BACKGROUND: This study was carried out to evaluate the efficacy of desferrioxamine as a chelating agent in iron overloaded patients with severe aplastic anemia due to multiple transfusion. METHODS AND MATERIALS: From Oct. 1995 to Aug. 1996, 15 patients with aplastic anemia, diagnosed from May 1995 to Jan. 1996 at St. Mary's Hospital, who had a transfusional hemosiderosis were included in this study. They received 19 courses of high-dose desfer-rioxamine therapy for 6 days(20 to 30 mg/kg daily as a 24-hour intravenous infusion) . Before and after treatment, we measured serum ferritin, iron, TIBC, 24-hour urinary excretion of iron. RESULTS: 1) The range of iron load before treatment was between 4.5 and 20.0 gram. 2) Because of limit of detection(1,800 microgram/L), it was difficult to compare the changes of serum ferritin level after therapy to those of before therapy. 3) There was no significant differences between the levels of serum iron before and after therapy(214.3+/-62.8 vs 220.0+/-53.3). And there was no significant differences between TIBC before and after therapy(235.8+/-64.6 vs 259.4+/-60.1). 4) Iron/TIBC ratios were significantly deceased after desferrioxamine treatment compared to those of before therapy(0.90+/-0.04 vs 0.85+/-0.04, P<0.001) and mean urinary excretions of iron were increased by high-dose desferrioxamine compared to those by test dose(6.5+/-7.6 vs 29.1+/-14.3, P<0.001) CONCLUSION: High-dose desferrioxamine therapy is very effective for chelating and excretion of iron in iron overloaded patients with severe aplastic anemia due to multiple transfusion. A repeat administration of desferrioxamine is necessary for the iron overloaded patient to eliminate the risk of a transfusional hemosidersis.
Anemia, Aplastic* ; Deferoxamine* ; Ferritins ; Hemosiderosis* ; Humans ; Iron ; Iron Overload

Background and Objectives: Iron is an essential element that plays a vital role in a wide variety of cellular processes. But when present in excess concentration in organs, it may increase the risk for liver disease, heart failure, and diabetes. Recently, siderophores, which are iron-chelating agents produced by microorganisms, have attracted tremendous attention because of their strong binding and high selectivity to the ferric form of iron. Thus, the use of siderophore in sequestering excess iron in the body as a form of therapy is very attractive. This study determined the effects of commercially available siderophore in sequestering excess iron in organs such as liver, heart, and pancreas under excess iron conditions. Methodology: First, iron-overload was induced by injecting iron dextran (20 mg) into male ICR mice for three consecutive days. The effects of iron to the liver, heart, and pancreas and the possible sequestration by siderophore were determined by scoring histological sections. The liver iron concentration was also assessed by atomic absorption spectroscopy (AAS). Results and Conclusion The study showed that iron-overloaded mice exhibited skin hyperpigmentation and hemosiderosis in liver, heart, and pancreas. Significant changes in the liver include hepatomegaly and development of tumor. Iron-overloaded mice had 2,935% increase in liver iron content compared to the salinetreated mice. However, when iron-overloaded mice were treated with either 100 µg or 200 µg siderophore, there was a 77% and 84% decrease in liver iron content, respectively. Moreover, the treatment of ironoverloaded mice with siderophore prevented the development of hemosiderosis, tumor, and structural changes in the tissues studied. The results showed that siderophore can effectively reduce excess iron and organ damage in iron-overloaded mice and can be potentially employed in chelation therapy of iron-overload diseases. Further studies on the possible mechanisms of siderophore aside from decreasing iron excess and lowering organ dysfunction are recommended.
Siderophores ; Iron Overload ; Iron Chelating Agents ; Hemosiderosis ; Hepatomegaly

BACKGROUND: In chronic renal failure (CRF) patients, iron deficiency is a common problem and a primary cause of resistance to recombinant human erythropoietin (rHuEPO) therapy. Serum ferritin and transferrin saturation (TS) are most commonly used parameters of iron status in CRF patients but may be influenced by the presence of inflammation and malnutrition. Recently soluble transfer-rin receptor (sTfR) has been advocated as a useful parameter of iron deficiency. We evaluated sTfR as an iron deficient marker in CRF patients. METHODS: Included in this study were 73 CRF patients, 30 uncomplicated iron deficiency anemia (IDA) patients, and 55 normal control. Serum sTfR, serum ferritin, TS, and complete blood count were measured. The CRF patients were classified as absolute iron deficient, functional iron deficient, non-iron deficient, and iron overload groups according to National Kidney Foundation Kidney Disease and Dialysis Outcome Quality Initiative (NKF-K/DOQI) guideline. RESULTS: The sTfR concentrations were significantly higher in uncomplicated IDA patients (3.9 +/-1.5 mg/L) and significantly lower in CRF patients (1.1 +/-0.4 mg/L) than in normal controls (1.4 +/-0.4mg/L). In uncomplicated IDA patients, sTfR was inversely correlated with MCV, MCH, and MCHC. In CRF patients, sTfR had a weak inverse correlation with TS and MCHC, but not significantly different between the four groups. The sTfR was not significantly different between the CRF patients with the normal CRP and those with an increased CRP. CONCLUSIONS: The sTfR is useful for diagnosis of uncomplicated IDA, but not for the detection of iron deficiency in CRF patients. Further studies are needed for the evaluation of sTfR as an erythro-poietic marker with rHuEPO therapy.
Anemia, Iron-Deficiency ; Blood Cell Count ; Diagnosis ; Dialysis ; Erythropoietin ; Ferritins* ; Humans ; Inflammation ; Iron ; Iron Overload ; Kidney ; Kidney Diseases ; Kidney Failure, Chronic* ; Malnutrition ; Receptors, Transferrin* ; Transferrin

Paroxysmal nocturnal hemoglobinuria(PNH) is a rare, acquired disease involving multiple hematopoietic lines. Characteristics of PNH are intrinsic hemolytic anemia, iron deficiency anemia and venous thrombosis. report a case of PNH with characterostoc MR and CT findings. The signal intensity of renal cortex was lower than that of medulla on both T1- and T2- weighted MR imaging. On T2 weighted MR images, the liver showed very low signal intensity but the signal intensity of the spleen was normal. On precontrast CT the attenuation of renal cortex was higher than that of renal medulla and the attenuation of liver was higher than that of the spleen. These findings of MR imaging and CT were the result from the deposition of hemosiderin in the cells of proximal convoluted tubules and transfusional hemosiderosis of liver.
Anemia, Hemolytic ; Anemia, Iron-Deficiency ; Hemoglobinuria, Paroxysmal* ; Hemosiderin ; Hemosiderosis ; Liver ; Magnetic Resonance Imaging ; Spleen ; Venous Thrombosis

Iron is critical for almost all living organisms because it serves as a cofactor for many proteins and enzymes necessary for oxygen and energy metabolism. Disruption of iron homeostasis is associated with a wide range of diseases. Thus mammals have developed sophisticated mechanisms to maintain optimal range of iron concentration. Iron regulation involves processes at the systemic and cellular levels. These processes are regulated by hepcidin and iron regulatory proteins. Hepcidin modulates systemic iron homeostasis with ability to impede cellular iron export via interaction with the iron export protein, ferroportin. Whereas, iron regulatory proteins control cellular iron homeostasis by translational regulation of proteins which involve iron metabolism. Recent advances in the study of iron metabolism have shown promising results that hepcidin-targeted strategies may help to improve the diagnosis and treatment of iron related diseases. Although these strategies are now under development, ongoing studies can help to elucidate its application possibilities.
Diagnosis ; Energy Metabolism ; Hepcidins ; Homeostasis ; Iron Metabolism Disorders ; Iron* ; Iron-Regulatory Proteins ; Mammals ; Metabolism* ; Oxygen

Hemochromatosis ; metabolism ; Humans ; Iron Overload ; Liver ; metabolism ; Male ; Middle Aged

Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.
Anemia ; Anemia, Iron-Deficiency ; Child ; Cough ; Diagnosis ; Dyspnea ; Fibrosis ; Hemoptysis ; Hemorrhage ; Hemosiderin ; Hemosiderosis* ; Humans ; Inhalation* ; Iron ; Macrophages ; Male ; Obesity ; Prednisolone ; Rare Diseases ; Steroids

Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.
Anemia ; Anemia, Iron-Deficiency ; Child ; Cough ; Diagnosis ; Dyspnea ; Fibrosis ; Hemoptysis ; Hemorrhage ; Hemosiderin ; Hemosiderosis* ; Humans ; Inhalation* ; Iron ; Macrophages ; Male ; Obesity ; Prednisolone ; Rare Diseases ; Steroids

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been suggested, but the etiology of IPH remains unknown. We report on a 9-year-old girl with idiopathic pulmonary hemosiderosis who showed seasonal recurrences without cause.
Anemia, Iron-Deficiency ; Child ; Hemoptysis ; Hemorrhage ; Hemosiderosis ; Humans ; Lung Diseases ; Macrophages ; Pulmonary Fibrosis ; Rare Diseases ; Recurrence ; Respiratory Insufficiency ; Seasons

Hemochromatosis is a disorder caused by excessive iron deposition in parenchymal cells that leads to cellular damage and organ dysfunction. The excessive iron overload of secondary hemochromatosis is associated with chronic disorders of erythropoiesis that are treated with prolonged repeated blood transfusions. We experienced two cases of transfusional hemochromatosis involving the pituitary gland, and we report the findings of the MR imaging.
Blood Transfusion ; Erythropoiesis ; Hemochromatosis* ; Humans ; Iron ; Iron Overload ; Magnetic Resonance Imaging* ; Pituitary Gland*