The Association of Health Literacy with Health-Related Quality of Life in Youth and Young Adults with Spina Bifida: A Cross-Sectional Study.

OBJECTIVE: The objective of the study was to determine if health literacy is associated with health-related quality of life (HRQOL) in adolescents and young adults (AYAs) with spina bifida. STUDY DESIGN: Between June 2019 and March 2020, the Patient-Reported Outcome Measurement Information System Pediatric Global Health-7 (PGH-7), a measure of HRQOL, and the Brief Health Literacy Screening Tool (BRIEF) were administered to patients ≥12 years old with a diagnosis of spina bifida seen in our multidisciplinary spina bifida center. Questionnaires were completed at scheduled clinic visits. The primary outcome was the PGH-7 normalized T-score. The primary exposure was the BRIEF score. Demographic and clinical characteristics were obtained from the medical record. Nested, multivariable linear regression models assessed the association between health literacy and the PGH-7 score. RESULTS: Of 232 eligible patients who presented to clinic, 226 (97.4%) met inclusion criteria for this study. The median age was 17.0 years (range: 12-31). Most individuals were female (54.0%) and had myelomeningocele (61.5%). Inadequate, marginal, and adequate health literacy levels were reported by 35.0%, 28.3%, and 36.7% of individuals. In univariable analysis, higher health literacy levels were associated with higher PGH-7 scores. In nested, sequentially adjusted multivariable linear regression models, a higher health literacy level was associated with a stepwise increase in the PGH-7 score. In the fully adjusted model, adequate health literacy and marginal health literacy, compared with inadequate health literacy, were associated with increases in a PGH-7 score of 3.3 (95% CI: 0.2-6.3) and 1.1 (95% CI: -2.0 to 4.2), respectively. CONCLUSIONS: Health literacy was associated with HRQOL after adjusting for demographic and clinical factors. Strategies incorporating health literacy are needed to improve HRQOL in AYAs with spina bifida.

Kidney Outcomes and Hypertension in Survivors of Wilms Tumor: A Prospective Cohort Study.

OBJECTIVE: To assess the prevalence of therapy-related kidney outcomes in survivors of Wilms tumor (WT). STUDY DESIGN: This prospective cohort study included survivors of WT who were ≥5 years old and ≥1 year from completing therapy, excluding those with preexisting hypertension, prior dialysis, or kidney transplant. Participants completed 24-hour ambulatory blood pressure monitoring (ABPM). Abnormal blood pressure (BP) was defined as ≥90th percentile. Masked hypertension was defined as having normal office BP and abnormal ABPM findings. Urine was analyzed for kidney injury molecule-1, interleukin-18, epidermal growth factor, albumin, and creatinine. The estimated glomerular filtration rate (eGFR) was calculated using the bedside chronic kidney disease in children equation. Recent kidney ultrasound examinations and echocardiograms were reviewed for contralateral kidney size and left ventricular hypertrophy, respectively. Clinical follow-up data were collected for approximately 2 years after study enrollment. RESULTS: Thirty-two participants (median age, 13.6 years [IQR, 10.5-16.3 years]; 75% stage 3 or higher WT) were evaluated at a median of 8.7 years (IQR, 6.5-10.8 years) after therapy; 29 participants underwent unilateral radical nephrectomy, 2 bilateral partial nephrectomy, and 1 radical and contralateral partial nephrectomy. In this cohort, 72% received kidney radiotherapy and 75% received doxorubicin. Recent median eGFR was 95.6 mL/min/1.73 m2 (IQR, 84.6-114.0; 11 [34%] had an eGFR of <90 mL/min/1.73 m2). Abnormal ABPM results were found in 22 of 29 participants (76%), masked hypertension in 10 of 29 (34%), and microalbuminuria in 2 of 32 (6%). Of the 32 participants, 22 (69%) had abnormal epidermal growth factor; few had abnormal kidney injury molecule-1 or interleukin-18. Seven participants with previous unilateral nephrectomy lacked compensatory contralateral kidney hypertrophy. None had left ventricular hypertrophy. CONCLUSIONS: In survivors of WT, adverse kidney outcomes were common and should be closely monitored.

Mortality and Morbidity after Laparoscopic Surgery in Children with and without Congenital Heart Disease.

OBJECTIVES: To determine the risk of morbidity and mortality after laparoscopic surgery among children with congenital heart disease (CHD). STUDY DESIGN: Cohort study using the 2013-2014 National Surgical Quality Improvement Program-Pediatrics, which prospectively collected data at 56 and 64 hospitals in 2013 and 2014, respectively. Primary exposure was CHD. Primary outcome was overall in-hospital postoperative mortality. Secondary outcomes included 30-day mortality and 30-day morbidity (any nondeath adverse event). Among 34?543 children who underwent laparoscopic surgery, 1349, 1106, and 266 had minor, major, and severe CHD, respectively. After propensity score matching within each stratum of CHD severity, morbidity and mortality were compared between children with and without CHD. RESULTS: Children with severe CHD had higher overall mortality and 30-day morbidity (OR 12.31, 95% CI 1.59-95.01; OR 2.51, 95% CI 1.57-4.01, respectively), compared with matched controls. Overall mortality and 30-day morbidity were also higher among children with major CHD compared with children without CHD (OR 3.46, 95% CI 1.49-8.06; OR 2.07, 95% CI 1.65-2.61, respectively). Children with minor CHD had similar mortality outcomes, but had higher 30-day morbidity compared with children without CHD (OR 1.71, 95% CI 1.37-2.13). CONCLUSIONS: Children with major or severe CHD have higher morbidity and mortality after laparoscopic surgery. Clinicians should consider the increased risks of laparoscopic surgery for these children during medical decision making.

Urological Findings in Beckwith-Wiedemann Syndrome With Chromosomal Duplications of 11p15.5: Evaluation and Management.

Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder with various congenital anomalies. Although the most classic constellation includes macrosomia, macroglossia, and omphalocele, nephrourological findings are commonly associated with BWS. Clinical presentation is highly variable because of its complex molecular heterogeneity, which involves changes in DNA methylation and disruption of growth regulatory genes. We report 3 pediatric patients, ages 13 months to 3 years old, who presented with clinical features consistent with BWS. A variety of nephrourological abnormalities were also noted, including posterior urethral valves, hydroureteronephrosis, and undescended testes. Genetic testing for all 3 patients revealed duplication of the region chromosome 11p15.5.