Comer Children's Hospital

Background: The best treatment for children with KD who fail to respond to the first dose of IVIG (refractory KD) is currently unknown. The purpose of this study was to determine treatment practices of pediatric rheumatologists in North America who manage IVIG-refractory KD. Methods: A 34-item web-based survey was sent to 102 randomly selected members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA). The anonymous survey addressed the use of primary intensification as well as the treatment of IVIG-refractory KD. Results: The response rate was 82%; 56% (all pediatric rheumatologists) completed the survey. Primary intensification was used for macrophage activation syndrome (MAS), KD shock, and those at high risk for coronary artery aneurysms (CAAs) by 84%, 76% and 52% of responders, respectively, with corticosteroids (CSs) used most frequently. For IVIG-refractory KD without CAA, a second dose of IVIG was used most often (63% alone; 23% plus CS). With non-giant CAAs, only 15% used a second IVIG alone, 40% used IVIG plus CS, and 35% took infliximab, usually with CS/IVIG. With giant CAA, treatments used most frequently were CS, a second IVIG, and infliximab (91%, 69%, and 58%, respectively), usually as combinations of two or more medications. Conclusions: Treatment of IVIG-refractory KD varies significantly among North American pediatric rheumatologists, particularly in the presence of CAAs. Our findings emphasize the need for research to identify the most effective therapy for this KD subgroup. The current use of primary intensification and the presence and size of the CAA will need to be considered as consensus treatment plans are developed.

Although survival rates for pediatric cancer diagnoses have improved significantly, persistent health disparities prevent many children and adolescents in the United States from reaping equitable benefit from this progress. This review examines inequities related to race and ethnicity, language preference, socioeconomic status, and geographic location. A literature search identified 33 articles highlighting how these factors intersect to affect diagnosis, treatment access, clinical trial participation, and outcomes. Black patients and Hispanic patients face consistently poorer survival, while language barriers are linked to delayed care and increased mortality. Low socioeconomic status and lack of insurance contribute to late-stage diagnoses and treatment nonadherence. Residence in rural areas or historically marginalized urban neighborhoods are associated with increased mortality. Disparities in clinical trial enrollment further limit access to cutting-edge therapies and weaken the generalizability of research. Addressing these disparities requires systemic reform, culturally informed care, and inclusive research practices to ensure equitable outcomes.

Background/Objectives: To determine whether adolescent (T1) mental health, quality of life, and adjustment to celiac disease (CeD) are associated with college-age (T2) perceived physical and mental health and gluten-free diet (GFD) adherence. Methods: In 2015, adolescents with CeD (n = 101, T1) completed standardized surveys assessing mental health (CSI-4), quality of life (PedsQL), and adjustment to CeD (CDDUX). Five years later, participants ≥18 years self-reported GFD adherence and physical and mental health perception in college (n = 59, T2). Patients who were current or recent college students that provided complete data at both time points were analyzed (n = 43) using Kendall’s tau to test: concurrent associations among T2 perceived physical/mental health and GFD adherence; and prospective associations between T1 measures and T2 perceived outcomes. Results: Higher T1 CSI-4 and PedsQL scores were negatively correlated with T2 perceived physical health (τ = −0.31, p = 0.02 and τ = −0.28, p = 0.04, respectively). There was trending association between T1 PedsQL and T2 mental health perception (τ = −0.23, p = 0.06). T2 physical and mental health perception were positively correlated (τ = 0.41, p = 0.001). No significant associations emerged between T1 measures and T2 GFD adherence, nor between T2 health perception and GFD adherence, although T2 physical health perception positively trended with GFD adherence (p = 0.78). Conclusions: Adolescents with CeD reporting more depressive symptoms or lower quality of life feel less physically and mentally healthy when in college. In college, feeling physically healthy aligns with feeling mentally healthy, although neither clearly predicts GFD adherence. Early mental health screening in adolescents with CeD may support transitions to independent dietary management.

Child neurodevelopment is shaped by complex social, environmental, and biological factors. Few studies have characterized these factors in Latin American populations. We aimed to evaluate the impact of social and environmental factors on neurodevelopmental outcomes in 5-6-year-old children from three geographic locations in Colombia. Our pilot, cross-sectional study included 105 typically developing Colombian children from two urban cities and one rural municipality. Measures assessed domains of IQ (Wechsler Preschool and Primary Scale of Intelligence-WPPSI), executive functioning, psychosocial functioning, and a range of home environmental factors. We compared outcomes across locations and the impact of covariates using linear regression models. Including covariates, adjusted mean WPPSI IQ was significantly lower in children living in a rural town (80.41 ± 2.07) compared to either city (96.30 ± 2.76; 91.69 ± 2.90). Maternal education, location, dietary diversity, and home resources explained 46% of the variance in IQ. Outcomes in other domains were significantly different, but functioning was all within age expectations across groups. Significant disparities in cognitive and other outcomes exist among typically developing Colombian children, influenced by location, maternal education, and home resources. Future research should ensure well-matched control populations and use culturally appropriate, validated assessments to minimize social, cultural, and environmental confounding. Maternal education, dietary diversity, total home resources, and location account for approximately 50% of the neurodevelopmental variability of children from urban and rural Colombian cohorts. Typically developing children from a rural community in Colombia have a lower Full-Scale IQ than peers from urban cities, even after including multiple covariates. Future research should continue to study the multiple factors that impact neurodevelopment, particularly in low- and middle-income countries. Well-matched control populations and the use of culturally appropriate, validated assessments are important to minimize confounding factors in neurodevelopmental outcomes research, as statistical correction is not sufficient to fully account for these differences.

Celiac disease is an autoimmune disease caused by a permanent sensitivity to gluten. It manifests as small intestinal enteropathy, which is ongoing intestinal damage, irritation, and swelling. Celiac disease can occur at any age and can present with both gastrointestinal and extraintestinal symptoms, making it challenging to diagnose. Lifelong, strict adherence to a gluten-free diet is currently the only treatment option. After diagnosis, multidisciplinary care, including regular visits with a gastroenterology physician or a dietitian, are useful to help families manage dietary modifications, lifestyle changes, and the psychological burden that adherence to a lifelong treatment regimen can incur. Children often first present with symptoms to their pediatrician, and it is reasonable for pediatricians to initiate celiac serology screening in the appropriate clinical scenario and consider referral to gastroenterology for ongoing care. Knowledge in the primary care setting regarding the presentation of celiac disease, identifying patient populations who are at risk, and ordering the appropriate tests are paramount to prevent diagnostic delay.

Breast reconstruction using tissue expanders is the primary treatment option following mastectomy. Although skin growth in response to chronic supra-physiological stretch is well-established, individual patient factors such as breast shape, volume, skin prestrain, and mechanical properties, create unique deformation and growth patterns. The inability to predict skin growth and deformation prior to treatment often leads to complications and suboptimal esthetic outcomes. Personalized predictive simulations offer a promising solution to these challenges. We present a pipeline for predictive computational models of skin growth in tissue expansion. At the start of treatment, we collect three-dimensional (3D) photos and create an initial finite element model. Our framework accounts for uncertainties in treatment protocols, mechanical properties, and biological parameters. These uncertainties are informed by surgeon input, existing literature on mechanical properties, and prior research on porcine models for biological parameters. By collecting 3D photos longitudinally during treatment, and integrating the data through a Bayesian framework, we can systematically reduce uncertainty in the predictions. Calibrated personalized models are sampled using Monte Carlo methods, which require thousands of model evaluations. To overcome the computational limitations of directly evaluating the finite element model, we use Gaussian process surrogate models. We anticipate that this pipeline can be used to guide patient treatment in the near future.

Intestinal ultrasound (IUS) is a noninvasive tool for detecting and monitoring disease activity in children with inflammatory bowel disease (IBD). Remission values for bowel wall thickness (BWT) are extrapolated from adult data. We aimed to define normal BWT in children with IBD in sustained deep remission. Multicenter, retrospective pediatric IUS database including children with IBD, without a history of surgery or complications, who underwent IUS after achieving sustained deep remission, defined as the absence of ulcerations on ileocolonoscopy and/or transmural healing on magnetic resonance enterography and steroid-free clinical remission for ≥6 months. Univariate and multivariable analyses were performed using mixed-effect modeling. Ninety-eight children (40 [41%] female; 64 [5%] Crohn's disease [CD]: 29 (30%) ulcerative colitis [UC]: 5 (5%) IBD-unspecified), median age 15.2 [13.8-16.8] years), and 484 bowel segments were included. Median BWT was 1.4 (1.1-1.6) mm, with no difference in BWT between bowel segments (p = 0.28). In multivariable analysis, a positive association remained between BWT and weight (univariate β = 0.004 [95% confidence interval, CI = 0.001-0.007], p = 0.016, multivariate β = 0.006 [95% CI = 0.001-0.011], p = 0.041). A negative association remained between BWT and disease duration (unadjusted β = -0.024 [95% CI = -0.047 to -0.001] p = 0.046, adjusted β = -0.028 [95% CI = -0.052 to -0.003], p = 0.039). Previously inflamed BWT for children with IBD in sustained deep remission was less than 2.5 mm, which is less than 3 mm in adults, and unaffected by age, sex, and bowel segment in this population. BWT may be affected by weight and disease duration. These findings are crucial to the standardized assessment of transmural healing in children.

BackgroundGiven the most recent report of the nationwide rise of congenital syphilis (CS), with over a 773% increase from 2012 to 2021 per the Center for Disease Control and Prevention, we sought to utilize penicillin administration and ICD-10 billing data as proxies to assess the accuracy of diagnosis of congenital syphilis among major tertiary care pediatric hospitals.MethodsThis retrospective cohort study drawing data from 49 major tertiary children's hospitals in the United States sought to determine whether administration of penicillin in infants 30days or younger, excluding other common infectious diseases treated with the medication, correlated with recently reported congenital syphilis epidemiologic data.Results2290 infants met inclusion criteria and received penicillin therapy, excluding infants with the most common secondary infections treated with penicillin, with 1123 (49.3%) of those included not having a syphilis diagnosis made. Of the neonates with a coded diagnosis of CS, (1107/1162) 95.3% received more than 1day of penicillin therapy, and those not coded for CS that received more than 1day of penicillin therapy was 37.9% (428/1128).ConclusionsOur findings found a significant number of infants who had penicillin administration and laboratory and procedural workup performed suggestive of congenital syphilis consideration, without a congenital syphilis diagnosis billed. This may reflect an underestimation of what is notably a growing nationwide pandemic. By addressing the screening and treatment needs of patients with CS, we can help to address some of the socioeconomic inequities in pediatric and maternal healthcare, and further accurately characterize the extent of this increasingly prevalent disease process.