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1.
The Role of the Gluten-Free Diet in the Management of Seronegative Enteropathy.
Szaflarska-Popławska, A
Nutrients. 2021;(11)
Abstract
The differential diagnosis and treatment of seronegative enteropathy, also termed seronegative villous atrophy (SNVA), is a clinical challenge. Although seronegative coeliac disease (CD) is a frequent cause of SNVA, the aetiology can include immune-mediated, inflammatory, infectious, and drug-related forms. As a misdiagnosis of SNVA can result in patients being unnecessarily placed on a lifelong strict gluten-free diet or even given incorrect immunosuppressive therapy, the aim of this paper is to provide an evidence-based and practical approach for the workup and management of SNVA.
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2.
The Interrelationships between Intestinal Permeability and Phlegm Syndrome and Therapeutic Potential of Some Medicinal Herbs.
Park, J, Choi, TJ, Kang, KS, Choi, SH
Biomolecules. 2021;(2)
Abstract
The gastrointestinal (GI) tract has an intriguing and critical role beyond digestion in both modern and complementary and alternative medicine (CAM), as demonstrated by its link with the immune system. In this review, we attempted to explore the interrelationships between increased GI permeability and phlegm, an important pathological factor in CAM, syndrome, and therapeutic herbs for two disorders. The leaky gut and phlegm syndromes look considerably similar with respect to related symptoms, diseases, and suitable herbal treatment agents, including phytochemicals even though limitations to compare exist. Phlegm may be spread throughout the body along with other pathogens via the disruption of the GI barrier to cause several diseases sharing some parts of symptoms, diseases, and mechanisms with leaky gut syndrome. Both syndromes are related to inflammation and gut microbiota compositions. Well-designed future research should be conducted to verify the interrelationships for evidence based integrative medicine to contribute to the promotion of public health. In addition, systems biology approaches should be adopted to explore the complex synergistic effects of herbal medicine and phytochemicals on conditions associated with phlegm and leaky gut syndromes.
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3.
Cerebrotendinous xanthomatosis, sitosterolemia, Smith-Lemli-Opitz syndrome and the seminal contributions of Gerald Salen, MD (1935-2020).
Schaefer, EJ, Tint, GS, Duell, PB, Steiner, RD
Journal of clinical lipidology. 2021;(4):540-544
Abstract
Cerebrotendinous xanthomatosis (CTX), sitosterolemia, and Smith-Lemli Opitz syndrome (SLOS) are rare inborn errors of metabolism. The diagnoses of CTX and sitosterolemia are often delayed for many years because of lack of physician awareness, often resulting in significant and unnecessary progression of disease. CTX may present with chronic diarrhea, juvenile onset cataracts, strikingly large xanthomas, and neurologic disease in the setting of a normal serum cholesterol, but markedly elevated serum or plasma cholestanol levels. These patients have a defect in producing the bile acid chenodoxycholate, and oral chenodeoxycholate therapy is essential for these patients in order to prevent neurologic complications. Sitosterolemia can present with xanthomas, anemia, thrombocytopenia, splenomegaly, very premature heart disease, and serum cholesterol levels that may be normal or elevated, along with marked elevations of plasma β-sitosterol. These patients have a defect causing overabsorption of β-sitosterol, and the treatment of choice is oral ezetimibe. SLOS presents with growth delay, intellectual disability, multiple structural anomalies, and low serum cholesterol levels, and the defect is reduced cholesterol production. Treatment consists of dietary cholesterol supplementation and oral bile acid therapy which raises serum cholesterol levels and may improve symptoms. The metabolic and genetic defects in these disorders have been defined. There is no one in our field that has contributed more to the diagnosis and treatment of these disorders than Gerald Salen, MD, who died in late 2020 at 85 years of age. He will be greatly missed by his family, friends, and colleagues from around the world.
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4.
A detailed analysis of the current status of intestinal transplantation in the middle east.
Nikeghbalian, S, Arasteh, P, Nikoupour, H
Current opinion in organ transplantation. 2020;(2):169-175
Abstract
PURPOSE OF REVIEW Intestinal transplantations are among the most complex transplantations, which are performed in few centers in the world. When patients develop intestinal failure, different treatment modalities including parenteral nutrition, autologous gastrointestinal tract reconstructive surgery, and intestinal transplantations are considered. The Middle East is a region where reports on intestinal failures and intestinal transplantations are mainly lacking. In the present review, we highlighted the status of intestinal transplantations in the Middle East and focused on existing reports from this region. RECENT FINDINGS Very few countries in the Middle East have the facilities for home parenteral nutrition and only two countries including Iran and Turkey perform intestinal transplantations in the region. With advances in intestinal rehabilitation units and development of autologous gastrointestinal tract reconstructive surgery, some centers have been able to reduce the number of patients in need of intestinal transplantations. SUMMARY An overview of the condition of intestinal transplantations in the Middle East shows that the issue of intestinal failure and the treatment facilities still remain an unsolved problem. Although there exists a high need for intestinal transplantation, advances in reconstructive surgeries and the development of parenteral nutrition in this region can significantly reduce the need for intestinal transplantations among patients with intestinal failure.
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5.
[Evolution of patients with home parenteral nutrition: a systematic review of available studies].
Seisdedos Elcuaz, R, Mejías Trueba, M, Rodríguez Ramallo, H, Pérez Blanco, JL, Pereira Cunill, JL, García Luna, PP
Nutricion hospitalaria. 2020;(3):599-608
Abstract
Introduction: home parenteral nutrition (HPN) is a fundamental treatment for patients with intestinal failure who do not require hospitalization. We aimed to conduct an epidemiological and clinical practice analysis of HPN through a systematic review. Methods: the systematic review was conducted according to the PRISMA guidelines. A search was performed using the Healthcare Databases Advanced Search of PubMed® and EMBASE®, to identify articles which followed patients treated with HPN for at least 5 years, published between 2009 and 2019 in English or Spanish language. In addition, we manually retrieved other publications of interest. We excluded articles about subgroups of patients with a specific pathology, cancer or pregnant patients. We excluded studies collecting exclusively HPN complications. Results: a total of 267 references were identified, of which 9 met criteria (3 of pediatric population and 6 of adults). In adults, the main difference found between publications was the underlying pathologies. The most common indication was short bowel syndrome and the main cause of exitus was the underlying pathology. Most of the pediatric patients received this support in the first months of life. In addition, children showed a higher conversion rate to oral intake and a lower number of deaths when compared to adults. Conclusions: the use of HPN in cancer pathology is subject to wide geographic variability. It would be advisable to establish indication guidelines in patients with cancer and conduct quality studies, which provide rigorous and homogeneous information.
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6.
Nutritional Feeding Strategies in Pediatric Intestinal Failure.
Olieman, J, Kastelijn, W
Nutrients. 2020;(1)
Abstract
Intestinal failure is defined as a critical reduction of the gut mass or function, below the minimum needed to absorb nutrients and fluids. The ultimate goal in intestinal failure is to promote bowel adaptation and reach enteral autonomy while a healthy growth and development is maintained. The condition is heterogeneous and complex. Therefore, recommendations for the type and duration of parenteral, enteral, and oral nutrition are variable, with the child's age as an additional key factor. The aim of this review is to provide an overview of nutritional feeding strategies in this heterogeneous population. Different perspectives on nutritional management, nutrition and adaptation, and microbiome and nutrition will be discussed.
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7.
Current status of pediatric intestinal transplantation in the United States.
Venick, RS, Duggan, E, Whatley, J
Current opinion in organ transplantation. 2020;(2):201-207
Abstract
PURPOSE OF REVIEW The present review aims to describe in detail the characteristics, outcomes, and recent trends in the field of pediatric intestinal transplantation in the United States. It will examine the route cause and future implications of these developments. The review will draw from recent publications in the field, the Intestinal Transplant Registry, and contemporary data from large U.S. single centers. RECENT FINDINGS More than 1500 pediatric intestinal transplants have been performed in the United States since 1985, however, over the past decade there have been fewer than 50 transplants/year nationwide. This trend is largely a result of stagnant long-term ITx outcomes and advancements in intestinal rehabilitation programs. Nationally the overall 1-year and 5-year graft survival are 68 and 50% respectively, whereas certain high-volume centers have experienced significantly better results. Sepsis is the leading cause of death following pediatric ITx, whereas rejection is the leading cause of graft loss. Chronic kidney disease and posttransplant lymphoproliferative disorder are significant and relatively prevalent long-term complications. The majority of pediatric ITx recipients receive T-cell depleting induction agents and are on Tacrolimus-based immunosuppression. Most recipient are off parenteral nutrition, but may require supplemental tube feeds. Many pediatric ITx recipients require special education, and in certain domains some report lower health related quality of life. SUMMARY As intestinal rehabilitation has improved in the modern era, the volume of pediatric ITx in the United States has decreased. Although pediatric ITx results have room for improvement nationwide, successful outcomes have been reported at experienced American centers.
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8.
A detailed analysis of the current status of intestinal transplantation in Latin America.
Gondolesi, GE, Doeyo, M, Solar-Muñiz, H
Current opinion in organ transplantation. 2020;(2):189-195
Abstract
PURPOSE OF REVIEW Latin America and the Caribbean represent a vast territory, with very different economic and healthcare realities, which result in significant disparities in the management of intestinal failure patients throughout the region. Since 1968, multiple attempts have been done to accomplish a successful intestinal transplant; but it was not until 2004, with the establishment of multidisciplinary programs, that large series with long-term results could be obtained. Currently, three countries (Colombia, Argentina, and Brazil) in the region are actively performing these procedures. RECENT FINDINGS A total number of 135 intestinal transplants have been performed; 11 attempts before 2004, and 124 after that period, 66 transplants were done in Argentina (42 in children), 40 in Colombia, 15 in Brazil (1 child), 2 in Costa Rica and 1 in México; 76% have been isolated, whereas 2 were done with living donors. SUMMARY Publications are still scarce, and compliance to existing registries remains limited. The challenge for the next years is to develop more 'comprehensive units' and extend home parenteral nutrition availability in the rest of the region. Regional cooperation and networking need to be set, in order to achieve regional self-sufficiency and improve long-term results.
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9.
The Role of a Nutrition Support Team in the Management of Intestinal Failure Patients.
Vlug, LE, Nagelkerke, SCJ, Jonkers-Schuitema, CF, Rings, EHHM, Tabbers, MM
Nutrients. 2020;(1)
Abstract
Parenteral nutrition (PN) is a complex and specialized form of nutrition support that has revolutionized the care for both pediatric and adult patients with acute and chronic intestinal failure (IF). This has led to the development of multidisciplinary teams focused on the management of patients receiving PN: nutrition support teams (NSTs). In this review we aim to discuss the historical aspects of IF management and NST development, and the practice, composition, and effectiveness of multidisciplinary care by NSTs in patients with IF. We also discuss the experience of two IF centers as an example of contemporary NSTs at work. An NST usually consists of at least a physician, nurse, dietitian, and pharmacist. Multidisciplinary care by an NST leads to fewer complications including infection and electrolyte disturbances, and better survival for patients receiving short- and long-term PN. Furthermore, it leads to a decrease in inappropriate prescriptions of short-term PN leading to significant cost reduction. Complex care for patients receiving PN necessitates close collaboration between team members and NSTs from other centers to optimize safety and effectiveness of PN use.
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10.
Validation of the Micronutrient and Environmental Enteric Dysfunction Assessment Tool and evaluation of biomarker risk factors for growth faltering and vaccine failure in young Malian children.
Arndt, MB, Cantera, JL, Mercer, LD, Kalnoky, M, White, HN, Bizilj, G, Boyle, DS, de Hostos, EL, Choy, RKM
PLoS neglected tropical diseases. 2020;(9):e0008711
Abstract
Environmental enteric dysfunction (EED) is an intestinal disorder common among children in low-resource settings and is associated with increased risk of growth stunting, cognitive deficits, and reduced oral vaccine immunogenicity. The Micronutrient and EED Assessment Tool (MEEDAT) is a multiplexed immunoassay that measures biomarkers previously associated with child growth faltering and/or oral vaccine immunogenicity: intestinal fatty acid-binding protein (I-FABP), soluble CD14 (sCD14), insulin-like growth factor 1 (IGF-1), and fibroblast growth factor 21 (FGF21). MEEDAT also measures systemic inflammation (α1-acid glycoprotein, C-reactive protein), ferritin, soluble transferrin receptor, retinol binding protein 4, thyroglobulin, and Plasmodium falciparum antigenemia (histidine-rich protein 2). The performance of MEEDAT was compared with commercially available enzyme-linked immunosorbent assays (ELISAs) using 300 specimens from Malian infant clinical trial participants. Regression methods were used to test if MEEDAT biomarkers were associated with seroconversion to meningococcal A conjugate vaccine (MenAV), yellow fever vaccine (YFV), and pentavalent rotavirus vaccine (PRV) after 28 days, or with growth faltering over 12 weeks. The Pearson correlations between the MEEDAT and ELISA results were 0.97, 0.86, 0.80, and 0.97 for serum I-FABP, sCD14, IGF-1, and FGF21, respectively. There were significant associations between I-FABP concentration and the probability of PRV IgG seroconversion and between IGF-1 concentration and the probability of YFV seroconversion. In multivariable models neither association remained significant, however there was a significant negative association between AGP concentration and YFV seroconversion. GLP-2 and sCD14 concentrations were significantly negatively associated with 12-week change in weight-for-age z-score and weight-for-height z-score in multivariable models. MEEDAT performed well in comparison to commercially-available ELISAs for the measurement of four analytes for EED and growth hormone resistance. Adoption of MEEDAT in low-resource settings could help accelerate the identification of interventions that prevent or treat child stunting and interventions that boost the immunogenicity of child vaccinations.