Prevention of Invasive Pneumococcal Disease Among Black or African American Children With and Without Sickle Cell Disease in the United States After Introduction of 13-Valent Pneumococcal Conjugate Vaccine, 2005 Through 2019.

Background: Administration of pneumococcal vaccines and oral penicillin prophylaxis has been recommended for children with sickle cell disease (SCD) to reduce the risk of invasive pneumococcal disease (IPD). Characterizing changes in IPD cases among children with SCD after 13-valent pneumococcal conjugate vaccine (PCV13) introduction could help inform the need for additional prevention measures.

Methods: Using data from Active Bacterial Core surveillance, we characterized IPD cases among Black or African American (Black) children aged less than 18 years with SCD, non-SCD IPD risk factors, and no IPD risk factors across three time periods (pre-PCV13 [2005-2009], early-PCV13 [2010-2014], and late-PCV13 [2015-2019]), and assessed proportion of IPD cases caused by serotypes in new pneumococcal conjugate vaccines (PCV15, PCV20) recommended after 2019.

Pneumococcal infections in children with sickle cell disease before and after pneumococcal conjugate vaccines.

Children with sickle cell disease (SCD) are at increased risk of invasive pneumococcal disease (IPD). Over 25 years, the Georgia Emerging Infections Program/Centers for Disease Control and Prevention Active Bacterial Core Surveillance network identified 104 IPD episodes among 3707 children with hemoglobin SS (HbSS) or HbSC aged <10 years, representing 6% of IPD in Black or African American children residing in Metropolitan Atlanta (reference population). Children with IPD and HbSS/SC were older than those with IPD in the reference population (P < .

Influenza vaccination rates and hospitalizations among Medicaid enrollees with and without sickle cell disease, 2009-2015.

Background: Personswith sickle cell disease (SCD) face increased risks for pulmonary and infection-related complications. This study examines influenza vaccination coverage and estimates influenza-related morbidity among Medicaid enrollees with and without SCD.

Procedure: Influenza vaccination coverage and hospitalizations related to influenza and pneumonia/acute chest syndrome (ACS) during each influenza season from 2009-2010 to 2014-2015 were assessed among enrollees in the IBM MarketScan® Multi-State Medicaid Database.

Observed and expected frequencies of structural hemoglobin variants in newborn screening surveys in Africa and the Middle East: deviations from Hardy-Weinberg equilibrium.

Purpose: Our objective was to compare observed and expected genotype proportions from newborn screening surveys of structural hemoglobin variants.

Methods: We conducted a systematic review of newborn screening surveys of hemoglobins S and C in Africa and the Middle East. We compared observed frequencies to those expected assuming Hardy-Weinberg equilibrium (HWE).

Contribution of Sickle Cell Disease to the Pediatric Stroke Burden Among Hospital Discharges of African-Americans-United States, 1997-2012.

Background: Approximately 10-20% of children with sickle cell disease (SCD) develop stroke, but few consistent national estimates of the stroke burden for children with SCD exist. The purpose of this study is to determine the proportion of diagnosed stroke among African-American pediatric discharges with and without SCD.

Procedure: Records for African-Americans aged 1-18 years in the Kids' Inpatient Database (KID) 1997-2012 with ≥1 ICD-9-CM diagnosis code for stroke were included.

Plasma BDNF and PDGF-AA levels are associated with high TCD velocity and stroke in children with sickle cell anemia.

Sickle cell anemia (SCA) associated cerebrovascular disease includes vascular remodeling, abnormal cerebral blood flow (CBF) and infarction. We studied the relationships between plasma brain derived neurotropic factor (BDNF), platelet derived growth factors (PDGF-AA and -AB/BB) and high trans-cranial Doppler (TCD) velocity, an indication of CBF velocity. Baseline plasma samples from 39 children (19 SCA with abnormal/high TCD [SATCD], 13 SCA with normal TCD [SNTCD] and 7 healthy non-SCA), were assayed for BDNF, PDGF-AA and -AB/BB plus 11 other cytokines.

Genetic polymorphisms linked to susceptibility to malaria.

The influence of host genetics on susceptibility to Plasmodium falciparum malaria has been extensively studied over the past twenty years. It is now clear that malaria parasites have imposed strong selective forces on the human genome in endemic regions. Different genes have been identified that are associated with different malaria related phenotypes.

Pulmonary, gonadal, and central nervous system status after bone marrow transplantation for sickle cell disease.

We conducted a prospective, multicenter investigation of human-leukocyte antigen (HLA) identical sibling bone marrow transplantation (BMT) in children with severe sickle cell disease (SCD) between 1991 and 2000. To determine if children were protected from complications of SCD after successful BMT, we extended our initial study of BMT for SCD to conduct assessments of the central nervous system (CNS) and of pulmonary function 2 or more years after transplantation. In addition, the impact on gonadal function was studied.

Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury.

Chronic blood transfusion is increasingly indicated in patients with sickle cell disease. Measuring resulting iron overload remains a challenge. Children without viral hepatitis enrolled in 2 trials for stroke prevention were examined for iron overload (STOP and STOP2; n = 271).

Effectiveness of the 7-valent pneumococcal conjugate vaccine in children with sickle cell disease in the first decade of life.

Background: The incidence of and mortality from invasive pneumococcal disease are significantly higher in children with sickle cell disease than in the general pediatric population. The objective of this population-based study was to assess the effect of pneumococcal conjugate vaccine on rates of invasive pneumococcal disease among children with sickle cell disease.

Patients And Methods: Records, including the history of pneumococcal conjugate vaccine administration, of 1247 children born after 1983 residing in metropolitan Atlanta, Georgia, with confirmed hemoglobinopathies were linked to an active surveillance database for invasive pneumococcal disease for the period of January 1, 1995, through January 1, 2003.

Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia.

A retrospective cohort study of children with sickle cell anemia (SCA) and strokes was used to test the hypothesis that exchange transfusion at the time of stroke presentation more effectively prevents second strokes than does simple transfusion. Children receiving simple transfusion had a 5-fold greater relative risk (95% confidence interval = 1.3 to 18.

Quinupristin-dalfopristin nonsusceptibility in pneumococci from sickle cell disease patients.

Sickle cell disease (SCD) is a risk factor for fatal pneumococcal infection. Nonsusceptibilty to quinupristin-dalfopristin (Q-D) was absent from 105 non-SCD-associated pneumococcal isolates but was present in 33/148 (22%) SCD-associated isolates. One-third of the isolates harbored a known resistance mechanism.

Identification of unrelated cord blood units for hematopoietic stem cell transplantation in children with sickle cell disease.

This study examined the theoretical availability of compatible unrelated umbilical cord blood (UCB) units for hematopoietic stem cell transplantation (HSCT) of children with sickle cell disease (SCD), matched for DRB1 at high resolution. UCB units registered via Bone Marrow Donors Worldwide were matched with patients who had been previously typed for possible HSCT. Suitable matching was determined after typing at antigen level at A and B loci and allele-level typing at DRB1.

Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination.

Rates and severity of pneumococcal infections in children with sickle cell disease were examined before licensure of pneumococcal-conjugated vaccine (PVC). Rates of peak invasive infection rates in 1-year-old children with hemoglobin SS and mortality in those 0 to 10 years of age were 36.5 to 63.

Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke.

Objective: To test the hypothesis that children with sickle cell disease (SCD) who have an initial stroke temporally unrelated to another medical event are at higher risk for recurrent stroke than are children who had strokes temporally related to medical events.

Methods: A retrospective cohort study of children with SCD and stroke who received regularly scheduled blood transfusions for a minimum of 5 years was conducted. Medical records were examined for the documentation of antecedent or concurrent medical events (hypertension, acute chest syndrome, aplastic crisis, fever associated with infection, exchange transfusion) associated with physician contact within 14 days before the initial stroke.