-
1.
[Sitosterolemia (phytosterolemia)].
Lütjohann, D
Der Internist. 2019;(8):871-877
Abstract
Sitosterolemia or phytosterolemia is a rare autosomal recessive hereditary lipid storage disorder. It is caused by homozygous or compound heterozygous mutations in one of the two ABCG5 and ABCG8 genes encoding the intestinal and hepatic heterodimer ABCG5 (sterolin 1)/ABCG8 (sterolin 2) efflux transporters. These mutations lead to intestinal hyperabsorption and reduced hepatic secretion of cholesterol and plant sterols with subsequent accumulation of phytosterols and cholesterol in plasma and deposition in tissue (xanthoma). Phytosterols are found mainly in vegetable oils, margarine, nuts, grains, soybeans and avocados. Patients with sitosterolemia show extreme phenotypic heterogeneity from almost asymptomatic individuals to those with combined severe hypercholesterolemia at a young age, leading to increased atherosclerosis and premature cardiac death. Early abnormalities include hemolytic anemia with stomatocytosis, macrothrombocytopenia and splenomegaly. In addition to strict avoidance of phytosterol-containing foods, the use of the sterol absorption inhibitor ezetimibe, possibly in combination with the bile acid-binding resin cholestyramine, is the most effective treatment option.
-
2.
Systematic Review: Noninvasive Assessments of Intestinal Failure-Associated Liver Disease in the Adult Population.
Huijbers, A, Koggel, LM, Bronkhorst, C, Verheij, J, Wanten, GJA
JPEN. Journal of parenteral and enteral nutrition. 2019;(5):615-626
Abstract
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.
-
3.
Imaging of adult intestinal failure.
Smith, J, Godfrey, E, Bowden, D, Hickman, K, Sharkey, L, Butler, A, Upponi, S
Clinical radiology. 2019;(8):603-612
Abstract
Intestinal failure is the inability to maintain adequate nutrition or hydration through the gut. It is caused by a diverse range of benign and malignant aetiologies. Imaging takes a central role in the multidisciplinary assessment of patients with intestinal failure.
-
4.
Tryptophan, glutamine, leucine, and micronutrient supplementation improves environmental enteropathy in Zambian adults: a randomized controlled trial.
Louis-Auguste, J, Besa, E, Zyambo, K, Munkombwe, D, Banda, R, Banda, T, Watson, A, Mayneris-Perxachs, J, Swann, J, Kelly, P
The American journal of clinical nutrition. 2019;(5):1240-1252
-
-
Free full text
-
Abstract
BACKGROUND Environmental enteropathy (EE) refers to villus blunting, reduced absorption, and microbial translocation in children and adults in tropical or deprived residential areas. In previous work we observed an effect of micronutrients on villus height (VH). OBJECTIVE We aimed to determine, in a randomized controlled trial, if amino acid (AA) or multiple micronutrient (MM) supplementation can improve intestinal structure or barrier dysfunction in Zambian adults with EE. METHODS AA (tryptophan, leucine, and glutamine) and/or MM supplements were given for 16 wk in a 2 × 2 factorial comparison against placebo. Primary outcomes were changes in VH, in vivo small intestinal barrier dysfunction assessed by confocal laser endomicroscopy (CLE), and mechanistic (or mammalian) target of rapamycin complex 1 (MTORC1) nutrient responsiveness in lamina propria CD4+ lymphocytes. RESULTS Over 16 wk AA, but not MM, supplementation increased VH by 16% (34.5 μm) compared with placebo (P = 0.04). Fluorescein leak, measured by CLE, improved only in those allocated to both AA and MM supplementation. No effect was seen on MTORC1 activation, but posttreatment MTORC1 and VH were correlated (ρ = 0.51; P = 0.001), and change in MTORC1 was correlated with change in VH in the placebo group (ρ = 0.63; P = 0.03). In secondary analyses no effect was observed on biomarkers of microbial translocation. Metabolomic analyses suggest alterations in a number of microbial- and host-derived metabolites including the leucine metabolite β-hydroxy-β-methylbutyrate, which was increased by AA supplementation and correlated with VH. CONCLUSIONS In this phase 2 trial, AA supplementation protected against a decline in VH over the supplementation period, and improved barrier function when combined with micronutrients. Leucine and MTORC1 metabolism may be involved in the mechanism of effect. This trial was registered at www.pactr.org as PACTR201505001104412.
-
5.
Current status of graft-versus-host disease after intestinal transplantation.
Ganoza, A, Mazariegos, GV, Khanna, A
Current opinion in organ transplantation. 2019;(2):199-206
Abstract
PURPOSE OF REVIEW Over the past decades, visceral transplantation has become the standard of care for patients with irreversible intestinal failure who suffer complications of total parenteral nutrition (TPN). Graft-versus-host disease (GVHD) after solid organ transplantation is a rare but often fatal complication with high mortality. GVHD after intestinal transplantation, given the large lymphoid content of the graft, is more frequent compared with other solid organs. It is a complex condition that may have varied clinical presentations. The therapy of GVHD is multifactorial and has evolved with visceral transplantation. RECENT FINDINGS In recent large series of intestinal transplantation performed in centers around the world, GVHD remained an important cause of death (40-70%). Advances in immunology and current treatment options come from the hematopoietic stem-cell transplantation (HSCT) experience given the high prevalence of GVHD in that patient population. Therapeutic options for GVHD are based on disease classification, overall grading, organs involved, and associated symptoms. SUMMARY Graft-versus-host disease (GVHD) is a serious complication that can occur after solid organ and allogenic HSCT. Intestinal or multivisceral transplantation have the highest incidence of GVHD among all solid organ transplants with very high mortality rates. Increased risk of GVHD is present after multivisceral and liver-included transplants compared with isolated intestinal transplant. Visceral transplantation is the all-encompassing term used for transplant that includes small bowel. It includes isolated small bowel transplant, small bowel/pancreas transplant, liver/small bowel/ pancreas transplant, stomach/pancreas/small bowel (modified multivisceral transplant), and stomach/liver/pancreas/small bowel (multivisceral). Each of these may or may not include the colon as part of the allograft. Steroids remain the first line of treatment along with modulation of the primary immunosuppression. Steroid-refractory patients remain a challenge and, to date, no consensus has been achieved for a single agent second-line therapy. Successful outcome depends on early diagnosis and prompt treatment.
-
6.
Performance measures for small-bowel endoscopy: A European Society of Gastrointestinal Endoscopy (ESGE) Quality Improvement Initiative.
Spada, C, McNamara, D, Despott, EJ, Adler, S, Cash, BD, Fernández-Urién, I, Ivekovic, H, Keuchel, M, McAlindon, M, Saurin, JC, et al
United European gastroenterology journal. 2019;(5):614-641
-
-
Free full text
-
Abstract
The European Society of Gastrointestinal Endoscopy (ESGE) together with the United European Gastroenterology (UEG) recently developed a short list of performance measures for small-bowel endoscopy (i.e. small-bowel capsule endoscopy and device-assisted enteroscopy) with the final goal of providing endoscopy services across Europe with a tool for quality improvement. Six key performance measures both for small-bowel capsule endoscopy and for device-assisted enteroscopy were selected for inclusion, with the intention being that practice at both a service and endoscopist level should be evaluated against them. Other performance measures were considered to be less relevant, based on an assessment of their overall importance, scientific acceptability, and feasibility. Unlike lower and upper gastrointestinal endoscopy, for which performance measures had already been identified, this is the first time small-bowel endoscopy quality measures have been proposed.
-
7.
Needs-based quality of life in adults dependent on home parenteral nutrition.
Burden, ST, Jones, DJ, Gittins, M, Ablett, J, Taylor, M, Mountford, C, Tyrrell-Price, J, Donnellan, C, Leslie, F, Bowling, T, et al
Clinical nutrition (Edinburgh, Scotland). 2019;(3):1433-1438
Abstract
BACKGROUND & AIMS Home parenteral nutrition (HPN) provides life sustaining treatment for people with chronic intestinal failure. Individuals may require HPN for months or years and are dependent on regular intravenous infusions, usually 12-14 h overnight between 1 and 7 days each week. This regime can have adverse impact on the life of people dependent on the treatment. The aim of this study was to establish mean values for the Parenteral Nutrition Impact Questionnaire (PNIQ) and to determine the effect of disease, frequency of infusions per week and patient characteristics on quality of life of patients fed HPN. METHOD The PNIQ was distributed to patients across nine UK HPN clinics. Data were analysed using linear regression, with PNIQ score as the dependent variable and potential confounders as independent variables. Unadjusted and adjusted models are presented. Higher PNIQ scores reflect poorer quality of life. RESULTS Completed questionnaires were received from 466 people dependent on HPN. Mean PNIQ score was 11.04 (SD 5.79). A higher PNIQ score (effect size 0.52, CI 0.184 to 0.853) was recorded in those dependent on a higher frequency of HPN infusions per week. Respondents with cancer had a similar mean PNIQ score to those with inflammatory bowel disease (mean 10.82, SD 6.00 versus 11.04, SD 5.91). Those with surgical complications reported a poorer QoL (effect size 3.03, CI 0.642 to 5.418) and those with severe gastro-intestinal dysmotility reported a better QoL (effect size -3.03, CI -5.593 to -0.468), compared to other disease states. CONCLUSIONS This large cohort study of quality of life in chronic intestinal failure demonstrates that HPN impacts individuals differently depending on their underlying disease. Furthermore, since the number of HPN infusions required per week is inversely related to an individual's needs-based quality of life, therapies that reduce PN burden should lead to an improvement in QoL.
-
8.
Therapeutic potential of natural plant products and their metabolites in preventing radiation enteropathy resulting from abdominal or pelvic irradiation.
Pathak, R, Shah, SK, Hauer-Jensen, M
International journal of radiation biology. 2019;(4):493-505
-
-
Free full text
-
Abstract
Radiation-induced gastrointestinal injury or radiation enteropathy is an imminent risk during radiation therapy of abdominal or pelvic tumors. Despite remarkable technological advancements in image-guided radiation delivery techniques, the risk of intestinal injury after radiotherapy for abdominal or pelvic cancers has not been completely eliminated. The irradiated intestine undergoes varying degrees of adverse structural and functional changes, which can result in transient or long-term complications. The risk of development of enteropathy depends on dose, fractionation, and quality of radiation. Moreover, the patients' medical condition, age, inter-individual sensitivity to radiation and size of the treatment area are also risk factors of radiation enteropathy. Therefore, strategies are needed to prevent radiotherapy-induced undesirable alteration in the gastrointestinal tract. Many natural plant products, by virtue of their plethora of biological activities, alleviate the adverse effects of radiation-induced injury. The current review discusses potential roles and possible mechanisms of natural plant products in suppressing radiation enteropathy. Natural plant products have the potential to suppress intestinal radiation toxicity.
-
9.
The hepatobiliary complications of malnutrition and nutritional support in adults.
McNeice, A, Scott, R, Rafferty, GP, Cash, WJ, Turner, GB
Irish journal of medical science. 2019;(1):109-117
Abstract
Hepatobiliary complications of hypoalimentation and parenteral nutrition (PN) are widely recognised. Hypoalimentation includes conditions such as anorexia nervosa (AN), obesity malnutrition and liver disease following bariatric surgery. Treatment of the underlying condition causing hypoalimentation can result in an improvement in liver dysfunction. Liver function test abnormalities are also commonly found in patients on PN, with the three main complications being steatosis, cholestasis and biliary system sludge/stones. Patients with intestinal failure receiving PN often have multiple possible aetiologies for liver dysfunction (rather than solely caused by the PN); hence, it is now more commonly referred to as intestinal failure-associated liver disease (IFALD). Liver enzyme abnormalities are very common with long-term PN use and do not always help with monitoring progression of IFALD. A systematic approach is required for investigating liver function abnormalities related to PN. The key management in IFALD is through prevention of sepsis, promoting intestinal health and restoring intestinal continuity where possible. A variety of imaging modalities can be used to investigate, and monitor, the liver disease. Most importantly, patients on PN for more than 28 days should be managed in a large centre with experience in managing intestinal failure to minimise the risk of such complications. Early identification of liver dysfunction is essential and, should it progress despite the above measures, early discussion with an intestinal transplant centre should be encouraged.
-
10.
Simulated amniotic fluid-like solution given enterally to neonates after obstructive bowel surgeries: A randomized controlled trial.
El-Farrash, RA, Gad, GI, Abdelkader, HM, Salem, DAD, Fahmy, SA
Nutrition (Burbank, Los Angeles County, Calif.). 2019;:187-191
Abstract
OBJECTIVES Withholding postoperative feeding is common in neonates recovering from surgeries for congenital abnormalities of the gastrointestinal tract (GIT), which leads to prolonged exposure to total parenteral nutrition, intestinal atrophy, and feeding intolerance. Because amniotic fluid plays a significant role in fetal gut maturation and development, the aim of this study was to test a hypothesis suggesting that feeding tolerance could be improved in neonates recovering from surgeries for congenital obstructive bowel abnormalities by enteral administration of simulated amniotic fluid-like solution given enterally (SAFE) containing recombinant human granulocyte colony-stimulating factor and erythropoietin. METHODS This prospective, double-blind, randomized, placebo-controlled trial was conducted with 40 late preterm/term neonates recovering from GIT surgeries. Neonates were randomly divided postoperatively into two groups: 20 neonates received the test solution (SAFE group) and 20 neonates received distilled water (placebo group) with a gestational age range (34.3-40.4 versus 34-40 wk, respectively) and mean gestational age (37.10 ± 1.68 versus 36.90 ± 1.83 wk, respectively). Treatment was started postoperatively and the test solution (or distilled water) was discontinued when daily enteral intake reached 100 mL/kg. RESULTS The study group showed better feeding tolerance as demonstrated as reflected by an earlier achievement of 50, 100, 120, and 150 mL/kg enteral feeding per day with a higher enteral caloric intake on day 7 post SAFE administration and a higher rate of weight gain (P < 0.05 for all). CONCLUSION Enteral administration of SAFE may improve postoperative feeding tolerance, enteral caloric intake, and weight gain.