Utility of Caregiver Signaling Questions to Detect Neurocognitive Impairment in Children with Sickle Cell Disease.

The American Society of Hematology cerebrovascular guidelines for sickle cell disease (SCD) recommend surveillance using signaling questions to screen for neurocognitive difficulties, though the clinical utility of these signaling questions has yet to be established. This study aimed to determine the clinical utility of caregiver signaling questions for detecting significant neurocognitive impairment (defined as >1.5 standard deviation (SD) below the normative mean on 2 or more measures) and domain-specific impairment (defined as >1.

Intrapulmonary administration of recombinant activated factor VII in pediatric, adolescent, and young adult oncology and hematopoietic cell transplant patients with pulmonary hemorrhage.

Introduction: Diffuse alveolar hemorrhage (DAH) is a devastating disease process with 50-100% mortality in oncology and hematopoietic cell transplant (HCT) recipients. High concentrations of tissue factors have been demonstrated in the alveolar wall in acute respiratory distress syndrome and DAH, along with elevated levels of tissue factor pathway inhibitors. Activated recombinant factor VII (rFVIIa) activates the tissue factor pathway, successfully overcoming the tissue factor pathway inhibitor (TFPI) inhibition of activation of Factor X.

Review on clinician bias and its impact on racial and socioeconomic disparities in pediatric heart transplantation.

This expert review seeks to highlight implicit bias in health care, transplant medicine, and pediatric heart transplantation to focus attention on the role these biases may play in the racial/ethnic and socioeconomic disparities noted in pediatric heart transplantation. This review breaks down the transplant decision making process to highlight points at which implicit bias may affect outcomes and discuss how the science of human decision making may help understand these complex processes.

Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis.

Background: Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation.

Methods: CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022.

Corrigendum: Academic performance of children with sickle cell disease in the United States: a meta-analysis.

[This corrects the article DOI: 10.3389/fneur.2021.

Proinflammatory state promotes red blood cell alloimmunization in pediatric patients with sickle cell disease.

We examined risk factors for red blood cell (RBC) alloimmunization in pediatric patients with sickle cell disease, focusing on the recipients' inflammatory state at the time of transfusion and anti-inflammatory role of hydroxyurea (HU). Among 471 participants, 55 (11.70%) participants were alloimmunized and formed 59 alloantibodies and 17 autoantibodies with an alloimmunization rate of 0.

Genomic Comparisons Reveal Selection Pressure and Functional Variation Between Nutritional Endosymbionts of Cave-Adapted and Epigean Hawaiian Planthoppers.

Planthoppers in the family Cixiidae (Hemiptera: Auchenorrhyncha: Fulgoromorpha) harbor a diverse set of obligate bacterial endosymbionts that provision essential amino acids and vitamins that are missing from their plant-sap diet. "Candidatus Sulcia muelleri" and "Ca. Vidania fulgoroidea" have been associated with cixiid planthoppers since their origin within the Auchenorrhyncha, whereas "Ca.

Neurocognitive functioning in children with sickle cell anemia and history of abnormal transcranial doppler ultrasonography.

Background: Transcranial doppler (TCD) ultrasonography can be used to identify stroke risk in children with sickle cell anemia. Previous studies have reported mixed findings on neurocognitive outcomes in children with elevated TCD. This study examined associations between TCD velocity and neurocognitive outcomes in children and adolescents without prior history of stroke.

Internalizing Symptoms in Adolescents With Sickle Cell Disease.

Objective: Sickle cell disease (SCD) is a genetic blood disorder that may affect patients' mood and behavior. However, measuring the prevalence of internalizing symptoms (anxiety and depression) in patients with SCD has been elusive. We assessed internalizing symptoms in adolescents with SCD to evaluate prevalence and to test whether neurocognitive performance and frequency of pain-related episodes were associated with internalizing concerns.

Optimizing haematopoietic stem and progenitor cell apheresis collection from plerixafor-mobilized patients with sickle cell disease.

We adjusted haematopoietic stem and progenitor cell (HSPC) apheresis collection from patients with sickle cell disease (SCD) by targeting deep buffy coat collection using medium or low collection preference (CP), and by increasing anticoagulant-citrate-dextrose-solution A dosage. In 43 HSPC collections from plerixafor-mobilized adult patients with SCD, we increased the collection efficiency to 35.79% using medium CP and 82.

Cardiac allograft vasculopathy in pediatric heart transplant recipients does early-onset portend a worse prognosis?

Purpose: We sought to evaluate the association between timing of cardiac allograft vasculopathy (CAV) occurrence post-heart transplant (HT) with graft survival and progression of CAV severity in pediatric HT recipients.

Methods: Data from the Pediatric Heart Transplant Society for pediatric (<18 years old) HT recipients between 1993-2019 with available angiographic data were obtained (N = 5,075). The timing of CAV diagnosis (<3; 3-<5; 5-<10; and ≥10 years post-HT) and severity of disease at each assessment (CAV 1-3) was determined.

Contemporary Provider Management Practices and Attitudes Toward Referral for Advanced Heart Failure Therapies in Fontan Patients Across North America.

Background: To date, no reports have described clinicians' management practices for patients with Fontan circulatory failure or their understanding of risk factors for mortality and transplant outcomes in these patients.

Methods And Results: A cross-sectional survey of caregivers across North America was conducted from February to September 2020. Responses were compared by primary specialty (heart failure/transplant vs non-heart failure/transplant), years of experience (early, mid, and late career), and Fontan center volume (low, medium, and high).

Academic Performance of Children With Sickle Cell Disease in the United States: A Meta-Analysis.

Students with sickle cell disease are at risk for poor academic performance due to the combined and/or interactive effects of environmental, psychosocial, and disease-specific factors. Poor academic performance has significant social and health consequences. To study academic achievement and attainment in children with sickle cell disease in the United States.

Breaking the Rules: Left Common Carotid Artery from the Main Pulmonary Artery in an Infant with a Vascular Ring.

Anomalous left common carotid artery is a unique and rare abnormality. Most aortic arch anomalies can be explained by the totipotential aortic arch. Rare anomalies may not fit traditional arch regression models.

Gabapentin for acute pain in sickle cell disease: A randomized double-blinded placebo-controlled phase II clinical trial.

Pain in sickle cell disease (SCD) can have a neuropathic component. This randomized phase II double-blinded placebo-controlled study evaluated the efficacy of gabapentin in reducing pain and opioid consumption (morphine-equivalent dose [MED]) during acute vaso-occlusive crisis (VOC). Of 90 patients aged 1-18 years with VOC pain, 45 were randomized to a single gabapentin dose (15 mg/kg) and 45 to placebo, in addition to standard treatment; 42 and 44 patients were evaluable in the gabapentin and placebo arms, respectively.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

Background: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported.

Fontan-associated plastic bronchitis waitlist and heart transplant outcomes: A PHTS analysis.

Plastic bronchitis is a rare post-Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis.

Role of implicit bias in pediatric cancer clinical trials and enrollment recommendations among pediatric oncology providers.

Background: Provider implicit bias can negatively affect clinician-patient communication. In the current study, the authors measured implicit bias training among pediatric oncology providers and exposure to implicit association tests (IATs). They then assessed associations between IATs for race and socioeconomic status (SES) and recommendations for clinical trial enrollment.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

Background: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making.

Methods: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers.

Should Decision Making Be Shared in High-Risk Pediatric Heart Donation?

This article considers complexities of shared decision making in pediatric heart transplantation and suggests that decisions about pediatric heart transplantation should be shared between a clinical team and parents. This article also considers goals of shared decision making involving Public Health Service increased-risk donors and recommends policy changes to strengthen decision sharing.

Abnormal Vital Signs Predict Critical Deterioration in Hospitalized Pediatric Hematology-Oncology and Post-hematopoietic Cell Transplant Patients.

Hospitalized pediatric hematology-oncology and post-hematopoietic cell transplant (HCT) patients have frequent deterioration requiring Pediatric Intensive Care Unit (PICU) care. Critical deterioration (CD), defined as unplanned PICU transfer requiring life-sustaining interventions within 12 h, is a pragmatic metric to evaluate emergency response systems (ERS) in pediatrics, however, it has not been investigated in these patients. The goal of this study was to evaluate if CD is an appropriate metric to assess effectiveness of ERS in pediatric hematology-oncology and post-HCT patients and if it is preceded by an actionable period of vital sign changes.

Review of interactions between high-risk pediatric heart transplant recipients and marginal donors including utilization of risk score models.

Background: Donor organ acceptance practices vary among pediatric heart transplant professionals. We sought to understand what is known about the interactions between the "high-risk" recipient and the "marginal donor," and how donor risk scores can impact this discussion.

Methods: A systematic review of published literature on pediatric HTx was undertaken with the assistance of a medical librarian.