Goryeb Children's Hospital

Background/Aim: We previously reported that late gadolinium enhancement (LGE) on cardiac MRI (CMR) was as high as 82% in pediatric patients with COVID-19 vaccine-associated myocarditis (C-VAM) despite mild clinical symptoms and normal left ventricular function. As LGE can be a harbinger for future adverse events including arrhythmias, heart failure or sudden cardiac death, we sought to identify predictors for LGE in C-VAM, specifically assessing troponin as a screening marker for C-VAM patients at risk for myocardial scarring who could then be referred for a confirmatory CMR with LGE. Methods: In this longitudinal multicenter retrospective observational study across 38 U.S. member institutions of the Myocarditis After COVID Vaccination (MACiV) study network, 333 patients with C-VAM based on CDC criteria were included from April 2021 to November 2022. Data collected included demographics, laboratory values, clinical and cardiac imaging characteristics and outcomes. Using logistic regression, troponin levels at presentation were assessed as a log transformed continuous variable and categorized into tertiles. Results: The C-VAM patients were predominantly white (67%) adolescent males (91%, 15.7± 2.8 years). There were 216/333 (65%) patients who had both a reported troponin value and had a CMR. On univariate analysis, elevated troponin increased the probability of having LGE (OR=1.29, 95% CI: 1.06, 1.58, p=0.012). Even after controlling for age, race, sex, number of vaccine doses and left ventricular ejection fraction (OR=1.32, 95% CI: 1.06, 1.65, p=0.013). Patients >15 years compared to those ≤15 years of age were 2.94 (95% CI: 1.28, 6.75, p=0.011) times more likely to have LGE at presentation. Patients with troponin levels in the highest tertile compared to lowest tertile were 2.66 times (95% CI: 1.04, 6.83, p=0.042) more likely to have LGE along with a greater involvement > 4 AHA myocardial segments with LGE (p=0.004) Conclusions: Higher troponin values are associated with presence of late gadolinium enhancement on cardiac MRI in patients with COVID-19 vaccine-associated myocarditis. Troponin levels at presentation may facilitate risk stratification and function as a screening tool to identify those C-VAM patients with the greatest likelihood of myocardial scarring, who may benefit from undergoing CMR for tissue characterization.

This case series details seven pediatric cases of nutritional rickets in an inner-city population of New Jersey. Eighty six percent of these patients were exclusively breast fed, and all self-identified as black or African American. Patients ranged in age from 8 months to 3 years at the time of initial diagnosis. All seven patients were male. Five patients were presented in an office setting, while two were found to have nutritional rickets in an emergency setting. Patients demonstrated classic pathognomonic findings of rickets, including three patients with rachitic rosary. The aim of this case series is to emphasize the importance of surveillance and high clinical suspicion for nutritional rickets, particularly in children with darker skin complexions and who were exclusively breast-fed during infancy. Rapid diagnosis and intervention may delay or negate the need for orthopedic surgical intervention.

• The efficacy of weekly somatrogon injections was no different from that of daily somatropin injections to treat children who don't make enough growth hormone to grow adequately. ○ Efficacy refers to how well a drug works in a clinical trial. ○ Children treated with weekly somatrogon had an increased growth rate, similar to that of children treated with daily somatropin. • The safety of weekly somatrogon injections was similar to that of daily somatropin injections. The original scientific article on which this summary is based was published in The Journal of Clinical Endocrinology & Metabolism and can be accessed for free at: https://academic.oup.com/jcem/article/107/7/e2717/6566444. The details of the original article are as follows: Cheri L. Deal, Joel Steelman, Elpis Vlachopapadopoulou, Renata Stawerska, Lawrence A Silverman, Moshe Phillip, Ho-Seong Kim, CheolWoo Ko, Oleg Malievskiy, Jose F. Cara, Carl L. Roland, Carrie Turich Taylor, Srinivas Rao Valluri, Michael P. Wajnrajch, Aleksandra Pastrak, and Bradley S. Miller. Efficacy and safety of weekly somatrogon vs daily somatropin in children with growth hormone deficiency: a phase 3 study. J Clin Endocrinol Metab 2022; 107(7): e2717-e2728. The purpose of this plain language summary is to help you to understand the findings from recent research. • Somatrogon is used to treat growth hormone deficiency (the condition under study that is discussed in this summary). Approval varies by country; please check with your local healthcare provider for more details. • The results of this study may differ from those of other studies. Physicians/providers should make treatment decisions based on all available evidence and not on the results of a single study.

Purpose of ReviewPediatric use of yoga as an integrative medicine modality has increased in prevalence over the last several decades. In this article, we review the available evidence for yoga in pediatric gastrointestinal disorders.Recent FindingsEvidence supports that in many pediatric disorders of gut brain interaction (DGBI), including irritable bowel syndrome, functional abdominal pain and functional dyspepsia, yoga decreases pain intensity and frequency and increases school attendance. Yoga has been shown to improve health-related quality of life and improve stress management as an effective adjunct to standard medical therapy in pediatric inflammatory bowel disease (IBD). Further studies are needed regarding optimal frequency, duration of practice and evaluation of the impact on IBD disease activity measures.SummaryYoga may benefit pediatric gastroenterology patients with DGBIs and IBD through improving quality of life and reducing pain. Future yoga studies could investigate biomarkers and continued research will help integrate this modality into routine pediatric gastroenterology care.

Abstract Outcomes of acute lymphoblastic leukemia (ALL) are associated with both host and environmental factors. We hypothesized that there is also an interaction between them, with genetic variants influencing dietary choices. The dopamine transporter 1 (DAT1) and D4 subtype of the dopamine receptor (DRD4) genes are polymorphic, with a variable number of nucleotide repeats (VNTR) that affect dopaminergic neurotransmission, and reward circuitry for food cravings. Specifically, the 9-tandem repeats (9R) allele of DAT1 and the 7-tandem repeats (7R) allele of DRD4 are risk factors for eating disorders or undereating behavior in children. The goal of this study was to determine whether these polymorphisms are associated with altered dietary intake during therapy for childhood ALL. DNA was isolated from peripheral blood mononuclear cells collected from 439 children being treated on Dana Farber Cancer Institute ALL Consortium Protocol 05-001 (NCT00400946). Target alleles in DAT1 and DRD4 were determined using PCR product length analysis. Each subject was classified as having either zero or at least one copy of the target alleles. Dietary intake was previously determined using food frequency questionnaires collected at three timepoints: time of diagnosis, end of induction, and continuation therapy. As previously published dietary composition in fat and carbohydrate differed significantly among three timepoints. There was no significant difference between those with or without the target alleles in DAT1 or DRD4 in percent from fat or percent from carbohydrate at any of the three timepoints. However, significant differences were observed when dietary intake was compared to the Dietary Reference Intake (DRI). Notably, a greater portion of male participants with DAT1 9R polymorphism had fat consumption below the DRI at the time of diagnosis. On the contrary, significantly decreased portion of females with DAT1 9R had fat consumption below the DRI during continuation therapy. When compared to normative values, a significantly increased number of children had total calorie consumption below DRI during induction therapy. The DRD4 7R polymorphism was associated with fat intake below DRI during induction therapy in males. However, neither DAT1 nor DRD4 polymorphisms were related to variation in carbohydrates consumption. In addition, a greater portion of participants that carry both DAT1 and DRD4 polymorphism exhibited a trend toward undereating behavior in fat intake compared to the controls. Our results are the first to indicate that DAT1 and DRD4 polymorphisms are associated with altered dietary intake during chemotherapy for childhood ALL. Future study will further elucidate whether this modulation is associated with changes in their body weight, BMI, glucose level, and disease relapse. Citation Format: Jing Wen, Arul Duggimpudi, Shengguo Li, Elena Ladas, Kara M. Kelly, Peter D. Cole. The effect of polymorphisms in DAT1 and DRD4 on dietary intake during chemotherapy for childhood leukemia. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 5216.

To determine if oropharyngeal therapy with mother's own milk (OPT-MOM) reduces late-onset sepsis (L-OS; primary outcome), NEC, death, length of stay, time to full enteral nutrition (FEN) and full oral feeds in preterm infants (BW < 1250 g). Infants (N = 220) were randomized to Group A (milk) or B (placebo) and received 0.2 mL every 2 h for 48 h, then every 3 h until 32 weeks CGA. There were no significant differences in L-OS, NEC or death. Group A trended towards an 8-day reduction in stay, 8-day reduction in time to FEN and a 6-day reduction in time to full oral feeds, compared to B. While clinically relevant, due to large variability in outcomes and lack of power, p values were > 0.05. OPT-MOM did not reduce L-OS, NEC or death. Group A trended towards a reduced stay and better nutritional outcomes, but results were not statistically significant. GOV: NCT02116699.

Research on the utilization and effectiveness of antitumor necrosis factor (TNF) biologics in children with very early onset inflammatory bowel disease (VEOIBD) is urgently needed. Here we describe anti-TNF use and durability in a multicenter cohort. We performed a retrospective cohort study of patients diagnosed with VEOIBD (<6 years) between 2008 and 2013 at 25 North American centers. We performed chart abstraction at diagnosis and 1, 3, and 5 years after diagnosis. We examined the rate of initiation and durability of infliximab and adalimumab and evaluated associations between treatment durability and the following covariates with multivariate Cox proportional hazard regression: age at diagnosis, sex, disease duration, disease classification, and presence of combined immunomodulatory treatment versus monotherapy. Of 294 children with VEOIBD, 120 initiated treatment with anti-TNF therapy and 101 had follow-up data recorded [50% Crohn disease (CD), 31% ulcerative colitis (UC), and 19% IBD unclassified (IBD-U)]. The cumulative probability of anti-TNF treatment was 15% at 1 year, 30% at 3 years, and 45% at 5 years from diagnosis; 56 (55%) were treated between 0 and 6 years old. Anti-TNF durability was 90% at 1 year, 75% at 3 years, and 55% at 5 years. The most common reason for discontinuation of anti-TNF were loss of response in 24 (57%) children. Children with UC/IBD-U had lower durability than those with CD (hazard ratio [HR] 0.17; 95% confidence interval [CI], 0.06-0.51; P = 0.001). Utilization and durability of anti-TNF in VEOIBD is relatively high and comparable with older children. Having Crohn disease (compared with UC/IBD-U) is associated with greater durability.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). “Cases of SCMR” is a case series hosted on the SCMR website (https://www.scmr.org) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. The COVID-19 Case Collection highlights the impact of coronavirus disease 2019 (COVID-19) on the heart as demonstrated on CMR. Each case in series consists of the clinical presentation and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2021 Cases of SCMR and the 2020 and 2021 COVID-19 Case Collection series of nine cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar literature search engine.