Short Bowel Syndrome: clinical management

The management of a case of intestinal failure due to Short Bowel Syndrome (SBS) is described. Patients’ care needs an expert multidisciplinary approach. Published data have demonstrated that the lack of a specialist staff is a risk factor for patients’ death. The creation of networks linking non-specialist doctors with dedicated centers is recommended

Assessment of Intestinal Failure Associated Liver Disease according to different diagnostic criteria

Background & aims: Intestinal failure associated liver disease (IFALD) has been defined using numerous criteria; however the clinical relevance of these criteria has never been compared. We therefore aimed to evaluate the prevalence, incidence, evolution of IFALD diagnosed by different criteria and to assess any clinical features that may be associated with its occurrence. Methods: A cross sectional (CS) and retrospective study were carried out on adults on home parenteral nutrition (HPN) for chronic intestinal failure (CIF) managed at a single center. Inclusion criteria at CS: age 6518 years, benign disease. Collected data included: patient demographics, CIF and HPN characteristics, episodes of central venous catheter related bloodstream infection (CRBSI). IFALD was diagnosed by 9 criteria based on liver function tests and liver ultrasound (US) imaging. IFALD diagnoses were categorized as steatosis (2 criteria), cholestasis (3 criteria) or fibrosis (2 criteria) and unclassified (2 criteria). Prevalence was assessed at CS and at starting HPN (baseline, BS). Evolution was assessed as change of IFALD between BS and CS. Incidence was calculated as patients who developed IFALD from BS to CS. Results: A total of 113 patients were included. At CS, IFALD prevalence range in each diagnostic categories was: cholestasis 5\u201315%; steatosis 17\u201343%; fibrosis 10\u201320%; unclassified 7\u201338%. A 28.5% of patients did not have IFALD according to any criteria. Two cholestasis criteria and one fibrosis criterion were significantly (P < 0.05) associated with a short bowel syndrome as the pathophysiological mechanism of CIF, HPN requirement and the number of CRBSI episodes. At BS, IFALD prevalence range was: cholestasis 13\u201340%; steatosis 27\u201390%; fibrosis 2\u20135%; unclassified 8\u201375%. The incidence range of IFALD was: cholestasis 0\u20137%; steatosis 0\u201339%; fibrosis 7\u201318%; unclassified 4\u20139%. IFALD steatosis diagnosed by US was the most frequent diagnosis at both CS prevalence and incidence assessments. Notably, IFALD criteria normalized in various percentages (2\u201370%), depending on the diagnostic categories, between BS and CS. Conclusions: This is the first study to systematically demonstrate that the frequency of IFALD varies greatly depending on diagnostic criteria used, confirming the need for a consensus definition to be used between different national and international IF units. IFALD can be present at HPN initiation but may resolve thereafter; further work is required to evaluate the factors associated with improvement

Functional lipidomics in patients on home parenteral nutrition: Effect of lipid emulsions

AIM To investigate the fatty acid-based functional lipidomics of patients on long-term home parenteral nutrition receiving different intravenous lipid emulsions. METHODS A cross-sectional comparative study was carried out on 3 groups of adults on home parenteral nutrition (HPN), receiving an HPN admixture containing an olive-soybean oil-based intravenous lipid emulsion (IVLE) (OO-IVLE; n = 15), a soybean- medium-chain triacylglycerol-olivefish oil-based IVLE (SMOF-IVLE; n = 8) or HPN without IVLE (No-IVLE; n = 8) and 42 healthy controls (HCs). The inclusion criteria were: duration of HPN â\u89¥ 3 mo, current HPN admixtures â\u89¥ 2 mo and HPN infusions â\u89¥ 2/wk. Blood samples were drawn 4-6 h after the discontinuation of the overnight HPN infusion. The functional lipidomics panel included: the red blood cell (RBC) fatty acid (FA) profile, molecular biomarkers [membrane fluidity: saturated/monounsaturated FA ratio = saturated fatty acid (SFA)/monounsaturated fatty acid (MUFA) index; inflammatory risk: n-6/n-3 polyunsaturated fatty acid (PUFA) ratio = n-6/n-3 index; cardiovascular risk: sum of n-3 eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) = n-3 index; free radical stress: sum of FA trans isomers = %trans index] and FA pathway enzyme activity estimate (delta-9-desaturase = D9D; delta-6-desaturase = D6D; delta-5-desaturase = D5D; elongase = ELO). Statistics were carried out using nonparametric tests. The amount of each FA was calculated as a percentage of the total FA content (relative%). RESULTS In the OO-IVLE group, the percentage of oleic acid in the RBCs was positively correlated with the weekly load of OO-IVLE (r = 0.540, p = 0.043). In the SMOFIVLE cohort, the RBC membrane EPA and DHA were positively correlated with the daily amount of SMOFIVLE (r = 0.751, p = 0.044) and the number of HPN infusions per week (r = 0.753; p = 0.046), respectively. The SMOF-IVLE group showed the highest EPA and DHA and the lowest arachidonic acid percentages (p < 0.001). The RBC membrane linoleic acid content was lower, and oleic and vaccenic acids were higher in all the HPN groups in comparison to the HCs. Vaccenic acid was positively correlated with the weekly HPN load of glucose in both the OO-IVLE (r = 0.716; p = 0.007) and the SMOF-IVLE (r = 0.732; p = 0.053) groups. The estimated activity of D9D was higher in all the HPN groups than in the HCs (p < 0.001). The estimated activity of D5D was lower in the SMOF-IVLE group than in the HCs (p = 0.013). The SFA/MUFA ratio was lower in all the HPN groups than in the HCs (p < 0.001). The n-6/n-3 index was lower and the n-3 index was higher in the SMOF-IVLE group in comparison to the HCs and to the other HPN groups (p < 0.001). The %trans index did not differ among the four groups. CONCLUSION The FA profile of IVLEs significantly influenced the cell membrane functional lipidomics. The amount of glucose in the HPN may play a relevant role, mediated by the insulin regulation of the FA pathway enzyme activities