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New Insights Into Intestinal Failure-Associated Liver Disease in Children.
Khalaf, RT, Sokol, RJ
Hepatology (Baltimore, Md.). 2020;(4):1486-1498
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Abstract
Development of intestinal failure-associated liver disease (IFALD) is a common complication of long-term parenteral nutrition (PN) in children and adults. The molecular and cellular mechanisms and the phases of IFALD are now being delineated. Components of PN lipid emulsions, including plant sterols, interact with hepatic innate immune activation promoted by products of gut bacterial overgrowth/dysbiosis and altered intestinal barrier function (gut-liver axis) and by episodes of sepsis to cause cholestasis and IFALD. New therapeutic strategies, including modifications of intravenous lipid emulsions to reduce pro-inflammatory fatty acids and plant sterol content, can lower the risk of IFALD, reverse cholestasis, and reduce complications, although the significance of persisting hepatic fibrosis is unknown. This review will provide an update on advances in the pathogenesis of IFALD, newer therapeutic and preventative strategies, and challenges that confront managing patients with IFALD.
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Mechanisms of Parenteral Nutrition-Associated Liver and Gut Injury.
Madnawat, H, Welu, AL, Gilbert, EJ, Taylor, DB, Jain, S, Manithody, C, Blomenkamp, K, Jain, AK
Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2020;(1):63-71
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Abstract
Parenteral nutrition (PN) has revolutionized the care of patients with intestinal failure by providing nutrition intravenously. Worldwide, PN remains a standard tool of nutrition delivery in neonatal, pediatric, and adult patients. Though the benefits are evident, patients receiving PN can suffer serious cholestasis due to lack of enteral feeding and sometimes have fatal complications from liver injury and gut atrophy, including PN-associated liver disease or intestinal failure-associated liver disease. Recent studies into gut-systemic cross talk via the bile acid-regulated farnesoid X receptor (FXR)-fibroblast growth factor 19 (FGF19) axis, gut microbial control of the TGR5-glucagon-like peptide (GLP) axis, sepsis, and role of prematurity of hepatobiliary receptors are greatly broadening our understanding of PN-associated injury. It has also been shown that the composition of ω-6/ω-3 polyunsaturated fatty acids given parenterally as lipid emulsions can variably drive damage to hepatocytes and cell integrity. This manuscript reviews the mechanisms for the multifactorial pathogenesis of liver disease and gut injury with PN and discusses novel ameliorative strategies.
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When is parenteral nutrition indicated in the hospitalized, acutely ill patient?
Fragkos, KC, Sebepos-Rogers, G, Rahman, F
Current opinion in gastroenterology. 2020;(2):129-135
Abstract
PURPOSE OF REVIEW The current review discusses current practices regarding appropriate indications for parenteral nutrition in acutely ill hospitalized patients. We address-specific indications for parenteral nutrition in the perioperative period, and in inflammatory bowel disease, oncology, hepatobiliary, critical care and end-stage renal disease patients. RECENT FINDINGS Acutely ill hospitalized patients can develop intestinal failure requiring parenteral nutrition. Recent studies have provided insight into the main indications. The most common indications for inpatient parenteral nutrition include postsurgical complications, including prolonged ileus, sepsis, fistula and leaks, and bowel obstruction, predominantly malignant. Severe or complicated inflammatory bowel disease and cancer treatment-related mucosal enteropathies (mucositis, enterocolitis, gut graft-versus-host disease) are the next commonest indications. Less frequent indications are primary motility disorders and inability to secure enteral access for enteral nutrition. Gastrointestinal failure of the intensive care patient is a separate entity resulting from multiple mechanisms, including an enteropathy and dysmotility. SUMMARY Despite the wider availability of nutrition support teams, use of parenteral nutrition is not without risk. The risks and benefits of parenteral nutrition in the acute setting need to be carefully considered even when it is indicated.
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Parenteral nutrition.
Bozzetti, F
Nutrition (Burbank, Los Angeles County, Calif.). 2019;:101-107
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Parenteral nutrition use in children with cancer.
McGrath, KH
Pediatric blood & cancer. 2019;(12):e28000
Abstract
Multiple disease and treatment-related factors contribute to intestinal insult and influence the nutritional status of children with cancer. Many children with cancer will experience intestinal dysfunction during their cancer journey and children with cancer are a common referral group for inpatient parenteral nutrition. Appropriate use of parenteral nutrition in children with cancer and intestinal failure may alleviate malnutrition and associated risks. However, proper selection of patients, correct parenteral nutrition prescription, and close monitoring is important to avoid unnecessary intestinal failure or parenteral nutrition-related complications, minimize long-term nutritional sequelae or additional costs to health services, and optimize intestinal rehabilitation.
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Evaluation and Management of Enterocutaneous Fistula.
Bhama, AR
Diseases of the colon and rectum. 2019;(8):906-910
Abstract
A 36-year-old woman presents with an abscess at her midline wound 4 weeks following an ileocecectomy for Crohn's disease. After the abscess is incised, there is purulent drainage followed by the drainage of enteric contents; the output is 750 mL per 24 hours.
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Pseudo-obstruction, enteric dysmotility and irritable bowel syndrome.
Lindberg, G
Best practice & research. Clinical gastroenterology. 2019;:101635
Abstract
New diagnostic techniques have advanced our knowledge about the irritable bowel syndrome. The majority of patients that we believed to have a psychosomatic disorder have received other diagnoses explaining their symptoms. Endoscopy makes it possible to diagnose celiac disease before it leads to malnutrition and allows the detection of microscopic colitis as a cause of watery diarrhea. At the severe end of the symptom spectrum enteric dysmotility marks the border at which IBS ceases to be a functional disorder and becomes a genuine motility disorder. Joint hypermobility or Ehlers-Danlos syndrome is present in a substantial proportion of patients with enteric dysmotility. Chronic intestinal pseudo-obstruction is the end-stage of a large number of very rare disorders in which failed peristalsis is the common denominator. Nutritional needs and symptom control are essential in the management of pseudo-obstruction. Home parenteral nutrition is life saving in more than half of patients with chronic intestinal pseudo-obstruction.
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The Long Road to the Development of Effective Therapies for the Short Gut Syndrome: A Personal Perspective.
Jeppesen, PB
Digestive diseases and sciences. 2019;(10):2717-2735
Abstract
The ability to provide parenteral support represents a revolutionary change in medical therapy for patients with temporary and inadequate intestinal absorptive capacity or for patients with chronic intestinal failure due to digestive diseases. Nevertheless, due to the rarity of intestinal failure, a de facto policy of "discrimination by organ failure treatment" exists in many countries whereby this problem is under-recognized and under-treated. With the increasing recognition of the pathophysiological consequences of intestinal resection and the occurrence of new pro-adaptive treatments for patients suffering from short bowel syndrome, this review reflects on the history of developments in this area and discusses current practice and future directions of the field.
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Parenteral Nutrition Drug Shortages: A Single-Center Experience With Rapid Process Change.
Nystrom, EM, Bergquist, WJ, Wieruszewski, PM, McMahon, MM, Barreto, EF
JPEN. Journal of parenteral and enteral nutrition. 2019;(5):583-590
Abstract
Disruptions in the medication supply chain and consequent drug product shortages, including shortages of parenteral products used for parenteral nutrition (PN) compounding, have become an increasingly common occurrence. The amino acid solution shortage that resulted from the devastating impact of Hurricanes Maria and Irma on manufacturing facilities in Puerto Rico in 2017 necessitated a rapid, coordinated shift from use of compounded PN to commercial multichamber-bag PN (MCB-PN) at our hospitals. We describe our experience operationalizing this intervention via a framework that may be adapted for addressing other drug product shortages to promote rapid yet safe use of therapeutic alternatives.
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Pediatric Intestinal Failure: A Review of the Scope of Disease and a Regional Model of a Multidisciplinary Care Team.
Mangalat, N
Missouri medicine. 2019;(2):129-133
Abstract
The term "intestinal failure" signifies the inability of the body to meet the digestive, absorptive and nutritive needs of the body. In children, intestinal failure is most often due to short bowel syndrome, often a result of necrotizing enterocolitis, a severe GI ischemic pathology that is generally associated with prematurity. With advances in neonatal care, more preterm infants are surviving, and subsequently we care for more children with SBS than ever before. These children require parenteral nutrition (PN) for survival. Neurodevelopmental outcomes are tied to nutrition in early years; thus these children are the most vulnerable to the sequelae of intestinal failure. As such, the development of multi-disciplinary intestinal rehabilitation programs have emerged as the state of the art in the care of children with intestinal failure.