Hypomorphic RAG2 Deficiency Promotes Selection of Self-Reactive B Cells.

Reduced function or hypomorphic variants in recombination-activating genes (RAG) 1 or 2 result in a broad clinical phenotype including common variable immunodeficiency (CVID) and even adult-onset disease. Milder RAG variants are less characterized. Here we describe the longitudinal course of a milder combined RAG deficiency in 3 of 7 siblings sharing the same RAG2 mutations over a 50-year study.

A choice experiment of older patients' preferences for kidney failure treatments.

Most older people with kidney failure choose between treatment with dialysis or conservative kidney management. The preferences underlying these decisions are poorly understood. Here, we performed a choice experiment, informed by qualitative research, to examine preferences for the characteristics of dialysis and conservative management among over-65-year-olds with eGFR of 20 mls or under/min/1.

A Multinational, Multicenter Study Mapping Models of Kidney Supportive Care Practice.

Introduction: Kidney supportive care (KSC) integrates kidney and palliative care to improve quality of life for people with chronic kidney disease (CKD). Despite increasing interest and global advocacy to integrate KSC into kidney care, evidence to guide optimal care delivery is limited.

Methods: This observational cross-sectional study used an online survey to describe current KSC models in Australia, Aotearoa-New Zealand, and the UK.

Predicting Kidney Failure, Cardiovascular Disease and Death in Advanced CKD Patients.

Introduction: Predicting the timing and occurrence of kidney replacement therapy (KRT), cardiovascular events, and death among patients with advanced chronic kidney disease (CKD) is clinically useful and relevant. We aimed to externally validate a recently developed CKD G4+ risk calculator for these outcomes and to assess its potential clinical impact in guiding vascular access placement.

Methods: We included 1517 patients from the European Quality (EQUAL) study, a European multicentre prospective cohort study of nephrology-referred advanced CKD patients aged ≥65 years.

'Mali Matters': reflections on a leadership and quality improvement project to improve blood transfusion safety at Maluti Adventist Hospital, Mapoteng, Lesotho.

Introduction: This report outlines a quality improvement (QI) project aiming to improve blood transfusion safety at Maluti Adventist Hospital (MAH), Lesotho, from August 2019 to January 2020.The project team comprised nine local staff members and two UK doctors working through the NHS 'Improving Global Health through Leadership Development' (IGH) programme.

Methods: Baseline data was gathered and a 'process mapping' meeting held to understand existing processes and identify methods for improvement.

Landscape of official development assistance for nutrition data and information systems.

Nutrition data and information systems (ND&IS) are critical to guide the prioritisation, collection, analysis and dissemination of nutrition data in countries. However, there is limited guidance for countries regarding how to invest in their ND&IS and little is known about current financing allocations by both countries and donors. This hinders our ability to identify the most critical funding gaps and to effectively advocate for increased financial commitments to ND&IS.

Developing effective data visualization tools for nutrition: reflections on the design of a Nutrition Scorecard in Nigeria.

There has been a growing number of nutrition data visualization tools (DVTs) to monitor progress towards targets and encourage action. However, there are few documented examples of how to go about designing effective DVTs for nutrition-related audiences. In this Open Letter, we summarize reflections from collaborative efforts between the Nigeria Governors' Forum (NGF) and the Data for Decisions to Expand Nutrition Transformation project (DataDENT) in 2019-2021 to design a sub-national nutrition scorecard that aims to hold Nigeria's 36 Governors accountable to nutrition commitments.

Treatment with High-Dose Prednisolone in Vigabatrin-Refractory Infantile Spasms.

Objectives: This research aimed to study the short-term seizure outcomes following treatment with 8 mg/kg/day prednisolone in children with infantile spasms (IS) refractory to vigabatrin. We hypothesized that high-dose prednisolone may result in similar rates of electroclinical remission when compared to published ACTH rates.

Methods: All consecutive children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without IS, who had been treated with vigabatrin as first-line anti-seizure medication (ASM) followed by high-dose oral prednisolone (8 mg/kg/day; maximum 60 mg/day) in cases who did not respond to vigabatrin, were included.

United States' Obstetrician/Gynecologists' Readiness to Care for Women Affected by Female Genital Cutting.

Female genital cutting (FGC) is a form of gender-based violence with obstetrical and gynecological complications that require recognition and care. Data suggest that United States' physicians are not prepared to care for those who have been affected by this practice. This study evaluated the knowledge and practices of United States' obstetricians and gynecologists to care for patients who have undergone FGC.

Jarosite formation in deep Antarctic ice provides a window into acidic, water-limited weathering on Mars.

Many interpretations have been proposed to explain the presence of jarosite within Martian surficial sediments, including the possibility that it precipitated within paleo-ice deposits owing to englacial weathering of dust. However, until now a similar geochemical process was not observed on Earth nor in other planetary settings. We report a multi-analytical indication of jarosite formation within deep ice.

Comparison of Tissue Molecular Biomarker Testing Turnaround Times and Concordance Between Standard of Care and the Biocartis Idylla Platform in Patients With Colorectal Cancer.

Objectives: Management of colorectal cancer warrants mutational analysis of KRAS/NRAS when considering anti-epidermal growth factor receptor therapy and BRAF testing for prognostic stratification. In this multicenter study, we compared a fully integrated, cartridge-based system to standard-of-care assays used by participating laboratories.

Methods: Twenty laboratories enrolled 874 colorectal cancer cases between November 2017 and December 2018.

Integrating evolution into medical education for women's health care practitioners.

Evolution is a fundamental principle in biology; however, it has been neglected in medical education. We argue that an evolutionary perspective is especially important for women's health care providers, as selection will act strongly on reproductive parameters, and the biological costs of female reproduction are generally more resource expensive than for men (e.g.

Early economic evaluation of MRI-guided laser interstitial thermal therapy (MRgLITT) and epilepsy surgery for mesial temporal lobe epilepsy.

Background: MRI-guided laser interstitial thermal therapy (MRgLITT) is a new minimally invasive treatment for temporal lobe epilepsy (TLE), with limited effectiveness data. It is unknown if the cost savings associated with shorter hospitalization could offset the high equipment cost of MRgLITT. We examined the cost-utility of MRgLITT versus surgery for TLE from healthcare payer perspective, and the value of additional research to inform policy decision on MRgLITT.

Role of Physician Gender in the Modern Practice of Obstetrics and Gynecology: Do Obstetrician-Gynecologists Perceive Discrimination from their Sex?

Objectives: It is unclear whether obstetrician-gynecologists (OBGYNs) experience gender discrimination as a result of patient and organizational gender preferences. Our objective was to evaluate whether the gender preference for OBGYNs resulted in perceptions of discrimination by the physician while simultaneously assessing their patients' views for choosing their OBGYN.

Methods: A survey assessed whether OBGYNs' perceptions of patients and employers' preferences for gender in selecting an OBGYN affected their clinical practice and resulted in feelings of discrimination.

Obstetrician-gynecologists' practices and attitudes on substance use screening during pregnancy.

Objective: To describe obstetrician-gynecologists' practices and attitudes related to substance use screening in pregnant patients.

Study Design: A 2017 cross-sectional survey assessed US obstetrician-gynecologists' (n = 462; response rate = 34%) practices (substance use screening frequency and methods) and attitudes (practice priority of screening, confidence in treating, and responsibility statements). Chi-squared tests and adjusted modified Poisson regression were used to estimate associations between practices and attitudes.

Obstetrician-gynecologists' practice patterns related to opioid use during pregnancy and postpartum-United States, 2017.

Objective: To describe obstetrician-gynecologists' practices and attitudes related to opioid use among pregnant and postpartum women.

Study Design: A 2017 cross-sectional survey assessed U.S.

Obstetricians' knowledge and practices regarding the management of preeclampsia.

Preeclampsia affects over 4% of pregnancies in the United States. Management of preeclampsia is dependent on the severity of the condition and can range from expectant management to early delivery and inpatient observation. After publication of the hypertension in Pregnancy Task Force guidelines in 2013, little is known about their implementation and acceptance by practicing obstetricians and maternal-fetal medicine (MFM) specialists.

Screening for History of Childhood Abuse: Beliefs, Practice Patterns, and Barriers Among Obstetrician-Gynecologists.

Background: A history of childhood abuse is strongly linked to adult health problems. Obstetrician-gynecologists will undoubtedly treat abuse survivors during their careers, and a number of patient presenting problems may be related to a history of childhood abuse (e.g.

Practice Variation in Antenatal Steroid Administration for Anticipated Late Preterm Birth: A Physician Survey.

Objective: The objective of this study was to measure knowledge and practice variation in late preterm steroid use.

Study Design: Electronic survey of American College of Obstetricians and Gynecologists (ACOG) members about data supporting the ACOG/Society for Maternal-Fetal Medicine (SMFM) recommendations and practice when caring for women with anticipated late preterm birth (PTB), 34 to 36 weeks.

Results: Of 352 administered surveys, we obtained 193 completed responses (55%); 82.

Systematic review and network meta-analysis of resective surgery for mesial temporal lobe epilepsy.

Objective: To evaluate the effectiveness of anterior temporal lobectomy (ATL) versus selective amygdalohippocampectomy (SAH) on seizure-free outcome in patients with temporal lobe epilepsy, using both direct and indirect evidence from the literature.

Methods: MEDLINE, Embase and Cochrane databases were searched for original research articles and systematic reviews comparing ATL versus SAH, and ATL or SAH versus medical management (MM). The outcome was seizure freedom at 12 months of follow-up or longer.

"Saved by the Bell": Near SUDEP during intracranial EEG monitoring.

SUDEP is the sudden unexpected death of a person with epilepsy, when no structural or toxicological cause of death can be found. The majority of witnessed cases are reported to be preceded by a convulsive seizure and postictal hypoventilation. Here, we report an 8-year-old girl with drug-resistant focal seizures secondary to a focal cortical dysplasia type IIb.

Benefits and Barriers to Teaching Medical Students in an Ob-Gyn Clinic.

Introduction: As the US health-care system has evolved over the past decade, access to obstetric care in rural communities has declined, and there has been a challenge in retaining obstetrics and gynecology (OB-GYN) providers to train the next generation of physicians. The current pilot study sought to identify the factors that influence faculty who train medical students within the field of OB-GYN with the hope of influencing recruitment and retention of providers for the future.

Methods: Clinical OB-GYN faculty within the University of Washington School of Medicine regional medical education program were surveyed about practice patterns and beliefs regarding medical student training as part of a pilot study on provider recruitment and retention.