Background: School difficulties are common in patients with sickle cell disease (SCD). The study aimed to assess the relationship between severity of SCD in children and their school absenteeism and achievement. Subjects and Methods: The sample included school-aged children (7–15 years old) with SCD who were enrolled from Hematology Clinic in Al-Sadaqa General Teaching Hospital, Aden, during 2013 through 2014. Data about school absence, academic score achievement, and grade retention were collected. Disease severity was assessed by frequency of clinical events and complications. Differences between groups were assessed by appropriate statistical analysis. Results: Sixty children were included for the study; their mean age was 11.5 ± 2.4 years and 51.7% were of the female sex. The number of days absent from school in a year ranged from 0 to 112 days, with a median (IQR) of 28 (14–45) days. Absence of more than 20 days in the previous academic year was reported in 60% of the children. Grade retention was reported in 45% of the children. Both school absence and grade retention were significantly correlated with age (r = 0.35, P = 0.006 and r = 0.32, P = 0.01, respectively). During the previous academic year, 48.3% of the children reported a below average final academic score. Severity assessment revealed that 65% of the children scored as severe. School absenteeism was significantly associated with disease severity score (r = 0.44, P < 0.001). Children with low academic achievement and those with grade retention had significantly higher disease severity score (all P < 0.05). Conclusion: This study suggests that disease severity has important influences on school attendance and performance. Interventions to modify disease severity and school absence might improve academic performance in the affected children.

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Objectives: Venous thromboembolism is an important and potentially life-threatening complication of nephrotic syndrome (NS). This study aims to evaluate the functional test of thrombin generation (TG) in different stages of NS; determine its relation with the coagulation screening tests (prothrombin time [PT] and activated partial thromboplastin time), hemostatic activation markers (thrombin–antithrombin complex [TAT] and prothrombin fragment 1+2 [PF1+2]), and von Willebrand factor (vWF) and its proteolytic enzyme ADAMTS-13; and determine the correlation between TG and NS severity, as reflected by the levels of proteinuria and albumin. Materials and Methods: This case–control cross-sectional study included 125 patients (n = 40, nephrotic range proteinuria; n = 45, NS; n = 40, remission) and 80 controls. Calibrated automated thrombogram assay (endogenous thrombin potential [ETP]) was performed to determine TG. TAT, PF1+2, vWF, and ADAMTS-13 were measured using enzyme-linked immunosorbent assay. Results: TG (ETP), TAT, PF1+2, and vWF levels were significantly higher in all of the patient groups (P < 0.0001) than in the control group. ADAMTS-13 levels were significantly lower in the NS group (P < 0.0001) than in the control group. Conclusion: Our findings confirm activation of the coagulation pathway in nephrotic patients. However, the degree of hypercoagulopathy (especially TG [ETP]) is positively correlated with proteinuria. Proteinuria could be considered an indirect indicator of the highest risk of thrombotic disease in patients with NS.

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Background: Fondaparinux (Arixtra) a synthetic pentasaccharide that causes an antithrombin III-mediated selective inhibition of factor Xa. The clearance of fondaparinux reduces in patients with renal impairment, and there are no dosage adjustments provided in the manufacturer’s labeling. In patients with creatinine clearance rate (CrCl) >50 ml/min, total clearance is reduced by 25% while in case of CrCl 30–50 ml/min, the total clearance could be 40% lower when compared to patients with normal renal function. Aim of the Study: To evaluate the risk of bleeding in patients with renal impairment treated with fondaparinux. Materials and Methods: We performed a retrospective chart review study of patients 18 years of age and older who received fondaparinux between 11/10/2003 and 30/12/2009 during their hospital stays, and who had a CrCl of ≤80 ml/min. The patients were classified according to their degree of renal dysfunction as either stage A (CrCl: 80–50 ml/min; mild dysfunction) or stage B (CrCl: <50 ml/min; moderate or severe dysfunction). The HAS-BLED scoring system (HAS-BLED mnemonic stands for: hypertension, abnormal renal and liver function, stroke, bleeding, labile international normalized ratios, elderly, drugs or alcohol) was used to categorize the bleeding risk as mild, moderate, or high. Additionally, the bleeding severity was categorized as either major bleeding or minor bleeding. Results: A total of 165 patients were included in the study; of which 87 were men. In that 52.7% of the total were classified as stage A and the remainder as stage B. The patients classified as stage B were more frequently classified at high risk of bleeding than stage A patients (48.7%, n = 38 of stage B patients vs. 23.0%, n = 20 of stage A patients). Twenty-three percent (n = 38) of the patients experienced bleeding, and most of which were stage B patients (55.3%, n = 21). The majority of the patients who bled experienced major bleeding (71.0%, n = 27). Ten percent (n = 16) of the total number of patients, whose fondaparinux doses were adjusted as per the drug monograph, were documented to have had a bleeding event during their hospital stay. By contrast, 13% of the total number of patients (n = 22) who required dose adjustments and received fondaparinux without adjustments had bleeding events. Conclusion: Fondaparinux increases the risk of bleeding in patients with mild-to-moderate renal impairment even with appropriate dose adjustments. The risk of bleeding and the incidence of major bleeding are increased in patients with moderate and severe renal dysfunction.

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Peliosis is an extremely rare condition of unknown etiology, and its pathogenesis is characterized by multiple, cyst-like, blood-filled cavities predominantly involving a mononuclear phagocytic system. Isolated splenic peliosis is extremely uncommon, but may present as a potentially life-threatening situation. An awareness of its clinical condition and prompt management may improve the clinical outcomes. The role of prophylactic splenectomy is not clear in this condition.

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Background: Tumor lysis syndrome (TLS) is an oncological emergency. Rasburicase (recombinant urate oxidase) has been proven to be an effective therapy for prevention of TLS and its serious consequences in patients with hematological malignancies such as acute leukemias with high white blood cells count, Burkitt lymphoma, and lymphoblastic lymphoma with high tumor burden. The US Food and Drug Administration recommended daily dosing regimen for 5 days is unaffordable by each and every patient in developing countries such as India. Recently, the conducted studies have clearly shown a similar efficacy for a single dose of rasburicase. Herein, we report a case series of 15 patients, including children and adults with hematologic malignancies, in whom TLS was managed by a single dose of rasburicase. Materials and Methods: We retrospectively analyzed the efficacy of single-dose rasburicase (SDR) (0.15 mg/kg intravenous infusion over 30 min) in patients with hematologic malignancies at risk for TLS. The drug was administered in five adult and 10 pediatric patients admitted to the Gujarat Cancer and Research Institute between January 2013 and December 2014. Results: The study included 15 patients, out of which 10 were pediatric (8 male:2 female) and five were adults (5 male:0 female). Patients with hematologic malignancies having Eastern Cooperative Oncology Group performance status 0–2 and at high risk or potential risk for TLS were selected. The median ages in pediatric and adult groups were 7.7 years and 32 years, respectively. The presence of hyperuricemia (plasma uric acid (UA) levels ≥7.5 mg/dl) or a diagnosis of very aggressive lymphoma or leukemia based on the World Health Organization classification of hematopoietic and lymphoid neoplasms in patients was classified as high-risk. Rasburicase was administered in a single dose of 0.15 mg/kg intravenously over 30 min. Patients were evaluated by clinical examination and blood biochemical tests at frequent intervals. Plasma samples for UA were collected at baseline before rasburicase, 6–24 h post-rasburicase, 48 h post-rasburicase, and daily during treatment. The blood samples for UA during the course of treatment were collected in prechilled tubes containing heparin and immediately immersed and transported on ice. The blood samples were analyzed within 4 h of collection. Serum electrolytes, blood urea nitrogen, creatinine, calcium, and phosphorous were monitored daily during this period. A single dose of rasburicase produced a rapid and sustained therapeutic effect of lowering the plasma UA levels in all the 15 patients. Renal parameters normalized within 72 h. UA levels remained below 4 mg/dl throughout the administration of chemotherapy until discharge, and none of the patients required a repeat dosing of rasburicase. Conclusion: SDR is a highly economical and clinically effective way of managing patients with TLS and could serve as an alternative to the 5-day treatment in a resource-limited country such as India.

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In haemophilia-A patients, if the plasma concentration of factor VIII could be maintained at an optimum level, an open wound can be easily covered with split-thickness skin grafting without any significant bleeding or complication. Removing a split-thickness layer of skin should cause no more difficulty than in a normal individual. This can be attributed to normal bleeding time, prothrombin time, clot retraction and platelet count found in such patients. Skin grafting over non-healing raw areas in haemophiliac patients can significantly decrease the morbidity and length of hospitalization required, thereby, decreasing health expenditure. We present a case report of a haemophilia-A patient having raw area over lower limb, which was managed successfully by skin grafting.

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