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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.
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2.
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.
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3.
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.
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4.
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
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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.
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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
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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.
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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.
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Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Technical Review.
Rondonotti, E, Spada, C, Adler, S, May, A, Despott, EJ, Koulaouzidis, A, Panter, S, Domagk, D, Fernandez-Urien, I, Rahmi, G, et al
Endoscopy. 2018;(4):423-446
Abstract
SMALL-BOWEL CAPSULE ENDOSCOPY (SBCE): 1: ESGE recommends that prior to SBCE patients ingest a purgative (2 L of polyethylene glycol [PEG]) for better visualization.Strong recommendation, high quality evidence.However, the optimal timing for taking purgatives is yet to be established. 2: ESGE recommends that SBCE should be performed as an outpatient procedure if possible, since completion rates are higher in outpatients than in inpatients.Strong recommendation, moderate quality evidence. 3: ESGE recommends that patients with pacemakers can safely undergo SBCE without special precautions.Strong recommendation, low quality evidence. 4: ESGE suggests that SBCE can also be safely performed in patients with implantable cardioverter defibrillators and left ventricular assist devices.Weak recommendation, low quality evidence. 5: ESGE recommends the acceptance of qualified nurses and trained technicians as prereaders of capsule endoscopy studies as their competency in identifying pathology is similar to that of medically qualified readers. The responsibility of establishing a diagnosis must however remain with the attending physician.Strong recommendation, moderate quality evidence. 6: ESGE recommends observation in cases of asymptomatic capsule retention.Strong recommendation, moderate quality evidence.In cases where capsule retrieval is indicated, ESGE recommends the use of device-assisted enteroscopy as the method of choice.Strong recommendation, moderate quality evidence. DEVICE-ASSISTED ENTEROSCOPY (DAE): 1: ESGE recommends performing diagnostic DAE as a day-case procedure in patients without significant underlying co-morbidities; in patients with co-morbidities and/or those undergoing a therapeutic procedure, an inpatient stay is recommended.Strong recommendation, low quality evidenceThe choice between different settings also depends on sedation protocols.Strong recommendation, low quality evidence. 2: ESGE suggests that conscious sedation, deep sedation, and general anesthesia are all acceptable alternatives: the choice between them should be governed by procedure complexity, clinical factors, and local organizational protocols.Weak recommendation, low quality evidence. 3: ESGE recommends that the findings of previous diagnostic investigations should guide the choice of insertion route.Strong recommendation, moderate quality evidence.If the location of the small-bowel lesion is unknown or uncertain, ESGE recommends that the antegrade route should be generally preferred.Strong recommendation, low quality evidence.In the setting of massive overt bleeding, ESGE recommends an initial antegrade approach.Strong recommendation, low quality evidence. 4: ESGE recommends that, for balloon-assisted enteroscopy (i. e., single-balloon enteroscopy [SBE] and double-balloon enteroscopy [DBE]), small-bowel insertion depth should be estimated by counting net advancement of the enteroscope during the insertion phase, with confirmation of this estimate during withdrawal.Strong recommendation, low quality evidence.ESGE recommends that, for spiral enteroscopy, insertion depth should be estimated during withdrawal.Strong recommendation, moderate quality evidence. Since the calculated insertion depth is only a rough estimate, ESGE recommends placing a tattoo to mark the identified lesion and/or the deepest point of insertion.Strong recommendation, low quality evidence. 5: ESGE recommends that all endoscopic therapeutic procedures can be undertaken at the time of DAE.Strong recommendation, moderate quality evidence.Moreover, when therapeutic interventions are performed, additional specific safety measures are needed to prevent complications.Strong recommendation, high quality evidence.
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Pediatric Intestinal Transplantation.
Celik, N, Mazariegos, GV, Soltys, K, Rudolph, JA, Shi, Y, Bond, GJ, Sindhi, R, Ganoza, A
Gastroenterology clinics of North America. 2018;(2):355-368
Abstract
Pediatric intestinal transplantation has moved from the theoretic to an actual therapy for children with irreversible intestinal failure who are suffering from complications of total parenteral nutrition. Owing to significant advancement in the management of intestinal failure and prevention of parenteral nutrition-related complications that have led to reduction in incidence of parenteral nutrition-associated liver disease and have improved intestinal adaptation, the indications for intestinal transplantation are evolving. Long-term outcomes have improved, but challenges in long-term graft function owing to chronic rejection and immunosuppressant-related complications remain the major opportunities for improvement.
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Insights into medical management of pediatric intestinal failure.
Oliveira, SB, Cole, CR
Seminars in pediatric surgery. 2018;(4):256-260
Abstract
Medical management of children with Intestinal failure continues to evolve. The development of specialized teams focused on the management of these children has made the most significant impact in improving outcomes. Medical management strategies are centered on the provision of adequate fluid electrolytes and calories to allow for appropriate growth and neurological development. Enteral therapy and drugs are required to enhance bowel adaptation while parenteral nutrition is the main source of nutrients, electrolytes and fluid. Modification in parenteral nutrition with the availability of lipid alternatives are contributing to decreasing incidence of Intestinal failure associated liver disease. Utilization of patient centered central line care bundles has also significantly contributed to the decrease in morbidity and mortality. This review provides insight into the current medical therapy available for managing intestinal failure in children.
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Skeletal muscle-gut axis: emerging mechanisms of sarcopenia for intestinal and extra intestinal diseases.
de Sire, R, Rizzatti, G, Ingravalle, F, Pizzoferrato, M, Petito, V, Lopetuso, L, Graziani, C, de Sire, A, Mentella, MC, Mele, MC, et al
Minerva gastroenterologica e dietologica. 2018;(4):351-362
Abstract
In recent years, there has been an increasing interest on muscle wasting, considering the reduction of quality of life and the increase of morbidity and mortality associated. Sarcopenia and cachexia represent two conditions of reduction of muscle mass, sharing several elements involved in their pathogenesis, such as systemic inflammation, impaired muscle protein synthesis, increased muscle apoptosis, mitochondrial dysfunction in skeletal muscle tissue and insulin resistance. These features often characterize cancer, inactivity or denervation, but also inflammatory diseases, such as chronic obstructive pulmonary disease, renal failure, cardiac failure, rheumatoid arthritis, inflammatory bowel disease and aging in general. The gastrointestinal tract and gut microbiota are thought to be deeply associated with muscle function and metabolism, although the exact mechanisms that link gut with skeletal muscle are still not well known. This review summarized the potential pathways linking gut with muscle, in particular in conditions as sarcopenia and cachexia. The main emerging pathways implicated in the skeletal muscle-gut axis are: the myostatin/activin signaling pathway, the IGF1/PI3K/AKT/mTOR signaling pathway, which results suppressed, the NF-kB signaling pathway and the FOXO signaling pathway. Further researches in this field are necessary to better explain the linkage between gut microbiota and muscle wasting and the possible emerging therapies associated.