ASPEN international survey on ethical competencies dealing with decision-making in the use of artificially administered nutrition and hydration and competency application in clinical practice.

BACKGROUND: Ethical competencies dealing with decision-making for clinicians involved in artificially administered nutrition and hydration (AANH) have not been defined in the literature. Although clinical assessments identify nutrition needs and appropriate routes of nutrition administration, an assessment of the ethical, cultural, and spiritual implications of the medical nutrition therapy may be overlooked. METHODS: Eleven competency statements were developed by members of two international sections of the American Society for Parenteral and Enteral Nutrition. This descriptive cross-sectional survey study was conducted to measure the importance of the competency statements to the membership of two sections using a five-point Likert scale of 1-5 (1-low to 5-high). RESULTS: A total of 113 responses (12.5% response rate) were obtained predominantly from physicians and dietitians from 25 countries. There was a wide range of world regions of the 49% respondents outside of the United States. Means and SDs were calculated for agreement with the 11 competency statements with overall means ranging from 4.32 to 4.67. Most of the participants cared for adult/older adult patients (63.7%) exclusively, and 12.4% cared for pediatric/neonate patients exclusively; the remainder (23.9%) cared for both populations. Respondents reported they were either experienced, competent, or expert (88.6%) in dealing with ethical issues related to AANH. CONCLUSION: This international interdisciplinary group agreed that the integration of ethical, cultural, and spiritual competencies into clinical decision-making regarding artificially AANH is important.

Gut microbiota and intestinal rehabilitation: a prospective childhood cohort longitudinal study of short bowel syndrome (the MIRACLS study): study protocol.

INTRODUCTION: Short bowel syndrome (SBS) is the predominant cause of paediatric intestinal failure. Although life-saving, parenteral nutrition (PN) is linked to complications and may impact quality of life (QoL). Most children will experience intestinal rehabilitation (IR), but the mechanisms underpinning this remain to be understood. SBS is characterised by abnormal microbiome patterns, which might serve as predictive indicators for IR. We aim to characterise the microbiome profiles of children with SBS during IR, concurrently exploring how parental perspectives of QoL relate to IR. METHODS AND ANALYSIS: This study will enrol a minimum of 20 paediatric patients with SBS (0-18 years). Clinical data and biological samples will be collected over a 2-year study period. We will apply 16S rRNA gene sequencing to analyse the microbiome from faecal and gut tissue samples, with additional shotgun metagenomic sequencing specifically on samples obtained around the time of IR. Gas chromatography with flame ionisation detection will profile faecal short-chain fatty acids. Plasma citrulline and urinary intestinal fatty acid binding proteins will be measured annually. We will explore microbiome-clinical covariate interactions. Furthermore, we plan to assess parental perspectives on QoL during PN and post-IR by inviting parents to complete the Paediatric Quality of Life questionnaire at recruitment and after the completion of IR. ETHICS AND DISSEMINATION: Ethical approval was obtained from the East Midlands-Nottingham 2 Research Ethics Committee (22/EM/0233; 28 November 2022). Recruitment began in February 2023. Outcomes of the study will be published in peer-reviewed scientific journals and presented at scientific meetings. A lay summary of the results will be made available to participants and the public. TRIAL REGISTRATION NUMBER: ISRCTN90620576.

Global availability of parenteral nutrition: Pre- and post-COVID-19 pandemic surveys.

OBJECTIVE: Parenteral nutrition represents a therapeutic option for patients with type 3 intestinal failure. If used exclusively, parenteral nutrition has to be complete to provide all essential nutrients. The aim was to assess the availability of parenteral nutrition in all parts of the world, to better comprehend the global situation, and to prepare an action plan to increase access to parenteral nutrition. METHODS: An international survey using an electronic questionnaire was conducted in August 2019 and repeated in May 2022. An electronic questionnaire was sent to 52 members or affiliates of the International Clinical Nutrition Section of the American Society for Parenteral and Enteral Nutrition. Questions addressed the availability of parenteral nutrition admixtures and their components, reimbursement, and prescribing pre- and post-COVID-19 pandemic. All participating countries were categorized by their economic status. RESULTS: Thirty-six country representatives responded, answering all questions. Parenteral nutrition was available in all countries (100%), but in four countries (11.1%) three-chamber bags were the only option, and in six countries a multibottle system was still used. Liver-sparing amino acids were available in 18 (50%), kidney-sparing in eight (22.2%), and electrolyte-free in 11 (30.5%) countries (30.5%). In most countries (n = 28; 79.4%), fat-soluble and water-soluble vitamins were available. Trace elements solutions were unavailable in four (11.1%) countries. Parenteral nutrition was reimbursed in most countries (n = 33; 91.6%). No significant problems due to the coronavirus pandemic were reported. CONCLUSIONS: Despite the apparent high availability of parenteral nutrition worldwide, there are some factors that may have a substantial effect on the quality of parenteral nutrition admixtures. These shortages create an environment of inequality.

Small and large bowel anatomy is associated with enteral autonomy in infants with short bowel syndrome: A retrospective cohort study.

BACKGROUND: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA. METHODS: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015. The cohort was stratified based on the achievement of EA. Statistical testing was completed using t-test, chi-square, Cox proportional hazards regression model, and Kaplan-Meier analysis. RESULTS: EA was achieved in 229 patients. In the multivariable analysis, the percentage of residual LB (hazard ratio [HR] = 1.02; 95% CI = 1.01-1.02) and SB (HR = 1.01; 95% CI = 1.01-1.02) length, presence of the ileocecal valve (HR = 2.02; 95% CI=1.41-2.88), and not coming from a high-volume transplantation center (HR = 2.42; 95% CI = 1.68-3.49) were positively associated with EA, whereas a negative association was seen with the presence of stoma at the time when shortest remnant was documented (HR = 0.72; 95% CI = 0.52-1.00). EA achievement was significantly different between the anatomical subgroups (log-rank test P < 0.001) with an EA rate of 80.4% in infants with ≥50% SB and LB (median time 209 days); 62.5% with ≥50% SB and <50% LB (397 days); 58.3% with <50% SB and ≥50% LB (1192 days), and 25.9% with <50% SB and LB. Necrotizing enterocolitis (NEC) was not associated with a better achievement of EA (NEC vs other etiologies: log-rank test P = 0.33). CONCLUSIONS: Overall, 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. A colon length of >50% can compensate for the loss of small bowel (<50%) and account for similar EA rates as those in children with residual SB > 50%.

Factors influencing enteral autonomy after autologous gastrointestinal reconstructive surgery: A two-centre UK perspective.

BACKGROUND AND AIMS: The implementation of multidisciplinary care and improvements in parenteral nutrition (PN) in patients with short bowel syndrome (SBS) have led to better outcomes and higher survivability. Autologous gastrointestinal reconstructive (AGIR) surgery can reduce the duration on PN and lead to earlier enteral autonomy (EA). Our aim was to investigate the effect of SBS aetiology and other predictors on the achievement of enteral autonomy following AGIR surgery. METHODS: Retrospective review of all patients undergoing AGIR surgery in two tertiary paediatric surgical units, between 2010 and 2021. Continuous data is presented as median (range). RESULTS: Twenty-seven patients underwent 29 AGIR procedures (20 serial transverse enteroplasties (STEP), 9 longitudinal intestinal lengthening and tailoring (LILT)) at an age of 6.6 months (1.5 - 104.5). EA rate was 44% at 13.6 months after surgery (1 - 32.8). AGIR procedures achieved an increase in small bowel length of 70% (pre-operative 46.5 vs 77 cm, p = 0.003). No difference was found between STEP and LILT (p = 0.84). Percentage of expected small bowel length (based on the child's weight) was a strong predictor of EA (bowel length >15% - EA 80% vs bowel length ≤15% - EA 17%, p = 0.008). A diagnosis of gastroschisis showed a negative non-significant correlation with the ability to achieve EA (25% vs 60%, p = 0.12). Overall survival rate was 96%. CONCLUSION: AGIR surgery is an important tool in the multidisciplinary management of children with SBS. Percentage of expected small length and aetiology of SBS are likely predictors of achievement of EA in patients undergoing AGIR surgery. LEVEL OF EVIDENCE: IV: Retrospective Case-Series.

An international multicenter validation study of the Toronto listing criteria for pediatric intestinal transplantation.

Deciding which patients would benefit from intestinal transplantation (IT) remains an ethical/clinical dilemma. New criteria* were proposed in 2015: ≥2 intensive care unit (ICU) admissions, loss of ≥3 central venous catheter (CVC) sites, and persistently elevated conjugated bilirubin (CB ≥ 75 µmol/L) despite 6 weeks of lipid modification strategies. We performed a retrospective, international, multicenter validation study of 443 children (61% male, median gestational age 34 weeks [IQR 29-37]), diagnosed with IF between 2010 and 2015. Primary outcome measure was death or IT. Sensitivity, specificity, NPV, PPV, and probability of death/transplant (OR, 95% confidence intervals) were calculated for each criterion. Median age at IF diagnosis was 0.1 years (IQR 0.03-0.14) with median follow-up of 3.8 years (IQR 2.3-5.3). Forty of 443 (9%) patients died, 53 of 443 (12%) were transplanted; 11 died posttransplant. The validated criteria had a high predictive value of death/IT; ≥2 ICU admissions (p < .0001, OR 10.2, 95% CI 4.0-25.6), persistent CB ≥ 75 µmol/L (p < .0001, OR 8.2, 95% CI 4.8-13.9). and loss of ≥3 CVC sites (p = .0003, OR 5.7, 95% CI 2.2-14.7). This large, multicenter, international study in a contemporary cohort confirms the validity of the Toronto criteria. These validated criteria should guide listing decisions in pediatric IT.

Fat soluble vitamin status in children on home parenteral nutrition in a tertiary paediatric intestinal rehabilitation unit.

BACKGROUNDS & AIMS: Children with intestinal failure on home parenteral nutrition are at risk of fat malabsorption and fat soluble vitamin deficiency. Fish oil containing mixed lipid emulsions (SMOFlipid®) with higher vitamin E content, have a theoretical risk of exceeding current recommendations for vitamin E dosing and, may influence other fat soluble vitamin status in these children. The aim of this study was to assess for fat soluble vitamin status in children on long-term home parental nutrition receiving a mixed lipid emulsion (SMOFlipid®) compared with those receiving traditional soy or soy/olive oil based (non- SMOFlipid®) lipid emulsions and whether this is influenced by the underlying cause of intestinal failure. METHODS: Retrospective longitudinal study in a tertiary referral paediatric hospital of children on home parental nutrition during the period January 2000 to June 2019. Data was retrieved using medical and pharmacy records, laboratory database, and summarised using inferential statistics. RESULTS: 111 patients (n = 58 female) received home parental nutrition in 121 discrete episodes (range 45-5329 days). N = 61 (55%) were diagnosed with anatomical short bowel syndrome, of which necrotising enterocolitis was the most common cause (n = 14). SMOFlipid® was used exclusively in n = 79 patients, non-SMOFlipid® exclusively in n = 19, and n = 13 changed from non-SMOFlipid® to SMOFlipid® during the study period. The median vitamin E level and vitamin E:lipid ratio were significantly higher for patients on SMOFlipid® compared to non-SMOFlipid® (27.9 vs 18.3 µmol/L respectively, p < 0.001; 7.10 vs 4.00 µmol/mmol; p < 0.001). Median vitamin A level was comparable (1.19 vs 1.12 µmol/L, p = 0.241), while median vitamin D level was significantly lower in the non-SMOFlipid® group consistent with mild deficiency (63.7 vs 43.0 nmol/L, p < 0.001). CONCLUSION: The use of SMOFlipid® correlated with higher Vitamin E level in paediatric home parental nutrition patients. Lower vitamin D level appears to correlate with the use of non-SMOFlipid®. A larger prospective cohort is required to delineate any clinical significance from these findings.

Trends in Pediatric Intestinal Failure: A Multicenter, Multinational Study.

OBJECTIVES: To assess the natural history and outcomes of children with intestinal failure in a large, multicenter, geographically diverse contemporary cohort (2010-2015) from 6 pediatric intestinal failure programs. STUDY DESIGN: Retrospective analysis of a multicenter intestinal failure cohort (n = 443). Competing-risk analysis was used to obtain cumulative incidence rates for the primary outcome (enteral autonomy, transplantation, or death). The χ2 test and Cox proportional hazard regression were used for bivariate and multivariable analyses. RESULTS: The study cohort comprised 443 patients (61.2% male). Primary etiologies included short bowel syndrome (SBS), 84.9%; dysmotility disorder, 7.2%; and mucosal enteropathy, 7.9%. Cumulative incidences for enteral autonomy, transplantation, and death at 6 years of follow-up were 53.0%, 16.7%, and 10.5%, respectively. Enteral autonomy was associated with SBS, ≥50% of small bowel length, presence of an ileocecal valve (ICV), absence of portal hypertension, and follow-up in a non-high-volume transplantation center. The composite outcome of transplantation/death was associated with persistent advanced cholestasis and hypoalbuminemia; age <1 year at diagnosis, ICV, and intact colon were protective. CONCLUSIONS: The rates of death and transplantation in children with intestinal failure have decreased; however, the number of children achieving enteral autonomy has not changed significantly, and a larger proportion of patients remain parenteral nutrition dependent. New strategies to achieve enteral autonomy are needed to improve patient outcomes.

Ethical Aspects of Artificially Administered Nutrition and Hydration: An ASPEN Position Paper.

The American Society for Parenteral and Enteral Nutrition (ASPEN) Position Paper focus is on applying the 4 ethical principles for clinician's decision-making in the use of artificially administered nutrition and hydration (AANH) for adult and pediatric patients. These basic principles are (1) autonomy, respect the patient's healthcare preferences; (2) beneficence, provide healthcare in the best interest of the patient; (3) nonmaleficence, do no harm; and (4) justice, provide all individuals a fair and appropriate distribution of healthcare resources. Preventing and resolving ethical dilemmas is addressed, with an emphasis on a collaborative, interdisciplinary approach. Optimizing early communication and promoting advance care planning, involving completion of an advance directive, including designation of a surrogate decision-maker, are encouraged. Clinicians achieve respect for autonomy when they incorporate the patient, family, community, country, geographical, and presumed cultural values and religious belief considerations into ethical decision-making for adults and children with a shared decision-making process. These discussions should be guided by the 4 ethical principles. Hospital committees and teams, limited-time trials, clinician obligation with conflicts, and forgoing of AANH are addressed. Specific patient conditions are addressed because of the concern for potential ethical issues: coma, decreased consciousness, and dementia; advanced dementia; cancer; eating disorders; and end-stage disease/terminal illness. Incorporated in the Position Paper are ethical decisions during a pandemic and a legal summary involving ethical issues. International authors presented the similarities and differences within their own country or region and compared them with the US perspective.

Parenteral Provision of Micronutrients to Pediatric Patients: An International Expert Consensus Paper.

INTRODUCTION: Micronutrients (vitamins and trace elements) are essential to all nutrition. For children and neonates who are dependent upon nutrition support therapies for growth and development, the prescribed regimen must supply all essential components. This paper aims to facilitate interpretation of existing clinical guidelines into practical approaches for the provision of micronutrients in pediatric parenteral nutrition. METHODS: An international, interdisciplinary expert panel was convened to review recent evidence-based guidelines and published literature to develop consensus-based recommendations on practical micronutrient provision in pediatric parenteral nutrition. RESULTS: The guidelines and evidence have been interpreted as answers to 10 commonly asked questions around the practical principles for provision and monitoring of micronutrients in pediatric patients. CONCLUSION: Micronutrients are an essential part of all parenteral nutrition and should be included in the pediatric nutrition therapy care plan.

Outcomes of Children With Chronic Intestinal Failure: Experience Over 2 Decades at a Tertiary Paediatric Hospital.

BACKGROUND AND AIMS: The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. METHODS: Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011. RESULTS: A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30-80) or 23.9% of the expected length for age (interquartile range 17.0%-40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2-29.2) with intestinal failure-associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients with chronic intestinal pseudo-obstruction syndrome and 2 of 4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both P < 0.001). CONCLUSIONS: Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

Intestinal Failure in Children.

Children with intestinal failure have had improved survival, particularly those with extreme short bowel syndrome, over the last 10-15 y. This has been attributed to better understanding of the pathophysiology of intestinal failure, improvement in line care, recognition of the importance of a team approach as well as the progress of intestinal transplant as a viable option. Parenteral nutrition remains the cornerstone for the continual survival of these patients. This review will cover contemporary approaches to intestinal failure including post surgical approaches, non-transplant surgery, dietetic and medication approaches during the adaptation process, considerations for home parenteral nutrition and latest in intestinal transplantation.

Parenteral trace elements in children: clinical aspects and dosage recommendations.

PURPOSE OF REVIEW: Since the 1970s, it has been known that the supplementation of trace elements with parenteral nutrition is required in order to avoid the clinical manifestations of their deficiencies. However, the correct level of requirements of these trace elements, particularly in paediatrics, has provided some debate. The recent developments might help revise some of the current recommendations, particularly in short-term parenteral nutrition provision. RECENT FINDINGS: Parenterally fed preterm neonates require routine addition of zinc. Provision of chromium and manganese in parenteral nutrition should be limited, particularly for short-term patients. Newer parenteral iron preparations provide the opportunity for a safer and larger dose of administration. SUMMARY: Clinicians should prescribe according to the individual requirements and revise the routine practice of providing 'all in one' parenteral trace elements preparations, as these products do not reflect or allow tailored provision in paediatrics and may increase the risk of toxicity.

A prospective study of catheter-related complications in HPN patients.

BACKGROUND & AIMS: Central venous access device (CVAD)-related complications such as catheter-related sepsis, occlusion and breakage contribute to both mortality and morbidity in home parenteral nutrition (HPN) patients. Prospectively collected data in these patients are scarce. METHODS: Data on all CVAD-related complications in four tertiary referral centres in Australia and New Zealand were prospectively collected between 01/01/2009 and 31/12/2009 together with costs of each episode that required admission. RESULTS: Of 53 patients (42 adults and 11 children), 27 suffered a total of 49 episodes of CVAD-associated complications (line infection 36, catheter blockage 5, line fracture 1, line migration 7), giving an incidence of 3.6 per 1000 CVAD days (11.6 per 1000 in patients with a multi-use CVAD). Thirty seven episodes resulted in hospital admission for a median duration of 8 days (range 1-29). Responsible microbes were mainly enteric with klebsiella being the most commonly isolated organism (10 episodes). The average cost of care per episode of CVAD-associated complication requiring inpatient admission was $A9,710 (€6480 approximately). CONCLUSION: CVAD complications, in particular line infection are still a major source of potentially avoidable HPN morbidity and mortality with a high cost to the healthcare system. The predominance of enteric organisms in our series raises the possibility of bacterial translocation as a significant component of the pathogenesis of line sepsis in HPN patients.

Central venous catheter thrombosis associated with 70% ethanol locks in pediatric intestinal failure patients on home parenteral nutrition: a case series.

Central venous catheter (CVC) ethanol locks may reduce catheter-related bloodstream infection (CRBSI). Four children with intestinal failure on home parenteral nutrition (HPN) were selected for 70% ethanol locks because of their high rate of CRBSI. The 70% ethanol locks were instilled at a volume equal to the estimated internal volume of the CVC. Two children (aged 4 and 11 years) received 70% ethanol locks as CRBSI prophylaxis; another 2 children (aged 10 and 11 years) received 70% ethanol locks as adjunctive treatment for CRBSI. All 4 children developed either visible thrombosis in the CVC or CVC occlusion. To the authors' knowledge, this is the first report of CVC thrombosis associated with ethanol lock therapy in the pediatric HPN population. Although none of the CVCs were removed due to occlusion, these events raise serious concerns about the use of high-concentration ethanol locks.

Chronic prenatal nicotine exposure alters enkephalin mRNA regulation in the perinatal rat adrenal medulla.

Prenatal exposure to nicotine significantly increases enkephalin mRNA levels in the rat adrenal medulla prenatally, and postnatally the normal up-regulation is obliterated. This may lead to a disturbed modulation or regulation of catecholamine release in the adrenal and may be one factor contributing to the attenuated capacity of nicotine-treated pups to survive severe hypoxia. We speculate that this may be part of the mechanism underlying the relation between maternal smoking and sudden infant death syndrome.