OBJECTIVE To evaluate the prevalence of hypoparathyroidism in adult transfusion-dependent patients with beta-thalassemia major in a teaching referral hospital in Oman. METHODS All adult (>13 years) patients with beta-thalassemia major seen at Royal Hospital in Oman between 2004 and 2006 were studied. Demographic, pharmaceutical, clinical and biochemical data were collected for all the subject...

OBJECTIVE Clinical predictors for acute respiratory distress syndrome (ARDS) have been studied in few prospective studies. Although transfusions are common in the intensive care unit, the role of submassive transfusion in non-trauma-related ARDS has not been studied. We describe here the clinical predictors of ARDS risk and mortality including the role of red cell transfusion. DESIGN Observat...

BACKGROUND AND OBJECTIVES Transferrin receptor (TfR) expression in erythroid cells is regulated by a number of factors, including iron status and erythropoietin (Epo) stimulation. However, the impact of these factors on reticulocyte TfR expression in vivo has never been studied. A soluble form of TfR (sTfR) is present in serum in proportion to the mass of cellular TfR. Although sTfR shedding by...

To cite: Singh A, Varma S. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2014-207637 DESCRIPTION A 19-year-old man presented with dysmorphic facies, progressive fatigability and exertional breathlessness from the age of 15 years. On examination the patient had classical ‘Chipmunk facies’ (figure 1), short stature, delayed puberty and mild splenomegaly. X-ray of...

BACKGROUND Hematopoieticic stem cell transplantation is the only therapeutic option that can cure thalassemia disease. Reduced intensity hematopoietic stem cell transplantation (RI-HSCT) has demonstrated a high cure rate with minimal complications compared to other options. Because RI-HSCT is very costly, economic justification for its value is needed. This study aimed to estimate the cost-util...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

IT IS BELIEVED that bone marrow contains a relatively large mass of reticuloendothelial (RE) cells. These provide a lining for the marrow sinusoidss 3 and are potentially capable of phagocytosing injected foreign particles such as carbon and electronegative colloidal dyes, much as do the RE cells of liver and spleen.4 However, there is little quantitative information concerning this population ...

The rapidity and duration of the response of non-transferrin-bound iron (NTBPI) to chelation therapy are largely unknown and have important implications for the design of optimal chelation regimens. Methodology was developed to measure simultaneously NTBPI, deferoxamine (DFO), and its major metabolite. NTBPI was present in all but 2 of 28 thalassaemia major (TM) patients who had received conven...

Resistance to malignant erythropoiesis induced by Friend spleen focus-forming virus and resistance to marrow stem cell allografts are under genetic control. Strains of mice, e.g., C57BL/6 and B10.D2, which are homozygous for resistance at the Fv-2 locus, are also good rejectors of most bone marrow allografts. (89)Sr, a bone-seeking isotope, irradiates marrow but not other lymphoid organs and ab...