Evidence-based recommendations of the Andalusian Group for Nutrition Reflection and Investigation (GARIN) for the management of adult patients with short bowel syndrome / Recomendaciones basadas en la evidencia del Grupo Andaluz para la Reflexión e Investigación en Nutrición (GARIN) para el manejo del paciente con síndrome de intestino corto

In order to develop evidence-based recommendations and expert consensus for the nutritional management of patients with short bowel syndrome (SBS), we conducted a systematic literature search using the PRISMA methodology plus a critical appraisal following the GRADE scale procedures. Pharmacological treatment with antisecretory drugs, antidiarrheal drugs, and somatostatin contributes to reducing intestinal losses. Nutritional support is based on parenteral nutrition; however, oral intake and/or enteral nutrition should be introduced as soon as possible. In the chronic phase, the diet should have as few restrictions as possible, and be adapted to the SBS type. Home parenteral nutrition (HPN) should be individualized. Single-lumen catheters are recommended and taurolidine should be used for locking the catheter. The HPN's lipid content must be greater than 1 g/kg per week but not exceed 1 g/kg per day, and omega-6 fatty acids (ω6 FAs) should be reduced. Trace element vials with low doses of manganese should be used. Patients with chronic SBS who require long-term HPN/fluid therapy despite optimized treatment should be considered for teduglutide treatment. All patients require a multidisciplinary approach and specialized follow-up. These recommendations and suggestions regarding nutritional management in SBS patients have direct clinical applicability. (AU)

Con el fin de desarrollar recomendaciones basadas en la evidencia y el consenso de expertos para el manejo nutricional de los pacientes con síndrome de intestino corto (SIC), realizamos una búsqueda bibliográfica sistemática utilizando la metodología PRISMA junto a una valoración crítica siguiendo los procedimientos de la escala GRADE. El tratamiento farmacológico con fármacos antisecretores, antidiarreicos y somatostatina contribuye a reducir las pérdidas intestinales. El apoyo nutricional se basa en la nutrición parenteral; sin embargo, la ingesta oral y/o la nutrición enteral deben introducirse lo antes posible. En la fase crónica, la dieta debe tener las menores restricciones posibles y adaptarse al tipo de SIC. La nutrición parenteral domiciliaria (NPD) debe individualizarse. Se recomiendan catéteres de un solo lumen y se debe utilizar taurolidina para bloquear el catéter. El contenido de lípidos de la HPN debe ser superior a 1 g/kg por semana, pero no debe exceder 1 g/kg por día, y debe reducirse el ácido graso omega-6 (AG ω6). Deben utilizarse viales de oligoelementos con dosis bajas de manganeso. Los pacientes con SIC crónico que requieren NPD/fluidoterapia a largo plazo a pesar del tratamiento optimizado deben considerarse para el tratamiento con teduglutida. Todos los pacientes requieren un abordaje multidisciplinar y un seguimiento especializado. Estas recomendaciones y sugerencias con respecto al manejo nutricional de los pacientes con SIC tienen aplicabilidad clínica directa. (AU)

Nutrición parenteral domiciliaria en España, 2019: informe del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria NADYA / Home and Ambulatory Artificial Nutrition (NADYA) Group report: home parenteral nutrition in Spain, 2019

Objetivo: comunicar los datos de nutrición parenteral domiciliaria (NPD) obtenidos del registro del grupo NADYA-SENPE (www.nadya-senpe.com) del año 2019. Material y métodos: análisis descriptivo de los datos recogidos de pacientes adultos y pediátricos con NPD en el registro NADYA-SENPE desde el 1 de enero al 31 de diciembre de 2019. Resultados: se registraron 283 pacientes (51,9 %, mujeres), 31 niños y 252 adultos procedentes de 47 hospitales españoles, lo que representa una tasa de prevalencia de 6,01 pacientes/millón de habitantes/año 2019. El diagnóstico más frecuente en los adultos fue “oncológico paliativo” y “otros” (21,0 %). En los niños fue la enfermedad de Hirschsprung junto a la enterocolitis necrotizante, las alteraciones de la motilidad intestinal y la pseudoobstrucción intestinal crónica, con 4 casos cada uno (12,9 %). El primer motivo de indicación fue el síndrome del intestino corto tanto en los niños (51,6 %) como en los adultos (37,3 %). El tipo de catéter más utilizado fue el tunelizado tanto en los niños (75,9 %) como en los adultos (40,8 %). Finalizaron 68 episodios, todos en adultos: la causa más frecuente fue el fallecimiento (54,4 %). Pasaron a la vía oral el 38,2 %. Conclusiones: el número de centros y profesionales colaboradores con el registro NADYA va incrementándose. Se mantienen estables las principales indicaciones y los motivos de finalización de la NPD. (AU)

Aim: to communicate the home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2019. Material and methods: a descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1 to December 31, 2019. Results: a total of 283 patients (51.9 % women), 31 children, and 252 adults from 47 Spanish hospitals were registered, which represents a prevalence rate of 6.01 patients per million inhabitants for year 2019. The most frequent diagnosis in adults was “palliative oncological” and “others” (21.0 %). In children, it was Hirschsprung's disease together with necrotizing enterocolitis, alterations in intestinal motility, and chronic intestinal pseudo-obstruction, with 4 cases each (12.9 %). The first reason for the indication was short-bowel syndrome in both children (51.6 %) and adults (37.3 %). The most used type of catheter was tunnelled both in children (75.9 %) and in adults (40.8 %). Sixty-eight episodes ended, all in adults, and the most frequent cause was death (54.4 %); 38.2 % were switched to oral. Conclusions: the number of collaborating centers and professionals in the NADYA registry is increasing. The main indications and reasons for HPN termination remain stable. (AU)

[Home and Ambulatory Artificial Nutrition (NADYA) Group report: home parenteral nutrition in Spain, 2019]. / Nutrición parenteral domiciliaria en España, 2019: informe del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria NADYA.

INTRODUCTION: Aim: to communicate the home parenteral nutrition (HPN) data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2019. Material and methods: a descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1 to December 31, 2019. Results: a total of 283 patients (51.9 % women), 31 children, and 252 adults from 47 Spanish hospitals were registered, which represents a prevalence rate of 6.01 patients per million inhabitants for year 2019. The most frequent diagnosis in adults was "palliative oncological" and "others" (21.0 %). In children, it was Hirschsprung's disease together with necrotizing enterocolitis, alterations in intestinal motility, and chronic intestinal pseudo-obstruction, with 4 cases each (12.9 %). The first reason for the indication was short-bowel syndrome in both children (51.6 %) and adults (37.3 %). The most used type of catheter was tunnelled both in children (75.9 %) and in adults (40.8 %). Sixty-eight episodes ended, all in adults, and the most frequent cause was death (54.4 %); 38.2 % were switched to oral. Conclusions: the number of collaborating centers and professionals in the NADYA registry is increasing. The main indications and reasons for HPN termination remain stable.

INTRODUCCIÓN: Objetivo: comunicar los datos de nutrición parenteral domiciliaria (NPD) obtenidos del registro del grupo NADYA-SENPE (www.nadya-senpe.com) del año 2019. Material y métodos: análisis descriptivo de los datos recogidos de pacientes adultos y pediátricos con NPD en el registro NADYA-SENPE desde el 1 de enero al 31 de diciembre de 2019. Resultados: se registraron 283 pacientes (51,9 %, mujeres), 31 niños y 252 adultos procedentes de 47 hospitales españoles, lo que representa una tasa de prevalencia de 6,01 pacientes/millón de habitantes/año 2019. El diagnóstico más frecuente en los adultos fue "oncológico paliativo" y "otros" (21,0 %). En los niños fue la enfermedad de Hirschsprung junto a la enterocolitis necrotizante, las alteraciones de la motilidad intestinal y la pseudoobstrucción intestinal crónica, con 4 casos cada uno (12,9 %). El primer motivo de indicación fue el síndrome del intestino corto tanto en los niños (51,6 %) como en los adultos (37,3 %). El tipo de catéter más utilizado fue el tunelizado tanto en los niños (75,9 %) como en los adultos (40,8 %). Finalizaron 68 episodios, todos en adultos: la causa más frecuente fue el fallecimiento (54,4 %). Pasaron a la vía oral el 38,2 %. Conclusiones: el número de centros y profesionales colaboradores con el registro NADYA va incrementándose. Se mantienen estables las principales indicaciones y los motivos de finalización de la NPD.

Evidence-based recommendations of the Andalusian Group for Nutrition Reflection and Investigation (GARIN) for the management of adult patients with short bowel syndrome.

INTRODUCTION: In order to develop evidence-based recommendations and expert consensus for the nutritional management of patients with short bowel syndrome (SBS), we conducted a systematic literature search using the PRISMA methodology plus a critical appraisal following the GRADE scale procedures. Pharmacological treatment with antisecretory drugs, antidiarrheal drugs, and somatostatin contributes to reducing intestinal losses. Nutritional support is based on parenteral nutrition; however, oral intake and/or enteral nutrition should be introduced as soon as possible. In the chronic phase, the diet should have as few restrictions as possible, and be adapted to the SBS type. Home parenteral nutrition (HPN) should be individualized. Single-lumen catheters are recommended and taurolidine should be used for locking the catheter. The HPN's lipid content must be greater than 1 g/kg per week but not exceed 1 g/kg per day, and omega-6 fatty acids (ω6 FAs) should be reduced. Trace element vials with low doses of manganese should be used. Patients with chronic SBS who require long-term HPN/fluid therapy despite optimized treatment should be considered for teduglutide treatment. All patients require a multidisciplinary approach and specialized follow-up. These recommendations and suggestions regarding nutritional management in SBS patients have direct clinical applicability.

INTRODUCCIÓN: Con el fin de desarrollar recomendaciones basadas en la evidencia y el consenso de expertos para el manejo nutricional de los pacientes con síndrome de intestino corto (SIC), realizamos una búsqueda bibliográfica sistemática utilizando la metodología PRISMA junto a una valoración crítica siguiendo los procedimientos de la escala GRADE. El tratamiento farmacológico con fármacos antisecretores, antidiarreicos y somatostatina contribuye a reducir las pérdidas intestinales. El apoyo nutricional se basa en la nutrición parenteral; sin embargo, la ingesta oral y/o la nutrición enteral deben introducirse lo antes posible. En la fase crónica, la dieta debe tener las menores restricciones posibles y adaptarse al tipo de SIC. La nutrición parenteral domiciliaria (NPD) debe individualizarse. Se recomiendan catéteres de un solo lumen y se debe utilizar taurolidina para bloquear el catéter. El contenido de lípidos de la HPN debe ser superior a 1 g/kg por semana, pero no debe exceder 1 g/kg por día, y debe reducirse el ácido graso omega-6 (AG ω6). Deben utilizarse viales de oligoelementos con dosis bajas de manganeso. Los pacientes con SIC crónico que requieren NPD/fluidoterapia a largo plazo a pesar del tratamiento optimizado deben considerarse para el tratamiento con teduglutida. Todos los pacientes requieren un abordaje multidisciplinar y un seguimiento especializado. Estas recomendaciones y sugerencias con respecto al manejo nutricional de los pacientes con SIC tienen aplicabilidad clínica directa.

[Mechanism of gut-microbiota-liver axis in the pathogenesis of intestinal failure-associated liver disease].

Intestinal failure (IF) is defined as the critical reduction of functional intestines below the minimum needed to absorb nutrients and fluids, so that intravenous supplementation with parenteral nutrition (PN) is required to maintain health and/or growth. Although the benefits are evident, patients receiving PN can suffer from serious cholestasis due to lack of enteral feeding and small intestinal bacterial overgrowth (SIBO). One such complication that may arise is intestinal failure-associated liver disease (IFALD). Evidences from recent studies suggest that alterations in the intestinal microbiota, as well as intraluminal bile acid driven signaling, may play a critical role in both hepatic and intestinal injury. Since Marshall first proposed the concept of the gut-liver axis in 1998, the role of gut-liver axis disorders in the development of IFALD has received considerable attention. The conversation between gut and liver is the key to maintain liver metabolism and intestinal homeostasis, which influences each other and is reciprocal causation. However, as a "forgotten organ" , intestinal microbiota on the pathogenesis of IFALD has not been well reflected. As such, we propose, for the first time, the concept of gut-microbiota-liver axis to emphasize the importance of intestinal microbiota in the interaction of gut-liver axis. Analysis and research on gut-microbiota-liver axis will be of great significance for understanding the pathogenesis of IFALD and improving the prevention and treatment measures.

Controversy in Nutrition Recommendations for Short Bowel Syndrome: How Type of SBS Impacts Response.

PURPOSE OF REVIEW: This review examines the current recommendations for dietary management of patients living with short bowel syndrome (SBS) and outlines the need for future research to provide optimal care for this unique group of patients. RECENT FINDINGS: Providers caring for patients with SBS lack sufficient data to help guide recommendations regarding diet. The majority of studies are conducted at a single medical institution on a small number of anatomically diverse patients. Multi-center studies would allow for inclusion of a larger number of patients and may lead to more individualized dietary recommendations. Patients with short bowel syndrome should be evaluated on an individual basis by a multidisciplinary team including physicians, dieticians, pharmacists, and nurses specializing in the care of these complex patients. Tailoring both medical and nutritional therapy will help realize the overarching goal for these patients of maintaining adequate nutrition with diet and medications, and achieving independence from parenteral support.

Cardiac Changes in Patients on Long-Term Parenteral Nutrition.

Patients with short bowel syndrome (SBS) on long-term home parenteral nutrition (HPN) chronically receive high fluid volumes directly into the right atrium (RA) through the superior vena cava. We retrospectively evaluated cardiac function measured by routine transthoracic echocardiography (TTE) in a population of 26 SBS patients on long-term HPN and compared their data on echocardiograph-derived right heart structure and function, with those of a control group of 26 patients also bearing a central venous catheter (CVC) for other reasons. Results showed that body weight and BMI were significantly higher in the control group. The echocardiographic estimate of RA pressure was higher in HPN patients than in controls (p = 0.01). An increased estimate of RA pressure indicates the need to consider TTE in the follow-up of long-term HPN patients to detect functional impairment early.

Effect of oral citrulline supplementation on whole body protein metabolism in adult patients with short bowel syndrome: A pilot, randomized, double-blind, cross-over study.

BACKGROUND & AIMS: As citrulline is produced by small intestine, plasma citrulline concentration is decreased and may become essential in patients with short bowel syndrome (SBS). In a rat model of SBS, citrulline supplementation enhanced muscle protein synthesis. The aim of the study was to determine whether citrulline impacts whole body protein metabolism in patients with SBS. METHODS: Nine adults with non-malignant SBS (residual small bowel 90 ± 48 cm; mean ± SD) who were in near-normal nutritional status without any artificial nutrition, were recruited long after surgery. They received 7-day oral supplementation with citrulline (0.18 g/kg/day), or an iso-nitrogenous placebo in a randomized, double-blind, cross-over design with a 13-day wash-out between regimens, and an intravenous 5-h infusion of L-[1-13C]-leucine in the postabsorptive state to assess protein metabolism after each regimen. RESULTS: Plasma citrulline concentration rose 17-fold (25 ± 9 vs. 384 ± 95 µmol/L) and plasma arginine 3-fold after oral citrulline supplementation (both p < 4 × 10-6). Supplementation did not alter leucine appearance rate (97 ± 5 vs. 97 ± 5 µmol kg-1.h-1; p = 0.88), leucine oxidation (14 ± 1 vs. 12 ± 1 µmol kg-1.h-1; p = 0.22), or non-oxidative leucine disposal (NOLD), an index of whole-body protein synthesis (83 ± 4 vs. 85 ± 5 µmol kg-1.h-1; p = 0.36), nor insulin or IGF-1 plasma concentrations. In each of the 3 patients with baseline citrulline<20 µmol/L, citrulline supplementation increased NOLD. Among the 7 patients with plasma citrulline <30 µmol/L, the effect of supplementation on NOLD correlated inversely (r2 = 0.81) with baseline plasma citrulline concentration. CONCLUSION: 1) Oral citrulline supplementation enhances citrulline and arginine bioavailability in SBS patients. 2) Oral citrulline supplementation does not have any anabolic effect on whole body protein metabolism in patients with SBS in good nutritional status, in the late phase of intestinal adaptation, and with near-normal baseline citrulline homeostasis. 3) Whether oral citrulline would impact whole body protein anabolism in severely malnourished SBS patients in the early adaptive period, and with baseline plasma citrulline below 20 µmol/L, warrants further study. Registered under ClinicalTrials.gov Identifier no. NCT01386034.

High amylase resistant starch to decrease stool output in people with short bowel syndrome: A pilot trial.

Short bowel syndrome (SBS) is defined as having less than 200 cm of functional small bowel. Malabsorptive diarrhoea and dehydration are difficult to manage despite medical therapy and dietary manipulations. Evidence shows that supplementing the diet with High Amylase Resistant Starch (HARS) can reduce diarrhoea from a number of causes including gastroenteritis. It is hypothesised HARS will decrease stool output via the production of short chain fatty acids and the resultant increased water reabsorption. This study aimed to determine if the addition of HARS can reduce diarrhoea in patients with SBS. METHODS: Patients with SBS with colon in continuity were recruited from the intestinal rehabilitation clinic at Austin Health. The study was a 2 week crossover trial. Each participant completed the control and the intervention (addition of 50 g HARS to usual diet). Total daily stool weight and number of bowel actions per day were compared between groups using paired t-tests. RESULTS: Eight adults (58% male, mean age 55.7 yrs) were recruited. Five participants completed the trial. Total daily stool weight was reduced in all participants when consuming HARS. Mean daily stool output was significantly decreased 1049 ± 519 g/d to 804 ± 585 g/d (p = 0.023). Number of bowel actions per day showed a trend to reduction. CONCLUSION: This study gives some support to the hypothesis that the addition of HARS into the diet of patients with short bowel syndrome reduces stool output. Longer trials are required to confirm the effect on nutritional/hydration status.

Examining the pathophysiology of short bowel syndrome and glucagon-like peptide 2 analogue suitability in chronic intestinal failure: experience from a national intestinal failure unit.

INTRODUCTION: Short bowel syndrome (SBS) is a leading cause of intestinal failure (IF). Home parenteral nutrition (HPN) remains the standard treatment, with small intestinal transplantation reserved for cases with severe complications to HPN. There have recently been significant developments in growth factor therapy. We aimed to develop a greater contemporary understanding of our SBS-IF subset. METHOD: We performed a retrospective observational study of a prospectively maintained HPN audit database in October 2017. Intestinal anatomical details and parenteral requirements were recorded. Each case was assessed for eligibility for growth factor therapy using recently published trials. RESULTS: Of 273 patients receiving HPN, 152 (55.7%) had type three IF as a result of SBS (SBS-IF), with a mean duration of HPN of 61 months (range 4-416). Mean length of small intestine was 98 cm. Furthermore, 114 (41.8%) patients had an end jejunostomy (SBS-J), 18 (6.6%) had an end ileostomy, and 7.3% of patients had all or part of the colon-in-continuity. Crohn's disease was the most common underlying pathology. Univariate analysis for the whole HPN cohort demonstrated SBS-IF and a longer duration of HPN to be associated with higher PN energy requirements, p ≤ 0.0001. Of all, 73 (48%) patients with SBS-IF were deemed suitable for GLP-2 analogue therapy, with co-morbidity being the most frequent cause of non-suitability (29.1%). CONCLUSION: We describe a large U.K. HPN cohort using ESPEN pathophysiological and clinical severity classification. The majority of patients with SBS-IF had a jejunostomy and relatively few had colon-in-continuity. Co-morbidity is the most common contra-indication to GLP-2 analogue therapy. CLINICAL RELEVANCY: GLP-2 analogues are emerging as an important treatment for patients with short bowel syndrome. Our study explores patient suitability in a large HPN cohort managed in a national IF centre. Furthermore, the international variation in the pathophysiology of SBS-IF varies significantly, which can have a bearing on PN requirements and outcomes when GLP-2 analogues are used.

[Dietetic treatment with fructose in a 5-year-old girl with recurrent D-lactic acidosis]. / Tratamiento dietético con fructosa en una niña de 5 años con acidosis D-láctica recurrente.

D-lactic acidosis is an infrequent complication, mainly reported in patients with short bowel syndrome. It is characterized by recurrent episodes of encephalopathy with elevated serum D-lactic acid, usually associating metabolic acidosis. The presence of D-lactate-producing bacteria is necessary for the development of this complication. Other factors, such as the ingestion of large amounts of carbohydrates or reduced intestinal motility, contribute to D-lactic acidosis. We report a case of recurrent D-lactic acidosis in a 5-year-old girl with short bowel syndrome, due to a midgut volvulus. She initially received oral antibiotics in order to treat bacterial overgrowth, together with oral carbohydrates restriction. Nevertheless, recurrences did occur. Subsequently, 25% of the enteral nutrition was replaced for a formula containing fructose exclusively, while other fermentable sugars were restricted from the diet. After 16 years of follow up, further recurrences of D-lactic acidosis were not observed.

The Role of Lipids in Human Milk and Infant Formulae.

The quantity and quality of dietary lipids in infant formulae have a significant impact on health outcomes, especially when fat storing and/or absorption are limited (e.g., preterm birth and short bowel disease) or when fat byproducts may help to prevent some pathologies (e.g., atopy). The lipid composition of infant formulae varies according to the different fat sources used, and the potential biological effects are related to the variety of saturated and unsaturated fatty acids. For example, since lipids are the main source of energy when the normal absorptive capacity of the digestive tract is compromised, medium-chain saturated fatty acids might cover this requirement. Instead, ruminant-derived trans fatty acids and metabolites of n-3 long-chain polyunsaturated fatty acids with their anti-inflammatory properties can modulate immune function. Furthermore, dietary fats may influence the nutrient profile of formulae, improving the acceptance of these products and the compliance with dietary schedules.

Nutritional therapy complications in children with ultra-short bowel syndrome include growth deficiency but not cholestasis.

AIM: Children with ultra-short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, using citrulline serum concentration and cholestasis. METHODS: We studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015. RESULTS: The median standard deviation score (SDS) was -1.2 for body mass according to chronological age, -1.72 according to height and -0.59 according to height for age. Patients requiring seven days per week parenteral nutrition had a citrulline concentration below 10 µmol/L. Decreased bone-mineral density was observed in 87% of the patients. Low values of 25-hydroxyvitamin D were found in 53% of the children. None of the patients had elevated conjugated bilirubin levels above 34.2 µmol/L. CONCLUSION: Children with USBS were growth deficient according to their chronological age, with frequent abnormal bone mineralisation and vitamin D deficiency. Children requiring parenteral nutrition seven days a week had citrulline concentrations below 10 µmol/L. Cholestasis was not seen.

Nutritional Therapy in Adult Short Bowel Syndrome Patients with Chronic Intestinal Failure.

Intestinal failure (IF) is the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that parenteral support (PS) is required to maintain health and/or growth. This article critically revises the gaps in and evidence for providing general nutritional therapy recommendations in the Short Bowel Syndrome-IF population. It addresses the need for an individualized approach, aiming to reduce or even eliminate the need for PS, and emphasizes a need to focus on effects of dietary interventions on the quality of life of these patients.

The protective effect of fish oil lipid emulsions on intestinal failure-associated liver disease in a rat model of short-bowel syndrome.

PURPOSE: Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurrence of IFALD. In this study, we evaluated the effect of SO and fish oil (FO) lipid emulsion on hepatic steatosis. METHODS: Sprague-Dawley rats underwent jugular vein catheterization and were divided into three groups: sham operation with normal chow (Sham group), 80% small bowel resection (80% SBR) + TPN with SO lipid emulsion (SO group), and 80% SBR + TPN with FO lipid emulsion (FO group). All rats were euthanized and the serum biochemistry and hepatic histology analyzed. RESULTS: No significant differences in the serum liver or biliary enzymes were noted between the SO and FO groups. The pathological findings and NAFLD score in the FO group did not show steatosis and were significantly lower than in the SO group. An analysis of the fatty acids profile in the both the SO and FO groups did not indicate essential fatty acid deficiency (EFAD). CONCLUSION: FO lipid emulsion may have a protective role against steatosis of IFALD without EFAD.

Prevalence and risk factors for complications in adult patients with short bowel syndrome receiving long-term home parenteral nutrition.

BACKGROUND AND OBJECTIVES: Short bowel syndrome (SBS) is a complicated and challenging disease where home parenteral nutrition (HPN) is widely used. The complications of long-term HPN-dependent in adult patients with SBS are poorly documented. This study was mainly aimed to assess the prevalence and risk factors of HPNassociated complications in adult patients with SBS, especially the catheter-related sepsis and HPN-associated liver/biliary disorders. METHODS AND STUDY DESIGN: 47 non-malignant adult patients with SBS who received HPN for more than 2 years in our clinical nutrition center were included. Patients were divided into two groups according to whether HPN-associated complications were present or not. Student's t-test and χ2 test were applied to compare the differences between the two groups. RESULTS: The mean frequency of catheter-related sepsis was 0.31±0.05 per catheter year of HPN. An higher incidence of catheter-related infections (p<0.001) and shorter delay between HPN onset and first infection (p<0.001) were identified as risk factors for catheter-related sepsis. A total of 25 patients (53.2%) developed HPN-associated liver/biliary diseases. The identified risk factors for HPNassociated liver/biliary disorders were higher rate of catheter-related infections (p=0.009), shorter delay between HPN onset and first infection (p=0.017), higher energy content of HPN (p=0.014), higher glucose rate of HPN (p=0.009), and lower lipid rate of HPN (p=0.022). CONCLUSION: Our study revealed that adult patients with SBS receiving long-term HPN treatment developed a low prevalence of catheter-related sepsis but a rather high prevalence of HPN-associated liver/biliary disorders. We also identified several risk factors for HPN-associated complications which should be taken notice of in clinical practice.

Management of short bowel syndrome in postoperative very low birth weight infants.

Short bowel syndrome is a potentially devastating morbidity for the very low birth weight infant and family with a high risk for mortality. Prevention of injury to the intestine is the ideal, but, if and when the problem arises, it is important to have a systematic approach to manage nutrition, use pharmaceutical strategies and tools to maximize the outcome potential. Safely maximizing parenteral nutrition support by providing adequate macronutrients and micronutrients while minimizing its hepatotoxic effects is the initial postoperative strategy. As the infant stabilizes and starts to recover from that initial injury and/or surgery, a slow and closely monitored enteral nutrition approach should be initiated. Enteral feeds can be complemented with medications and supplements emerging as valuable clinical tools. Engaging a multidisciplinary team of neonatologists, gastroenterologists, pharmacists, skilled clinical nutrition support staff including registered dietitians and nutrition support nurses will facilitate optimizing each and every infant's long term result. Promoting intestinal rehabilitation and adaptation through evidence-based practice where it is found, and ongoing pursuit of research in this rare and devastating disease, is paramount in achieving optimal outcomes.

Eficacia y seguridad de fórmulas extesamente hidrolizada en pacientes pediátricos con sindrome de intestino corto y/o falla intestinal / Efficacy and safety of excessively hydrolyzed formulas in pediatric patients with short bowel syndrome and / or intestinal failure

INTRODUCCIÓN: Antecedentes: El presente informe expone la evaluación del produco nutricional fórmula extensamente didrolizada (FEH) respecto a sua uso en pacientes pediátricos con síndroe de intestiono corto y/o falla intestinal. Aspectos Generales: La falla intestinal (FI) se define como la reducción de la función por debajo del mínimo necesario para la absorción de macronutrientes y/o agua y electrolitos, por lo que se requíere la suplementación intravenosa para mantener un buen estado de salud y/o crecimiento. Aspectos Generales: La falla intestinal (FI) se define como la reducción de la función intestinal por debajo del mínimo necesario para la absorción de macronutrientes y/o agua y electrolitos, por lo que se requíere la suplementación intravenosa para mantener un buen estado de salud y/o crecimiento. La causa principal de FI en niños es el síndrome de instestino corto (SIC), condición en la cual la capacidad de absorción del instestino delgado se encuentra comprometida en respuesta a una resección intestinal. METODOLOGÍA: Estrategia de Búsqueda: se realizó una busqueda de literatura científica en relación a la eficacia y seguridad de fórmulas extensamente hidrolizadas (FEH) en pacientes peidátricos con diagnóstico de síndrome de intestino corto (SIC) y/o falla intestinal (FI) que no toleran alimentos con proteínas intactas. Se dio preferencia a guías de práctica clínica, revisiones sistemáticas con o sin meta-análisis y ensayos clínicos aleatorizados. RESULTADOS: Sinopsis de la Evidencia: Se realizó la búsqueda bibliográfica y de evidencia científica que sustente el uso de fórmula extensamente hidrolizada (FEH) en pacientes pediátricos con diagnóstico de síndrome de instestino corto (SIC) y/o falla intestional (FI) que no toleran alimentos con proteínas intactas. CONCLUSIONES: En la presente evaluación de tecnología sanitaria se presente la evidencia recabada sobre el beneficio de las fórmulas extensamente hidrolizadas (FEH) en pacientes pediátricos con diagnóstico de síndrome de intestino corto (SIC) y/o falla intestinal (FI) que no toleran alimentos con proteínas intactas. La evidencia encontrada que evalúa el uso de una FEH en pacientes con SIS es escaza e indirecta. Se ha evidencia proveniente de un ensayo clínico aleatorizado que compara el efecto de una FEH y una fórmula polimérica (FP) en el aumento de peso y el balance nitrogenado de pacientes con SIS. El Instituto de Evaluación de Tecnologías en Salud e Investigación-IETSI, aprueba el uso de la fórmula extensamente hidrolizadas en pacientes pediátricos con diagnóstico de síndrome de instestino corto y/o falla intestinal que no toleran alimentos con proteínas intactas. El presente Dictamen Preliminar tiene yna vigencia de dos anõs a partir de la fecha de publicación.

Eficacia y seguridad de fórmula a base de aminoácidos libres en pacientes pediátricos con sindrome de intestino corto y/o falla intestinal / Efficacy and safety of formula based on free amino acids in pediatric patients with short bowel syndrome and/or intestinal failure

INTRODUCCIÓN: Antecedentes: El presente informe expone la evaluación del producto nutricional Fórmula a base de aminoácidos libres (FAA) respecto a su uso en pacientes pediátricos con síndrome de intestino corto y/o falla intestinal. Aspectos Generales: El Síndrome de Intestino Corto (SIC) es el resultado de una resección quirúrgica, defectos congénitos, o pérdida de absorción asociada a enfermedad, y se caracteriza por una longitud insuficiente de intestino funcional incapaz de mantener balances proteicos, energéticos, de fluidos, de electrolitos y de micronutrientes. Tecnología Sanitaria de Interés: Las fórmulas a base de aminoácidos libres (FM) son fórmulas hipoalergénicas que están diseñadas para pacientes que requieren ingerir proteínas en su estructura más simple con el objetivo de evitar complicaciones de su enfermedad de base. Estas fórmulas aportan el nitrógeno en forma de L-aminoácidos razón por la cual son consideradas como fórmulas elementales. METODOLOGÍA: Estrategia de Búsqueda: Se realizó una búsqueda de literatura científica en relación a la eficacia y seguridad de fórmulas a base de aminoácidos libres (FAA) en pacientes pediátricos con diagnóstico de síndrome de intestino corto (SIC) y/o falla intestinal (FI) que no responden al tratamiento con fórmulas extensamente hidrolizadas (FEH). Se dio preferencia a guías de práctica clínica, revisiones sistemáticas con o sin meta-análisis y ensayos clínicos aleatorizados. Asimismo, se consideró extraer información con una estrategia de "bola de nieve" mediante la revisión de listas de referencias de las guías de práctica clínica, revisiones sistemáticas, estudios primarios, estudios descriptivos y revisiones narrativas seleccionadas. RESULTADOS: Se realizó la búsqueda bibliográfica y de evidencia científica que sustente el uso de una fórmula a base de aminoácidos libres (FAA) en pacientes pediátricos con diagnóstico de síndrome de intestino corto (SIC) y/o falla intestinal (FI) que no responden al tratamiento con fórmulas extensamente hidrolizadas (FEH). En resumen, luego de revisar un total de 1377 referencias resultados de la búsqueda bibliográfica, logramos filtrar 34 estudios relevantes para nuestra pregunta PICO de interés. Luego, 2 referencias fueron finalmente seleccionadas para ser analizadas, ya que responden a nuestra pregunta PICO. Sinopsis de la Evidencia: A continuación se detalla el resumen de los hallazgos según el tipo de evidencia científica encontrada para sustentar el uso de fórmulas a base de aminoácidos esenciales en pacientes pediátricos son síndrome de intestino corto y/o falla intestinal que no responden al tratamiento con fórmulas extensamente hidrolizadas. Guías de práctica clínica: la búsqueda no identificó guías de práctica clínica relevantes a la pregunta PICO de interés. Revisiones sistemáticas: No se encontraron revisiones sistemáticas asociadas a la pregunta PICO de interés. Ensayos clínicos: No se encontraron ensayos clínicos aleatorizados relevantes a la pregunta PICO de interés. Ensayos Clínicos no publicados: Se encontró un ensayo clínico no publicado que corresponde a Chan et al. Estudios observacionales: No se identificaron estudios observacionales relevantes a la pregunta PICO de interés. Otros: Se identificó un estudio descriptivo de serie de casos que corresponde a Bines et al. CONCLUSIONES: En la presente evaluación de tecnología sanitaria se presenta la evidencia recabada sobre el beneficio de las fórmulas a base de aminoácidos libres \r\n(FAA) en pacientes pediátricos con diagnóstico de síndrome de intestino corto (SIC) y/o falla intestinal (FI) que no responden al tratamiento con fórmulas extensamente hidrolizadas (FEH). La evidencia encontrada que evalúa el uso de una FAA en pacientes con SIC es escaza. Se ha identificado evidencia proveniente de un estudio de serie de casos que describe el uso de FAA en pacientes pediátricos con SIC que no responden al tratamiento con FEH, y un ensayo clínico no publicado que tiene como objetivo evaluar la tolerancia y el tiempo en que se logra el destete de la nutrición parenteral (NP) al ingerir una FAA en comparación a una fórmula parcialmente hidrolizada. El estudio descriptivo de serie de casos sugiere que el consumo progresivo de de FAA vía enteral disminuye la frecuencia del flujo fecal, la presencia de vómitos y el tiempo de destete de la NP en los infantes con SIC que no responden al tratamiento con FEH. La calidad de evidencia que presenta el estudio evaluado es baja debido al diseño propio del estudio y las limitaciones metodológicas que este presenta. El Instituto de Evaluación de Tecnologías en Salud e Investigación ­ IETSI, aprueba el uso de la fórmula a base de aminoácidos libres en pacientes pediátricos con diagnóstico de síndrome de intestino corto y/o falla intestinal que no responden al tratamiento con FEH. El presente Dictamen Preliminar tiene \r\nuna vigencia de dos años a partir de la fecha de publicación.