Two-Year Follow-up Survey: Views of US Anesthesiologists About Health Care Costs and Future Practice Roles.

Anesthesiologists' perspectives on US health care finance reform are increasingly germane to recent policy reforms. The aim of this follow-up survey was to examine how anesthesiologists' views of health care costs and future practice roles have changed since 2014. Six thousand randomly chosen active members of the American Society of Anesthesiologists were again surveyed and were also asked several new questions regarding specialties and perioperative management. Results showed an increase in self-reported understanding of the perioperative surgical home. Government, insurance companies, and pharmaceutical companies saw an increase in perceived "major responsibility" for cost reduction. Respondents vastly preferred that patient care under the perioperative surgical home be multidisciplinary.

Sphingosine phosphate lyase insufficiency syndrome: a systematic review.

BACKGROUND: Sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) or nephrotic syndrome type-14 is caused by biallelic mutations in SGPL1. Here, we conducted a systematic review to delineate the characteristics of SPLIS patients. METHODS: A literature search was performed in PubMed, Web of Science, and Scopus databases, and eligible studies were included. For all patients, demographic, clinical, laboratory, and molecular data were collected and analyzed. RESULTS: Fifty-five SPLIS patients (54.9% male, 45.1% female) were identified in 19 articles. Parental consanguinity and positive family history were reported in 70.9% and 52.7% of patients, respectively. Most patients (54.9%) primarily manifested within the first year of life, nearly half of whom survived, while all patients with a prenatal diagnosis of SPLIS (27.5%) died at a median [interquartile (IQR)] age of 2 (1.4-5.3) months (P = 0.003). The most prevalent clinical feature was endocrinopathies, including primary adrenal insufficiency (PAI) (71.2%) and hypothyroidism (32.7%). Kidney disorders (42, 80.8%) were mainly in the form of steroid-resistant nephrotic syndrome (SRNS) and progressed to end-stage kidney disease (ESKD) in 19 (36.5%) patients at a median (IQR) age of 6 (1.4-42.6) months. Among 30 different mutations in SGPL1, the most common was c.665G > A (p.Arg222Gln) in 11 (20%) patients. Twenty-six (49.1%) patients with available outcome were deceased at a median (IQR) age of 5 (1.5-30.5) months, mostly following ESKD (23%) or sepsis/septic shock (23%). CONCLUSION: In patients with PAI and/or SRNS, SGPL1 should be added to diagnostic genetic panels, which can provide an earlier diagnosis of SPLIS and prevention of ESKD and other life-threatening complications.

Patterns of allergenic food introduction in Los Angeles inner-city children.

Background: Early introduction of allergenic foods is recommended to reduce the risk of developing food allergies, but it is unclear whether recommendations are being followed. Objective: We examine patterns of allergenic food introduction in inner-city children enrolled in an academic pediatric practice in the greater Los Angeles area. Methods: This was a prospective study with patients ages 12 to 24 months recruited from the pediatrics continuity clinic at an inner-city tertiary medical center in the greater Los Angeles area. Caregivers were asked via anonymous surveys about their child's history of atopic diseases and at what age they first introduced egg, soy, wheat, peanut, tree nuts, fish, shrimp, and shellfish into their child's diet. Results: Two hundred caregivers responded to the survey. The average age of introduction of egg was 9.2 months, soy 10 months, wheat 9.3 months, peanut 10.5 months, tree nuts 10.9 months, fish 10.9 months, shrimp 11.3 months, and shellfish 11.5 months. Between ages 4-11 months, 65.3% of children were introduced egg, 19.1% soy, 55.8% wheat, 28.6% peanut, 17.1% tree nuts, 28.1% fish, 13.6% shrimp, and 7.0% shellfish. By age 24 months, 92% of children were introduced egg, 37.7% soy, 85.4% wheat, 67.3% peanut, 47.7% tree nuts, 67.8% fish, 48.2% shrimp, and 30.2% shellfish. Of the 14 children with eczema or egg allergy, 26.1% were introduced peanut by age 4-6 months and 50% by age 4-11 months. Conclusion: Despite recommendations, inner-city caregivers may not be introducing allergenic foods in a timely manner to their children.

ANCA-associated vasculitis in Caucasian and Hispanics of the Inland Empire of Southern California.

INTRODUCTION: ANCA-associated vasculitis is a disease with high morbidity and mortality which has shown to have different phenotypes in different ethnic and racial groups. This disease has been most frequently studied in Caucasians. We studied a group in Southern California where the Hispanics make up half of the population. We believe there will be different phenotypes between the two. METHODS: A retrospective study of 114 patients was conducted at two tertiary care centers between 2003 and 2019. Demographic data, ICU admission, ANCA antibody status, BVAS on presentation, VDI per the last clinic visit, the number of hospitalizations, the number of follow-up years, and treatment were recorded. We calculated odds ratios for the categorical data and ran independent sample T test for the continuous data with alpha equal to 0.05 for statistical significance. RESULTS: Difference was found in antibody status, disease presentation, morbidity, and age at diagnosis. Hispanics had greater number of AAV flares despite BVAS and VDI being comparable. Caucasians had more frequent follow-up. Hispanics had a 4.39 increase in odds of being admitted to the ICU, a 1.33 increased odds of developing acute respiratory failure, and a 67% increased odds of developing hemoptysis or pulmonary alveolar hemorrhage. Further, Hispanics had a 1.22 increase in odds of having ESRD. CONCLUSIONS: Clinicians treating Hispanic patients with AAV should have a high index of suspicion for severe disease in this patient population. Further, epidemiologic and disparities research should be conducted to evaluate the discrepancy in outcomes in these groups. Key Points • This is the first study to examine the phenotype and severity of ANCA associated vasculitis in Southern California, a population which is comprised largely of Hispanics. • Hispanics in this population were found to be more likely to be admitted to the ICU, have more flares, reach end-stage renal disease, have severe pulmonary manifestations, and had fewer outpatient follow-up visits than their Caucasian counterparts. • Clinicians should have a high suspicion for more severe disease in Hispanics in this region when compared to Caucasians. • More research is needed to assess the degree social determinants of health contribute to these findings and if progress can be made with decreasing health disparities between these populations in this disease.

Eosinophilic gastrointestinal disorders in patients with inborn errors of immunity: Data from the USIDNET registry.

Rationale: Eosinophilic gastrointestinal disorders (EGID), including eosinophilic esophagitis (EoE), are inflammatory disorders of the gastrointestinal mucosa mediated by complex immune mechanisms. Although there have been initial reports of EGID in patients with inborn errors of immunity (IEI), little is known about the presentation of EGID in immunodeficient individuals. Methods: We queried the U.S. Immunodeficiency Network (USIDNET) for patient records including the terms eosinophilic esophagitis, gastritis, enteritis, or colitis. We analyzed 74 patient records from the database, including diagnoses, demographics, infectious history, laboratory findings, genetic studies, therapeutic interventions, and clinical outcomes. Results: We examined 74 patient records. A total of 61 patients had isolated EoE, and 13 had distal gastrointestinal involvement consistent with EGID. The most common IEI were common variable immunodeficiency (43.2%), some form of combined immunodeficiency (21.6%), chronic granulomatous disease (8.1%), hyper-IgE syndrome (6.8%), and autoimmune lymphoproliferative syndrome (6.8%). The median age at presentation with IEI was 0.5 years (IQR 1.725, max 39 years) and 56.76% were male. Approximately 20% of the patients in the cohort received a hematopoietic stem cell transplantation for treatment of IEI, but the timing of the HSCT in relationship to the EGID diagnosis was unknown. Conclusions: Here, we report EGID in a diverse cohort of IEI patients, suggesting that both non-EoE EGID and EoE can be seen as comorbid conditions with a variety of IEI. Our data suggests that EGID may be more common in patients with IEI than would be expected based on estimates of EGID in the general population.

The evolution of the sexually selected sword in Xiphophorus does not compromise aerobic locomotor performance.

Sexual selection can increase morphological diversity within and among species. Little is known regarding how interspecific variation produced through sexual selection affects other functional systems. Here, we examine how morphological diversity resulting from sexual selection impacts aerobic locomotor performance. Using Xiphophorus (swordtail fish) and their close relatives (N = 19 species), we examined whether the evolution of a longer sexually selected sword affects critical swimming speed. We also examined the effect of other suborganismal, physiological, and morphological traits on critical swimming speed, as well as their relationship with sword length. In correlation analyses, we found no significant relationship between sword length and critical swimming speed. Unexpectedly, we found that critical swimming speed was higher in species with longer swords, after controlling for body size in multiple regression analyses. We also found several suborganismal and morphological predictors of critical swimming speed, as well as a significant negative relationship between sword length and heart and gill mass. Our results suggest that interspecific variation in sword length is not costly for this aspect of swimming performance, but further studies should examine potential costs for other types of locomotion and other components of Darwinian fitness (e.g., survivorship, life span).

As the sword grows: individual variation and ontogenetic effects of a sexually selected trait on locomotor performance in Xiphophorus hellerii.

Previous studies aimed at detecting costs of sexually selected traits have yielded mixed results partly because of variable methods. We present a novel approach: a repeated-measures design to examine individual variation in locomotor performance of male Xiphophorus hellerii as the sexually selected sword develops ontogenetically and to determine whether the growth of a sexually selected trait alters consistency of performance. Individual differences in sprint speed, critical swimming speed (stamina), and relative sword length were statistically repeatable over 9 wk. However, using the Akaike Information Criterion corrected for small sample sizes, the best-fit predictive models for swimming performance did not include sword length or relative sword length. Furthermore, in less supported models and within-week comparisons, there was no statistically significant effect of sword length on performance. These results suggest little effect of the sword on locomotor abilities, which is inconsistent with results from some previous experimental manipulations, possibly because compensatory traits develop ontogenetically in parallel with the sword. However, our results are consistent with correlational studies of natural variation that suggest no locomotor cost of the sword. These results do not necessarily imply a complete lack of a cost to the sword but rather lack of a functional cost for swimming performance.