Pediatric Chronic Intestinal Failure: Something Moving?

Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient's intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords "intestinal failure", "parenteral nutrition" and "pediatric". Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use.

Usefulness of central venous catheter replacement with a guidewire in patients with intestinal failure: a single-center study.

PURPOSE: Patients with intestinal failure (IF) require long-term parenteral nutrition using central venous catheters (CVCs), which often require replacement. We adopted a less fallible guidewire replacement (GWR) method and verified its effectiveness and validity. METHODS: We enrolled 108 cases that underwent a CVC replacement with "GWR" method with IF at our department between 2013 and 2023. We retrospectively reviewed patients' clinical details with tunneled CVC (Hickman/Broviac catheter). For the analysis, we compared for the same time period the catheter exchange method "Primary placement"; newly inserted catheter by venipuncture. RESULTS: The success rate of catheter replacement using GWR was 94.4%. There were six unsuccessful cases. A log-rank test showed no significant difference in catheter survival between primary placement and the GWR, and the time to first infection was significantly longer in the GWR (p = 0.001). Furthermore, no significant differences were observed between the two methods until the first infection, when the exchange indication was limited to infections. In the same way, when the indication was restricted to catheter-related bloodstream infection, there was no significant difference in catheter survival between the two approaches. CONCLUSION: Our GWR procedure was easy to perform and stable, with a high success rate and almost no complications. Moreover, using a guidewire did not increase the frequency of catheter replacement and the infection rate.

Current Status of Chronic Intestinal Failure Management in Adults.

BACKGROUND: Chronic intestinal failure (CIF) is a heterogeneous disease that affects pediatric and adult populations worldwide and requires complex multidisciplinary management. In recent years, many advances in intravenous supplementation support, surgical techniques, pharmacological management, and intestinal transplants have been published. Based on these advances, international societies have published multiple recommendations and guidelines for the management of these patients. The purpose of this paper is to show the differences that currently exist between the recommendations (ideal life) and the experiences published by different programs around the world. METHODS: A review of the literature in PubMed from 1980 to 2024 was carried out using the following terms: intestinal failure, CIF, home parenteral nutrition, short bowel syndrome, chronic intestinal pseudo-obstruction, intestinal transplant, enterohormones, and glucagon-like peptide-2. CONCLUSIONS: There is a difference between what is recommended in the guidelines and consensus and what is applied in real life. Most of the world's countries are not able to offer all of the steps needed to treat this pathology. The development of cooperative networks between countries is necessary to ensure access to comprehensive treatment for most patients on all continents, but especially in low-income countries.

Transition from pediatric to adult care in patients with chronic intestinal failure on home parenteral nutrition: How to do it right?

BACKGROUND: Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. AIM: To create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. METHODS: A survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. RESULTS: A total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient's transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1-2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. CONCLUSION: This study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient's chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed.

Bioengineering of Intestinal Grafts.

Intestinal failure manifests as an impaired capacity of the intestine to sufficiently absorb vital nutrients and electrolytes essential for growth and well-being in pediatric and adult populations. Although parenteral nutrition remains the mainstay therapeutic approach, the pursuit of a definitive and curative strategy, such as regenerative medicine, is imperative. Substantial advancements in the field of engineered intestinal tissues present a promising avenue for addressing intestinal failure; nevertheless, extensive research is still necessary for effective translation from experimental benchwork to clinical bedside applications.

Avoiding the use of long-term parenteral support in patients without intestinal failure: A position paper from the European Society of Clinical Nutrition & Metabolism, the European Society of Neurogastroenterology and Motility and the Rome Foundation for Disorders of Gut-Brain Interaction.

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.

Avoiding the use of long-term parenteral support in patients without intestinal failure: A position paper from the European Society of Clinical Nutrition & Metabolism, the European Society of Neurogastroenterology and Motility and the Rome Foundation for Disorders of Gut-Brain Interaction.

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.

Association between chronic intestinal failure etiology and eGFR trajectory in adults receiving home parenteral nutrition: A retrospective longitudinal cohort study.

BACKGROUND: Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF. METHODS: This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow-up on December 31, 2021. RESULTS: There were 357 patients included in the analysis. Median follow-up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m2 to 86.30 ml/min/1.73 m2 at the first year of follow-up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow-up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD. CONCLUSION: This is the largest study of renal function in patients receiving long-term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes.

Body composition of adults with chronic intestinal failure receiving home parenteral nutrition: A descriptive cohort study.

BACKGROUND: Chronic intestinal failure (CIF) refers to the long-lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF. METHODS: This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat-free mass index (FFMI), and fat percentage (fat%). RESULTS: Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25-68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m2, FFMI was 14.2 (1.9) kg/m2 in females and 17.0 (2.0) kg/m2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%. CONCLUSION: This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in-depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function.

Funding and resource availability for home parenteral nutrition in Australia: A national cross-sectional survey.

BACKGROUND: Home parenteral nutrition (HPN) is a life-saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally. METHODS: This was a cross-sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges. RESULTS: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6-25) patients vs 16 (IQR: 9-17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0-12 min) in adult centers and 14 min (IQR: 10-21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics. CONCLUSION: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework.

Parenteral fish oil lipid emulsion use in adults: a case series and review from an intestinal failure referral center.

BACKGROUND: Intestinal failure-associated liver disease (IFALD) is a complication of long-term PN use, attributed to the use of ω-6 injectable lipid emulsions (ILE). Fish oil (FO) ILE have been successful in reversing liver injury in neonates. Evidence for pure FO ILE use in adult patients is limited. METHODS: Case series of the use of FO lipid emulsions in adults with IFALD from the University of Chicago PN registry. Analysis of medical charts and PN formulations was performed. RESULTS: Three cases of IFALD treated with FO ILE were identified. The first case was a 30-year-old man with short bowel syndrome (SBS), hyperbilirubinemia, and biopsy-proven IFALD. Following a change from a soy lipid emulsion to FO lipid emulsion, his liver tests rapidly improved and remained stable over 202 weeks of use. The second case was a 76-year-old woman with intestinal failure (IF) due to a frozen bowel. A change from a soy ILE to a composite lipid and later to a pure FO ILE did not result in improvement in her liver tests. The third case was a 28-year-old man with SBS and biopsy-proven IFALD. Change to a composite ILE and subsequently FO lipid emulsion resulted in a gradual improvement in liver tests. No clinical essential fatty acid (EFA) deficiencies were identified during treatment. CONCLUSION: FO ILE may be effective in the treatment of adult patients with cholestatic IFALD. Use is safe with no EFA deficiencies detected in up to 4 years of use.

Approximately 50% of acute intestinal failure (AIF) patients on short-term parenteral nutrition (PN) have intestinal failure-associated liver disease (IFALD) without effect on hospital length of stay and mortality.

INTRODUCTION: Patients with intestinal failure (IF) are often dependent on PN for provision of calories and nutrients for survival. Similar to chronic intestinal failure (CIF) patients, those who have AIF are also at risk of IFALD, which is a poorly understood but potentially fatal condition. The local incidence of IFALD amongst AIF patients is not known. OBJECTIVES: The primary objective of this study was to determine the incidence of IFALD in AIF patients on short-term PN. Secondary objectives were to analyse patient and PN risk factors of IFALD, and clinical outcomes of length of stay (LOS) and inpatient mortality. DESIGN: This was a retrospective cross-sectional cohort study of hospitalised adult patients with AIF prescribed with short-term PN. All adult patients aged 21 years and above who received PN for at least 5 consecutive days and had normal liver function tests (LFTs) at the time of PN initiation were included in this study. RESULTS: A total of 171 patients were enrolled in this study, with 77 (45%) having deranged LFTs at the end of PN therapy and categorised under the IFLAD group. The patient cohort was predominantly male (92 [54%]) and had a median age of 68 years (IQR 59-76). Patients with IFALD at the end of PN therapy had higher diabetes prevalence (36% vs 26%, p = 0.2) and were on PN for a longer duration (median [IQR]: 12 [8-17] vs 8 [6-15] days, p = 0.003) than those without IFALD. There were no significant differences in patient and PN characteristics between the IFLAD and non-IFALD group. The multivariable models showed that the IFALD cohort had longer hospital stays (HR 0.90, 95% CI 0.65-1.23) and lower odds of inpatient death (OR 0.75, 95% CI 0.12-4.60), though both findings are not statistically significant (p = 0.5, 0.7). CONCLUSION: In this study, IFALD is a common phenomenon in AIF and the incidence was found to be an estimated 50% amongst patients on short-term PN with similar clinical outcomes between the two groups.

Pediatric Intestinal Failure Associated Eating Disorder: An Overview of the Importance of Oral Feeding in a Population at Risk for Feeding Difficulties.

Achieving feeding skills and food acceptance is a multi-layered process. In pediatric intestinal failure (PIF), oral feeding is important for feeding skills development, physiologic adaptation, quality of life and the prevention of eating disorders. In PIF, risk factors for feeding difficulties are common and early data suggests that feeding difficulties are prevalent. There is a unique paradigm for the feeding challenges in PIF. Conventional definitions of eating disorders have limited application in this context. A pediatric intestinal failure associated eating disorder (IFAED) definition that includes feeding/eating skills dysfunction, psychosocial dysfunction, and the influence on weaning nutrition support is proposed.

Indications for Intestinal Transplantation.

Outcomes for patients with chronic intestinal failure have improved with organization of experts into multidisciplinary teams delivering care in intestinal rehabilitation programs. There have been improvements in understanding of intestinal failure complications as well as development of newer therapies that have amplified the improvements in survival. In spite of this encouraging trend, patients who fail PN are often referred too late for intestinal transplantation. The author proposes a more rational framework that might allow earlier identification of intestinal failure patients at risk for PN-failure, who could appropriately be considered earlier for intestinal transplantation with improvements in overall outcomes.

Strategies to Promote Success in Oral Feedings in Infants and Children with Intestinal Failure due to Short Bowel Syndrome.

Infants and children with intestinal failure are at risk for pediatric feeding disorders, which challenge their oral feeding development. This article explores these challenges and offers several practical strategies that can be used by multidisciplinary care teams and at-home caregivers to help support the development of oral feeding in these children and eventually lead to their attaining enteral autonomy.

Life on the line - Incidence and management of central venous catheter complications in intestinal failure.

BACKGROUND & AIMS: Loss of venous access is threatening for patients with intestinal failure (IF) under long-term parenteral nutrition (PN). We aimed to identify the incidence of central venous catheter (CVC) complications, compare different devices, and analyze interventional recanalizing procedures to restore the patency of occluded CVCs. METHODS: For this retrospective cohort study, patient data from a prospective IF database spanning 16 years was analyzed at a tertiary referral center. Catheter dwell times (CDTs) were distinguished by Kaplan-Meier survival analysis and subgroup analyses were performed for different CVC types (tunneled/port catheters). Specific complications (occlusion, catheter-related infection (CRI), displacement, and material defect) were analyzed. Explantation rates and CDTs were compared. RESULTS: Overall, 193 CVCs in 77 patients with IF under PN could be enrolled (62.524 "CVC-days"). Broviac type "B" was found to be significantly superior to type "A" regarding occlusion, CRI, and material defects (log-rank test: p = 0.05; p = 0.026; p = 0.005 respectively). Port catheters were displaying the highest incidence of CRI (2.13 events/1000 catheter days). Interventional catheter recanalization was performed 91 times and significantly increased the CDT from a median of 131 days (IQR: 62; 258) to 389 days (IQR: 262; 731) (Mann-Whitney-U-test: p= <0.001) without increasing complications. CONCLUSIONS: Different complication rates and CDT were seen depending on CVC type. Tunneled catheters were significantly superior concerning CRI. Interventional catheter recanalization is a viable alternative to fibrinolytics to restore CVC patency, but long-term patency data is scarce.

Association between oral nutrition and inflammation after intestinal transplantation.

Intestinal transplantation (Itx) can be a life-saving treatment for certain patient populations, including those patients with intestinal failure (IF) who develop life-threatening complications due to the use of parenteral nutrition (PN). Most patients who have undergone Itx are eventually able to tolerate a full oral diet. However, little guidance or consensus exists regarding optimizing the specific components of an oral diet for Itx patients, including macronutrients, micronutrients and dietary patterns. While oral dietary prescriptions have moved to the forefront of primary and preventive care, this movement has yet to occur across the field of organ transplantation. Evidence to date points to the role of systemic chronic inflammation (SCI) in a wide variety of chronic diseases as well as post-transplant graft dysfunction. This review will discuss current trends in oral nutrition for Itx patients and also offer novel insights into nutritional management techniques that may help to decrease SCI and chronic disease risk as well as optimize graft function.

[Taurolidine lock in pediatric patients with intestinal failure. A practical guideline from the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP)]. / Sellado con taurolidina en el fracaso intestinal pediátrico. Guía práctica de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP).

Introduction: Objectives: the prevention of central line-associated bloodstream infections is a critical aspect of care for patients with intestinal failure who are treated with parenteral nutrition. The use of taurolidine in this context is becoming increasingly popular, however there is a lack of standardization in its pediatric application. The objective of this work is to develop a guide to support its prescription. Methodology: the guide is based on a review of the literature and expert opinions from the Intestinal Failure Group of the SEGHNP. It was developed through a survey distributed to all its members, addressing aspects of usual practice with this lock solution. Results: this manuscript presents general recommendations concerning taurolidine indications, commercial presentations, appropriate forms of administration, use in special situations, adverse reactions, and contraindications in the pediatric population Conclusions: taurolidine is emerging as the primary lock solution used to prevent central line-associated bloodstream infections, proving to be safe and effective. This guide aims to optimize and standardize its use in pediatrics.

Introducción: Objetivo: la prevención de las infecciones asociadas a catéter ocupa un papel fundamental en los cuidados del paciente en situación de fracaso intestinal en tratamiento con nutrición parenteral. El empleo del sellado del catéter con taurolidina con ese fin se ha generalizado sin que exista una estandarización sobre su uso en población pediátrica. El objetivo de este trabajo es elaborar una guía clínica que sirva de apoyo en su utilización. Métodos: la guía se basa en una revisión de la literatura y en la opinión de expertos del Grupo de Trabajo de Fracaso Intestinal de la SEGHNP recogida a través de una encuesta realizada a todos sus integrantes sobre aspectos de la práctica habitual con este sellado. Resultados: este manuscrito expone unas recomendaciones en cuanto a las indicaciones, presentaciones comerciales disponibles, forma adecuada de administración, uso en situaciones especiales, reacciones adversas y contraindicaciones de la taurolidina en población pediátrica. Conclusiones: el sellado con taurolidina para la prevención de la infección asociada a catéter venoso central se ha mostrado como un tratamiento eficaz y seguro. La presente guía pretender optimizar y homogeneizar su uso en pediatría.

The Anxiety Burden in Patients with Chronic Intestinal Failure on Long-Term Parenteral Nutrition and in Their Caregivers.

Background and aims: Home parenteral nutrition (HPN) is a life-saving treatment for patients affected by chronic intestinal failure (CIF). Both this clinical condition and its therapy require radical lifestyle modifications, affecting life quality and psychological balance in patients as well as family members. Patient psychological burden has rarely been taken into consideration, not to mention that of caregivers. This study aims to evaluate the levels of anxiety in CIF patients on HPN, and their caregivers, consequently determining their impact on the psychological and physical aspects. Methods: After a brief introductory interview, adult patients on HPN for CIF and their caregivers were asked to fill in the HAMA-A questionnaire. Results: Fifty patients and their respective caregivers were enrolled. Mean HAMA-A scores were similar in patients and caregivers and testified the presence of a mild to severe impact of CIF and HPN in both groups, with a significantly higher impact on female patients and caregivers. After adjusting age, education level, duration of CIF and HPN dependence, and degree of kinship, no differences were revealed in the scores. Conclusions: The study confirms that CIF patients on HPN and their caregivers have a significant anxiety burden independently from the duration of the disease, therefore needing appropriate support.

Catheter salvage or removal in catheter-related bloodstream infections with Staphylococcus aureus in children with chronic intestinal failure receiving home parenteral nutrition and the use of prophylactic taurolidine catheter lock solution: A descriptive cohort study.

BACKGROUND: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands. METHODS: We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality. RESULTS: A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI. CONCLUSION: Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center.