Requiring Home Parenteral Nutrition Research Articles

Hospital inpatient training for patients with intestinal failure who require home parenteral nutrition; the experience of a regional HPN centre in England

Hospital inpatient training for patients with intestinal failure who require home parenteral nutrition; the experience of a regional HPN centre in England

An Interprofessional Quality Improvement Project to Reduce Community-Acquired Central Line-Associated Bloodstream Infections in the Pediatric Intestinal Failure Population

Highlights Abstract Aim: Central line-associated bloodstream infections (CLABSIs) result in significant morbidity but are largely preventable. Methods to reduce hospital-acquired CLABSIs are well described, but few recommendations exist for how these practices might translate to patients with central lines in the ambulatory setting. This team sought to reduce the mean monthly community-acquired CLABSI rate by 25% from 11.7 to 8.8 per 1000 community line-days in children with intestinal failure who require home parenteral nutrition within 12 months. Methods: First, an interprofessional quality improvement team was formed. After baseline data collection, plan-do-study-act cycles began. Tests of change consisted of a central venous catheter (CVC) maintenance bundle. This bundle included a CVC care tote with additional supplies, improved educational materials, and ethanol lock prophylaxis. A control chart was used to display the monthly community-acquired CLABSI rate and observe for special cause variation. Results: At baseline, the average monthly community-acquired CLABSI rate was 11.7 per 1000 community catheter-days. The home CVC maintenance bundle was implemented, after which there were 5 consecutive months with no infections. After enhancements to the care bundle and tote, special cause variation was noted, and the average monthly community-acquired CLABSI rate declined by 66% to 4 per 1000 community catheter-days. Special cause variation was noted at 2 other time points without an identifiable cause. Conclusion: A lower community-acquired CLABSI rate has been sustained for 24 months and reflects approximately 12 fewer infections per year in this at-risk population.

Risk factors for catheter-related bloodstream infections in patients with intestinal failure undergoing home parenteral nutrition: a single-center study.

The incidence and risk factors of catheter-related bloodstream infections (CRBSI) in patients with intestinal failure (IF) have not been established, partly because catheter management methods vary from different facilities. This study aimed to identify the risk factors and incidence rate of CRBSIs in patients with IF who were given prophylactic treatment. Sixteen patients with IF who required home parenteral nutrition were enrolled in this study. Prophylactic management of CRBSI included monthly ethanol lock therapy and standardized infection prevention education. The outcomes included the incidence and risk factors of CRBSI. The median incidence rate of CRBSI was 1.2 per 1000 catheter days. Univariate analysis showed that the risk of developing CRBSI was significantly associated with short bowel syndrome (< 30cm) (p = 0.016). Other relevant findings included a significant negative correlation between serum albumin and CRBSI rate (r = -0.505, p = 0.046), and past history of mixed bacterial infections was significantly associated with increased CRBSI rate (p = 0.013). CRBSIs can still develop despite undergoing prophylactic management. Risk factors for CRBSI include the residual intestinal length, nutritional status, and susceptibility to certain microorganisms.

Effectiveness of salvage catheters in home parenteral nutrition: A single-center study and systematic literature review

There are sparse data regarding the rate of catheter salvage and long-term effectiveness of antibiotic lock treatment outcome after central line-associated bloodstream infections (CLABSI). Objectives were to analyze the effectiveness of central venous catheter (CVC) rescue strategy and its impact on catheter lifespan. Secondary objective included effectiveness of taurolidine+4% citrate in primary prevention, compared to a secondary prevention strategy, by analyzing infection incidence during two successive periods. Real-life 5-year observational study assessing CLABSI occurrence and CVC salvage outcomes in adult patients requiring Home Parenteral Nutrition (HPN) managed in a single-center Intestinal Failure Unit. Over the 5-year period, there were 106 confirmed infections (63/143 patients (44%)). Infection incidence was 0.92/1000 catheter-days. Incidence was 1.02/1000 catheter-days during the taurolidine+4% citrate period while lower at 0.84/1000 catheter-days (p=0.034) during the systematic taurolidine lock period. Of the total number of infections, 89 CVCs were immediately removed and 17 were salvaged. The success rate of catheter salvage with antibiotic lock was 82.4%, with 53% remaining CLABSI-free at one year. The salvage strategy extended catheter lifespan by a median 165 days (IQR 50-214). However, the rate of new infection was significantly higher in instances of salvage (71.4%) vs. removal (36%). Parenteral Nutrition (PN) ≥12 months (p=0.002), PN (vs. hydroelectrolytic support) (p=0.028) and self-management by patients (p=0.049) were independent risk factors of CLABSI. Catheter salvage appears to be an effective long-term strategy with >50% of CVCs remaining CLABSI-free at one year and a prolonged catheter life, although may expose to a more frequent and earlier infection recurrence. Cohort approved by the French CNIL (National Committee for Data Protection, authorization number CNIL 2015-25). referred to as "observational research", "non-interventional", or«non-RIPH ».

A287 HOME PARENTERAL NUTRITION FOR INDIVIDUALS WITH SHORT BOWEL SYNDROME SECONDARY TO CROHN’S DISEASE VERSUS OTHER ETIOLOGIES: A PROSPECTIVE COHORT STUDY

Abstract Background Individuals with short bowel syndrome (SBS) have reduced intestinal absorptive capacity and many require home parenteral nutrition (PN) support. One of the common causes of SBS is secondary to intestinal resections in the management of Crohn’s disease (CD). Complication rates and survival in SBS secondary to CD on PN versus other etiologies remains unknown. Purpose To determine whether patients with SBS secondary to CD versus SBS secondary to other etiologies on home PN, have increased risk of hospitalizations and complications including central line associated bloodstream infection (CLABSI) and venous thromboembolism and whether there is a difference in overall survival between groups. Method This is a multicentre prospective cohort study using the Canadian Home Parenteral Nutrition (HPN) Registry on individuals with defined SBS separated into two cohorts: 1) Patients with SBS secondary to Crohn’s disease vs. 2) Patients with SBS secondary to other aetiologies (trauma, surgical complication, vascular event, volvulus, malignancy). Patient characteristics and clinical factors are presented as mean (standard deviation) for continuous variables and as frequency (percentage) for categorical variables. Comparison between groups (SBS CD vs SBS other) were performed using 2-sample t-test for continuous variables and Chi-square or Fisher exact tests when appropriate for categorical variable. Survival probabilities will be estimated using the Kaplan-Meier method. Result(s) The study included a total of 379 patients with short bowel syndrome on home PN. There are 170 (45%) patients with SBS secondary to CD and 209 (55%) patients with SBS from other secondary causes. The average age of those with CD is 52 and 65% female patients. The average age of those with other causes of SBS is 56 with similar percentage of female patients (65%). There were significant differences in baseline medications with higher use of immunosuppressant therapy (39% vs. 7%, p&amp;lt;0.001) in those with CD. There was no significant difference in total number of hospitalizations, hospitalizations related to PN and CLABSI. Image Conclusion(s) Individuals with SBS secondary to CD do not appear to be at increased risk of central line infections or hospitalizations compared those with SBS from other causes. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

Nonsense mutation in the novel PERCC1 gene as a genetic cause of congenital diarrhea and enteropathy

Congenital diarrheas and enteropathies (CODEs) constitute a heterogeneous group of individually rare disorders manifesting with infantile-onset chronic diarrhea. Genomic deletions in chromosome 16, encompassing a sequence termed the ‘intestine-critical region (ICR)’, were recently identified as the cause of an autosomal recessive congenital enteropathy. The regulatory sequence within the ICR is flanked by an unannotated open reading frame termed PERCC1, which plays a role in enteroendocrine cell (EEC) function. We investigated two unrelated children with idiopathic congenital diarrhea requiring home parenteral nutrition attending the Irish Intestinal Failure Program. Currently 12 and 19-years old, these Irish male patients presented with watery diarrhea and hypernatremic dehydration in infancy. Probands were phenotyped by comprehensive clinical investigations, including endoscopic biopsies and serum gastrin level measurements. Following negative exome sequencing, PCR and Sanger sequencing of the entire coding region and intron boundaries of PERCC1 were performed for each proband and their parents. In both patients, serum gastrin levels were low and failed to increase following a meal challenge. While no deletions involving the ICR were detected, targeted sequencing of the PERCC1 gene revealed a shared homozygous c.390C > G stop gain variant. We report clinical and molecular findings in two unrelated patients harboring a shared homozygous variant in PERCC1, comprising the first description of a point mutation in this gene in association with CODE. That both parenteral nutrition dependent children with unexplained diarrhea at our institution harbored a PERCC1 mutation underscores the importance of its inclusion in exome sequencing interpretation.

Reducing COVID-19 risk by optimising delivery frequency of home parenteral nutrition

During the COVID-19 pandemic patients with intestinal failure requiring home parenteral nutrition (HPN) or intravenous fluids (IVF) were instructed to shield at home and avoid contact with others. However, maintenance of the delivery of their nutrition or fluids was essential. Could the delivery frequency of their treatment be optimised in order to keep their contact with others to a minimum?

An evaluation of plasma vitamin C concentrations in individuals requiring home parenteral nutrition.

Ascorbic acid (VitC) is an essential coenzyme to maintain health, but there are minimal data on the adequacy of VitC supply in patients requiring home parenteral nutrition (HPN). A prospective pilot study was carried out measuring plasma VitC, serum vitamins A, D and E, and the minerals zinc, copper, selenium and magnesium in 28 adult HPN-dependent (≥6 months) patients. Fifty-seven percent of patients had insufficient VitC status. There was a strong, positive correlation between HPN provision of VitC and plasma VitC concentrations (rs = 0.663, p = 0.00) with an 83% insufficiency rate below a provision of 800 mgweek-1 . There was no association seen between plasma VitC and number of HPN daysweek-1 (p = 0.539), number of months on HPN (p = 0.773) or dependency on HPN (86% ± 31% of energy requirements met via HPN (77% ± 23%, p = 0.39). VitC insufficiency is prevalent in HPN-dependent patients. Our data highlight the need for regular monitoring of VitC in those living with type III intestinal failure.

New cine magnetic resonance imaging parameters for the differential diagnosis of chronic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction (CIPO) is a severe and refractory intestinal motility disorder whose diagnosis currently relies on subjective imaging assessments. Cine magnetic resonance imaging (MRI) may potentially improve the quantitative analysis of gastrointestinal motility; however, suitable CIPO detection parameters should be determined. Cine MRI was performed in seven patients with CIPO and 11 healthy controls. The logarithm of the Mahalanobis distance (x1) and distance variation per time (x2) were used as the original parameters to determine CIPO diagnostic thresholds. Furthermore, the correlation between cine MRI findings and CIPO severity was investigated. Threshold values of α = 1.10 and β = 0.15 for x1 and x2, respectively, produced a CIPO diagnosis sensitivity of 1.00 (7/7) and specificity of 0.82 (9/11). The resulting error was 0.11 (2/18). The two parameters were correlated (Pearson’s correlation coefficient: − 0.52). Any of the intestinal tracts of patients with severe CIPO requiring home parenteral nutrition belonged to the region defined by x1 ≥ 1.10 and x2 ≤ 0.15. Cine MRI is effective for the quantitative evaluation of small intestinal motility and CIPO diagnosis when using the abovementioned parameters and can be useful for treatment decision-making. However, these parameters have a wide distribution in healthy volunteers; this may complicate the detection of other disorders.

NeoCHIRP: A model for intestinal rehabilitation in the neonatal intensive care unit.

Multidisciplinary intestinal rehabilitation (IR) teams have transformed care in pediatric intestinal failure (IF).1 Although most children with IF are identified in the neonatal intensive care unit (NICU), IR teams may not be involved at this stage. We describe our collaborative model, blending NICU and IR expertise to optimize care. Over 6 years, the NeoCHIRP (Neonatal Children's IR Program) team followed 164 babies for weekly visits (median, 8; range, 1-27). Bedside rounds included CHIRP team physician and surgeons, neonatologist champion, attending neonatologist and fellow, NICU dietitian, bedside nurse, and family. Medical and nutrition status, nutrition history, and laboratory data were discussed, and a nutrition plan to support IR, considering the child's other medical needs, was created to guide the next week's management. Typical issues addressed included parenteral nutrition (PN) composition, enteral nutrition plan, oral feeding, management of small-intestinal bacterial overgrowth and sodium status, and cholestasis. A total of 164 babies were followed by the NeoCHIRP team. Of 153 survivors, IF resolved by discharge in 89% (136 of 153). Seventeen of 153 babies (11%) went on to require home PN and were transferred from NICU directly to the CHIRP team. By discharge, 99% of babies were orally fed (69/136, 50% fully, 67/136, 49% partially), and cholestasis improved or resolved in 80/105 (76%). Eleven babies (7%) died; four deaths were unrelated to IF, but in seven babies, IF was at least a contributing factor. In this high-risk cohort, most babies achieved good outcomes, and those who required longer-term IR transitioned smoothly to the CHIRP team.

Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction.

Chronic intestinal pseudo-obstruction (CIPO) is an extremely rare condition with symptoms of recurrent intestinal obstruction without any lesions. The outcomes of pediatric CIPO and predictors for the outcomes have not yet been well established. We analyzed the clinical outcomes and associated factors for the outcomes of pediatric CIPO. We retrospectively reviewed 66 primary CIPO patients diagnosed between January 1985 and December 2017. We evaluated parenteral nutrition (PN) factors such as PN duration, PN use over 6 months, home PN, and mortality as outcomes. We selected onset age, presence of urologic symptoms, pathologic type, and involvement extent as predictors. The early-onset CIPO was found in 63.6%, and 21.2% of the patients presenting with urologic symptoms. Of the 66 patients, 47 and 11 had neuropathy and myopathy, respectively. The generalized involvement type accounted for 83.3% of the cases. At the last follow-up, 24.2% of the patients required home PN management. The mean duration of PN was 11.8 ± 21.0 months. The overall mortality rate of primary CIPO was 18.2%. PN factors were predicted by the urologic symptoms and extent of involvement. However, mortality was predicted by pathologic type. The onset age was not significantly associated with the outcomes. CIPO with urologic symptoms and generalized CIPO had poor PN outcomes. Myopathy is suggested as a predictor of mortality in children with primary CIPO.

Factors affecting antidepressant use by patients requiring home parenteral nutrition.

Home parenteral nutrition (HPN) is a life-saving therapy for patients with chronic intestinal failure but can be associated with a degree of psychological distress. The factors associated with the need for antidepressants (ADs) in this cohort have not yet been described. The study involved prospective data collection from patients attending an HPN clinic at a national intestinal failure referral center. Patients requiring HPN as a result of active malignancy were excluded. Patients were divided in 2 groups according to AD usage; demographic, anthropometric, socioeconomic characteristics, and intravenous supplementation (IVS) regimens were compared between groups. A total of 184 patients were recruited between July 2018 and April 2019, with an overall prevalence of AD use of 41.7% (70/168 patients). Daily mean IVS volume was significantly higher among patients taking AD ("AD" group; 2125.48 ± 991.8 ml/day, "no-AD" group; 1828.54 ± 847.0 ml/day, P = .039), with the proportion of patients needing high-volume IVS (≥3000 ml/day) being 3 times higher in the AD group (20.0%(14/70 patients) vs 6.1% (6/98 patients), P = .006). The average energy IVS infusion per day was similar between the groups. This is the first study to demonstrate that AD use correlates with higher IVS volume rather than energy requirements in HPN patients, suggesting that high IVS volume requirements may be better associated with the patient's disease burden. Early and tailored mental health intervention may be beneficial in those with high IVS volume requirements.

State-of-the-art colorectal disease: conservative surgical management of intestinal failure in adults.

Intestinal failure (IF) is defined by the reduction of gut function under the minimum necessary for adequate absorption of macronutrients and/or electrolytes, requiring home parenteral nutrition (HPN) to maintain health and/or growth. Among the different causes of IF, short bowel syndrome (SBS) is one of the main causes of IF in adults. Management of IF and SBS is complex and requires a multidisciplinary approach. The aim of this study was to review the place and the modalities of conservative surgical procedures performed for IF in adults. HPN has drastically modified the poor prognosis of these patients, leading to an overall survival around 70-75% at 5 years in patients without cancer. However, HPN is associated with life-threatening long-term complications, including liver failure and catheter-related complications, main causes of deaths for these patients. Surgery can be proposed, in order to try to reduce SBS consequences with either conservative or non-conservative procedures. Simple bowel continuity restoration should be performed as often as possible in order to wean or at least to reduce HPN. Lengthening procedures are proposed to increase the absorption surface of the remnant bowel. These procedures can be only performed on a dilated small bowel. The slowing transit time procedure is represented by segmental reversal of the small bowel. Intestinal transplantation is the last surgical option for patients with IF but is still today associated with high mortality and failure rates. Thus, conservative procedures have a major role in the surgical armentarium for patients with IF.

Use of instructional videos to reduce central venous catheter complications in children with intestinal failure receiving home parenteral nutrition.

Children with intestinal failure (IF) require parenteral nutrition (PN) at home, delivered through a central venous catheter (CVC) to support growth. CVC-related complications including infection, breakage, and blockage are the most common cause of readmission to the hospital. The objective of this study was to evaluate the use of instructional videos as part of the caregiver home PN-teaching program to reduce CVC-related complications. Caregivers of children with IF requiring home PN were surveyed to assess skill confidence and interest in instructional videos for skill acquisition. Videos were then created using a smartphone and free video-editing software. Input from stakeholders (families, care providers) was incorporated in video production. Families were given access to the videos, and CVC-related complications were compared for 2 years prior to and 1 year following video introduction with Welch t-test analysis. After obtaining ethics approval, 11 caregivers were surveyed. Thirty percent reported feeling underconfident in their skills at the time of discharge. After viewing the videos, 100% of caregivers reported that these videos were useful. Catheter-related complication rates significantly decreased in the year following the video introduction from 7.88 to 2.65 complications per 1000 catheter days (P = .046). This included reductions in catheter-related infections, catheter occlusions, and breakages. Children with IF receiving home PN are at high risk for CVC-related complications, and caregivers are the first line of defense for catheter care. Instructional videos were low-cost to create, were well received by all families, and may contribute to reduced catheter-related complications.

The effectiveness of buccal Vitamin D replacement in patients requiring home parenteral nutrition.

Patients with intestinal failure requiring home parenteral nutrition are at risk of vitamin D and other micronutrient deficiencies. Conventional enteral replacement of Vitamin D may not be sufficient for this patient group. This study examines whether buccal Vitamin D provides an alternative, effective route for supplementing Vitamin D in patients with intestinal failure. A retrospective review of patients who received buccal Vitamin D replacement between 1st January 2013 and 1st January 2020at our hospital in Northern England was carried out. Demographics were recorded as were patients' daily intravenous fluid requirements using standard ESPEN definitions. Serum Vitamin D levels were recorded prior to buccal replacement and then at a minimum interval of 3 months after commencement. A cost comparison of a 6 month course of this preparation was also made with an equivalent duration of replacement using oral cholecalciferol capsules. 17 patients were identified. The mean level of Vitamin D prior to replacement was 28.4nmol/l with 65% of patients classed as Vitamin D deficient (<25nmol/l) prior to replacement. The average duration of buccal replacement prior to a repeat level was 5 months. Following buccal Vitamin D replacement no patients were classed as Vitamin D deficient with all levels ≥25nmol/l and a mean of 62.3nmol/l. There was a statistically significant increase in post buccal replacement serum Vitamin D concentrations (p=0.001). Using costings from our hospital pharmacy a 6 month course of this buccal Vitamin D preparation was 38% less expensive than 6 months of replacement with oral cholecalciferol capsules. This study shows that in patients with intestinal failure on home parenteral nutrition, buccal Vitamin D is both a use and cost-effective method of replacement.

Repair of central venous access devices in intestinal failure patients is safe and cost-effective: A retrospective single centre cohort study

Patients with chronic intestinal failure (IF) require home parenteral nutrition (HPN). Central venous access is needed for prolonged use of PN, usually via a long term central venous access device (CVAD). Post insertion there may be mechanical complications with a CVAD such as catheter rupture or tear. Repair of damaged CVADs is possible to avoid risks associated with catheter replacement in patients with IF. However, catheter related blood stream infections (CRBSI) are a concern when CVAD's are accessed or manipulated. To investigate the success of repair of CVADs in patients with IF on HPN, related to repair longevity and incidence of CRBSI following repair. Nutrition team records of CVAD repairs carried out in patients with IF were reviewed retrospectively for the period April 2015 to March 2019. Nutrition Clinical Nurse Specialists carried out 38 repairs in 27 patients. Male n=5, female n=22; mean age 55 years. Catheter longevity before first repair (n=27): median 851 days, IQR 137-1484 days. 30/38 (78.9%) of repairs were successful lasting ≥30days. Hospital admission was avoided in 76% of cases. 4 patients in the failed repair group underwent catheter re-insertion where 4 had a further, subsequently successful, repair, an overall success rate of 89.4% (34/38). 30-day CRBSI rate was 0.09/1000 catheter days in repaired catheters. In comparing costs, there is a potential cost saving of 2766GBP for repair compared to replacement of damaged CVADs. Repair of tunnelled CVADs in patients with IF is successful and safe with no increased risk of CRBSI. Significant cost savings may be made.

Factors affecting antidepressant use by patients requiring home parenteral nutrition

Rationale: Home parenteral nutrition (HPN) is a life-saving therapy for patients with chronic intestinal failure. However, HPN is associated with impaired quality of life and a degree of psychological distress, although the factors associated with the need for antidepressants in this cohort have not yet been described.

Short bowel syndrome and the impact on patients and their families: a qualitative study.

Short bowel syndrome (SBS) is a malabsorptive condition that can result in intestinal failure (SBS-IF). Many patients with SBS-IF require home parenteral nutrition (PN) for survival. However, PN has profound effects on patients and their family members. The present study aimed to understand the lived experience of SBS-IF for patients and their families. In-depth semi-structured qualitative interviews were conducted with 15 patients with SBS-IF and five adult family members living with someone with SBS-IF. A patient-centric approach was taken, with a patient steering group providing input and guidance to develop the interview guide. Key concepts were identified using thematic analysis of interview transcripts. Patients' lives were dominated by having SBS-IF. They described physical impacts that included patient-reported signs and symptoms and physical restrictions comprising of restrictions on daily life, actives of daily living and physical functioning. In addition, they encountered emotional impacts with a plethora of negative feelings and social impacts, such as difficulties socialising and maintaining relationships. Patients coped by adapting their life around SBS-IF, having support and adopting an attitude of gratitude and acceptance. Family members were also affected and, along with patients, appreciated the respite of a night off from infusions. Patients and families face many difficulties with SBS-IF. Healthcare professionals can support patients by facilitating them explore what others have found beneficial; adapting their life around PN, viewing PN with acceptance and trying to cultivate gratitude. Further research into the support required for families may be beneficial.

Influence of different intravenous lipid emulsions on fatty acid status and laboratory and clinical outcomes in adult patients receiving home parenteral nutrition: A systematic review

Patients who have chronic intestinal failure require home parenteral nutrition (HPN) support. Intravenous lipid emulsions (IVLEs) are a vital part of HPN. The conventional IVLE is based on pure soybean oil, which contains a high concentration of omega-6 fatty acids. Alternative IVLEs are commercially available. These contain various oil blends and have different fatty acid compositions from soybean oil that could provide benefit to patients on HPN. The aim of this systematic review is to assess the effects of different IVLEs in adult patients requiring HPN. A systematic literature search was conducted up to October 2019 using relevant search terms in the Medline, EMBASE and CINAHL databases. Only randomised controlled trials (RCTs) in adults on HPN that compared two or more IVLEs were included. Data were extracted and the Cochrane Collaboration's tool for assessing risk of bias was used. Six articles were identified for inclusion in this systematic review. Studies differed according to sample size, duration and the IVLEs compared. Four studies found no increased risk of adverse effects related to the different IVLEs, whilst one study found a higher frequency of serious adverse events with soybean oil. One study found higher serum α-tocopherol with the blend of soybean oil, medium chain triglycerides, olive oil and fish oil. Inflammatory markers were not affected by different IVLEs in three studies. Differences in liver function tests were minimal, but one study found slight abnormalities in patients receiving soybean oil. IVLEs containing olive oil or fish oil modified the blood fatty acid profile. No studies reported essential fatty acid deficiency. There may be benefits of using alternative IVLEs to soybean oil-based emulsions in adults requiring HPN, although there is currently insufficient evidence to determine superiority of one formulation over another. More and larger RCTs are required in this area.

Patients With Severe Gastrointestinal Dysmotility Disorders Receiving Home Parenteral Nutrition Have Similar Survival As Those With Short-Bowel Syndrome: A Prospective Cohort Study.

Severe gastrointestinal dysmotility disorder (GID) constitute approximately 20% of patients requiring home parenteral nutrition (HPN), whereas short-bowel syndrome (SBS) remains the most frequent indication for HPN. This study's aim was to characterize GID patients and compare clinical parameters and survival to SBS patients. Similarly, clinical comparisons between sclerodermaand nonscleroderma patients were made. Demographic and clinical data for all patients was extracted from the Canadian HPN Registry from January 1, 2003, to November 1, 2018. Kaplan-Meier method was used to estimate the unadjusted survival probability, and log-rank test was used to compare the survival probability between groups. 270 patients (52 GID and 218 SBS) were included in the analysis. For all patients, higher mortality was associated with age (hazard ratio [HR], 1.02 [1.00-1.04]; P = .05), PN dependence (HR, 1.01 [1.00-1.02]; P = .04), hospitalizations (HR, 1.21 [1.10-1.33]; P < .001), and use of immunosuppressant (HR, 1.97 [1.02-3.82]; P = .04). The 5- and 10-year actuarial survival probabilities between GID and SBS were not significantly different (5-year: 70.0% vs 59.2%; 10-year: 79.6% vs 66.2% [P = .5], respectively). There was no difference in survival between scleroderma and nonscleroderma patients (P = .67). T5- and 10- year survival probabilities were similar between GID and SBS patients. The diagnosis of scleroderma had no effect on survival. Use of immunosuppressant, older age, PN dependence, and number of hospitalizations per PN duration are risk factors for mortality in both the GID and SBS groups.