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 < .

Sex and frequency of pain episodes are associated with acute pain trajectories in adolescents with sickle cell disease.

Introduction/objective: Acute pain episodes are a major cause of health care utilization (HCU) in sickle cell disease (SCD), and adolescence is associated with increased pain frequency. We sought to determine whether there were differences in acute pain trajectories by sex and frequency of pain episodes among adolescents with SCD who presented to the emergency department (ED).

Methods: Retrospective review of electronic health records from a large, multicampus, pediatric SCD program.

Are children with sickle cell disease at particular risk from the harmful effects of air pollution? Evidence from a large, urban/peri-urban cohort.

Introduction: Pathophysiologic pathways of sickle cell disease (SCD) and air pollution involve inflammation, oxidative stress, and endothelial damage. It is therefore plausible that children with SCD are especially prone to air pollution's harmful effects.

Methods: Patient data were collected from a single-center, urban/peri-urban cohort of children with confirmed SCD.

Trends in blood transfusion, hydroxyurea use, and iron overload among children with sickle cell disease enrolled in Medicaid, 2004-2019.

Background: There have been significant changes in clinical guidelines for sickle cell disease (SCD) over the past two decades, including updated indications for hydroxyurea, transfusions, and iron overload management. In practice however, there are few studies that examine SCD care utilization over time.

Methods: We conducted a serial cross-sectional cohort study of pediatric SCD patients from 2004 to 2019 using Georgia Medicaid claims data.

Sickle Cell Transplantation Evaluation of Long-term and Late Effects Registry (STELLAR) to Compare Long-term Outcomes After Hematopoietic Cell Transplantation to Those in Siblings Without Sickle Cell Disease and in Nontransplanted Individuals With Sickle Cell Disease: Design and Feasibility Study.

Background: There are sparse data on the long-term and late effects of hematopoietic cell transplantation (HCT) for sickle cell disease (SCD).

Objective: This study aims to establish an international registry of long-term outcomes post-HCT for SCD and demonstrate the feasibility of recruitment at a single site in the United States.

Methods: The Sickle Cell Transplantation Evaluation of Long-Term and Late Effects Registry (STELLAR) was designed to enroll patients with SCD ≥1 year post-HCT, their siblings without SCD, and nontransplanted controls with SCD to collect web-based participant self-reports of health status and practices by using the Bone Marrow Transplant Survivor Study (BMTSS) surveys, health-related quality of life (HRQOL) using the Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Profile-25 or Pediatric Profile-29 survey, chronic graft-versus-host disease (cGVHD) using the symptom scale survey, daily pain using an electronic pain diary, the economic impact of HCT using the financial hardship survey, sexual function using the PROMIS Sexual Function SexFSv2.

Bloodstream Infections in Children With Sickle Cell Disease: 2010-2019.

Background: Children with sickle cell disease (SCD) are at increased risk for bloodstream infections (BSIs), mainly because of functional asplenia. Immunizations and antibiotic prophylaxis have reduced the prevalence of invasive bacterial infections, but contemporary analysis of BSI in children with SCD is limited.

Methods: We conducted a retrospective cohort study of children aged <18 years with SCD who had blood cultures collected at our institution from 2010 to 2019 to identify BSI.

A pilot study of the acceptability, feasibility and safety of yoga for chronic pain in sickle cell disease.

Objectives: To determine the acceptability, feasibility and safety of yoga for chronic pain in sickle cell disease.

Design And Setting: In Part A of this two-part study, adolescents with SCD and chronic pain (Group 1) and their parent (Group 2) completed a survey designed to capture pain characteristics, attitudes and practices related to yoga, and potential acceptability of a yoga program. In Part B, the study assessed the feasibility and safety of an instructor-led group yoga program.

Blockade of tumor necrosis factor superfamily members CD30 and OX40 abrogates disease activity in murine immune-mediated glomerulonephritis.

Co-stimulation is a prerequisite for pathogenic activity in T cell-mediated diseases and has been demonstrated to achieve tolerance in organ-specific autoimmunity as a therapeutic target. Here, we evaluated the involvement of the tumor necrosis factor family members CD30 and OX40 in immune-complex mediated kidney disease. In vitro stimulation and proliferation studies were performed with CD4 cells from wild type and CD30/OX40 double knock-out (CD30OX40) mice.

The burden of respiratory syncytial virus infections among children with sickle cell disease.

Background: Although respiratory syncytial virus (RSV) is the leading cause of pediatric lower respiratory tract infections, the burden of RSV in children with sickle cell disease (SCD) is unknown.

Methods: We conducted a retrospective, nested, case-control study of children with SCD <18 years who had respiratory viral panels (RVPs) performed at Children's Healthcare of Atlanta from 2012 to 2019. We abstracted the medical records to describe the demographics, clinical features, and outcomes of children who tested positive for RSV (cases) versus children who tested negative (controls).

An Integrated Hospital Protocol for Persons With Injection-Related Infections May Increase Medications for Opioid Use Disorder Use but Challenges Remain.

Background: Hospital-based strategies that link persons with infectious complications of opioid use disorder (OUD) to medications for OUD (MOUD) are of great interest. The objective of this study is to determine whether a hospital-based protocol would increase the use of MOUD and to identify barriers to MOUD during admission and at the time of discharge.

Methods: This study included participants with a documented or suspected history of injection drug usage receiving care for an infection at the University of Alabama at Birmingham Hospital from 2015 to 2018.

Microbiology and radiographic features of osteomyelitis in children and adolescents with sickle cell disease.

Background: Children with sickle cell disease (SCD) are at increased risk for bacterial infections including osteomyelitis (OM). Fever and bone pain, key presenting symptoms of OM, are common in SCD, thus complicating diagnosis. We reviewed presentation, imaging features, and microbiologic etiologies of children with SCD treated for OM.

Influenza vaccine effectiveness and disease burden in children and adolescents with sickle cell disease: 2012-2017.

Background: Data are limited on the burden of influenza and seasonal influenza vaccine effectiveness (VE) in children with sickle cell disease (SCD).

Methods: We used a prospectively collected clinical registry of SCD patients 6 months to 21 years of age to determine the influenza cases per 100 patient-years, vaccination rates, and a test-negative case-control study design to estimate influenza VE against medically attended laboratory-confirmed influenza infection. Influenza-positive cases were randomly matched to test-negative controls on age and influenza season in 1:1 ratio.

Impact of arginine therapy on mitochondrial function in children with sickle cell disease during vaso-occlusive pain.

Altered mitochondrial function occurs in sickle cell disease (SCD), due in part to low nitric oxide (NO) bioavailability. Arginine, the substrate for NO production, becomes acutely deficient in SCD patients with vaso-occlusive pain episodes (VOE). To determine if arginine improves mitochondrial function, 12 children with SCD-VOE (13.

In-Hospital Illicit Drug Use and Patient-Directed Discharge: Barriers to Care for Patients With Injection-Related Infections.

Background: Hospitalized persons who inject drugs are at a greater risk of adverse hospital outcomes including discharge against medical advice, inpatient illicit drug use, overdose, and death. However, there are limited data on the frequency and outcomes of these events in the United States.

Methods: This retrospective analysis included patients with injection-related infections receiving a protocol for injection drug use (IDU) at University of Alabama at Birmingham Hospital from 2016 to 2017.

Preliminary evaluation of the clinical implementation of cognitive-behavioral therapy for chronic pain management in pediatric sickle cell disease.

Objectives: Evaluate the implementation of cognitive-behavioral therapy (CBT) for chronic pain in a clinical setting by comparing youth with sickle cell disease (SCD) who initiated or did not initiate CBT.

Design: Youth with SCD (ages 6-18; n = 101) referred for CBT for chronic pain were compared based on therapy attendance: Established Care; Early Termination; or Comparison (i.e.

Comparative Effectiveness of a Web-Based Patient Decision Aid for Therapeutic Options for Sickle Cell Disease: Randomized Controlled Trial.

Background: Hydroxyurea, chronic blood transfusions, and bone marrow transplantation are efficacious, disease-modifying therapies for sickle cell disease but involve complex risk-benefit trade-offs and decisional dilemma compounded by the lack of comparative studies. A patient decision aid can inform patients about their treatment options, the associated risks and benefits, help them clarify their values, and allow them to participate in medical decision making.

Objective: The objective of this study was to develop a literacy-sensitive Web-based patient decision aid based on the Ottawa decision support framework, and through a randomized clinical trial estimate the effectiveness of the patient decision aid in improving patient knowledge and their involvement in decision making.

Improving an Administrative Case Definition for Longitudinal Surveillance of Sickle Cell Disease.

Objective: Several states are building infrastructure and data collection methods for longitudinal, population-based surveillance systems for selected hemoglobinopathies. The objective of our study was to improve an administrative case definition for sickle cell disease (SCD) to aid in longitudinal surveillance.

Methods: We collected data from 3 administrative data sets (2004-2008) on 1998 patients aged 0-21 in Georgia who had ≥1 encounter in which an SCD International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code was recorded, and we compared these data with data from a laboratory and medical record review.

Health literacy and knowledge of chronic transfusion therapy in adolescents with sickle cell disease and caregivers.

Background: Patients with sickle cell disease (SCD) may require chronic transfusion therapy (CTT) for prevention of stroke or other complications. Limited health literacy (HL) is common and is associated with poor health-related knowledge and outcomes in chronic disease. We sought to assess HL and transfusion knowledge in patients with SCD on CTT and their caregivers.

Incidence of invasive Haemophilus influenzae infections in children with sickle cell disease.

Background: Children with sickle cell disease (SCD) are at increased risk for invasive infection with encapsulated bacteria. Antibiotic prophylaxis and immunizations against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib) have decreased the overall incidence of invasive infections and have shifted distribution of serotypes causing disease toward those not covered by immunizations. We sought to determine the current incidence of invasive H.

Weather effects on birds of different size are mediated by long-term climate and vegetation type in endangered temperate woodlands.

Species occurrence is influenced by a range of factors including habitat attributes, climate, weather, and human landscape modification. These drivers are likely to interact, but their effects are frequently quantified independently. Here, we report the results of a 13-year study of temperate woodland birds in south-eastern Australia to quantify how different-sized birds respond to the interacting effects of: (a) short-term weather (rainfall and temperature in the 12 months preceding our surveys), (b) long-term climate (average rainfall and maximum and minimum temperatures over the period 1970-2014), and (c) broad structural forms of vegetation (old-growth woodland, regrowth woodland, and restoration plantings).

A 9-Point Risk Assessment for Patients Who Inject Drugs and Require Intravenous Antibiotics: Focusing Inpatient Resources on Patients at Greatest Risk of Ongoing Drug Use.

A 9-point risk assessment identified persons with a history of injection drug use who were safe for discharge. "Low-risk" patients were discharged with outpatient antibiotics; others continued inpatient treatment. Use of the assessment reduced the mean length of stay by 20 days and total direct cost by 33%, creating capacity for an additional 333 patients.

The accuracy of hospital ICD-9-CM codes for determining Sickle Cell Disease genotype.

Sickle cell disease affects more than 100,000 individuals in the United States, among whom disease severity varies considerably. One factor that influences disease severity is the sickle cell disease genotype. For this reason, clinical prevention and treatment guidelines tend to differentiate between genotypes.