Implementation and participation of an online nutrition curriculum for pediatric gastroenterology fellows.

Our team of nutrition experts developed an online nutrition curriculum consisting of 21 modules to serve as a resource for a stand-alone nutrition curriculum or as a supplement to existing nutrition electives during the Pediatric Gastroenterology fellowship. From April 2020 through January 2023, 2090 modules were completed by 436 fellows from 75 different programs across North America. The program was accessed most during tight restrictions on in-person learning during the COVID-19 pandemic. Overall, participants posttest scores improved from baseline pretest scores indicating retention of information from the modules. The overall success of this program suggests that there should be continued efforts to develop and offer online learning opportunities in clinical nutrition. There is an opportunity to expand the audience for the curriculum to include pediatric gastroenterologists from across the globe.

Pediatric-to-Adult Transfer of Care in Patients With Pancreas Disease: Recommendations for Care and Research Opportunities.

Young adults who have experienced recurrent acute pancreatitis and chronic pancreatitis as children or adolescents are vulnerable to poor follow-up and disease management during the transfer from the pediatric to adult healthcare system. Although formalized transition programs for young adults have been developed and described for other disease conditions, no such program has been described for young adults with pancreatic disease. This document is the first expert opinion outlining the important aspects of a transitional care and transfer program tailored to youth with recurrent acute and chronic pancreatitis. We emphasize the unique needs of these patients as they transfer to adult health care and the need for further research. The goal of improved transitional care and transfer is to enhance the services provided to adolescents/young adults with pancreatic disease in both healthcare settings and improve continuity of follow-up care.

Pancreatitis in the Complex Care Population: Presentation, Incidence, and Severity.

OBJECTIVES: To describe the incidence and presentation of pancreatitis in Children with Medical Complexity (CMC) while evaluating severity of disease and outlining risk factors. METHODS: This was a retrospective chart review between January 2010 and December 2019 of patients seen in the complex care clinic at Nationwide Children's Hospital (NCH) and Cincinnati Children's Hospital Medical Center (CCHMC). Data collected included sex, underlying diagnosis, family history of pancreatitis, type of pancreatitis, signs/symptoms, abdominal imaging, severity of attack, and presence of various risk factors associated with pancreatitis. Severity and diagnosis of pancreatitis was determined based on North American Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria. RESULTS: One hundred and twelve patients from both institutions were included, 62% from NCH, median age 11.5 [interquartile range (IQR): 5-16 years], 50% male. Most patients were less than 18 years of age with a median age of 8 years (IQR: 4-13 years). Underlying diagnoses included seizures (67%), cerebral palsy/spastic quadriplegia (65%), diabetes (3.6%), and mitochondrial disease (3%). Majority of patients were found to have multiple underlying diagnoses (88%). Incidence of pancreatitis for both institutions was 336 of 100,000 patients/year which is significantly higher than the general pediatric population ( P < 0.0001). Majority of first episodes of pancreatitis were mild (82%) with abdominal pain as the predominant symptom (50%). Adult patients were more likely to have pancreatitis related to medication use than pediatric patients (70% vs 38%, respectively P = 0.007). CONCLUSIONS: Individuals in the CMC population at our institutions have a high incidence of pancreatitis with unique risk factors compared to the general pediatric/young adult populations.

Should Clinicians Prescribe Non-FDA Regulated Dietary Supplements When Caring for Children With Hypovitaminosis D?

Hypovitaminosis D is a prevalent micronutrient deficiency that can be severe and hard to treat in children with short bowel syndrome, a condition treated with substantial bowel resection. Surgically altered bowel anatomy then results in iatrogenic digestion and absorption limitations that require short- and long-term management and follow-up. Care of children with hypovitaminosis D standardly includes prescription dietary micronutrient supplementation, sometimes in irregularly high doses. This commentary responds to a pediatric case of vitamin D toxicity and suggests micronutrient-prescribing risk mitigation strategies in light of the absence of regulatory oversight of over-the-counter dietary supplements, inadequate insurance coverage, and easily available commercial retail products.

The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations.

BACKGROUND: Body mass index (BMI) correlates with clinical outcomes in cystic fibrosis but has limitations.  Body composition aberrations in CF are multifactorial. We sought to evaluate body composition and relationships with pulmonary function, bone health, and hospital admissions. Other aims included defining body composition indices in a cohort of children with CF. METHODS: We conducted a retrospective review of patients with CF, age 8-18 years, seen at Nationwide Children's Hospital (2015-2020). Indices of body composition measured by dual-energy x-ray absorptiometry(DXA) scans.  Data included fat mass, fat-free mass (FFM), forced expiratory volume in one second (FEV1%), bone mineral density (BMD), and hospital admissions. NWA was defined as BMI 5th-85th percentile, body fat percentage >85th percentile. FFMD defined as FFMI <10th percentile. STATISTICS: T-tests compared NWA, FFMD and clinical measurements. Pearson correlations analyzed fat-free mass index (FFMI), fat mass index (FMI), BMI and clinical measurements. RESULTS: This study included 114 patients.  Mean age 12 years, 72 female. A high prevalence of FFMD existed (n=66, 38.6%).  FMI and FFMI correlated with FEV1% (r: 0.23, p:0.01, r: 0.36, p<0.001, respectively) and BMD (r: 0.29, p:0.002). FMI and hospital admissions were related (r:-0.23,p:0.01). FFMD was associated with 9.5% lower FEV1% (p=0.001) and lower BMD Z-score by 1.1 (p<0.001) when compared to no FFMD. CONCLUSION: This cohort of children with CF had a high prevalence of FFMD and low prevalence of NWA.  FFMD was associated with worsened clinical measurements. Patients with FFMD need additional exercise or nutritional intervention. Heterogeneity of body composition definitions creates need for more research.

Molecular classification of a complex structural rearrangement of the RB1 locus in an infant with sporadic, isolated, intracranial, sellar region retinoblastoma.

Retinoblastoma is a childhood cancer of the retina involving germline or somatic alterations of the RB Transcriptional Corepressor 1 gene, RB1. Rare cases of sellar-suprasellar region retinoblastoma without evidence of ocular or pineal tumors have been described. A nine-month-old male presented with a sellar-suprasellar region mass. Histopathology showed an embryonal tumor with focal Flexner-Wintersteiner-like rosettes and loss of retinoblastoma protein (RB1) expression by immunohistochemistry. DNA array-based methylation profiling confidently classified the tumor as pineoblastoma group A/intracranial retinoblastoma. The patient was subsequently enrolled on an institutional translational cancer research protocol and underwent comprehensive molecular profiling, including paired tumor/normal exome and genome sequencing and RNA-sequencing of the tumor. Additionally, Pacific Biosciences (PacBio) Single Molecule Real Time (SMRT) sequencing was performed from comparator normal and disease-involved tissue to resolve complex structural variations. RNA-sequencing revealed multiple fusions clustered within 13q14.1-q21.3, including a novel in-frame fusion of RB1-SIAH3 predicted to prematurely truncate the RB1 protein. SMRT sequencing revealed a complex structural rearrangement spanning 13q14.11-q31.3, including two somatic structural variants within intron 17 of RB1. These events corresponded to the RB1-SIAH3 fusion and a novel RB1 rearrangement expected to correlate with the complete absence of RB1 protein expression. Comprehensive molecular analysis, including DNA array-based methylation profiling and sequencing-based methodologies, were critical for classification and understanding the complex mechanism of RB1 inactivation in this diagnostically challenging tumor.

Promoting bone health in children and adolescents following solid organ transplantation.

Solid organ transplantation in children and adolescents provides many benefits through improving critical organ function, including better growth, development, cardiovascular status, and quality of life. Unfortunately, bone status may be adversely affected even when overall status is improving, due to issues with pre-existing bone disease as well as medications and nutritional challenges inherent post-transplantation. For all children and adolescents, bone status entering adulthood is a critical determinant of bone health through adulthood. The overall health and bone status of transplant recipients benefits from attention to regular physical activity, good nutrition, adequate calcium, phosphorous, magnesium and vitamin D intake and avoidance/minimization of soda, extra sodium, and obesity. Many immunosuppressive agents, especially glucocorticoids, can adversely affect bone function and development. Minimizing exposure to "bone-toxic" medications is an important part of promoting bone health in children post-transplantation. Existing guidelines detail how regular monitoring of bone status and biochemical markers can help detect bone abnormalities early and facilitate valuable bone-directed interventions. Attention to calcium and vitamin D supplementation, as well as tapering and withdrawing glucocorticoids as early as possible after transplant, can provide best bone outcomes for these children. Dual-energy X-ray absorptiometry can be useful to detect abnormal bone mass and fracture risk in this population and newer bone assessment methods are being evaluated in children at risk for poor bone outcomes. Newer bone therapies being explored in adults with transplants, particularly bisphosphonates and the RANKL inhibitor denosumab, may offer promise for children with low bone mass post-transplantation.

Variations in Nutrition Practices in Cystic Fibrosis: A Survey of the DIGEST Program.

BACKGROUND: Evidence-based management of gastrointestinal (GI) and nutrition manifestations of cystic fibrosis (CF) is limited, and practice variations have not been studied. METHODS: Thus, a survey was developed with the purpose of evaluating current nutrition practices of CF-focused gastroenterologists, specifically utilizing awardees and mentors of the Cystic Fibrosis Foundation (CFF) Developing Innovative GastroEnterology Specialty Training (DIGEST) Program. Topics included appetite stimulation, tube feeding (TF), and aspects of nutrition assessment, specifically urine sodium and essential fatty acid (EFA) status. RESULTS: The response rate was 61% (22/36). About half (55%; 12/22) of respondents had 5-10 years of experience in GI, and 23% (5/22) had >10 years. In regard to appetite stimulation, the majority used cyproheptadine; however, duration and pattern of prescribing varied. Variation was noted in TF management pertaining to tube placement, formula choice, and prescribing pancreatic enzyme replacement therapy with overnight TF. The majority did not check EFAs or urine sodium. Treatment for deficiencies in EFA or abnormal urine sodium was inconsistent. CONCLUSION: The survey reveals wide variation in management of some aspects of nutrition-related manifestations of CF among experienced providers. This reflects the need for research to provide evidence-based guidelines.

Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor: A Case Series.

Cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators have revolutionized care for individuals with cystic fibrosis (CF) with positive effects on the gastrointestinal (GI) tract. There is emerging evidence linking CFTR dysfunction to celiac disease (CD). We present 3 cases of patients with CF, genotype F508del/G551D, treated with CFTR modulator, ivacaftor, and diagnosed with CD. These patients tested for CD because they had persistent GI symptoms that had partially improved with ivacaftor. This case series highlights the importance of a better understanding of how CFTR modulators impact the GI tract, their possible link to CD, and the importance of considering CD when evaluating GI symptoms in individuals with CF.

Implementing Culinary Medicine Training: Collaboratively Learning the Way Forward.

There is a documented substantial gap between the level of nutrition education for medical trainees and the need to provide nutrition counseling. Culinary medicine offers a solution, but there are multiple barriers and no guides to implementation. This article identifies core components and strategies to overcome barriers on the basis of experiences of multiple institutions. The outline forms a foundation to be built upon by future collaborators to empower more widespread implementation of culinary medicine education and improve medical nutrition education and ultimately, patient outcomes.

Single High-dose Vitamin D3 Supplementation in Pediatric Patients With Inflammatory Bowel Disease and Hypovitaminosis D.

OBJECTIVES: The primary aim was to determine the effectiveness of a single high-dose of oral vitamin D3 (stoss therapy) in children with inflammatory bowel disease (IBD) and hypovitaminosis D. Our secondary aim was to examine the safety of stoss therapy. METHODS: We conducted a randomized, prospective study of 44 patients, ages 6 to 21 years, with IBD and 25-hydroxyvitamin D (25-OHD) concentrations <30 ng/mL. Patients were randomized to receive 50,000 IU of vitamin D3 once weekly for 6 weeks (standard of care, SOC group) or 300,000 IU once (stoss group). Serum 25-OHD levels were obtained at baseline, 4 and 12 weeks. Safety monitoring labs were performed at week 4. RESULTS: Thirty-nine of 44 enrolled patients (19 stoss, 20 SOC) completed the study. Baseline vitamin D levels were not significantly different between the groups. Stoss therapy resulted in a substantial rise in 25-OHD levels at week 4, equivalent to the weekly regimen (53.6 ±â€Š17.3 vs 54.6 ±â€Š17.5 ng/mL). At week 12, serum 25-OHD levels decreased in both groups, significantly lower in the stoss group, but remained close to 30 ng/mL (29.8 ±â€Š7.1 vs 40.4 ±â€Š11.9 ng/mL, P = 0.04). A significant interaction with treatment group over time was observed (P = 0.0003). At the week-4 time point, all patients who received stoss therapy had normal serum calcium and PTH levels. Eighty percentage of patients preferred stoss therapy to the weekly regimen. CONCLUSIONS: Stoss therapy was safe and effective in raising 25-OHD in children with IBD commensurate to that of the weekly regimen.

Development and Pilot Implementation of a Nutrition Curriculum and Rotation in Pediatric Gastroenterology Fellowships.

Structured nutrition rotations are rarely offered in pediatric gastroenterology fellowships. The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) Nutrition Committee developed a curriculum to serve as the basis for a rotation in clinical nutrition. We worked directly with 5 fellowship programs to tailor the experience to individual institutions. As part of our pilot study, fellows completed knowledge assessments and self-assessment of comfort level at the start and end of the experience. We saw a trend in improvement of comfort level and increase in mean score on knowledge assessments, but the differences did not meet statistical significance. Fellows who completed the rotation had an increase in comfort level in all topics with most dramatic increases in nutrition management of cystic fibrosis, refeeding syndrome, and cholestasis. Objective measures of nutrition knowledge attainment and use of programmatic feedback to continually improve the learners' experience will help expand the nutrition curriculum to a broader audience.

Unique Patterns of Body Composition and Anthropometric Measurements During Maturation in Neonatal Intensive Care Unit Neonates: Opportunities for Modifying Nutritional Therapy and Influencing Clinical Outcomes.

BACKGROUND: Body composition is an important predictor of long-term outcomes in neonates and may be altered by several factors. Innovative methods like air displacement plethysmography (ADP) can safely and reliably measure body composition, potentially assisting in individualization of nutrition therapy. OBJECTIVES: 1) To characterize patterns of body composition change in convalescing neonates in the neonatal intensive care unit (NICU) and examine factors leading to variation. 2) To evaluate if the time interval between 2 measurements via ADP can detect significant changes. METHODS: NICU infants underwent anthropometry and body composition measurements by ADP at 37.5±0.7 weeks (Time 1) and 41.0±0.7 weeks (Time 2) postmenstrual age. Nutrition data were recorded. Data were analyzed using paired t-tests and linear regression models, presented as mean±SE, median (IQR), or %. RESULTS: Twenty-two neonates (54% males, 32.2±0.9 weeks gestation) were evaluated with a median interval of 3.6 (2.9-4.0) weeks between studies. Mean weight and % body fat increased significantly. There was no significant difference between mean weight and mean % body fat compared with normal references. Abdominal girth increased and mid-arm circumference decreased (p<0.01). Preterm group had higher mean % body fat (10.1) than term infants (6.5), p = 0.03. CONCLUSION: Longitudinal assessment of body composition can effectively assess nutrition status of fragile NICU infants. Although, an interval of 2.9-4.0 weeks between consecutive measurements detected significant changes in body composition, more frequent measurements are needed to determine nutrition factors responsible for body composition changes.

Enteral Nutrition for Pediatric Crohn's Disease: An Underutilized Therapy.

Enteral nutrition (EN) for the treatment of Crohn's disease (CD) involves administration of a liquid nutrition product, administered orally or through tube feeding, while excluding typical dietary components. It is a safe and effective, but largely underused, therapy in the United States as a treatment for CD. EN is a particularly attractive option for pediatric CD as it avoids side effects of corticosteroids, improves growth, and may have a higher likelihood of achieving mucosal healing than some traditional medications. However, there are multiple real and perceived barriers to its use among providers. A comprehensive approach to addressing these barriers to EN may result in its increased use. This paper reviews the literature on the efficacy of EN, methods of utilization, and potential barriers and solutions to those barriers.

Using Quality Improvement to Increase Utilization of Enteral Therapy in Pediatric Crohn Disease: Results and Outcomes.

INTRODUCTION: Exclusive enteral nutrition (EEN) for induction of remission in children with Crohn disease (CD) is recommended as first-line therapy, but underutilized in the United States related to real and perceived barriers. We hypothesized that quality improvement (QI) methodology could increase use of EEN. METHODS: We developed, implemented, and revised an algorithm and a set of tools to facilitate use of EEN. Through a series of Plan Do Study Act cycles, the approach was modified to overcome provider and patient/family barriers. The primary outcome, the percentage of newly diagnosed CD patients who receive EEN per month between July 2013 and October 2015, assessed using statistical process control. Secondary outcomes, including the short pediatric Crohn disease activity index (sPCDAI), body mass index (BMI) z score, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), albumin, and hemoglobin were compared before and after EEN. RESULTS: Among patients newly diagnosed with CD, 73 patients initiated EEN and were included (mean age 12.7 ±â€Š2.9 years, 49% girls, 86% white). Rates of utilization of EEN increased significantly from a baseline of <5% to an average of approximately 50%. Of the 73 patients who started EEN, 37 (50%) completed a minimum of 8 weeks. Of those completing therapy, 25 (71%) achieved remission, with a significant reduction of sPCDAI (33.6 ±â€Š14.4 to 10.7 ±â€Š12.3, P < 0.0001) CONCLUSIONS:: Use of QI methodology to systematically implement tools designed to improve utilization was effective in increasing the use of EEN. Among those completing therapy, EEN was effective in inducing remission.

Enteral zinc supplementation and growth in extremely-low-birth-weight infants with chronic lung disease.

OBJECTIVE: Zinc deficiency causes growth deficits. Extremely-low-birth-weight (ELBW) infants with chronic lung disease (CLD), also known as bronchopulmonary dysplasia, experience growth failure and are at risk for zinc deficiency. We hypothesized that enteral zinc supplementation would increase weight gain and linear growth. METHODS: A cohort of infants was examined retrospectively at a single center between January 2008 and December 2011. CLD was defined as the need for oxygen at 36 weeks postmenstrual age. Zinc supplementation was started in infants who had poor weight gain. Infants' weight gain and linear growth were compared before and after zinc supplementation using the paired t test. RESULTS: A total of 52 ELBW infants with CLD met entry criteria. Mean birth weight was 682 ± 183 g, and gestational age was 25.3 ± 2 weeks. Zinc supplementation started at postmenstrual age 33 ± 2 weeks. Most infants received fortified human milk. Weight gain increased from 10.9 before supplementation to 19.9 g · kg(-1) · day(-1) after supplementation (P < 0.0001). Linear growth increased from 0.7 to 1.1 cm/week (P = 0.001). CONCLUSIONS: Zinc supplementation improved growth in ELBW infants with CLD receiving human milk. Further investigation is warranted to reevaluate zinc requirements, markers, and balance.