Progress and Gaps in Respiratory Disease Research and Treatment: Highlights of the IRM 2024 in Shanghai.

Respiratory diseases pose a major public health challenge globally, necessitating collaborative efforts between basic researchers and clinicians for effective solutions. China, which is heavily impacted by a broad spectrum of respiratory disorders, has made notable strides in both research and clinical management of these diseases. The International Respiratory Medicine (IRM) meeting was organized with the primary goal of facilitating the exchange of recent research developments and promoting collaboration between Chinese and American scientists in both basic and clinical research fields.

Conscientious Objection: Understanding When and Why Primary Care Physicians Object to Providing Health Care to Transgender and Gender-Diverse Patients in an Appalachian Medical Center.

Introduction: Transgender and gender-diverse (TGD) individuals face barriers to accessing primary and gender-affirming care, especially in rural regions where a national shortage of medical providers with skills in caring for TGD people is further magnified. This care may also be impacted by individual providers' strongly held personal or faith beliefs and associated conscientious objection to care.

Purpose: This study assesses the prevalence of conscientious objection to providing care and gender-affirming hormone (GAH) therapy to TGD individuals among physicians in an Appalachian academic medical center.

Accuracy of Screening Tests for the Diagnosis of Urinary Tract Infections in Young Children.

Background: The objective of this study was to compare the accuracy of available tests for pyuria, including newer automated tests, and to examine the implications of requiring them for the diagnosis of urinary tract infections (UTIs).

Methods: We included children between 1 and 36 months of age undergoing bladder catheterization for suspected UTIs who presented to 1 of 3 pediatric centers. Using a positive urine culture result as the reference standard, we compared the sensitivity of 5 modalities for assessing pyuria at the cutoffs most often used clinically for detecting children with a positive culture result: leukocyte esterase on a dipstick, white blood cell (WBC) count on manual microscopy with and without using a hemocytometer, automated WBC enumeration using flow cytometry, and automated WBC enumeration using digital imaging with particle recognition.

ZDHHC3-mediated SCAP S-acylation promotes cholesterol biosynthesis and tumor immune escape in hepatocellular carcinoma.

Cholesterol metabolism reprogramming plays essential roles in hepatocellular carcinoma (HCC). However, precisely how cholesterol metabolism is dysregulated is not clear. Here, we show that the palmitoyltransferase ZDHHC3 and depalmitoylase ABHD17A regulate HCC cell cholesterol biosynthesis by dynamically S-acylating SREBP cleavage-activating protein (SCAP).

Dual-labeled anti-GD2 targeted probe for intraoperative molecular imaging of neuroblastoma.

Background: Surgical resection is integral for the treatment of neuroblastoma, the most common extracranial solid malignancy in children. Safely locating and resecting primary tumor and remote deposits of disease remains a significant challenge, resulting in high rates of complications and incomplete surgery, worsening outcomes. Intraoperative molecular imaging (IMI) uses targeted radioactive or fluorescent tracers to identify and visualize tumors intraoperatively.

Increases in 4-Acetaminobutyric Acid Generated by Phosphomevalonate Kinase Suppress CD8 T Cell Activation and Allow Tumor Immune Escape.

Certain metabolites in the tumor microenvironment (TME) can alter innate immunity. Here, it is shown how phosphomevalonate kinase (PMVK) allows hepatocellular carcinoma (HCC) cells to overcome the anti-tumor immunity mediated by CD8 T cells. In HCCs, depletion of PMVK is required to facilitate CD8 T cell activation and their subsequent suppression of tumor growth.

Impact of Elevated Serum Triglycerides on Children with Acute Recurrent or Chronic Pancreatitis from INSPPIRE-2.

Objective: To determine if mild-moderate hypertriglyceridemia (HTG) is associated with increased development of chronic pancreatitis (CP) or pancreatitis-associated complications in children with acute recurrent or CP.

Study Design: Longitudinal data from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE-2 (INSPPIRE-2) cohort of children with acute recurrent or CP (n = 559) were analyzed. Subjects were divided into normal triglycerides (<150 mg/dL; 1.

Measuring Pectus Excavatum Severity, External Caliper, or Cross-Sectional Imaging: Family Perceptions.

Introduction: Patients with pectus excavatum (PE) often undergo cross-sectional imaging (CSI) to quantify severity for insurance authorization before surgical repair. The modified percent depth (MPD), an external caliper-based metric, was previously validated to be similar to the pectus index and correction index. This study explored family perceptions of CSI and MPD with respect to value and costs.

Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease.

Background: Childhood interstitial lung disease (chILD) encompasses a group of rare heterogeneous respiratory conditions associated with significant morbidity and mortality. Reports suggest that many patients diagnosed with chILD continue to have potentially progressive or fibrosing disease into adulthood. Over the last decade, the spectrum of conditions within chILD has widened substantially, with the discovery of novel entities through advanced genetic testing.

Evaluating Procedural Performance: A Composite Outcome for Aortic and Pulmonary Valvuloplasty in Congenital Cardiac Catheterization.

Background: Safety events and technical success (TS) have been previously reported for aortic and pulmonary valvuloplasty, but a composite performance measure as a novel, patient-centered strategy has neither been developed nor been studied. This study aims to refine a procedural performance (PP) variable, a composite of TS and procedural safety, for isolated, standard-risk aortic and pulmonary valvuloplasty.

Methods: A multicenter review was performed using data from the Congenital Cardiac Catheterization Project on Outcomes registry.

The protein disulfide isomerase A3 and osteopontin axis promotes influenza-induced lung remodelling.

Background And Purpose: Fibrotic lung remodelling after a respiratory viral infection represents a debilitating clinical sequela. Studying or managing viral-fibrotic sequela remains challenging, due to limited therapeutic options and lack of understanding of mechanisms. This study determined whether protein disulfide isomerase A3 (PDIA3) and secreted phosphoprotein 1 (SPP1), which are associated with pulmonary fibrosis, can promote influenza-induced lung fibrotic remodelling and whether inhibition of PDIA3 or SPP1 can resolve viral-mediated fibrotic remodelling.

Improvement of Perioperative Efficiency via Pediatric Preanesthesia Respiratory Risk Training.

Purpose: This process improvement to the registered nurse (RN) preanesthesia telephone call interview applied an evidence-based bundle of interventions to improve perioperative efficiency. The overarching aim was to decrease RN subjectivity regarding pediatric upper respiratory tract infection (URI) symptoms during the preoperative telephone call family outreach interview to allow for early identification of respiratory illness that could lead to a day of surgery (DOS) cancellation.

Design: The design was an evidence-based process improvement with a pretest post-test design.

Mitigation of Fetal Radiation Injury from Mid-Gestation Total-body Irradiation by Maternal Administration of Mitochondrial-Targeted GS-Nitroxide JP4-039.

Victims of a radiation terrorist event will include pregnant women and unborn fetuses. Mitochondrial dysfunction and oxidative stress are key pathogenic factors of fetal radiation injury. The goal of this preclinical study is to investigate the efficacy of mitigating fetal radiation injury by maternal administration of the mitochondrial-targeted gramicidin S (GS)-nitroxide radiation mitigator JP4-039.

Inhibition of DUSP18 impairs cholesterol biosynthesis and promotes anti-tumor immunity in colorectal cancer.

Tumor cells reprogram their metabolism to produce specialized metabolites that both fuel their own growth and license tumor immune evasion. However, the relationships between these functions remain poorly understood. Here, we report CRISPR screens in a mouse model of colo-rectal cancer (CRC) that implicates the dual specificity phosphatase 18 (DUSP18) in the establishment of tumor-directed immune evasion.

Long-Term PM Exposure and Upregulation of Expression in Nasal Epithelium from Youth with Asthma.

Background: Little is known about long-term PM exposure and airway epithelial gene expression.

Objective: To test for association between long-term PM exposure and nasal epithelial gene expression in youth with asthma.

Methods: Transcriptome-wide association study (TWAS) of long-term PM in nasal epithelium from youth aged 6-20 years in the: 1) Epigenetic Variation and Childhood Asthma in Puerto Ricans study (EVA-PR, n=182); 2) Vitamin D Kids Asthma Study (VDKA, n=58); and 3) Stress and Treatment Response in Puerto Rican and African American Children with Asthma study (STAR, n=81).

Intestinal Transplant for Hirschsprung's Disease: Stoma for Life or Not?

Hirschsprung's disease is a dysmotility disease caused by lack of ganglion cells in the bowel wall that can affect varying lengths of the intestine. In extreme circumstances, there can be little remaining ganglionated bowel, and the patient becomes dependent on parental nutrition (PN) for survival. Intestinal transplant has been utilized to salvage these patients suffering terminal complications of PN.

Recurrent Retinal Detachment in Stickler Syndrome.

Objective: To assess clinical factors leading to recurrent retinal detachment (RD) and characteristics of recurrence in patients with Stickler Syndrome.

Methods: Retrospective case series study of patients with clinical diagnosis of Stickler Syndrome who underwent rhegmatogenous RD repair. Recurrent RD after initial surgery was categorized as "early" if the recurrence was within 1 year or "late" if greater than 1 year.