Five-year survival and causes of death in patients on home parenteral nutrition for severe chronic and benign intestinal failure

Joly, Francisca; Baxter, J.; Staun, Michael; Kelly, Darlene G.; Hwa, Yi L.; Corcos, Olivier; Francesco, Antonella De; Agostini, F.; Kłęk, Stanisław; Santarpia, Lidia; Contaldo, Franco; Jonker, Cora; Wanten, Geert; Chicharro, L.; Burgos, R.; Gossum, A. Van; Cuerda, C.; Virgili, N.; Pironi, Loris

doi:10.1016/j.clnu.2017.06.016

Cited by 65 publications

(57 citation statements)

References 26 publications

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“…As such, it is 1 of only 2 lifesaving indications for intestinal transplantation or combined intestinal/liver transplantation . In adults using long‐term HPN for benign CIF, 1%–4% of all deaths is attributed to IFALD …”

Section: Introductionmentioning

confidence: 99%

“…10 In adults using long-term HPN for benign CIF, 1%-4% of all deaths is attributed to IFALD. 11,12 Liver complications are a particular concern in CIF patients, as patients often remain dependent on PN therapy for life. 1 Progression in adults is typically insidious, and therefore IFALD is often only recognized at a stage in which liver injury is irreversible.…”

Section: Introductionmentioning

confidence: 99%

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Systematic Review: Noninvasive Assessments of Intestinal Failure–Associated Liver Disease in the Adult Population

Huijbers

Koggel

Bronkhorst

et al. 2019

J Parenter Enteral Nutr

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Chronic intestinal failure (CIF) patients are at risk for developing intestinal failure‐associated liver disease (IFALD), which can progress to end‐stage liver disease. Liver biopsy is the current reference standard to diagnose and monitor IFALD. However, due to its associated complications, biopsy is an unattractive tool in this respect. Our aim was to assess the evidence regarding non‐invasive assessment of IFALD in the adult population and provide ideas to take this field further. We searched the PubMed, EMBASE and Web of Science databases in accordance with the PRISMA guideline. We included studies in the adult/mixed intestinal failure population, performing non‐invasive diagnostic assessment of IFALD and using liver biopsy, 1H‐MRS or MRI‐PDFF as reference. Quality of the included studies was assessed using the QUADAS‐2 tool. Four studies were included, assessing two serum (vitamin B12, FGF21) and two imaging tests (Fibroscan, CAUS). Three used liver biopsy as reference, all according to a different histological scoring system. One used 1H‐MRS as reference. Vitamin B12 did not correlate with liver injury, Fibroscan did not correlate with fibrosis, but with cholestasis. FGF21 correlated with steatosis grade. Several CAUS parameters correlated with the degree of steatosis assessed by 1H‐MRS. In conclusion, three tests show promise to non‐invasively assess IFALD, but the limited data do not justify conclusions on the diagnostic value of the tested biomarkers. Hence, additional studies are needed. Identification of and validation for grading and staging of clinically relevant histomorphological parameters of IFALD is also crucial and a conceptual study set up is provided.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Systematic Review: Noninvasive Assessments of Intestinal Failure–Associated Liver Disease in the Adult Population

Huijbers

Koggel

Bronkhorst

et al. 2019

J Parenter Enteral Nutr

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“…The probability of HPN dependency at 5 years is variable depending on the cause of the original HPN requirement, with a significantly increased risk of remaining on HPN at 5 years in those with SBS versus a much lower risk in those with an intestinal fistula. When 1,2, and 5-year survival in patients with CIF is compared between literature from 1999 [33] and 2017 [90], very little change has been observed (87 vs. 88%, 77 vs. 80%, and 62 vs. 64%). The underlying disease process remains responsible for 65% of deaths within this cohort.…”

Section: The Economic and Social Burdenmentioning

confidence: 96%

“…The ESPEN guidelines for CIF [12] recommend that a HPN programme includes the "provision of evidence-based therapy, prevention of HPN-related complications and ensure quality of life is maximised". A recently published international retrospective study [90] of 472 patients with severe chronic and benign IF who commenced HPN in 2000 demonstrated a survival probability of 88%, 74% and 64% at 1, 3, and 5 years, with survival inversely associated with increasing age, the presence of Crohn's disease or chronic idiopathic pseudo-obstruction. At 5-year follow up, 39% were alive on HPN with a mean age of 55 years, 36% had been weaned from HPN with a mean age of 52 years, 22% had died on HPN with a mean age of 60 years, 2% were alive following intestinal transplant with a mean age of 42 years and 1% had died following intestinal transplant with a mean age of 36 years.…”

Section: The Economic and Social Burdenmentioning

confidence: 99%

Intestinal failure in adults: Recommendations from the ESPEN expert groups

et al. 2018

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Both AIF and CIF are relatively rare conditions and most of the published work presents evidence from small, single-centre studies. Much remains to be investigated to improve the diagnosis and management of IF and future studies should rely on multidisciplinary, multicentre and multinational collaborations that gather data from large cohorts of patients. Emphasis should also be placed on partnership with patients, carers and government agencies in order to improve the quality of research that focuses on patient-centred outcomes that will help to improve both outcomes and quality of life in patients with this devastating condition.

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“…Patients with CIF are metabolically stable and receive IVS at home (home parenteral nutrition, HPN) for months, years or lifelong 2. Single or multicentre, mostly retrospective, surveys have described risk factors associated with the patient’s outcome, such as survival and reversibility of CIF, and with the risk of HPN/IF-related major complications 3–5. However, no simple indicator, such as creatinine for kidney disease and arterial oxygen saturation for respiratory disease, has yet been identified to categorise the severity of CIF.…”

Section: Introductionmentioning

confidence: 99%

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

Pironi

Steiger

Joly

et al. 2020

Gut

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Background and aimNo 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.MethodsAt 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).ResultsFifty-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).ConclusionsThe 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.

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Five-year survival and causes of death in patients on home parenteral nutrition for severe chronic and benign intestinal failure

Cited by 65 publications

References 26 publications

Systematic Review: Noninvasive Assessments of Intestinal Failure–Associated Liver Disease in the Adult Population

Systematic Review: Noninvasive Assessments of Intestinal Failure–Associated Liver Disease in the Adult Population

Intestinal failure in adults: Recommendations from the ESPEN expert groups

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

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