1/12. Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin.Juvenile hemochromatosis is a rare genetic disorder that causes iron overload. Clinical complications, which include liver cirrhosis, heart failure, hypogonadotropic hypogonadism and diabetes, appear earlier and are more severe than in HFE-related hemochromatosis. This disorder, therefore, requires an aggressive therapeutic approach to achieve iron depletion. We report here the case of a young Italian female with juvenile hemochromatosis who was unable to tolerate frequent phlebotomy because of coexistent ss-thalassemia trait. The patient was successfully iron-depleted by combining phlebotomy with recombinant human erythropoietin.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
2/12. Complete heart block in thalassemia major: a case report.Cardiac complications of iron overload are the most common cause of death in patients with thalassemia major. These complications include recurrent pericarditis, refractory congestive heart failure and rhythm disorders. The usual rhythm disturbances are supraventricular or ventricular premature contractions and first-or second-degree heart block. Complete heart block is a very rare complication of thalassemia major. Herein, we report a case of complete heart block with thalassemia major. The patient also had serious congestive heart failure. Management of the heart block with pacemaker brought no clinical improvement, and she died in the second month of hospitalization.- - - - - - - - - - ranking = 1.4keywords = thalassemia (Clic here for more details about this article) |
3/12. Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia.Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
4/12. Pulmonary hypertension and beta-thalassemia major: report of a case, its treatment, and a review of the literature.Pulmonary hypertension is a common complication of beta-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with beta-thalassemia major and review the literature on pulmonary hypertension and beta-thalassemia major. A 28-year-old man with beta-thalassemia major, splenectomy, hepatitis c, and hemosiderosis who presented with increasing dyspnea on exertion was diagnosed with pulmonary hypertension. After receiving continuous epoprostenol infusion and desferoxamine, his functional capacity and hemodynamic status improved. To our knowledge, this is the first case of pulmonary hypertension associated with beta-thalassemia treated with continuous epoprostenol infusion and desferoxamine. epoprostenol, beneficial in the treatment of other types of pulmonary hypertension, may ameliorate the morbidity and mortality of pulmonary hypertension associated with thalassemia.- - - - - - - - - - ranking = 2keywords = thalassemia (Clic here for more details about this article) |
5/12. Successful recovery of acute hemosiderotic heart failure in beta-thalassemia major treated with a combined regimen of desferrioxamine and deferiprone.We report the case of a 25-years-old male with beta-thalassemia major who developed acute heart failure, with severe systolic dysfunction, resulting from iron overload. Combined iron chelation with desferrioxamine and deferiprone together with standard cardiological treatment induced prompt and complete restoration of the cardiac function.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
6/12. Hepatocellular carcinoma in thalassemia major.The occurrence of hepatocellular carcinoma in a 22-year-old man with thalassemia major is reported. As a result of transfusional hemochromatosis, this patient had already developed diabetes, hypogonadism, heart failure, and the sicca syndrome; he was serum and tissue HBsAg negative. Liver iron concentration measured postmortem was found to be 50 times normal. Multiply transfused patients are at risk of developing hepatocellular carcinoma. Serial measurements of serum alpha-fetoprotein should permit early detection of the tumor and reduce mortality. Preventive measures include early immunisation against hepatitis b virus and prevention of iron accumulation by intensive use of desferrioxamine. Treatment of hemochromatosis-associated hypogonadism with androgens should be considered with caution.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
7/12. yersinia enterocolitica osteomyelitis in a child.yersinia enterocolitica systemic infections are uncommon and osteomyelitis due to this organism is very rare. We report a 9-year-old girl with thalassemia major, liver cirrhosis, and hemosiderosis who developed osteomyelitis of the rib caused by Y enterocolitica type 0:3. Serologic response to the infection was confirmed. No definite source of infection was identified.- - - - - - - - - - ranking = 0.2keywords = thalassemia (Clic here for more details about this article) |
8/12. magnetic resonance imaging of transfusional hemosiderosis complicating thalassemia major.Tissue deposits of hemosiderin, a paramagnetic iron-protein complex, resulted in marked abnormalities of magnetic resonance (MR) spin-echo signal intensity within the viscera of three children with transfusional hemosiderosis and thalassemia major. In all patients the liver and bone marrow demonstrated abnormally low spin-echo intensities and the kidneys and muscles had abnormally high intensities. These observations correlate with in vitro MR observations of ferric (Fe 3) solutions, in which concentrations of ferric salts greater than 20 mmol yielded a low MR intensity signal and ferric concentrations less than 15 mmol yielded higher intensities than did water alone. MR imaging is sensitive to the tissue deposition of hemosiderin, and MR intensity appears to provide a rough measure of the amount of iron deposited.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
9/12. iron overload in a non-transfused patient with thalassaemia intermedia.A patient with thalassemia intermedia and haemosiderosis is reported. This patient did not receive transfusions or iron therapy. The iron absorption and the plasma iron turnover (PIT) were increased. Transfusions were carried out in order to decrease the amount of abnormal erythropoiesis. After that, the erythropoietin and PIT were decreased to normal levels and the iron absorption also returned to normal. The data presented suggest that increased erythropoiesis was responsible for the abnormally high iron absorption and subsequently for the haemosiderosis of the patient presented.- - - - - - - - - - ranking = 0.2keywords = thalassemia (Clic here for more details about this article) |
10/12. A case of hypoparathyroidism in a child with beta-thalassemia successfully treated with 1 alpha-hydroxy-vitamin D3.A 13-year-old boy with homozygous beta-thalassemia who developed clinically manifest and biochemically proven hypoparathyroidism is described. The latter disease was attributed to hemosiderosis. Laboratory abnormalities were corrected after 1 alpha-hydroxy-vitamin D3 was administered.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
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