to pancreas or pituitary MRI values. Interestingly, endocrine assessments correlated with heart T2 values and peak (but not current) ferritin levels. We concluded that iron overload may contribute to organ damage after HSCT, and MRI assessment may be useful in its detection and treatment monitoring.
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PMID:A magnetic resonance imaging study of iron overload in hemopoietic stem cell transplant recipients with increased ferritin levels. 1808 87

The benefits of combined deferoxamine (DFO) and deferiprone (L1) chelation therapy, focusing on reducing myocardial iron loading, have been widely reported. Herein, we present the efficacy of combined chelation and its effects on iron load indices. Five thalassemia major (TM) patients who were undergoing chelation monotherapy with DFO were enrolled. Inclusion criteria were magnetic resonance imaging (MRI) T2* values, indicating serious heart and/or liver transfusional hemosiderosis. Combined therapy was started with the same dose of DFO and the addition of L1. The MRI T2* studies were repeated 18 months later. An Echo-Doppler study was performed in order to further evaluate the left ventricular (LV) systolic function. Within the 18 months' follow-up period, there was a significant statical decrease in mean serum ferritin levels. All patients increased their MRI T2* liver values, while two patients with very low MRI T2* also increased their myocardial values. The MRI ejection fraction (EF) and Echo-Doppler study measurements confirmed the improvement of systolic function. No adverse effects were reported. Combined L1 and DFO therapy seems to be effective in reducing iron excess in organ iron overloaded thalassemic patients. Magnetic resonance imaging can accurately quantify iron load, while echocardiography remains a reliable monitoring technology.
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PMID:Effects of combined deferiprone and deferoxamine chelation therapy on iron load indices in beta-thalassemia. 1827 80

Hepatic hemosiderosis is one of the most common postmortem findings in captive callitrichid species. Noninvasive evaluation of hematologic iron analytes has been used to diagnose hepatic iron storage disease in humans, lemurs, and bats. This study evaluated the relationship between hematologic iron analyte values (iron, ferritin, total iron binding capacity, and percent transferrin saturation) and hepatic hemosiderosis in callitrichids at the Wildlife Conservation Society's Central Park and Bronx Zoos. Results revealed that both ferritin and percent transferrin saturation levels had strong positive correlations with hepatic iron concentration (P<0.001, r=0.77, n=20; P<0.001, r=0.85, n=10, respectively). Serum iron levels positively correlated with hepatic iron concentration (P=0.06, r=0.56, n=11), but this finding was not significant. Serum total iron binding capacity did not significantly correlate with hepatic iron concentration (P=0.47, r=0.25, n=10). Both ferritin and hepatic iron concentration positively correlated with severity of hepatic iron deposition on histology (P<0.05, r=0.49, n=21; P<0.001, r=0.67, n=21, respectively). This study suggests that ferritin, serum iron concentration, and percent transferrin saturation are convenient, noninvasive, antemortem methods for assessing severity of hemosiderosis in callitrichids.
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PMID:Hematologic iron analyte values as an indicator of hepatic hemosiderosis in Callitrichidae. 1836 7

The purpose of this study is to investigate the role of femoral marrow MR imaging as predictor of outcome for hemopoietic stem cell transplantation (HSCT) in beta-thalassemia major. MR imaging of the proximal femur, including T1- and T2-weighted spin echo and short-tau inversion recovery and in-phase and out-of-phase fast field echo images, was prospectively performed in 27 thalassemia major patients being prepared for HSCT. The area of red marrow and its percentage of the proximal femur were measured, and the presence of marrow hemosiderosis was assessed. Age-adjusted multivariate logistic regression was used to determine the relationship between red marrow area percentage and marrow hemosiderosis and HSCT outcome. Red area percentage were less in patients with successful (90.25 +/- 4.14%) compared to unsuccessful transplants (94.54% +/- 2.93%; p = 0.01). Red marrow area percentage correlated positively with duration of symptoms(r = 0.428, p = 0.026) and serum ferritin (r = 0.511, p = 0.006). In multivariate-adjusted logistic regression analyses, red marrow area percentage was significantly inversely associated with successful HSCT (OR = 1.383, 95% CI: 1.059-1.805, p = 0.005). Marrow hemosidersosis and duration of sympotms and serum ferritin were not associated with HSCT outcome(p = 0.174, 0.974, 0.762, respectively). Red marrow area percentage of proximal femur on MR imaging is a useful predictor of HSCT outcome.
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PMID:Bone marrow MR imaging as predictors of outcome in hemopoietic stem cell transplantation. 1838 43

In a retrospective study of 126 adult patients with French-American-British-defined refractory anemia with ringed sideroblasts (RARS), staging by the International Prognostic Scoring System was highly predictive of survival outcome (P < 0.0001). In addition, red blood cell (RBC) transfusion requirement at diagnosis (P = 0.001), but not the number of RBC units transfused during the disease course (P = 0.17), was independently associated with inferior survival. There were no correlations between survival and serum ferritin level, measured either at diagnosis (median 567 ng/mL, range 16-3,475; P = 0.24) or during follow-up (median 1,108 ng/mL; range 238-43,500; P = 0.72). Similarly, there was no difference in survival when patients were stratified by serum ferritin levels of < or > or =1,000 ng/mL at diagnosis or peak serum ferritin levels of <1,000, 1,000-5,000, or >5,000 ng/mL during follow-up. The current study does not support the contention that transfusional hemosiderosis is an adverse prognostic factor in "good risk" myelodysplastic syndrome.
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PMID:Neither serum ferritin nor the number of red blood cell transfusions affect overall survival in refractory anemia with ringed sideroblasts. 1844 62

We performed MRI and endocrine assessment in 27 adult Chinese patients with severe aplastic anemia (SAA) and myelodysplasia (MDS). Despite high ferritin levels, cardiac hemosiderosis and heart failure was not common, and were not concordant in most cases. However, significant correlation was found between cardiac T2/T2* MRI assessment, ejection fraction and ferritin levels. Furthermore, there was a high incidence subclinical pancreatic abnormality that was related to liver MRI T2* readings. Hence, chelating agents may still have a role to improve organ function. Prospective data with serial functional and imaging monitoring is needed.
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PMID:A pilot MRI study of organ specific hemosiderosis and functional correlation in Chinese patients with myelodysplasia and aplastic anemia with raised ferritin levels. 1848 75

In beta-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies demonstrating proteinuria, aminoaciduria, low urine osmolality, and excess secretion of the tubular damage markers, such as urinary N-acetyl-D-glucosaminidase (U(NAG)) and beta2 microglobulin, in patients with thalassemia. The object of this study was to analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia and to correlate the renal findings to iron overload. Thirty-seven patients with beta-thalassemia major and 11 with thalassemia intermedia were studied. Twelve children without iron metabolism disorders or renal diseases served as a control group. No difference in blood urea nitrogen (BUN), serum creatinine, creatinine clearance, electrolytes, fractional excretion of sodium and potassium, and tubular phosphorus reabsorption was found. Serum uric acid was equal in the two groups, but its urine excretion was significantly higher in the thalassemic group. U(NAG) and U(NAG) to creatinine ratio (U(NAG/CR)) were elevated in all patients with thalassemia compared with the control group (p < 0.001) and were directly correlated to the amount of transfused iron but not to actual ferritin level. We found that renal tubular function is impaired in children with beta- thalassemia major and intermedia. It is not known whether these functional abnormalities would have any long-term effects on the patients. Further studies are needed, and means of preventing these disturbances should be sought.
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PMID:Renal function in children with beta-thalassemia major and thalassemia intermedia. 1858 Nov 45

IgA nephropathy associated with liver cirrhosis is a common disease, unlike hemochromatosis-associated renal involvement, which is uncommon. A 55-year-old man was admitted to our hospital for the acute deterioration of renal function. Laboratory tests showed extremely high transferrin saturation and serum ferritin level. Furthermore, magnetic resonance imaging revealed low-intensity signals in both T1- and T2-weighted images within the liver, diagnosed as primary hemochromatosis. Renal biopsy showed mesangial IgA deposition with cellular crescent and hemosiderin in both glomerular and tubular epithelial cells. Renal function worsened progressively after admission, and thus steroid pulse therapy was started. Renal dysfunction improved, but the patient died of cerebral hemorrhage. The present case was considered IgA nephropathy and renal hemosiderosis secondary to primary hemochromatosis. To our knowledge, this is the first report that describes the above complications in association with primary hemochromatosis.
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PMID:A case of IgA nephropathy and renal hemosiderosis associated with primary hemochromatosis. 1879 57

We performed MRI assessment in 37 adult Chinese patients with thalassemia intermedia and hemoglobin H disease. Despite abnormal ferritin and liver T2*, only 5% of patients had cardiac hemosiderosis. The two patients with reduced ejection fraction had normal cardiac T2*. Half of the cases showed pituitary and pancreatic iron loading. Subclinical endocrine abnormalities (HOMA, insulin growth factor) showed correlation with pancreatic, pituitary, and cardiac MRI values. Prospective data with serial functional and imaging monitoring is needed to verify the utility for chelation to improve cardiac and endocrine function in this group of patients.
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PMID:Organ-specific hemosiderosis and functional correlation in Chinese patients with thalassemia intermedia and hemoglobin H disease. 1916 82

Many patients with primary myelofibrosis (PMF) become red blood cell (RBC) transfusion dependent (TD), risking iron overload (IOL). Iron chelation therapy (ICT) may decrease the risk of haemosiderosis associated organ dysfunction, though its benefit in PMF is undefined. To assess the effect of TD and ICT on survival in PMF, we retrospectively reviewed 41 patients. Clinical data were collected from the database and by chart review. The median age at PMF diagnosis was 64 (range 43-86) years. Median white blood cell (WBC) count at diagnosis was 7.6 (range 1.2-70.9) x 10(9)/L; haemoglobin 104 (62-145) G/L; platelets 300 (38-2088) x 10(9)/L. Lille, Strasser, Mayo and International Prognostic System (IPS) scores were: low risk, n = 15, 8, 11, 3; intermediate, n = 15, 19, 9, 16; high, n = 5, 11, 5, 7; respectively. Primary PMF treatment was: supportive care, n = 23; hydroxyurea, n = 10; immunomodulatory, n = 4; splenectomy, n = 2. Sixteen patients were RBC transfusion independent (TI) and 25 TD; of these 10 received ICT for a median of 18.3 (0.1-117) months. Pre-ICT ferritin levels were a median of 2318 (range 263-8400) and at follow up 1571 (1005-3211 microg/L (p = 0.01). In an analysis of TD patients, factors significant for overall survival (OS) were: WBC count at diagnosis (p = 0.002); monocyte count (p = 0.0001); Mayo score (p = 0.05); IPS (p = 0.02); number of RBC units (NRBCU) transfused (p = 0.02) and ICT (p = 0.003). In a multivariate analysis, significant factors were: NRBCU (p = 0.001) and ICT (p = 0.0001). Five year OS for TI, TD-ICT and TD-NO ICT were: 100, 89 and 34%, respectively (p = 0.003). The hazard ratio (HR) for receiving >20 RBCU was 7.6 (95% Confidence Intervals [CI] 1.2-49.3) and for ICT was 0.15 (0.03-0.77). In conclusion, 61% of PMF patients developed RBC-TD which portended inferior OS; however patients receiving ICT had comparatively improved OS, suggesting a clinical benefit. Prospective studies of IOL and the impact of ICT in PMF are warranted.
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PMID:Improved survival in red blood cell transfusion dependent patients with primary myelofibrosis (PMF) receiving iron chelation therapy. 1955 69


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