Composite lipid emulsion use and essential fatty acid deficiency in pediatric patients with intestinal failure with high parenteral nutrition dependence: A retrospective cohort study.

BACKGROUND: Reports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction. METHODS: We retrospectively evaluated patients, ages 0-17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE. Demographic data, PN composition, PN days, growth, and plasma fatty acid profile were collected. A plasma triene-tetraene (T:T) ratio >0.2 indicated EFAD. Summary statistics and Wilcoxon rank sum test evaluated to compare between PNDI category and ILE administration (grams/kilograms/day). P < 0.05 was considered significant. RESULTS: Twenty-six patients (median age, 4.1 years [interquartile range (IQR) = 2.4-9.6]) were included. The median duration of PN was 1367 days (IQR = 824-3195). Sixteen patients had a PNDI of 80%-120% (61.5%). Fat intake for the group was 1.7 g/kg/day (IQR = 1.3-2.0). The median T:T ratio was 0.1 (IQR = 0.1-0.2) with no values >0.2. Linoleic and arachidonic acid were low in 85% and 19% of patients, respectively; however, Mead acid was normal in all patients. CONCLUSION: This report is the largest to date on the EFA status of patients with IF on PN. These results suggest that, in the absence of lipid restriction, EFAD is not a concern when using MO ILEs in children receiving PN for IF.

Body Composition and Physical Activity in Pediatric Intestinal Failure On and Off Parenteral Nutrition.

OBJECTIVES: Data on the relationship between body composition (BC) and physical activity (PA) in children with intestinal failure (IF) are lacking. The objectives were to collect data on PA and BC in children with IF, both parenterally and enterally fed, and to assess the relationship between PA and BC. METHODS: Cross-sectional study in children 5-18 years with IF including those receiving parenteral nutrition (PN) and those fully enterally fed. PA levels were measured using accelerometry. BC was measured by dual-energy X-ray absorptiometry. Data were compared to age- and sex-matched population norms using t tests. Regression analysis assessed the relationship between BC and PA. RESULTS: Fifty-eight children with IF (38 males), mean (SD) age of 10.0 (3.5) years, 20 dependent on PN were included. Patients with IF had significantly fewer steps per day ( P ≤ 0.001) compared with literature controls, with a mean (SD) of 7,972 (3,008) and 11,749 (1,106), respectively. There were no significant differences between patients receiving PN and those enterally fed, but both groups were significantly less active than literature controls ( P < 0.001). Patients with IF had higher fat mass and lower fat-free mass compared to literature controls ( P = 0.008). PA had a significant effect on BC ( r2 = 0.32, P < 0.001). CONCLUSIONS: Children with IF, those receiving PN and those fully enterally fed, are at risk of decreased PA and altered BC. PA should be part of ongoing rehabilitation and management to optimize outcomes.

Assessment of body composition in pediatric intestinal failure: A comparison study.

BACKGROUND: The objective of the study was to compare bioelectrical impedance analysis (BIA) and skinfolds with dual energy x-ray absorptiometry (DXA) in the assessment of body composition of children with intestinal failure. DXA is the reference method for body composition assessment in clinical settings. METHODS: Children aged 1-18 years with intestinal failure whohave DXA as part of routine clinical monitoring were eligible. BIA measured total body water on the same day as DXA. Skinfold measurements were taken at four sites: triceps, biceps, subscapular, and suprailiac. Percentage of fat mass (%FM) and fat-free mass (%FFM) were derived from resistance and reactance measured by BIA by using age-specific equations. Percentage of FM was calculated from skinfold measures by using age-specific equations. Data on patient characteristics, intestinal failure-related factors, and feeding method were collected. Paired t test examined differences in %FM and %FFM and Bland-Altman analysis determined the agreement between BIA, skinfolds, and DXA. Marginal linear model assessed the effect of age, sex, and feeding method on the difference in body composition obtained between DXA and BIA and between DXA and skinfolds. RESULTS: Sixty-eight children with intestinal failure, mean age 8.9 ± 4.2 years, were studied. There was no difference between %FFM and %FM obtained by DXA and BIA (P = 0.26), with a mean bias (95% CI) of -0.69 (-1.9 to 0.5) for %FFM. Sex and age were individually and jointly associated with the bias observed between DXA and BIA (P < 0.05). Skinfold and DXA measurements were significantly different (P < 0.05). CONCLUSIONS: BIA is an acceptable clinical tool for assessing body composition in pediatric intestinal failure.

Evaluation and Management of Iron Deficiency in Children Undergoing Intestinal Rehabilitation-A Position Paper From the NASPGHAN Intestinal Rehabilitation Special Interest Group.

Iron deficiency (ID) is the most common nutritional deficiency affecting children undergoing intestinal rehabilitation (IR). Patients may be asymptomatic or present with nonspecific symptoms including fatigue, irritability, and dizziness. The diagnosis of ID in this population can be complicated by the coexistence of systemic inflammation or other nutritional deficiencies which may mimic ID. Many routinely available laboratory tests lack specificity and no consensus on screening is available. Success in oral and enteral treatment is impeded by poor tolerance of iron formulations in a population already challenged with intolerance. Newer parenteral iron formulations exhibit excellent safety profiles, but their role in repletion in this population remains unclear. The following report, compiled by a multidisciplinary group of providers caring for children undergoing IR and representing the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Special Interest Group for Intestinal Rehabilitation, seeks to address these challenges. After discussing iron physiology and population-specific pathophysiology, we make recommendations on iron intake, iron status assessment, and evaluation for alternative causes of anemia. We then provide recommendations on iron supplementation and treatment of ID anemia specific to this nutritionally vulnerable population.

Normal anthropometry does not equal normal body composition in pediatric intestinal failure.

BACKGROUND: Published reports on abnormal body composition in pediatric patients with intestinal failure have been in patients with poor growth. The goal of the current study is to report the body composition of normally growing patients with intestinal failure. METHODS: Children 8-18 years old with a dual-energy x-ray absorptiometry (DXA) between January 1, 2013, and July 15, 2018, were included in the study. Data were retrospectively collected from the medical charts and included demographics, residual bowel anatomy, nutrition support, height, and weight. DXA data, including total body less head bone mineral density (BMD), fat mass (FM), and fat-free mass (FFM), were collected and compared with published literature controls matched for age and sex. RESULTS: Thirty-four children met inclusion criteria. Mean age at the time of DXA was 9.6 ± 1.8 years. Weight- and height-for-age z-scores were -0.4 ± 0.9 and -0.5 ± 1.0, respectively. Mean BMD z-score was -1.0 ± 1.3. Twenty-six percent of patients (n = 9) had reduced BMD. Patients with intestinal failure had higher FM (P = .02) and lower FFM (P = .02) compared with controls. CONCLUSIONS: These data show that, despite reference range z-scores for height and weight, children with intestinal failure are at risk for abnormal body composition. Body composition should be routinely measured in children with intestinal failure to direct nutrition interventions.

Trace Elements' Contamination of Pediatric Parenteral Nutrition Solutions in Canada: A Cause for Concern.

BACKGROUND: Trace elements' (TEs) contamination of parenteral nutrition (PN) solutions is an ongoing concern. The aims of this study were 1) to measure actual TE concentrations in PN admixtures compared with ordered concentrations and 2) compare TE intake with current recommendations. METHODS: PN admixtures from discarded bags were collected from patients receiving home PN and on inpatient wards. Samples were collected from 72 patients (39 inpatients, 33 receiving home PN). Age, percentage energy intake from PN, and PN orders were collected from patients' charts. PN samples were analyzed for TEs, including chromium (Cr) and manganese (Mn), and concentration measurements compared with ordered concentrations and current recommendations. RESULTS: Measured Cr and Mn concentrations were higher than ordered concentrations: 5.3 ± 1.7 vs 2.8 ± 1.5 µg/L; P < 0.0001 and 11.9 ± 5.9 vs 0.00 µg/L; P < 0.0001, respectively. Chromium contamination alone accounted for over 100% of current recommendations for patients 0-12 months and between 63% and 92% for children >1 year. Contamination of Mn provided all the measured Mn in PN admixtures, since Mn is excluded from PN orders at our institution. Between 70% and 120% of current Mn recommendations were met from contamination. CONCLUSIONS: Cr should be excluded from PN admixtures for children 0-12 months and only one-fourth the current recommendation should be added for pediatric patients >1 year. Manganese should also be excluded from PN admixture for pediatric patients but plasms monitoring 2-3 times per year is recommended for those on long-term PN.

Implementing a Clinical Practice Change: Adopting the Nutrition Care Process.

The Nutrition Care Process (NCP), created by the Academy of Nutrition and Dietetics, provides a framework that encourages critical thinking and promotes uniform documentation by Registered Dietitians (RD). Additionally, it creates a link between the nutrition assessment, nutrition intervention, and the predicted or actual nutrition outcome. NCP has been integrated into a number of institutions in Canada and internationally. A committee of nonmanagement RDs at the Hospital for Sick Children led the Department of Clinical Dietetics in adopting the NCP. The committee developed and consecutively delivered a tailored education plan to 5 groups of RDs within the department. Additional resources were developed to complement the learning plan. The committee administered informal pre- and post-education surveys to measure outcomes. RDs reported receiving adequate training and felt confident implementing NCP into their practice. Adopting the NCP was well-received and RDs within the department continue to integrate it into their current practice.