Characteristics of adult patients with chronic intestinal failure due to short bowel syndrome: An international multicenter survey.

BACKGROUND AND AIMS: The case-mix of patients with intestinal failure due to short bowel syndrome (SBS-IF) can differ among centres and may also be affected by the timeframe of data collection. Therefore, the ESPEN international multicenter cross-sectional survey was analyzed to compare the characteristics of SBS-IF cohorts collected within the same timeframe in different countries. METHODS: The study included 1880 adult SBS-IF patients collected in 2015 by 65 centres from 22 countries. The demographic, nutritional, SBS type (end jejunostomy, SBS-J; jejuno-colic anastomosis, SBS-JC; jejunoileal anastomosis with an intact colon and ileocecal valve, SBS-JIC), underlying disease and intravenous supplementation (IVS) characteristics were analyzed. IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorized as <1, 1-2, 2-3 and >3 L/day. RESULTS: In the entire group: 60.7% were females and SBS-J comprised 60% of cases, while mesenteric ischaemia (MI) and Crohn' disease (CD) were the main underlying diseases. IVS dependency was longer than 3 years in around 50% of cases; IVS was infused ≥5 days/week in 75% and FE in 10% of cases. Within the SBS-IF cohort: CD was twice and thrice more frequent in SBS-J than SBS-JC and SBS-JIC, respectively, while MI was more frequent in SBS-JC and SBS-JIC. Within countries: SBS-J represented 75% or more of patients in UK and Denmark and 50-60% in the other countries, except Poland where SBS-JC prevailed. CD was the main underlying disease in UK, USA, Denmark and The Netherlands, while MI prevailed in France, Italy and Poland. CONCLUSIONS: SBS-IF type is primarily determined by the underlying disease, with significant variation between countries. These novel data will be useful for planning and managing both clinical activity and research studies on SBS.

Intravenous supplementation type and volume are associated with 1-year outcome and major complications in patients with chronic intestinal failure.

BACKGROUND AND AIM: No marker to categorise the severity of chronic intestinal failure (CIF) has been developed. A 1-year international survey was carried out to investigate whether the European Society for Clinical Nutrition and Metabolism clinical classification of CIF, based on the type and volume of the intravenous supplementation (IVS), could be an indicator of CIF severity. METHODS: At baseline, participating home parenteral nutrition (HPN) centres enrolled all adults with ongoing CIF due to non-malignant disease; demographic data, body mass index, CIF mechanism, underlying disease, HPN duration and IVS category were recorded for each patient. The type of IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorised as <1, 1-2, 2-3 and >3 L/day. The severity of CIF was determined by patient outcome (still on HPN, weaned from HPN, deceased) and the occurrence of major HPN/CIF-related complications: intestinal failure-associated liver disease (IFALD), catheter-related venous thrombosis and catheter-related bloodstream infection (CRBSI). RESULTS: Fifty-one HPN centres included 2194 patients. The analysis showed that both IVS type and volume were independently associated with the odds of weaning from HPN (significantly higher for PN <1 L/day than for FE and all PN >1 L/day), patients' death (lower for FE, p=0.079), presence of IFALD cholestasis/liver failure and occurrence of CRBSI (significantly higher for PN 2-3 and PN >3 L/day). CONCLUSIONS: The type and volume of IVS required by patients with CIF could be indicators to categorise the severity of CIF in both clinical practice and research protocols.

Home parenteral nutrition provision modalities for chronic intestinal failure in adult patients: An international survey.

BACKGROUND & AIMS: The safety and effectiveness of a home parenteral nutrition (HPN) program depends both on the expertise and the management approach of the HPN center. We aimed to evaluate both the approaches of different international HPN-centers in their provision of HPN and the types of intravenous supplementation (IVS)-admixtures prescribed to patients with chronic intestinal failure (CIF). METHODS: In March 2015, 65 centers from 22 countries enrolled 3239 patients (benign disease 90.1%, malignant disease 9.9%), recording the patient, CIF and HPN characteristics in a structured database. The HPN-provider was categorized as health care system local pharmacy (LP) or independent home care company (HCC). The IVS-admixture was categorized as fluids and electrolytes alone (FE) or parenteral nutrition, either commercially premixed (PA) or customized to the individual patient (CA), alone or plus extra FE (PAFE or CAFE). Doctors of HPN centers were responsible for the IVS prescriptions. RESULTS: HCC (66%) was the most common HPN provider, with no difference noted between benign-CIF and malignant-CIF. LP was the main modality in 11 countries; HCC prevailed in 4 European countries: Israel, USA, South America and Oceania (p < 0.001). IVS-admixture comprised: FE 10%, PA 17%, PAFE 17%, CA 38%, CAFE 18%. PA and PAFE prevailed in malignant-CIF while CA and CAFE use was greater in benign-CIF (p < 0.001). PA + PAFE prevailed in those countries where LP was the main HPN-provider and CA + CAFE prevailed where the main HPN-provider was HCC (p < 0.001). CONCLUSIONS: This is the first study to demonstrate that HPN provision and the IVS-admixture differ greatly among countries, among HPN centers and between benign-CIF and cancer-CIF. As both HPN provider and IVS-admixture types may play a role in the safety and effectiveness of HPN therapy, criteria to homogenize HPN programs are needed so that patients can have equal access to optimal CIF care.

Candida catheter-related bloodstream infection in patients on home parenteral nutrition - Rates, risk factors, outcomes, and management.

BACKGROUND: Catheter-related bloodstream infections (CRBSIs) are life-threatening complications for home parenteral nutrition (HPN) patients. This review clarifies Candida CRBSI rates by species, risk factors, outcomes, and management to improve effectiveness of HPN programs. METHODS: A review of Candida CRBSIs in HPN patients was conducted around the following questions: 1. How often do adult and paediatric HPN patients contract Candida CRBSIs? 2. What is the proportion of different Candida species? 3. What are the risk factors? 4. How are outcomes in Candida versus other CRBSIs? 5. What are current guidelines to manage Candida CRBSIs? Specifically, should catheters be removed? What antimicrobial therapy is indicated? Are catheter lock techniques effective? RESULTS: 20 studies were included - six paediatric and 14 adult. Candida represented 9.8% of paediatric CRBSIs and 11.7% of adult CRBSIs. Paediatric candidal CRBSIs featured these species: C. albicans (46.2%), C. parapsilosis (34.6%), Candida guilliermondii (11.5%), Candida tropicalis (3.8%), and mixed or other types of Candida (3.8%). Adult candidal CRBSIs featured these species: C. albicans (37.3%), C. glabrata (33.3%), C. parapsilosis (22.4%), mixed or other types of Candida (5.7%), and C. tropicalis (1.3%). Risk factors for paediatric HPN CRBSIs include underlying haematological disease and previous fungaemia. Candida infection is associated with mortality rates around 30%. In Candida CRBSIs, major guidelines advocate catheter removal prior to systemic antifungal treatment (fluconazole, amphotericin B, echinocandins), ideally until 14 days after the first negative blood culture; some studies suggest the possibility of systemic therapy while catheters remain in-situ to preserve crucial line access. Various catheter lock solutions are effective as treatment and prophylaxis, but are not yet firmly established. CONCLUSIONS: Candida CRBSI is a significant danger to HPN patients causing high mortality; gold standard treatment is catheter removal and antifungal treatment, although treatments with catheters in-situ and catheter locks as prophylaxis appear to be gaining traction.

An international study of the quality of life of adult patients treated with home parenteral nutrition.

BACKGROUND & AIMS: Home parenteral nutrition-quality of life (HPN-QOL©) is a self-assessment tool for the measurement of QOL in patients on HPN. The aims of this study were: to re-assess the basic psychometric properties of the HPN-QOL© in a multinational sample of adult patients; to provide a description of QOL dimensions by short and long HPN treatment duration; to explore clinical factors potentially associated to QOL scores. METHODS: Patients (n = 699) from 14 countries completed the HPN-QOL©. The questionnaires were analysed to evaluate data completeness, convergent/discriminant validity and internal-consistency reliability. The association of overall QOL and HPN treatment duration as well as other clinical factors were investigated using multivariable linear regression models. RESULTS: The analysis of the multitrait-scaling and internal consistency indicates a good fit with the questionnaire structure for most items. Item discriminant validity correlation was satisfactory and psychometric evaluation of the HPN-QOL© in the different English, French and Italian language patient sub-groups confirmed psychometric equivalence of the three questionnaire versions. The results of the multivariable linear regression showed that QOL scores were significantly associated with HPN duration (better in long-term), underlying disease (better in Crohn's disease and mesenteric ischaemia) and living status (worse in living alone) and, after adjusting for the other factors, with the number of days of HPN infusion per week. CONCLUSIONS: The HPN-QOL©, is a valid tool for measurement of QOL in patients on HPN, to be used in the clinical practice as well as in research.

Clinical classification of adult patients with chronic intestinal failure due to benign disease: An international multicenter cross-sectional survey.

BACKGROUND & AIMS: The aim of the study was to evaluate the applicability of the ESPEN 16-category clinical classification of chronic intestinal failure, based on patients' intravenous supplementation (IVS) requirements for energy and fluids, and to evaluate factors associated with those requirements. METHODS: ESPEN members were invited to participate through ESPEN Council representatives. Participating centers enrolled adult patients requiring home parenteral nutrition for chronic intestinal failure on March 1st 2015. The following patient data were recorded though a structured database: sex, age, body weight and height, intestinal failure mechanism, underlying disease, IVS volume and energy need. RESULTS: Sixty-five centers from 22 countries enrolled 2919 patients with benign disease. One half of the patients were distributed in 3 categories of the ESPEN clinical classification. 9% of patients required only fluid and electrolyte supplementation. IVS requirement varied considerably according to the pathophysiological mechanism of intestinal failure. Notably, IVS volume requirement represented loss of intestinal function better than IVS energy requirement. A simplified 8 category classification of chronic intestinal failure was devised, based on two types of IVS (either fluid and electrolyte alone or parenteral nutrition admixture containing energy) and four categories of volume. CONCLUSIONS: Patients' IVS requirements varied widely, supporting the need for a tool to homogenize patient categorization. This study has devised a novel, simplified eight category IVS classification for chronic intestinal failure that will prove useful in both the clinical and research setting when applied together with the underlying pathophysiological mechanism of the patient's intestinal failure.

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.