Sensitivity and Specificity of a Signaling Question for Surveillance of Cognitive Functioning in Pediatric Sickle Cell Disease.

Background: Sickle cell disease (SCD) confers neurological risks that contribute to cognitive and academic difficulties. Clinical guidelines state that cognition should be monitored using signaling questions. However, evidence is lacking regarding the extent to which signaling questions accurately identify children with cognitive issues.

Adding hydroxyurea to chronic transfusion therapy for sickle cell anemia reduces transfusion burden.

Background: Chronic red blood cell (RBC) transfusion is an established therapy to prevent stroke in patients with sickle cell anemia (SCA). It is unclear if adding daily hydroxyurea treatment to chronic transfusion is beneficial.

Study Design And Methods: We conducted a phase 2 clinical trial (NCT03644953) investigating the addition of dose-escalated hydroxyurea to chronic transfusion for patients with SCA receiving simple chronic transfusion for stroke prevention.

Disparity in HLA typing rates among hospitalized pediatric patients with sickle cell disease.

Hematopoietic stem cell transplantation (HSCT) using an HLA-identical sibling donor is a well-established cure for sickle cell disease (SCD). The proportion of patients with SCD who have completed HLA typing, a key first step in considering HSCT, is unknown. We sought to determine the prevalence of HLA typing among patients with SCD hospitalized at our institution, identify characteristics associated with having had typing, and describe the acceptability of this testing.

Hematopoietic Cell Transplant compared with Standard Care in Adolescents and Young Adults with Sickle Cell Disease.

Disease-modifying therapies are standard of care (SOC) for sickle cell disease (SCD), but hematopoietic cell transplantation (HCT) has curative potential. We compared outcomes prospectively through 2-years after biologic assignment to a Donor or No Donor (SOC) Arm based on the availability of an HLA-matched sibling or unrelated donor (BMTCTN 1503; NCT02766465). A donor search was commenced after eligibility confirmation.

Time-Resolved X-ray Emission Spectroscopy and Synthetic High-Spin Model Complexes Resolve Ambiguities in Excited-State Assignments of Transition-Metal Chromophores: A Case Study of Fe-Amido Complexes.

To fully harness the potential of abundant metal coordination complex photosensitizers, a detailed understanding of the molecular properties that dictate and control the electronic excited-state population dynamics initiated by light absorption is critical. In the absence of detectable luminescence, optical transient absorption (TA) spectroscopy is the most widely employed method for interpreting electron redistribution in such excited states, particularly for those with a charge-transfer character. The assignment of excited-state TA spectral features often relies on spectroelectrochemical measurements, where the transient absorption spectrum generated by a metal-to-ligand charge-transfer (MLCT) electronic excited state, for instance, can be approximated using steady-state spectra generated by electrochemical ligand reduction and metal oxidation and accounting for the loss of absorptions by the electronic ground state.

A Team Approach to Bundle Compliance.

Hospitals are always looking to improve the quality of patient care and avoid hospital-acquired conditions such as ventilator-associated pneumonia (VAP). Currently, there are no set standards regarding interventions to prevent VAP, and there is not a single element that has a direct impact on VAP prevention. By creating an interprofessional team to work together, the quality improvement project was able to evaluate current practice compared with evidence-based practice in the literature to develop a critical care VAP bundle practice, which demonstrated improvement in compliance.

Nanoscale Size Effects on Push-Pull Fe-O Hybridization through the Multiferroic Transition of Perovskite ϵ-FeO.

Multiferroics have tremendous potential to revolutionize logic and memory devices through new functionalities and energy efficiencies. To reach their optimal capabilities will require better understanding and enhancement of the ferroic orders and couplings. Herein, we use ϵ-FeO as a model system with a simplifying single magnetic ion.

Optimising the screening for haemoglobinopathies in pregnancy planning.

Haemoglobinopathies are among the most common inherited disorders around the world. In the United States the diagnosis of haemoglobinopathy or a carrier state is made by universal newborn screening. However, many individuals of childbearing age do not know they are a haemoglobinopathy carrier.

Clinical outcomes of children and adolescents with sickle cell disease and COVID-19 infection: A year in review at a metropolitan tertiary pediatric hospital.

Background: COVID-19 was declared a global pandemic in March 2020. Early reports were primarily in adults, and sickle cell disease (SCD) was classified as a risk factor for severe COVID-19 disease. However, there are a limited number of primarily multi-center studies reporting on the clinical course of pediatric patients with SCD and COVID-19.

Quantifying DNA loss in laboratory-created latent prints due to fingerprint processing.

Fingerprints, which are associated with touch samples, typically contain a limited amount of DNA. The amount of available DNA can be further reduced when the same touch samples undergo fingerprint processing [1]. The fingerprint development process consists of high-powered lighting (inherent luminescence and UV light) and chemical compounds (ninhydrin, black powder, cyanoacrylate, and rhodamine 6 G) which could reduce DNA quality and quantity.

Development of an instrument for measuring the work functioning of persons with epilepsy in clinical settings.

Objective: To describe the development process of the Work Functioning Assessment for Epilepsy (WOFAE), an instrument recently developed in Brazil for measuring the work functioning of persons with epilepsy (PwE) in clinical settings, and to evaluate to what extent this instrument is in line with existing generic and epilepsy-specific tools used to measure general and work functioning.

Methods: The development process included four phases: the content development, based on a literature review and using the International Classification of Functioning, Disability and Health (ICF) as a reference framework; a preliminary field test, conducted with 20 PwE; an expert consultation, applying the Delphi Method; and the mapping and content comparison of the WOFAE to other five functioning assessments, using the ICF linking rules.

Results: The WOFAE containing 46 items structured into eight domains was developed in an evidence-based and participatory process.

Design of an adaptive randomized clinical trial of intravenous citrulline for sickle cell pain crisis in the emergency department.

Background: Vaso-occlusive pain crisis (VOC) is the most frequent cause for Emergency Department (ED) visits and hospital admissions for patients with sickle cell disease (SCD). Nitric oxide plays a critical role in the pathogenesis of vaso-occlusion. The amino acid, citrulline, is the main endothelial nitric oxide booster that offers the potential to ameliorate vaso-occlusion and decrease the risk of hospitalization.

Long-Term Organ Function After HCT for SCD: A Report From the Sickle Cell Transplant Advocacy and Research Alliance.

Hematopoietic cell transplantation (HCT) is an established cure for sickle cell disease (SCD) supported by long-term survival, but long-term organ function data are lacking. We sought to describe organ function and assess predictors for dysfunction in a retrospective cohort (n = 247) through the Sickle cell Transplant Advocacy and Research alliance. Patients with <1-year follow-up or graft rejection/second HCT were excluded.

Reduction in vaso-occlusive events following stem cell transplantation in patients with sickle cell disease.

Hematopoietic stem cell transplantation (HSCT) is potentially curative for patients with sickle cell disease (SCD). Patients with stable donor engraftment after allogeneic HSCT generally do not experience SCD-related complications; however, there are no published data specifically reporting the change in vaso-occlusive events (VOE) after HSCT. Data regarding the number of VOEs requiring medical attention in the 2 years before allogeneic HSCT were compared with the number of VOEs in the 2 years (0-12 months and 12-24 months) after allogeneic HSCT in patients with SCD.

Amino acid signature during sickle cell pain crisis shows significant alterations related to nitric oxide and energy metabolism.

Nitric oxide depletion secondary to arginase induced arginine deficiency has been shown to be important in the pathophysiology of vaso-occlusion in sickle cell pain crisis. Our objective of this study was to perform a comprehensive amino acid evaluation during sickle cell pain crisis. In a total of 58 subjects (29 in steady-state sickle cell disease and 29 with sickle cell pain crisis), the amino acids related to nitric oxide pathway was significantly decreased during sickle cell pain crisis compared to steady-state sickle cell disease: arginine (p = 0.

Fertility after Curative Therapy for Sickle Cell Disease: A Comprehensive Review to Guide Care.

Curative therapy for sickle cell disease (SCD) currently requires gonadotoxic conditioning that can impair future fertility. Fertility outcomes after curative therapy are likely affected by pre-transplant ovarian reserve or semen analysis parameters that may already be abnormal from SCD-related damage or hydroxyurea treatment. Outcomes are also likely affected by the conditioning regimen.

Preventing antibody positive delayed hemolytic transfusion reactions in sickle cell disease: Lessons learned from a case.

Introduction: Red blood cell (RBC) transfusions are important in the management of patients with sickle cell disease (SCD). However, a potentially catastrophic complication of transfusion in this population is the delayed hemolytic transfusion reaction (DHTR). The pathophysiology of all DHTRs is not understood, but some are known to be caused by an anamnestic resurgence of RBC alloantibodies.

Artificial urethral sphincter in male dogs with urethral sphincter mechanism incompetence: 19 cases (2010-2017).

Objective: To assess the outcome and safety of surgically placed artificial urethral sphincters in male dogs with urethral sphincter mechanism incompetence.

Materials And Methods: We included dogs with urethral sphincter mechanism incompetence treated by placing an artificial urethral sphincter from January 1, 2010 to December 31, 2017. The continence score (scale 1 to 5, with 5 indicating complete continence) was evaluated before and after implantation.

Patients with Cervical Dystonia Demonstrated Decreased Cognitive Abilities and Visual Planning Compared to Controls.

Background: Cervical dystonia (CD) involves clinical and motor manifestations, and visual and cognitive dysfunctions may also be frequent.

Objective: To evaluate functional vision, visual attention, and cognitive aspects in patients with CD compared with a control group.

Methods: Fifty patients with CD were assessed using the Useful Field of View Test (UFOV), the Mini Mental State Examination (MMSE), and the Trail Making Tests (TMT-A and TMT-B), and compared with an identical number of health controls matched by sex, age, and educational level.

Impact of universal irradiation on chronic transfusion for sickle cell disease.

Background: Irradiation of blood products prevents transfusion-associated graft-versus-host disease, but most patients do not require this modification which could have an adverse impact on transfusion outcomes. We hypothesized that irradiation may increase transfusion requirements for patients with sickle cell disease (SCD) receiving chronic transfusion.

Study Design And Methods: Our pediatric hospital implemented a new policy of universal blood product irradiation in May 2018.

Screening for new red blood cell alloantibodies after transfusion in patients with sickle cell disease.

Background: Patients with sickle cell disease (SCD) are frequent recipients of red blood cell (RBC) transfusions and are at risk for RBC alloimmunization. RBC alloimmunization is diagnosed by identifying RBC alloantibodies as part of pre-transfusion testing, but this testing fails to detect alloantibodies that have evanesced. It may be beneficial to screen for new RBC alloantibody development after transfusion before possible antibody evanescence.