Children's of Alabama is a pediatric acute care children's hospital located in Birmingham, Alabama. The main hospital has 332 beds and 48 bassinets. The hospital is affiliated with the University of Alabama at Birmingham School of Medicine. The hospital provides comprehensive pediatric specialties and subspecialties to pediatric patients aged 0–21 throughout Alabama and surrounding states. Children's of Alabama features the only level 1 pediatric trauma center in the state. The hospital was founded in 1911. The system's main hospital is located on the city's Southside, with additional outpatient facilities and primary care centers throughout central Alabama. It is the third largest children's hospital in the United States in terms of square footage.The hospital is nationally ranked in multiple pediatric specialties.
Neonates with congenital kidney failure or acute kidney injury often require continuous kidney replacement therapy (CKRT). Historically, venous access for CKRT in this population has relied on non-approved adult catheters, commonly via the internal jugular (IJ) vein. Limited international reports describe using the umbilical vein for CKRT, but this practice is not widespread in the USA. The Nuwellis® dual lumen extended length catheter (dELC), originally created for adult ultrafiltration, may support alternative access for neonatal CKRT. This case series describes the clinical course of four neonates requiring CKRT using a 6 French Nuwellis® dELC placed in the umbilical vein at University of Iowa Stead Family Children’s Hospital and Children’s of Alabama. Patient demographics, CKRT prescriptions, access characteristics, and complications were reviewed. All four neonates underwent successful umbilical vein placement of the 6 Fr dELC. Weight at initiation ranged from 2.23 to 4.1 kg, with blood flow rates 20–40 mL/min. Epoprostenol anticoagulation was used in three patients. Half of the cohort later transitioned to a tunneled catheter, and one transitioned to an IJ catheter following fluid removal. One catheter developed hub cracks likely related to lumen caps, and one patient experienced flow limitations attributed to positioning rather than catheter function. Two patients had no catheter-related complications. Umbilical venous access using the 6Fr dELC may represent a viable alternative for neonatal CKRT. Coil reinforcement within the catheter may support patency and better functionality. Additional studies are needed to assess safety and broader applicability. A higher resolution version of the Graphical abstract is available as Supplementary information
PURPOSE:Molecular biomarkers are increasingly used for risk stratification, particularly in up-front surgery settings (Children's Oncology Group trials), whereas in preoperative chemotherapy setting, the ongoing International Society of Pediatric Oncology (SIOP)-Renal Tumor Study Group-2016 UMBRELLA study aims to validate selected biomarkers for future risk-adapted treatment strategies. This systematic review summarizes all literature on the prognostic value of these biomarkers. MATERIALS AND METHODS:A systematic literature review (PubMed and Embase; up to January 2025) included studies with ≥50 de novo Wilms tumors (WTs). Eligible biomarkers included copy number variations; 1q gain, 1p and/or 16q loss of heterozygosity (LOH)/loss, 12 gain, 14q loss, 22 loss, 11p15 LOH/loss of imprinting (LOI), and structural somatic variants (TP53 [and/or 17p loss], MYCN, FBXW7, WT1, WTX, SIX1/SIX2, DROSHA, DGCR8, AMER1, CTNNB1, GPC3, MLLT1, DICER1, DIS3L2). Outcome included relapse-free survival, event-free survival (EFS), and overall survival (OS). Risk of bias was assessed with quality in prognosis studies tool. RESULTS:Low-bias multivariable/stratified analyses identified 1q gain as worse EFS and 1p and/or 16q LOH/loss as worse EFS/OS prognostic factors, in up-front nephrectomy settings. Preoperative chemotherapy settings revealed similar trends with lacking significance. TP53 and MYCN were adverse prognostic in univariate analyses. No prognostic data were available for the remaining variants. CONCLUSION:1q gain and 1p and/or 16q LOH/loss emerge as independent prognostic biomarkers in up-front nephrectomy settings. Evidence remains limited in preoperative chemotherapy settings, particularly when using SIOP-oriented treatment algorithms. Prognostic value of TP53, MYCN, and 11p15 LOH/LOI warrants further validation in both settings. This highlights the need for adequately powered prospective studies, specifically in the preoperative chemotherapy setting, to establish reliable molecular biomarkers.
BACKGROUND:Smoke exposure affects one-third of children with CF (CwCF), but smoking cessation strategies for their caregivers have not been tested. METHODS:We developed and tested CEASE-CF, a randomized controlled smoking cessation intervention tailored to smoking caregivers of CwCF and delivered by a tobacco treatment specialist (TTS) integrated into the CF care team. The intervention arm received CF-specific smoke exposure education, intake assessment, and treatment (nicotine replacement therapy and 12 counseling sessions over 6 months). The control arm received education. Primary outcomes were feasibility and acceptability among caregivers and providers. Secondary outcomes were 6-month quit rates and child hair nicotine concentrations. RESULTS:36 caregivers (24 intervention, 12 control) were recruited. Feasibility (64% accrual, 100% retention) and acceptability (86% caregivers satisfied and likely to recommend CEASE-CF, 67% finding it helpful) were high. Providers' (n = 19) mean scores were 4.7 (1-5 scale) for satisfaction, implementation, and recommendation to other clinics. At 6 months, 17% participants had quit smoking, and 78% had a 7-day point prevalence reduction of at least 17%, with an average reduction of 54%. Hair nicotine decreased from 2.7 (SE 0.17) ng/mg at baseline to 1.1 (SE 0.17) ng/mg at 6 months (p < 0.001). At baseline, all hair nicotine specimens were above the exposure threshold of 1.0 ng/mg, but only 41% at 6 months. CONCLUSIONS:Tobacco treatment delivered by a TTS part of the CF care team reduces smoke exposure in CwCF. CEASE-CF is a feasible and acceptable model for smoking cessation that can be incorporated into CF clinical care.
Glomerulocystic renal disease has numerous etiologies, including HNF1B mutations. In addition to cysts, morphologic renal findings in a setting of HNF1B mutations include cystic renal dysplasia, solitary functioning kidney, horseshoe kidney, and oligomeganephronia. Embryonal hyperplasia resembling nephrogenic rests has been reported in rare cases of glomerulocystic disease, but none have been genetically characterized. We report a case of bilateral glomerulocystic kidney disease (GCKD) showing extensive embryonal hyperplasia in a setting of germline HNF1B mutation with progressive renal failure. Explant showed numerous epithelial proliferations throughout the intervening stroma. GCKD may be seen in a setting of HNF1B mutation; however, the additional finding of extensive embryonal hyperplasia in a case with a known mutation has never been reported. Reports of similar embryonal hyperplasia, associated with either cystic kidney disease or other disease processes, appear to represent a heterogeneous population of presentations and etiologies, though there is sufficient evidence to suggest that this is a finding, that is, recurrently associated with cystic kidney diseases. The underlying pathobiology of embryonal hyperplasia and the neoplastic potential in this setting is unknown. We report this case to highlight a novel combination of morphologic and genetic findings in GCKD and to raise awareness of this rare finding.
Among pediatric institutions surveyed, administration of MMR, VAR, and RV was frequently allowed, though infection-prevention practices varied, particularly for VAR. Most institutions used standard precautions and reported no vaccine-derived transmission. Despite ongoing safety concerns and limited formal policies, most respondents believed benefits outweighed risks.
