Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited.
We aimed ...to establish the frequency and types of pediatric brain injury in a cross-sectional study at a large SCA clinic in Kampala, Uganda in a randomly selected sample of 265 patients with HbSS ages 1-12 years. Brain injury was defined as one or more abnormality on standardized testing: neurocognitive impairment using an age-appropriate test battery, prior stroke by examination or transcranial Doppler (TCD) velocities associated with stroke risk in children with SCA (cerebral arterial time averaged mean maximum velocity ≥ 170 cm/second).
Mean age was 5.5 ± 2.9 years; 52.3% were male. Mean hemoglobin was 7.3 ± 1.01 g/dl; 76.4% had hemoglobin < 8.0 g/dl. Using established international standards, 14.7% were malnourished, and was more common in children ages 5-12. Overall, 57 (21.5%) subjects had one to three abnormal primary testing. Neurocognitive dysfunction was found in 27, while prior stroke was detected in 15 (5.7%). The most frequent abnormality was elevated TCD velocity 43 (18.1%), of which five (2.1%) were in the highest velocity range of abnormal. Only impaired neurocognitive dysfunction increased with age (OR 1.44, 95%CI 1.23-1.68), p < 0.001). In univariate models, malnutrition defined as wasting (weight-for-height ≤ -2SD), but not sex or hemoglobin, was modestly related to elevated TCD (OR 1.37, 95%CI 1.01-1.86, p = 0.04). In adjusted models, neurocognitive dysfunction was strongly related to prior stroke (OR 6.88, 95%CI 1.95-24.3, p = .003) and to abnormal TCD (OR 4.37, 95%CI 1.30, p = 0.02). In a subset of 81 subjects who were enriched for other abnormal results, magnetic resonance imaging and angiography (MRI/MRA) detected infarcts and/or arterial stenosis in 52%. Thirteen subjects (25%) with abnormal imaging had no other abnormalities detected.
The high frequency of neurocognitive impairment or other abnormal results describes a large burden of pediatric SCA brain disease in Uganda. Evaluation by any single modality would have underestimated the impact of SCA. Testing the impact of hydroxyurea or other available disease-modifying interventions for reducing or preventing SCA brain effects is warranted.
Most countries in Sub‐Saharan Africa (SSA) are either low‐income or low‐middle income countries, that is countries whose gross national income per capita is $995 (USD) or less or $996–3895, ...respectively. Added to this, they have very few health care professionals specifically trained in transfusion medicine and are the countries whose populations have a high prevalence of transfusion‐transmissible agents (especially HIV, hepatitis B and malaria) and whose patients (women haemorrhaging at birth, men in motor vehicle or motorcycle accidents, children with malaria or sickle cell anaemia) are often in urgent need of blood transfusion. This combination of few resources, both financial and human, combined with many potential donors at risk of transmitting infection and patients with urgent transfusion requirements renders the provision of a safe and adequate blood supply in SSA extremely challenging. In this review, we will discuss the current literature addressing how these challenges are being met and present one example of a SSA national blood transfusion service, the Uganda Blood Transfusion Service.
BACKGROUND: Acute transfusion reactions (ATRs) are probably underdiagnosed in critically ill children because associated symptoms can frequently be attributed to the patient’s underlying disease. ...This study was undertaken to determine the incidence, type, imputability, and severity of ATRs observed in a tertiary care pediatric intensive care unit (PICU).
STUDY DESIGN AND METHODS: All transfusions of labile blood product administered to consecutive patients admitted to our PICU, between February 2002 and February 2004, were prospectively recorded. For each transfusion, the bedside nurse recorded the patient’s status before, during, and up to 4 hours after the transfusion, as well as the presence of any new sign or symptom suggesting an ATR. Three independent experts retrospectively reviewed all transfusion event reports and hospital charts. The presence, type, imputability, and severity of ATRs were adjudicated by consensus of two of three experts (Delphi method), with predefined criteria.
RESULTS: A total of 2509 transfusions were administered to 305 patients during the study. Forty transfusion events (1.6%) were confirmed to be ATRs by expert consensus: 24 febrile nonhemolytic, 6 minor allergic, 4 isolated hypotension, 3 bacterial contamination, 1 major allergic (anaphylactic shock), 1 TRALI, and 1 hemolytic reaction. Imputability of ATRs was probable or possible in 35 cases (88%). ATRs led to an immediate vital threat in 15 percent of cases.
CONCLUSION: Improved surveillance of transfusions given to PICU patients and better knowledge of these reactions by health care professionals should improve the safety of transfusions in the PICU.
To determine the most effective, evidence-based approach to the use of platelet transfusions in patients with cancer.
Outcomes of interest included prevention of morbidity and mortality from ...hemorrhage, effects on survival, quality of life, toxicity reduction, and cost-effectiveness.
A complete MedLine search was performed of the past 20 years of the medical literature. Keywords included platelet transfusion, alloimmunization, hemorrhage, threshold and thrombocytopenia. The search was broadened by articles from the bibliographies of selected articles.
Levels of evidence and guideline grades were rated by a standard process. More weight was given to studies that tested a hypothesis directly related to one of the primary outcomes in a randomized design. BENEFITS/HARMS/COST: The possible consequences of different approaches to the use of platelet transfusion were considered in evaluating a preference for one or another technique producing similar outcomes. Cost alone was not a determining factor.
Appendix A summarizes the recommendations concerning the choice of particular platelet preparations, the use of prophylactic platelet transfusions, indications for transfusion in selected clinical situations, and the diagnosis, prevention, and management of refractoriness to platelet transfusion.
Five outside reviewers, the ASCO Health Services Research Committee, and the ASCO Board reviewed this document.
American Society of Clinical Oncology
BACKGROUND: Canadian Blood Services' disposition reports suggested considerable variation in cryoprecipitate use and prompted this national audit.
STUDY DESIGN AND METHODS: Thirty‐one institutions ...were invited to participate in a 2‐month audit. Patient information and relevant laboratory and transfusion data were collected. Cryoprecipitate transfusions were categorized as appropriate if a fibrinogen level (taken 6 hr before/after transfusion) was not more than 1.0 g per L and inappropriate if the pretransfusion fibrinogen level was more than 1.0 g per L and posttransfusion fibrinogen level was more than 1.0 g per L or not performed. Appropriateness was categorized as undetermined if the pretransfusion fibrinogen level was not performed and the posttransfusion fibrinogen level was more than 1.0 g per L or not performed.
RESULTS: Overall, 25 of 31 invited hospitals agreed to participate. A total of 4370 units of cryoprecipitate were transfused in 603 events to 453 patients representing 62 percent of cryoprecipitate issued to hospitals during the time period. Comparison of the number of units of cryoprecipitate per 100 units of red blood cells (RBCs) transfused by each institution showed significant variation in practice (mean, 9 per 100 RBCs; range, 2 to 27 units). The single most common indication for cryoprecipitate was cardiac surgery (45.4% of events). Overall, 24 percent of cryoprecipitate transfusions were considered to be appropriate (pretransfusion fibrinogen level ≤1 g/L in 19% and posttransfusion fibrinogen level ≤1.0 g/L in another 5%), 34 percent were inappropriate, and in 42 percent appropriateness could not be determined.
CONCLUSION: A 2‐month audit of cryoprecipitate use in Canada revealed that the majority of cryoprecipitate use in Canada is not in accordance with published guidelines.
Objective: To compare the efficacy of Shirodkar to McDonald cerclage in patients with singleton pregnancies undergoing an ultrasound-indicated cerclage. Methods: Historical cohort of all patients ...with singleton pregnancies undergoing cerclage for the indication of a short cervix on ultrasound (ultrasound indicated) at one institution in 2005-2010. We compared outcomes based on cerclage type, Shirodkar or McDonald. Outcome measures were gestational age (GA) at delivery, delivery ≥35 weeks, ≥32 weeks, and PPROM. Multivariable regression analysis was performed to control for significant variables. Results: Seventy-four patients with singleton pregnancies underwent an ultrasound-indicated cerclage in the study period (47 Shirodkar, 27 McDonald). Shirodkar was associated with later GA at delivery (mean GA at delivery 36.98 +/− 3.39 vs. 33.34 +/− 6.37 weeks, p = 0.006), a higher likelihood of delivering ≥35 weeks (83 vs. 55.6%, p = 0.011) and ≥32 weeks (91.5 vs. 59.3%, p = 0.001), and a lower likelihood of preterm premature rupture of membrane (PPROM) (13.0 vs. 46.2%, p = 0.002). On adjusted analysis controlling for differing baseline characteristics, Shirodkar remained significantly associated with an increased incidence of delivery ≥32 weeks (odds ratio OR: 5.180, 95% CI: 1.024-26.205). Conclusion: Compared to the McDonald technique, the Shirodkar technique was more effective in prolonging pregnancy in patients with singleton pregnancies undergoing ultrasound-indicated cerclage. A prospective trial is needed to compare these two techniques.
BackgroundRecognition of the burden of sickle cell anaemia (SCA) in sub-Saharan African (SSA) countries is increasing, with few therapies available for clinical management. Hydroxyurea is the only ...disease-modifying therapy that has proven feasible and clinically efficacious in low-income countries in SSA; however, the health economic implications of its use in this region have not been quantified. Thus, we examined the incremental cost-effectiveness of hydroxyurea given as a fixed-dose regimen or at the maximum tolerated dose (MTD).MethodsWe estimated the cost of outpatient treatment at a specialized sickle cell clinic in Kampala, Uganda, from a provider’s perspective. These estimates were used in a discrete-event simulation model to project mean costs (US$), disability-adjusted life years (DALYs), and consumption of blood products per patient (450 ml units). We calculated cost-effectiveness as the ratio of incremental costs over incremental DALYs averted, discounted at 3% annually.FindingsFor Ugandan patients under the age of 18, we predicted that hydroxyurea at the MTD would avert an expected 1.38 DALYs and save US$ 111 per patient compared to standard care, while hydroxyurea at a fixed dose would avert 0.81 DALYs per patient at an incremental cost of US$ 21. Additionally, we predicted that the fixed-dose alternative would save 9.2 (95% CI 9.0–9.3) units of whole-blood equivalents per patient, while the MTD strategy saved 11.3 (95% CI 11.1–11.4) units of blood per patient.
Purpose To provide evidence-based guidance on the use of platelet transfusion in people with cancer. This guideline updates and replaces the previous ASCO platelet transfusion guideline published ...initially in 2001. Methods ASCO convened an Expert Panel and conducted a systematic review of the medical literature published from September 1, 2014, through October 26, 2016. This review builds on two 2015 systematic reviews that were conducted by the AABB and the International Collaboration for Transfusion Medicine Guidelines. For clinical questions that were not addressed by the AABB and the International Collaboration for Transfusion Medicine Guidelines (the use of leukoreduction and platelet transfusion in solid tumors or chronic, stable severe thrombocytopenia) or that were addressed partially (invasive procedures), the ASCO search extended back to January 2000. Results The updated ASCO review included 24 more recent publications: three clinical practice guidelines, eight systematic reviews, and 13 observational studies. Recommendations The most substantial change to a previous recommendation involved platelet transfusion in the setting of hematopoietic stem-cell transplantation. Based on data from randomized controlled trials, adult patients who undergo autologous stem-cell transplantation at experienced centers may receive a platelet transfusion at the first sign of bleeding, rather than prophylactically. Prophylactic platelet transfusion at defined platelet count thresholds is still recommended for pediatric patients undergoing autologous stem-cell transplantation and for adult and pediatric patients undergoing allogeneic stem-cell transplantation. Other recommendations address platelet transfusion in patients with hematologic malignancies or solid tumors or in those who undergo invasive procedures. Guidance is also provided regarding the production of platelet products, prevention of Rh alloimmunization, and management of refractoriness to platelet transfusion ( www.asco.org/supportive-care-guidelines and www.asco.org/guidelineswiki ).
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