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Establishing Sickle Cell Disease Stroke Prevention Teams in Africa is Feasible: Program Evaluation Using the RE-AIM Framework
We used the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework to evaluate a Stroke Prevention Team’s readiness to prevent strokes in children with sickle cell anemia living in northern Nigeria. The NIH sponsored Stroke Prevention Trial in Nigeria included a goal of a sustainable stroke prevention program. The program’s 1-year reach for transcranial Doppler screening was 14.7% (4710/32,000) of which 6.0% (281/4710) had abnormal velocities (≥200 cm/s). All participants with abnormal transcranial Doppler velocities were started on hydroxyurea (effectiveness). The leaders of all 5 hospi...
Source: Journal of Pediatric Hematology Oncology - January 1, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Transcranial Doppler Screening in a Regional Care Network for Sickle Cell Disease in the United Kingdom
The risk of stroke in children screened with transcranial Doppler ultrasound in the United Kingdom is not known. We evaluated a clinician-led program using a risk assessment modified from the STOP protocol. High-risk classification included abnormal velocities in the anterior cerebral artery, and single abnormal scan if initial velocity>220 cm/s (high abnormal) or if preceded by at least 2 conditional scans. In total, 1653 scans were performed in 542 children, followed for 2235 patient-years. Fifty-eight (10.7%) high-risk subjects were identified, including 18 (31%) with high abnormal, and 15 (26%) with previous conditiona...
Source: Journal of Pediatric Hematology Oncology - September 30, 2016 Category: Hematology Tags: Original Articles Source Type: research

Transcranial Doppler Screening in a Current Cohort of Children With Sickle Cell Anemia: Results From the DISPLACE Study
This report describes results from Part 1 of the 28-site DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study, a baseline assessment of TCD implementation rates. This report describes TCD implementation by consortium site characteristics; characteristics of TCDs completed; and TCD results based on age. The cohort included 5247 children with SCA, of whom 5116 were eligible for TCD implementation assessment for at least 1 study year. The majority of children were African American or Black, non-Hispanic and received Medicaid. Mean age at first recorded TCD was 5.9 and 10.5 yea...
Source: Journal of Pediatric Hematology Oncology - October 30, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Associations Between Transcranial Doppler Flow Velocities, Laboratory Parameters, and Clinical Features in Turkish Children With Sickle Cell Disease
Conclusions: High ferritin and low hemoglobin levels, a high mean platelet volume, a high red cell distribution width, low weight (below the third percentile), and a short height (below the third percentile) may be associated with elevated cerebral blood flow velocities and an increased stroke risk in children with SCD. Children with such features should be closely followed-up through transcranial Doppler ultrasonography examination.
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

The Role of Neighborhoods in the Receipt of Transcranial Doppler Screening Among Children With Sickle Cell Disease
Although transcranial Doppler (TCD) screening assesses the need for stroke prevention efforts among children with sickle cell disease (SCD), screening rates remain low across many parts of the United States. We sought to identify neighborhoods with low TCD screening rates and neighborhood-level factors related to screening to inform the utility of community-level interventions to improve TCD screening. Children ages 2 to 16 years with SCD (HbSS/HbS/β-thalassemia) living in Wayne County, MI, were identified in Michigan Medicaid (2007 to 2011) through newborn screening records. Children were enrolled for ≥1 year and could...
Source: Journal of Pediatric Hematology Oncology - April 21, 2015 Category: Hematology Tags: Original Articles Source Type: research

Elevated Tricuspid Regurgitant Velocity as a Marker for Pulmonary Hypertension in Children With Sickle Cell Disease: Less Prevalent and Predictive Than Previously Thought?
In conclusion, elevated TRV in children with sickle cell disease is less prevalent than previously thought and is not independently associated with increased short-term morbidity.
Source: Journal of Pediatric Hematology Oncology - February 18, 2015 Category: Hematology Tags: Original Articles Source Type: research

Splenectomy to Optimize Hemoglobin S Control in Children With Sickle Cell Anemia on Chronic Transfusion Therapy for Stroke Prevention
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S
Source: Journal of Pediatric Hematology Oncology - February 21, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Use of Tranexamic Acid in an Uncommon Triad: Hemorrhagic Stroke, Sickle Cell Disease and Severe Coagulopathy
No abstract available
Source: Journal of Pediatric Hematology Oncology - February 21, 2019 Category: Hematology Tags: Letter to the Editor Source Type: research

Reversible Cerebral Vasoconstriction Syndrome and Sickle Cell Disease: A Case Report
In this report, we focus on the typical features of RCVS and discuss the underlying risk factors that may increase the risk in patients with HbSS disease.
Source: Journal of Pediatric Hematology Oncology - December 16, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Mortality and Causes of Death From Sickle Cell Disease in The Netherlands, 1985-2017
In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5.1 y). Their vital status was determined up to January 2017. After a total follow-up period of 4565 patient years and a median time of follow-up of 15 ye...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

Establishing Sickle Cell Disease Stroke Prevention Teams in Africa is Feasible: Program Evaluation Using the RE-AIM Framework: Erratum
No abstract available
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Errata Source Type: research

Treatment-related Correlates of Growth in Children With Sickle Cell Disease in the DISPLACE Cohort
This study investigated growth and hemolysis in children with SCD in the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) cohort. The database includes 5287 children, of which, 3305 had at least 2 growth measurements over a 5-year period. Body mass index was converted to z-scores (zBMI), and 19.8%, 66.1%, 14.2% of children were classified as underweight, normal, and overweight/obese, respectively. Multivariable analysis of growth was conducted and included variables: age, sex, blood pressure, hemoglobin, reticulocyte count, treatment with chronic red cell transfusion therapy ...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research