Citations

A task force of pediatric surgical specialists with the support of The American College of Surgeons recently launched a verification program for pediatric surgery, the Children’s Surgery Verification quality improvement program, with the goal of improving pediatric surgical, procedural, and perioperative care. Included in this program are specific standards for the delivery of pediatric anesthesia care across a variety of practice settings. We review the background, available evidence, requirements for verification, and verification process and its implications for the practice of pediatric anesthesia across the country. In addition, we have included a special roundtable interview of 3 recently Children’s Surgery Verification–verified program directors to provide an up-to-date real-world perspective of this children’s surgery quality improvement program.

Background/Aims Studies have shown that nonalcoholic fatty liver disease (NAFLD) is associated with an increased risk of chronic kidney disease (CKD). However, it is unknown whether severe liver fibrosis is associated with a higher prevalence of CKD among NAFLD. We examined the diagnostic performance of noninvasive fibrosis markers in identifying prevalent CKD among NAFLD. Methods This study represents an analysis of the National Health and Nutrition Examination Survey conducted in the USA between 1988 and 1994. NAFLD was defined by ultrasonographic evidence of hepatic steatosis without other liver diseases. CKD was defined by estimated glomerular filtration rate less than 60 ml/min/1.73 m2. The presence and severity of hepatic fibrosis were determined by the NAFLD fibrosis score (NFS), FIB-4 score, APRI score, and BARD score. Multiple logistic regression was performed to generate odds ratios (ORs) and receiver operating characteristic curves were used to assess the predictive values of each marker. Results A total of 4142 individuals with NAFLD were included; 200 (4.8%) had CKD. The area under the receiver operating characteristic curves was 0.77, 0.75, 0.62, and 0.51 for the multivariable equation with FIB-4, NFS, BARD, and APRI score, respectively. Compared with patients with a low probability of developing advanced liver fibrosis, individuals with a high probability of developing fibrosis showed significantly increased odds of CKD as estimated by NFS (adjusted OR: 4.92, 95% confidence interval: 2.96–8.15) and FIB-4 (adjusted OR: 2.27, 95% confidence interval: 1.05–4.52). Conclusion Advanced liver fibrosis, defined by NFS and FIB-4 scores, is associated independently with CKD among individuals with NAFLD. FIB-4 is the best predictor of an increased risk of prevalent CKD.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a rare but potentially life-threatening cutaneous hypersensitivity reaction characterized by extensive mucocutaneous eruption, fever, hematologic abnormalities, and extensive organ involvement. Here, we present a case of a young woman with DRESS syndrome following exposure to vancomycin with renal, cutaneous, and gastrointestinal involvement. To the best of our knowledge, this is the first case description in the literature of DRESS of the gastrointestinal tract with autoimmune enteropathy.

The SCN5A-encoded voltage-gated mechanosensitive Na+channel NaV1.5 is expressed in human gastrointestinal smooth muscle cells and interstitial cells of Cajal. NaV1.5 contributes to smooth muscle electrical slow waves and mechanical sensitivity. In predominantly Caucasian irritable bowel syndrome (IBS) patient cohorts, 2–3% of patients have SCN5A missense mutations that alter NaV1.5 function and may contribute to IBS pathophysiology. In this study we examined a racially and ethnically diverse cohort of IBS patients for SCN5A missense mutations, compared them with IBS-negative controls, and determined the resulting NaV1.5 voltage-dependent and mechanosensitive properties. All SCN5A exons were sequenced from somatic DNA of 252 Rome III IBS patients with diverse ethnic and racial backgrounds. Missense mutations were introduced into wild-type SCN5A by site-directed mutagenesis and cotransfected with green fluorescent protein into HEK-293 cells. NaV1.5 voltage-dependent and mechanosensitive functions were studied by whole cell electrophysiology with and without shear force. Five of 252 (2.0%) IBS patients had six rare SCN5A mutations that were absent in 377 IBS-negative controls. Six of six (100%) IBS-associated NaV1.5 mutations had voltage-dependent gating abnormalities [current density reduction (R225W, R433C, R986Q, and F1293S) and altered voltage dependence (R225W, R433C, R986Q, G1037V, and F1293S)], and at least one kinetic parameter was altered in all mutations. Four of six (67%) IBS-associated SCN5A mutations (R225W, R433C, R986Q, and F1293S) resulted in altered NaV1.5 mechanosensitivity. In this racially and ethnically diverse cohort of IBS patients, we show that 2% of IBS patients harbor SCN5A mutations that are absent in IBS-negative controls and result in NaV1.5 channels with abnormal voltage-dependent and mechanosensitive function.NEW & NOTEWORTHY The voltage-gated Na+channel NaV1.5 contributes to smooth muscle physiology and electrical slow waves. In a racially and ethnically mixed irritable bowel syndrome cohort, 2% had mutations in the NaV1.5 gene SCN5A. These mutations were absent in irritable bowel syndrome-negative controls. Most mutant NaV1.5 channels were loss of function in voltage dependence or mechanosensitivity.