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.

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.