Hematology
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Review Case Reports
Exertional rhabdomyolysis and renal failure in patients with sickle cell trait: is it time to change our approach?
Based upon numerous reported cases and despite widespread beliefs to the contrary, sickle cell trait (SCT) may be deemed a quantifiable risk factor in certain subsets of patients. As a result of common misconceptions regarding SCT, most individuals with the condition are generally not informed regarding the possible consequences of certain activities such as venturing to high altitudes or participating in overly exertional physical activities. Acute exertional rhabdomyolysis is a potentially serious clinical illness and is caused by skeletal muscle injury resulting in the release of myoglobin and other cellular contents, including creatine kinase, into the circulatory system. ⋯ Several case reports have been published since the early 1970s describing significant morbidity and mortality of acute exertional rhabdomyolysis in patients with SCT. We present the case of a 27-year-old male with a past medical history significant only for SCT who presented after a 1.5 mile run with severe exertional rhabdomyolysis and subsequent acute renal failure requiring hemodialysis (HD). In presenting this case, we hope to raise awareness of a possible underlying cause to many cases of exertional rhabdomyolysis and encourage physicians to counsel their patients with SCT in order to avoid the significant morbidity and mortality that may be associated with the condition.
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Disorders of coagulation in children often prove challenging to the medical care team. The aims of this study were to assess the spectrum and prevalence of coagulation disorders among children attending Mansoura University Children Hospital (MUCH), Mansoura, Egypt. A total of 105 pediatric patients were referred to MUCH. ⋯ Anti-clotting factors inhibitor was detected in 18.2% of patients with hemophilia A and in 9.1% with hemophilia B. In conclusion, our study found that hemophilias are the most prevalent congenital coagulation disorders among children. Attention must be given for detection of hemophilia carriers and inhibitors of clotting factors.
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Both bone marrow stromal cells (BMSCs) and transcription factors (GATA-1, GATA-2 and GATA-3) are important in the normal hematopoiesis and the pathogenesis of hematopoietic disease. The purpose of this study was to investigate the expression of GATA-1, GATA-2 and GATA-3 genes in the bone marrow (BM) microenvironment from patients with chronic aplastic anemia (cAA) and normal individuals. Mononuclear cells (MNCs) were isolated from BM of patients with cAA (8 cases) and normal controls (9 cases). ⋯ The dominant expression of GATA-3 gene was found in the BMSCs from cAA and normal controls. GATA genes can be expressed in the BMSCs and may play a role in the regulation of hematopoiesis in normal individuals, as well as in patients with cAA. The change of expression levels of GATA genes may influence the hematopoiesis in BM microenvironment and relate to the pathogenesis and development of aplastic anemia.