No abstract available.
Hemosiderosis*

Repeated blood transfusions in transfusion dependent thalassemia (TDT) leads to iron overload-related endocrine complications. Hypoparathyroidism (HPT) with severe signs of hypocalcemia is a recognized complication among these patients. A 14-year-old thalassaemic boy, on regular transfusion and on anticonvulsant therapy with a presumptive diagnosis of epilepsy for the last 1 year, was admitted with high fever and severe muscle cramps with positive Trousseau’s sign. He was diagnosed as a case of primary HPT and magnesium deficiency on the basis of low serum calcium, high phosphate, normal alkaline phosphates, very low intact parathyroid hormone (iPTH), normal serum vitamin D and very low serum magnesium level. His calcium, magnesium and phosphate level normalised following treatment with intravenous magnesium and calcium. His iPTH improved but remained at low normal. He was discharged from hospital with oral calcium, calcitriol, and magnesium supplementation. The anticonvulsant (Phenobarbitone) was successfully withdrawn gradually over the next six months without any recurrence of seizure in the subsequent 3 years of follow up. Acquired HPT (apparently from hemosiderosis) is a common cause of hypocalcemia; and magnesium depletion further complicated the situation leading to severe hypocalcemia with recurrent episodes of convulsion. Magnesium replacement improved the parathyroid hormone (PTH) value proving its role in acquired HPT. Very high phosphate level on admission and poor PTH response with respect to the low serum calcium, indicates intrinsic parathyroid pathology. Metabolic abnormalities should always be evaluated in thalassaemic subject with seizure disorder and it appears that the initial convulsive episodes were due to hypocalcemia. Muscle pain, cramps or convulsion may occur from HPT and simultaneous magnesium deficiency in transfusion dependent thalassaemic subjects. Metabolic correction is more important than anticonvulsant medication. Calcium and magnesium should both be assessed routinely in transfusion dependent thalassemic patients.
Hemosiderosis ; Hypoparathyroidism ; Thalassemia

No abstract available.
Hemosiderosis* ; Humans ; Kidney Failure, Chronic* ; Seizures*

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

Idiopathic pulmonary hemosiderosis is characterized by cough, hemoptysis, dyspnea, diffuse pulmonary infiltrates, and microcytic and hypochromic anemia. The cause of this illness is unknown. We experienced a case of idiopathic pulmonary hemosiderosis in a 2 year and 8 month-old boy. Hemosiderin-laden macrophages are demonstrated in smears of material obtained from tracheal aspirates. There were no specific causes for pulmonary hemorrhage. We report a case of idiopathic pulmonary hemosiderosis with brief review of related literatures.
Anemia, Hypochromic ; Cough ; Dyspnea ; Hemoptysis ; Hemorrhage ; Hemosiderosis* ; Humans ; Infant ; Macrophages ; Male

An 18-Year-old male hemophiliac with high titer of factorVIII inhibitor, stage V hemophilic arthropathy in right knee joint and a history of hematuria and retroperitoneal hemorrhage was admitted because of acute and massive bleeding of epistaxis, pulmonary hemorrhage and intestinal bleeding. The bleeing was not controolled by massive infusion of factorVIII concentrates but by prothrombin complex concentrates and high dose of factorVIII concentrates. He showned symptoms of sustained fever and diffuse pulmonary infiltration which was diagnosed as pulmonary hemosiderosis by MRI. We suppressed his immune reaction by prednisolne to prevent the formation of factorVIII inhibitor. He has been followed up for 3 years and shown no massive bleeding there-after.
Adolescent ; Epistaxis ; Fever ; Hematuria ; Hemophilia A* ; Hemorrhage ; Hemosiderosis* ; Humans ; Knee Joint ; Magnetic Resonance Imaging ; Male ; Prothrombin

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

We report a case of biopsy-proven secondary hemosiderosis of the lung in a 58-year-old patient with mitralvalvular heart disease. Both chest radiography and high-resolution CT demonstrated patchy areas of ground-glassopacity; the former indicated that it was in both lungs, while the latter showed inter- and intralobular septalthickening. These findings were reversible when pulmonary venous hypertension was corrected.
Heart Diseases ; Heart Valve Diseases* ; Hemosiderosis* ; Humans ; Hypertension ; Lung ; Middle Aged ; Radiography ; Thorax

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.
Body Mass Index ; Child ; Cough ; Dyspnea ; Ferritins ; Hemoptysis ; Hemosiderosis ; Humans ; Obesity ; Pathology ; Prednisolone ; Weight Gain