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Palliative cancer care patients may live for a long time, but malnutrition worsens the prognosis. Parenteral nutrition (PN) is suitable for replenishing a calorie deficit, but its advantages and tolerance late in the cancer trajectory are debated. We examined symptom development in hospitalized patients with and without PN. A total of 21 palliative cancer care patients receiving PN and 155 palliative cancer care patients not receiving PN during hospitalization in a specialized unit were retrospectively compared. We studied symptom intensity at admission, symptom relief during the hospital stay, and survival. The patients had locally advanced or metastatic cancer, a mean age of 70 years, and their median ECOG performance status was III. Symptom burden at admission was similar in the compared groups. Symptom relief during hospitalization was also similar. However, patients already on PN at admission reported more nausea and patients receiving PN during hospitalization reported better nausea relief compared to patients not receiving this intervention. Overall median survival was less than two months and similar in the compared groups. Based on a limited number of observations and a suboptimal study design, we were not able to demonstrate an increased symptom burden for palliative cancer care patients receiving PN late in the disease trajectory. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome (SBS) in adults is defined as having less than 180 to 200 cm of remaining small bowel. Many literature sources do not provide precise epidemiological data, and challenges in estimating the prevalence of SBS include its multifactorial etiology and varying definitions. The most common pathologies leading to SBS include Crohn disease, mesenteric ischemia, radiation enteritis, post-surgical adhesions, and post-operative complications. Case presentation: This article presents a clinical case of a 76-year-old Lithuanian patient who underwent parenteral nutrition for four months due to SBS. Before the following diagnosis, the patient had undergone two surgeries. During the hospitalization, life-threatening conditions such as stercoral peritonitis, septic shock, and acute respiratory failure, were observed and treated. As a result of SBS, hypoproteinemia and hypoalbuminemia developed, leading to the prescription of full parenteral nutrition. After correcting the malnutrition, a third surgery was performed, resulting in the discontinuation of parenteral nutrition and the resumption of a regular diet. Conclusions: Parenteral nutrition is the sole effective method for preserving the lives of patients with a short segment of the intestine. While on parenteral nutrition, patients can be prepared for reconstructive surgery. [ABSTRACT FROM AUTHOR]

From January 2015 to February 2021, retrospective review was conducted for 24 USBS infants. Clinical characteristics, treatments and outcomes were recorded. There were 17 boys and 7 girls with a gestational age of (26-39+5) week and a range of birth weight (720-3 600) gram. The infants were premature (n=20) and full-term (n=4). Results Among them, 7 infants of temporary USBS were discharged upon completing short-term nutritional therapy after closing stoma; 7/17 infants of non-temporary USBS were weaned off parenteral nutrition to achieve intestinal adaptation; 5/17 relinquished further treatments during operation; 5/17 required continued parenteral nutrition. Among 19 survivors, there were intravenous nutrition-related cholestasis (n=9), catheter infection (n=9), acquired zinc deficiency dermatitis (n=3), vitamin D deficiency (n=6) and metabolic bone disease (n=2). All symptoms improved after symptomatic measures. Conclusions It is rather troublesome to manage USBS children and there are numerous complications. After proper treatments, some infants may finally achieve intestinal adaptation without parenteral nutrition. [ABSTRACT FROM AUTHOR]

Hyperammonemia syndrome is a complication that has been reported to occur in 1–4% of lung transplant patients with mortality rates as high as 60–80%, making detection and management crucial components of post-transplant care. Patients are treated with a multimodal strategy that may include renal replacement therapy, bowel decontamination, supplementation of urea cycle intermediates, nitrogen scavengers, antibiotics against Mollicutes, protein restriction, and restriction of parenteral nutrition. In this review we provide a framework of pharmacologic mechanisms, medication doses, adverse effects, and available evidence for commonly used treatments to consider when initiating therapy. In the absence of evidence for individual strategies and conclusive knowledge of the causes of hyperammonemia syndrome, clinicians should continue to design multimodal regimens based on suspected etiologies, institutional drug availability, patient ability to tolerate enteral medications and nutrition, and availability of intravenous access. [ABSTRACT FROM AUTHOR]

• Gastrointestinal involvement is common in Duchenne Muscular Dystrophy. • Bowel dysmotility can manifest as life-threatening pseudo-obstruction or volvulus. • Cardiorespiratory co-morbidities can make surgical interventions high-risk. • Parenteral nutritional has a role in the management of this bowel dysmotility. Duchenne muscular dystrophy is a progressive muscle wasting disease caused by pathogenic variants in DMD. Gastrointestinal involvement is increasingly recognised in older patients and can manifest as life-threatening bowel dysmotility. We describe a series of adults with Duchenne muscular dystrophy who developed either severe colonic pseudo-obstruction or sigmoid volvulus requiring urgent assessment and intervention. The presentations varied in their clinical picture and outcomes, but together highlight the complexity of managing gastrointestinal complications in this cohort of patients. Key considerations include pre-existing cardiorespiratory compromise and the increased risk associated with surgery and general anaesthesia. We also outline a role for home parenteral nutrition in the long-term management of associated bowel dysmotility. [ABSTRACT FROM AUTHOR]

Objective: To investigate the effect of different proportions of enteral combined with parenteral nutrition treatment on patients with severe traumatic brain injury (s TBI). Methods: The subjects of study were s TBI patients admitted to the Neurointensive Care Unit of Chongqing Emergency Medical Center from October 2020 to August 2022. 70 patients who met the inclusion and exclusion criteria were randomly divided into the experimental group and the control group with 35 cases each. Calculate the daily caloric requirements of patients and provide nutritional support according to different proportion of enteral and parenteral nutrition programs. The experimental group was given enteral and parenteral nutrition support at a ratio of 2:1, while the control group was given enteral and parenteral nutrition support at a ratio of 1:1. The differences of nutritional status indicators (hemoglobin, albumin, prealbumin, total protein), immune function indicators [CD3, CD4, CD4/CD8, immunoglobulin (Ig)A, Ig M, Ig G], inflammatory indicators [C-reactive protein (CRP), procalcitonin (PCT), interleukin-6 (IL-6)] and complications between the two groups were statistically compared. Results: In terms of immune function, the levels of CD4, CD4/CD8 and Ig A in the experimental group were significantly higher than those in the control group 14 d after nutritional support (P＜0. 05); There was no significant difference between the two groups in nutritional status, inflammatory indicators and complications (P＞0. 05). Conclusions: Enteral nutrition-based combined nutritional support can promote the recovery of immune function in patients with s TBI, but there is no significant difference in nutritional status, inflammatory indicators and complications between different proportions of enteral combined parenteral nutrition treatment. [ABSTRACT FROM AUTHOR]

Intestinal-failure-associated liver disease (IFALD) is a common complication of prolonged parenteral nutrition (PN). Risk factors for IFALD include clinical features, as well as medical interventions, and its management was initially based on the decrease or interruption of parenteral nutrition while increasing enteral nutrition. However, the tolerance of full enteral nutrition in children with intestinal failure may require prolonged intestinal rehabilitation over a period of years. As a consequence, infants unable to wean from PN are prone to develop end-stage liver disease. We describe the case of an infant receiving long-term PN who was diagnosed with IFALD wherein we were able to reverse IFALD by switching lipid emulsions to fish oil monotherapy. A systemic review of case reports and case series on reversing IFALD using fish oil lipid emulsion follows the case description. [ABSTRACT FROM AUTHOR]

Background: Pediatric intestinal pseudo‐obstruction (PIPO) encompasses a variety of rare, heterogeneous, and disabling disorders that severely affect gastrointestinal motility and are associated with high morbidity and mortality. PIPO management is complex and focuses on maintaining an optimal nutritional status, improving gut function, relieving symptoms, and treating complications. Nutritional issues prevail, and PIPO patients often experience severe undernutrition and faltering growth. Thus, nutritional management plays a pivotal role for achieving the most favorable clinical outcomes. The calorie and nutrient intake of each patient needs to be tailored to age, extent and severity of gut involvement and nutritional needs to support an optimal nutritional status. After defining the extent and severity of gut dysmotility, an experienced team should perform a careful nutritional assessment. An oral diet should always be encouraged and might include bite and dissolve solids, liquid diet or simple oral stimulation. If oral caloric intake is inadequate, liquid gastric feeds should provide the subsequent step. In the presence of severe gastric dysmotility, continuous post‐pyloric feeding represents a viable option. In the most severe cases, parenteral nutrition (PN) is required to meet appropriate nutritional requirements. Purpose: Pediatric data on this topic are scarce and mainly extrapolated from adult studies. In this review, we discuss current evidence and knowledge regarding nutritional options, implications of the use of different feed types, including a blended diet, and the use of PN. Moreover, based on our experience and the evidence from the literature, we propose a flow chart to guide the nutritional management of PIPO patients. [ABSTRACT FROM AUTHOR]

The authors express gratitude to those who show interest in the journal's supplement, based on the International Safety and Quality of Parenteral Nutrition (PN) Summit held on November 8 to 10, 2021. They discuss the summit's main objectives, the summit format, and the goal of the summit. They also mention the importance of PN as an important therapeutic intervention as noted in the summit and the differences that emerged between PN practices according to geographical location.

Objectives: This study aimed to examine the role of total parenteral nutrition in the treatment of chilotorax. Methods: A total of 1149 patients who were operated for esophagus cancer were screened between 2010 and 2021. Of these patients, 49 were identified with chilorotax. Patients were divided into three groups. Some of these were treated surgically (first group) and through conventional approaches such as plorodesis, and somatostatin (second group). The only method of treatment used for the remaining patients was total parenteral feeding (third group). These three groups were statistically compared using clinical data to demonstrate that total parenteral nutrition is as effective as other treatments for chilotorax. Results: A total of 1144 patients who received surgical treatment for esophageal cancer were analyzed. Chilotorax was used to diagnose 49 of these patients. Fewer deaths and complications occurred in the third group who were treated with just total parenteral nutrition. When the groups were compared using post hoc multiple comparison tests, based on the length of stay in the hospital after diagnosis and treatment initiation, it was discovered that; the average length of stay in the hospital in group 3 was less than the other two groups. Conclusion: The use of total parenteral nutrition alone or in conjunction with surgical and another interventional processes in the treatment of chylothorax importantly reduces the risk of difficulties as well as the death rate. In all patients with chylothorax, parenteral nutrition should be included to the treatment protocol. [ABSTRACT FROM AUTHOR]

Hemodialysis is associated with high morbidity and mortality rates as well as low quality of life. Altered nutritional status and protein-energy wasting are important indicators of these risks. Maintaining optimal nutritional status in patients with hemodialysis is a critical but sometimes overlooked aspect of care. Nutritional support strategies usually begin with dietary counseling and oral nutritional supplements. Patients may not comply with this advice or oral nutritional supplements, however , or compliance may be affected by other complications of progressive chronic kidney disease. Intradialytic parenteral nutrition (IDPN) may be a possibility in these cases, but lack of knowledge on practical aspects of IDPN delivery are seldom discussed and may represent a barrier. In this review, we, as a consensus panel of clinicians experienced with IDPN, survey existing literature and summarize our views on when to use IDPN, which patients may be best suited for IDPN, and how to effectively deliver and monitor this strategy for nutritional support. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is the commonest cause of intestinal failure in neonates. SBS results from widespread damage to the small intestine, leading to loss of functional capacity of this organ. This is generally secondary to conditions like necrotizing enterocolitis, gastroschisis, intestinal atresia, and midgut volvulus. The small bowel usually adapts to this damage in due course of time. The clinician's role usually entails the management of parenteral nutrition and the fluid and electrolyte balance to tide over this phase. The management should be initiated as soon as the diagnosis is suspected, especially postsurgical resection of the bowel. This should comprise enteral nutrition, with proactive monitoring and supplementation of electrolytes and micronutrients. Intestinal lengthening procedures like the Serial transverse enteroplasty (STEP), and Longitudinal intestinal lengthening and tailoring (LILT) may be considered in infants, where medical therapy fails to correct the pathology. The intricate nature of the condition warrants a multi-disciplinary approach, involving clinicians, intensivists, and surgeons, which ensures the best neonatal outcomes, in terms of the survival rates in these babies. [ABSTRACT FROM AUTHOR]

Patients with recurrent platinum-resistant ovarian cancer often present with inoperable malignant bowel obstruction (MBO) from a large burden of abdominal disease. Interventions such as total parenteral nutrition (TPN) and chemotherapy may be used in this setting. We aim to describe the relative cost-effectiveness of these interventions to inform clinical decision making. Four strategies for management of platinum-resistant recurrent ovarian cancer with inoperable MBO were compared from a societal perspective using a Monte Carlo simulation: (1) hospice, (2) TPN, (3) chemotherapy, and (4) TPN + chemotherapy. Survival, hospitalization rates, end-of-life (EOL) setting, and MBO-related utilities were obtained from literature review: hospice (survival 38 days, 6% hospitalization), chemotherapy (42 days, 29%), TPN (55 days, 25%), TPN + chemotherapy (74 days, 47%). Outcomes were the average cost per strategy and incremental cost-effectiveness ratios (ICERs) in US dollars per quality-adjusted life year (QALY) gained. In the base case scenario, TPN + chemotherapy was the most costly strategy (mean; 95% CI) ($49,741; $49,329–$50,162) and provided the highest QALYs (0.089; 0.089–0.090). The lowest cost strategy was hospice ($14,591; $14,527–$14,654). The TPN alone and chemotherapy alone strategies were dominated by a combination of hospice and TPN + chemotherapy. The ICER of TPN + chemotherapy was $918,538/QALY compared to hospice. With a societal willingness to pay threshold of $150,000/QALY, hospice was the strategy of choice in 71.6% of cases, chemotherapy alone in 28.4%, and TPN-containing strategies in 0%. TPN with or without chemotherapy is not cost-effective in management of inoperable malignant bowel obstruction and platinum-resistant ovarian cancer. • Parenteral nutrition is not cost-effective management of recurrent ovarian cancer & inoperable malignant bowel obstruction. • Hospice is an appropriate strategy for patients with recurrent ovarian cancer & inoperable malignant bowel obstruction. • Goals of care, quality of life, survival, and cost should be considered when making clinical decisions in this setting. [ABSTRACT FROM AUTHOR]

Purpose: Short bowel syndrome (SBS) with intestinal failure (SBS-IF) requires long-term parenteral nutrition (PN). This study investigated the real-world etiologies of SBS, treatment patterns, and PN-related outcomes among adult patients with SBS-IF in Japan. Methods: This retrospective, observational cohort study was based on data from April, 2008 to January, 2020 from one of the largest hospital-based claim databases in Japan. Analyzed patients were aged ≥ 16 years, had received continuous PN for ≥ 6 months, and had SBS or undergone SBS-related surgery with a diagnosis of a causative disease. The primary endpoint was PN weaning. Results: We analyzed data for 393 patients. The most frequent causes of SBS-IF were ileus (31.8%), Crohn's disease (20.1%), and mesenteric ischemia (16.0%). Of 144/393 (36.6%) patients who were weaned off their PN, 48 (33.3%) were subsequently restarted on PN. Of 276/393 (70.2%) patients whose PN was initiated in hospital, 156 (56.5%) transitioned to home management. The mean duration of initial PN was 450.4 and 675.5 days for patients who were able or unable to be weaned off PN, respectively. Sepsis (67.4%), catheter-related bloodstream infections (49.1%), and liver disorders (45.0%) were the most reported PN-related complications. Conclusions: Most patients with SBS-IF in Japan could not be weaned off PN and suffered life-threatening complications. [ABSTRACT FROM AUTHOR]

Purpose: To undertake a pilot study estimating patient-level costs of care for paediatric short bowel syndrome (SBS) from the healthcare provider perspective.Methods: A pilot group of patients with anatomical SBS was selected at a single specialist tertiary centre in the United Kingdom. The Patient Level Information and Costing System (PLICS) was used to extract costing data for all hospital-based activities related to SBS, from the implementation of PLICS in 2016 to April 2021. Patient-specific and pooled data were reported descriptively in per patient-year terms.Results: Five patients had full PLICS data available for the 5-year study period and 2 patients had 4 years of data. The median cost for hospital care of SBS was £52,834 per patient-year (range £1804-£331,489). The key cost drivers were inpatient beds, pharmacy, and staffing costs, which made up > 60% of annual costs. In the first 3 years following index admission (n = 2), there was a steady decline in the annual cost of care to a level comparable with patients with established SBS.Conclusion: Patient-level cost of care analysis for SBS is feasible using PLICS. Hospital-related costs vary widely between and within individual patients over time. Key drivers of cost are related to complications of SBS. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome (SBS) is an uncommon malabsorptive condition, most often caused by massive surgical resection of the small intestine, although it can also be congenital, especially in pediatric cases. SBS is associated with significant morbidity and mortality, reduced quality of life, and high healthcare costs. Aim: To report a case of SBS in a newborn treated at the Pediatrics Service of Hospital Universitário São Francisco in Providência de Deus (HUSF), located in the city of Bragança Paulista, SP, Brazil. Case Report: It was a newborn who was referred to the Neonatal Intensive Care Unit. Subsequently, the patient underwent an exploratory laparotomy with enterectomy, right partial colectomy, ileostomy, transverse colon burial using the Hartmann technique, appendectomy and cavity drainage. With severe malnutrition, the newborn received peripheral parenteral nutrition (PPN) until weight gain began (15g/day). After his nutritional status improved, he was discharged with outpatient follow-up. Conclusion: Although it is associated with high morbidity and mortality, pediatric SBS can be circumvented with the help of surgical procedures and subsequent PN, as demonstrated in the case reported here, which had a favorable outcome. [ABSTRACT FROM AUTHOR]

Purpose: This study aims to define the extent of additional surgical procedures after abdominal wall closure (AWC) in patients with gastroschisis (GS) and omphalocele (OC) with special focus on gastrointestinal related operations.Methods: A retrospective chart review was performed including all operations in GS and OC patients in the first year after AWC (2010-2019). The risk for surgery was calculated using the one-year cumulative incidence (CI).Results: 33 GS patients (18 simple GS, 15 complex) and 24 OC patients (12 without (= OCL), 12 OC patients with liver protrusion (= OCL +)) were eligible for analysis. 43 secondary operations (23 in GS, 20 in OC patients) occurred after a median time of 84 days (16-824) in GS and 114.5 days (12-4368) in OC. Patients with complex versus simple GS had a significantly higher risk of undergoing a secondary operation (one-year CI 64.3% vs. 24.4%; p = 0.05). 86.5% of surgical procedures in complex GS and 36.3% in OCL + were related to gastrointestinal complications. Complex GS had a significantly higher risk for GI-related surgery than simple GS. Bowel obstruction was a risk factor for surgery in complex GS (one-year CI 35.7%).Conclusion: Complex GS and OCL + patients had the highest risk of undergoing secondary operations, especially those with gastrointestinal complications. [ABSTRACT FROM AUTHOR]

L'insuffisance intestinale chronique (IIC) est une situation clinique rare dont la principale cause est le syndrome de grêle court (SGC). La pseudo-obstruction intestinale chronique (POIC) est également une cause d'IIC. La nutrition parentérale à domicile (NPAD) a permis d'augmenter la survie et la qualité de vie des patients ayant une insuffisance intestinale chronique. Plusieurs grossesses sous NPAD ont été décrites. L'objectif de cette revue est de faire la synthèse sur l'état des connaissances sur les grossesses en NPAD, et de faire le point sur les adaptations nécessaires des traitements et de la nutrition. En NPAD prolongée, les grossesses sont donc possibles, mais le taux de complications maternelles liées à la maladie sous-jacente et à la NP est élevé. Un suivi rapproché et une préparation à la grossesse doivent être réalisés afin de prévenir et traiter d'éventuelles carences et leurs effets délétères. Chronic intestinal failure is a rare disease and short bowel syndrome is its main etiology. Chronic intestinal pseudo obstruction (CIPO) is also a cause of IIC. Home parenteral nutrition (HPN) has improved survival and quality of life of patients with IIC. Some pregnancy during HPN have been reported. The aim of this review was to summarize the state of knowledge on pregnancy during HPN, and to resume the needed adaptations of treatments and PN. During long term HPN, pregnancy are possible but the complication rate, due to PN and to underlying disease, is high. A close follow-up end pregnancy preparation are necessary, in order to prevent and treat some deficiencies and their deleterious effects. [ABSTRACT FROM AUTHOR]

Background and Aims: Parenteral nutrition (PN) has become an efficient, safe, and convenient treatment over years for patients suffering from intestinal failure. Home PN (HPN) enables the patients to have a high quality of life in their own environment. The therapy management however implies many restrictions and potentially severe lethal complications. Prevention and therapy of the latter are therefore of utmost importance. This study aims to assess and characterize the situation of patients with HPN focusing on prevalence of catheter-related complications and mortality. Methods: Swiss multicentre prospective observational study collecting demographic, anthropometric, and catheter-related data by means of questionnaires every sixth month from 2017 to 2019 (24 months), focusing on survival and complications. Data were analysed using descriptive statistics. Logistic regression models were fitted to investigate association between infection and potential co-factors. Results: Seventy adult patients (50% women) on HPN were included (≈5 patients/million adult inhabitants/year). The most common underlying diseases were cancer (23%), bariatric surgery (11%), and Crohn's disease (10%). The most prevalent indication was short bowel syndrome (30%). During the study period, 47% of the patients were weaned off PN; mortality rate reached 7% for a median treatment duration of 1.31 years. The rate of catheter-related infection was 0.66/1,000 catheter-days (0.28/catheter-year) while the rate of central venous thrombosis was 0.13/1,000 catheter-days (0.05/catheter-year). Conclusion: This prospective study gives a comprehensive overview of the adult Swiss HPN patient population. The collected data are prerequisite for evaluation, comparison, and improvement of recommendations to ensure best treatment quality and safety. [ABSTRACT FROM AUTHOR]

Purpose: Vitamins and trace elements are essential nutrients for growth and intestinal adaptation in children with short bowel syndrome (SBS). This study aimed to assess micronutrients' status during and after weaning off PN in pediatric SBS. Methods: This retrospective study evaluated the follow-up of 31 children with SBS between Jan 2010 and Sep 2019. Clinical data were reviewed from the patients' electric medical record. Serum electrolytes, trace elements, vitamin B12, vitamin D, and folate concentrations were collected before and after enteral autonomy. Results: Thirty-one SBS cases were reviewed (median onset age 11 days after birth, 51.6% boys, mean PN duration 4 months, and mean residual small intestine length 58.2 cm). Median duration of follow-up was 10 months (interquartile range [IQR]: 4, 19). The common micronutrient deficiencies were zinc (51.6%), copper (38.7%), vitamin D (32.3%), and phosphorus (25.8%) after the transition to EN. The proportion of patients deficient in vitamin D decreased dramatically from 93.5% to 32.3% (P < 0.001), and serum concentrations of vitamin D increased significantly (27.4 ± 12.3 vs. 60.3 ± 32.9 nmol/l, P = 0.03) after achieving full enteral feeding more than 1 month. Additionally, serum magnesium levels significantly increased (0.76 ± 0.17 vs. 0.88 ± 0.14 mmol/l, P = 0.03). Hemoglobin levels elevated significantly after weaning off PN (104.3 ± 10.7 vs. 117.8 ± 13.7 g/l, P = 0.03). Conclusions: Micronutrient deficiencies remain a common problem in pediatric SBS through intestinal rehabilitation. Therefore, we strongly recommend supplementation of more vitamin D and trace elements (zinc, copper, and phosphorus) under regular monitoring during long-term intestinal rehabilitation. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome (SBS) is a malabsorptive condition that can result in intestinal failure (SBS‐IF). Many patients with SBS‐IF require home parenteral nutrition (PN) for survival. However, PN has profound effects on patients and their family members. The present study aimed to understand the lived experience of SBS‐IF for patients and their families. Methods: In‐depth semi‐structured qualitative interviews were conducted with 15 patients with SBS‐IF and five adult family members living with someone with SBS‐IF. A patient‐centric approach was taken, with a patient steering group providing input and guidance to develop the interview guide. Key concepts were identified using thematic analysis of interview transcripts. Results: Patients' lives were dominated by having SBS‐IF. They described physical impacts that included patient‐reported signs and symptoms and physical restrictions comprising of restrictions on daily life, actives of daily living and physical functioning. In addition, they encountered emotional impacts with a plethora of negative feelings and social impacts, such as difficulties socialising and maintaining relationships. Patients coped by adapting their life around SBS‐IF, having support and adopting an attitude of gratitude and acceptance. Family members were also affected and, along with patients, appreciated the respite of a night off from infusions. Conclusions: Patients and families face many difficulties with SBS‐IF. Healthcare professionals can support patients by facilitating them explore what others have found beneficial; adapting their life around PN, viewing PN with acceptance and trying to cultivate gratitude. Further research into the support required for families may be beneficial. [ABSTRACT FROM AUTHOR]

Intestinal failure is a complex and debilitating condition characterised by inadequate small intestinal function requiring parenteral or intravenous nutrition to maintain health and, for children, to enable growth and development. Although parenteral nutrition can be prescribed in many hospitals, children with chronic intestinal failure have improved outcomes when managed at a paediatric centre by a multidisciplinary team with specialised expertise in the comprehensive management of intestinal failure. Recent advances in the medical, surgical and nutritional approach have been effective at optimising intestinal rehabilitation and achieving enteral autonomy while limiting complications of intestinal failure. The role of intestinal transplantation in the management of the child with intestinal failure continues to evolve as an option for children with life-threatening complications of intestinal failure. The aim of this review is to highlight key advances in the care of children with intestinal failure. [ABSTRACT FROM AUTHOR]

Background: Few studies have examined the metabolic consequences of short bowel syndrome (SBS) and its effects on body composition in adults. We hypothesized that body composition of SBS patients is altered compared to a normal age-, race-, and sex-matched population, regardless of parenteral nutrition (PN) dependence.Aim: To compare the body composition of adult patients with SBS to age-, sex-, and race-matched healthy controls.Methods: Twenty patients with SBS underwent body composition analysis using the GE Lunar iDXA scanner. Patients were age-, sex-, and race-matched to controls from the National Health and Nutrition Examination Survey (1999-2004). Mean differences in body mass index, fat-free mass, fat mass, percent body fat, visceral adipose tissue mass and volume, and bone mineral density were measured. Statistical analysis was performed using SAS 9.4 software.Results: Fifty-five percent of subjects had a history of PN use, and 30% were current PN users. Mean percent body fat for SBS patients was 35.1% compared to 30.9% for healthy controls (p = 0.043). Fat-free mass was reduced in SBS (p = 0.007). Patients with reduced bone mass had a trend toward significantly more years of PN exposure compared to those with normal bone mass (p = 0.094), and a trend toward older age (p = 0.075).Conclusions: SBS is associated with increased percent body fat and reduced fat-free mass, suggesting that improved dietary and therapeutic interventions are needed to restore normal metabolic indices and avoid risk of metabolic syndrome in SBS patients. [ABSTRACT FROM AUTHOR]

Distal bowel reinfusion enteroclysis or fistuloclysis or mucous fistula re-feeding is described as the method of collecting proximal bowel effluents and re-infusing the same into the distal viable bowel with or without effluent processing. Patients with anatomical or functional short bowel syndrome (SBS) are very difficult to manage with great risk of fluid electrolyte imbalance and protein calorie malnutrition. We describe our experience with reinfusion of stoma effluent in these patients, whether it can be used as a stand-alone modality after an initial bridging period of total parenteral nutrition therapy (TPN). Reinfusion enteroclysis was attempted in 30 patients with functional SBS but with distal viable continuous bowel. After an initial bridging period of TPN therapy, gradually increasing quantities of chyme reinfusion was done with a Foley's catheter in the distal bowel until the whole of daily requirements was met by oral feeds and reinfusion enteroclysis. Gradually, the parenteral nutrition (PN) was withdrawn. Daily assessment of patient's oral nutritional adequacy, fluid, and electrolyte balance was done while in the hospital. Patients who were nutritionally stable on exclusive enteroclysis were discharged and were kept on stand-alone "home enteroclysis." The patients were followed until definitive surgery. Even though median length of proximal bowel was just 40 cm from duodeno-jejunal junction, PN could be completely withdrawn in 24 patients; they sustained only on reinfusion enteroclysis. Eighteen patients were discharged and sustained independently on "home enteroclysis." All 30 attained a positive nitrogen balance with weight gain and increasing albumin titers. There were no complications associated to reinfusion and no associated mortality. Successful definitive surgery was carried out in 16 patients, within the 3-month study period. However, 14 patients required more than 3 months for their definitive surgery. During this period, 8 of them continued to be on "exclusive re infusion enteroclysis" and 6 on a combination of PN and reinfusion enteroclysis until the second stage of definitive operative intervention. Reinfusion enteroclysis/fistuloclysis can act as effective stand-alone nutritional therapy in selected patients and adjunctive therapy in all patients of functional SBS with distal viable continuous bowel. [ABSTRACT FROM AUTHOR]

Acute intestinal failure (AIF), "reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, requiring parenteral nutrition", is common, but very often neglected part of multiorgan dysfunction syndrome (MODS) in the critically ill patients. It is now increasingly being recognized as a cause of prolonged ICU and hospital stay and poor outcome. Multidisciplinary team approach, systematic approach to management with treatment of sepsis, early mobilization and enteral feeding with prokinetics if required, control of intra-abdominal pressure and surgical intervention, when mandated, can help treat AIF and improve patient outcomes. [ABSTRACT FROM AUTHOR]

Purpose: Differences in body weight changes and serum liver tests (LTs) in acute myeloid leukemia (AML) patients receiving parenteral nutrition (PN) versus no PN during remission induction (RI) treatment were assessed. Methods: Retrospectively, differences in body weight changes and serum LTs in AML patients (n = 213) who received PN versus no PN during RI treatment in one of three Dutch hospitals between 2004 and 2015 were assessed. Weekly body weight and serum LT registrations were collected from medical records. Patients' body weight changes were compared between the hospitals where PN is applied upon first indication of inadequate oral intake (PN hospitals) and the hospital where use of PN is limited to severe cases only (no-PN hospital) using repeated measures mixed model analysis. Differences in severity of serum LT elevations, according to the Common Terminology Criteria for Adverse Events (CTCAE) version 4.0, were assessed between patients who did and did not receive PN using chi-square or Fisher's exact tests, and multiple logistic regression analysis. Results: Compared with patients of the PN hospitals, patients of the no-PN hospital experienced significantly more body weight loss during RI treatment (between-group difference 7.2%, 95% CI 4.0–10.3%). Furthermore, PN was associated with transient mild to moderate elevations of liver enzymes, but not with raised median total bilirubin levels nor with occurrence of CTCAE grade 3–4 LT elevations. Conclusion: Frequent compared with exceptional use of PN in AML patients during RI treatment better preserved body weight, without clinically relevant (CTCAE grade 3–4) elevations in serum LTs. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a rare disease that results from extensive resection of the intestine. When the remaining absorption surface of the intestine cannot absorb enough macronutrients, micronutrients, and water, SBS results in intestinal failure (IF). Patients with SBS who suffer from IF require parenteral nutrition for survival, but long-term parenteral nutrition may lead to complications such as catheter sepsis and metabolic diseases. Spontaneous intestinal adaptation occurs weeks to months after resection, resulting in hyperplasia of the remnant gut, modification of gut hormone levels, dysbiosis, and hyperphagia. Oral nutrition and presence of the colon are two major positive drivers for this adaptation. This review aims to summarize the current knowledge of the mechanisms underlying spontaneous intestinal adaptation, particularly in response to modifications of luminal content, including nutrients. In the future, dietary manipulations could be used to treat SBS. [ABSTRACT FROM AUTHOR]

Introduction: Introduction: home parenteral nutrition (NPD) is a complex technique that involves multidisciplinary follow-up. Objectives: descriptive analysis of all patients included in the NPD program. Methods: retrospective study of patients with NPD between 1985 and 2017 in our center, a tertiary university hospital. Results: we analyzed 61 patients (32 men, mean age: 51.2 years). The most common underlying pathology was neoplasia (32.8%), with short bowel syndrome (SIC) being the main indication of NPD (70.5%). Forty-five patients received partial NPD and 16 total. The tunnelled vein catheter was the most common venous access used. 20 patients suspended it for complete oral intake (19 the first 5 years), 26 were deceased (18 the first 5 years) and 15 maintain it. Neoplasia was the most frequent cause of death (46.2%) and in 15.4% liver disease was associated with NPD. The median duration of NPD was 25 months [1-394]; being in 24 patients longer than 5 years (8 dead, only 1 for oncologic cause not related to the NPD). Fifty-four per cent had catheter infections, being isolated 55.2% Staphylococcus coagulase negative, with an infection rate of 1.04 per 1000 days of catheterization. Conclusions: NPD is a useful therapeutic strategy in intestinal failure. The SIC is the most frequent indication in our case study. The underlying pathology, such as neoplasia, will determine the prognosis. Catheter infection is the more frequent complication, so it is necessary to strengthen health education and antiseptic prophylaxis. [ABSTRACT FROM AUTHOR]

Background: Intestinal failure is the most critical complication of Crohn's disease. Intestinal failure requires home parenteral nutrition, which worsens the quality of life of the patients and sometimes causes life-threatening complications.Aims: The purpose of this study was to investigate the incidence and risk factors for intestinal failure in Crohn's disease.Methods: We performed a retrospective analysis of Crohn's disease patients (162 cases) at Osaka University Hospital between January 2000 and December 2017. Kaplan-Meier analysis was used to investigate the cumulative incidence of intestinal failure. To identify the risk factors of intestinal failure, patient characteristics were analyzed by multivariate analysis, including disease classification, surgical history, medical treatment other than surgery, and cumulative inflammation was calculated using the average C-reactive protein value and disease duration.Results: The cumulative incidence of intestinal failure 5, 10, and 15 years after Crohn's disease diagnosis was 2.6%, 3.4%, and 8.6%, respectively. Multivariate analysis identified the following as independent risk factors for intestinal failure in Crohn's disease: residual small intestinal length < 200 cm (odds ratio 7.51, 95% confidence interval 2.14-29.96), non-use of anti-tumor necrosis factor-alpha therapy (3.34, 1.22-10.74), and cumulative inflammation (1.01, 1.001-1.038). We created a new predictive nomogram consisting of these risk factors.Conclusions: Intestinal failure occasionally occurred during long-term treatment of Crohn's disease. Cumulative inflammation for the first time, in addition to short residual small intestinal length and non-use of anti-tumor necrosis factor-alpha therapy, was shown to be potential risk factors for intestinal failure in Crohn's disease. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx. [ABSTRACT FROM AUTHOR]

Background: Teduglutide, a glucagon-like peptide 2 analog, has demonstrated efficacy in treating adult patients with short bowel syndrome (SBS) and dependence on parenteral nutrition (PN), but its role in chronic malabsorptive states that do not necessitate PN remains uncertain.Aims: To evaluate teduglutide use beyond its approved indications and to discuss the results of this adjunctive treatment in patients resistant to established therapy.Results: This series reports four patients treated with teduglutide off-label. The first case had Crohn's disease (CD) with persistent colocutaneous fistulae that demonstrated complete closure after 8 months of teduglutide therapy. The second case involved a PN-dependent CD patient with persistent fistulae and intra-abdominal abscesses who weaned off PN and had a significant improvement in her nutritional status after 3 months of teduglutide therapy. The third case had CD complicated by severe malnutrition and previous PN-associated line infections, but by 9 months of teduglutide therapy, she gained 5 kg and no longer required re-initiation of PN. The fourth case had a high-output diverting ileostomy with resultant impaired healing of a stage IV decubitus ulcer, and after 2 months of therapy, the patient's pre-albumin increased by 250% and the ulcer had decreased by 40% in size.Conclusion: The use of teduglutide might be broadened to include patients with functional SBS not meeting strict criteria for intestinal failure. Further studies should evaluate the efficacy of teduglutide in patients who may require short-term small intestine rehabilitation or who have chronically impaired absorptive capacity not yet requiring PN. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a syndrome in which a significant amount of intestine is lost due to functional or congenital anomalies, resulting in maldigestion, malabsorption, and malnutrition. Children with SBS rely on parenteral nutri tion (PN) for energy and growth. However, this life-sustaining treatment is associated with liver failure and intestinal atrophy, among other complications that can lead to morbidity and mortality. Thus, the goal of SBS therapy is to achieve enteral autonomy. The purpose of this article is to evaluate therapies aimed at protecting the bowel, promoting intestinal adaption, and increasing intestinal absorptive capabilities, with the goal of weaning PN support and achieving enteral autonomy in pediatric SBS. These therapies include bowel-lengthening procedures, bowel tissue engineering, and new hormonal treatments. Promising hormonal treatments include teduglutide, ghrelin, growth hormone, and glutamine. Additionally, new parenteral lipids formulations, Smoflipid® and Omegaven®, can result in improved long-term outcomes in children with SBS when compared to the current standard soy lipid. Nurses should consider these lipid options and therapies to help wean PN support and achieve enteral autonomy in pediatric SBS. [ABSTRACT FROM AUTHOR]

Aim: to communicate HPN data obtained from the HPN registry of the NADYA-SENPE group (www.nadya-senpe.com) for the year 2017.Material and Methods: descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1st, 2017 to December 31st, 2017.Results: there were 308 patients from 45 Spanish hospitals (54.5% women), 38 children and 270 adults, with 3,012 episodes, which represent a prevalence rate of 6.61 patients/million inhabitants/year 2017. The most frequent diagnosis in adults was "palliative cancer" (25.6%), followed by "others". In children, it was Hirschsprung's disease with six cases (15.8%). The first indication was short bowel syndrome in both children (55.3%) and adults (33.7%). The most frequently used type of catheter was tunneled in both children (73.4%) and adults (38.2%). Ending 81 episodes, the most frequent cause was death (62.9%) and transition to oral feeding (34.7%).Conclusions: the progressive increase of collaborating centers and professionals in the registry of patients receiving NPD is maintained. The main indications of HPN and the motive for ending have remained stable. [ABSTRACT FROM AUTHOR]

We report a case of vanishing gastroschisis visualized by antenatal ultrasound with a 7-year long term follow-up. Currently, the child is still dependent on daily parenteral nutrition with no signs of hepatotoxicity. To our knowledge, it's the fourth case with a long-term follow-up. Vanishing gastroschisis is a rare complication of gastroschisis. However, physicians should be aware of it because its prognosis is worse than classical gastroschisis. When a vanishing gastroschisis is visualized or suspected by antenatal ultrasound, prenatal counseling is required with explanations about the risk of short bowel syndrome, the need of parenteral nutrition and related complications (inflammatory colitis, sepsis, liver failure and organ transplant). Mortality rate was initially around 93%, and dropped to 27% after the years 2000 (versus 10% for classical gastroschisis). After birth, all children will require surgery, and sometimes autologous gastro-intestinal reconstruction. Most survivors (68%) could be taken off the TPN. Unfortunately, long-term outcomes for children with vanishing gastroschisis are still missing in current literature. [ABSTRACT FROM AUTHOR]

Background Since short bowel syndrome (SBS) patients face life-threatening conditions, the development of therapeutic strategies to induce intestinal adaptation has been investigated. Ghrelin, a ligand of growth hormone (GH) secretagogue-receptor that stimulates the release of GH and insulin like growth factor-1 (IGF-1), has several pleiotropic effects. We investigated whether ghrelin induces intestinal adaptation in parenterally fed rats with SBS. Methods Sprague-Dawley rats underwent venous catheterization and were divided into 3 groups: those receiving 90% small bowel resection while leaving the proximal jejunum and distal ileum (90% SBR) with TPN (SBS/TPN group), those receiving 90% SBR with TPN + ghrelin (SBS/TPN/ghrelin group), and those receiving sham operation and fed chow (sham group). Ghrelin was administered intravenously at 10 μg/kg/day. On Day 13, the rats were euthanized and the small intestine harvested, and the histology and crypt cell proliferation rates (CCPR), apoptosis, and nutrient transporter protein levels were analyzed and the plasma hormones were measured. Results The villus height and crypt depth of the ileum in the SBS/TPN/ghrelin group were significantly higher than in the SBS/TPN group. The CCPR of the jejunum and the ileum significantly increased by the administration of ghrelin; however, the apoptosis rates did not significantly differ between the SBS/TPN and SBS/TPN/ghrelin groups. Significant differences did not exist in the plasma IGF-1 and nutrient transporter protein levels among three groups. Conclusions The intravenous administration of ghrelin stimulated the morphological intestinal adaptation of the ileum to a greater degree than the jejunum due to the direct effect of ghrelin. [ABSTRACT FROM AUTHOR]

A study of 4,311 trauma ICU patients reported bowel ischemia in 13 patients (0.3%). In another study of 259 adult ICU patients receiving simultaneous EN and vasopressor agents for at least 1 hour, there were three episodes of bowel ischemia or perforation (0.9%) ([23]). Future RCTs of EN in critically ill patients need to incorporate bowel ischemia as a priori study outcome and perhaps should stratify enrolled patients based on hemodynamic status and on the grade of acute gastrointestinal injury. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Résumé But/Objectif En raison de l’hypersécrétion gastrique et/ou d’importantes pertes intestinales, certains patients nécessitent un apport en ranitidine dans leur mélange de nutrition parentérale (NP). L’objectif était de réévaluer l’intérêt clinique de la ranitidine dans les prescriptions de NP suite à des tensions d’approvisionnement en ranitidine injectable. Patients et méthodes Un suivi clinique prospectif des patients en NP à domicile (NPAD) a été réalisé pendant 4 mois selon trois critères : l’arrêt, la substitution ou le maintien de la ranitidine dans les prescriptions de NP. Résultats Parmi les 109 patients en NPAD, 64 (59 %) bénéficiaient de ranitidine dans leur NP. L’arrêt définitif, tenté chez 23 patients (36 %), s’est soldé par deux échecs avec retour à la prescription initiale. Le relais per os a concerné 22 patients (34 %) et a conduit à quatre échecs. Pour ces 6 patients, des nausées/vomissements et des accélérations du transit intestinal ont été observés. Ces effets secondaires ont concerné des patients atteints d’un syndrome du grêle court (SGC). Enfin, chez 19 patients (30 %) atteint soit d’un SGC sévère, d’une Maladie de Hirschsprung étendue ou d’une Pseudo obstruction intestinale chronique, la ranitidine a dû être maintenue. Conclusion Une réévaluation des prescriptions a été menée en respectant les profils cliniques particuliers des patients avec l’arrêt d’un grand nombre de prescription de ranitidine. Cependant, la ranitidine reste indispensable chez les patients présentant de graves troubles digestifs (nausées, vomissements, pertes élevées). Néanmoins, la voie intraveineuse peut être relayée par la voie orale quand cela est possible, associée à un suivi clinique. Objectives Because of gastric hypersecretion and/or high water-electrolytes losses, some patients require ranitidine in their parenteral nutrition (PN) mixture. Our goal was to reassess the clinical utility of adding ranitidine in NP mixtures following tensions in the supply of ranitidine for parenteral injection. Patients and methods A prospective clinical follow-up of patients on home parenteral nutrition (HPN) over a period of 4 months was carried out according to three criteria: “stopping”, “substituting” or “maintaining” ranitidine in NP prescriptions. Results At the beginning of the study, 109 patients were followed on HPN program. Most of them (48 %) having short bowel syndrome (SBS). Initially, 64 patients were receiving ranitidine in association with PN. Attempt to stop IV Ranitidine was achieved in 23 patients (36 %): 2 failures led to reverse to the initial prescription. The change to the oral route involved 22 patients (34 %) with 4 failures. These failures occurred shortly after modifications, with nausea/vomiting, increased water-electrolytes losses in SBS patients. On 19 patients (30 %), ranitidine was maintained, due to insufficient intestinal absorption from children with extreme SBS, extended Hirschsprung Disease, and Chronic Intestinal Pseudo Obstruction. Conclusion A review of ranitidine prescriptions was conducted according to specific clinical profiles of patients. Ranitidine appears necessary for patients with severe digestive disorders. Nevertheless, an oral administration of ranitidine can be considered with clinical monitoring. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a well-known cause of intestinal failure (IF) (1). SBS occurs after extensive resection of the small bowel (RSB) resulting in a bowel length of less than 150/200 cm. The colon may have been partially or completely removed. SBS patients experience severe water and nutrient malabsorption, so that they are often managed with parenteral nutrition (PN) to supplement their oral intake (2-4). A complete understanding of the pathophysiology of SBS and postoperative adaptations may allow identifying the spontaneous processes that compensate for the reduction in absorptive surface. A better knowledge of these adaptive mechanisms may help to improve the management of patient nutrition, to reduce the need for PN and to prevent D-encephalopathy episodes. This review focuses on the overall adaptations described in adult SBS patients but does not review pediatric cases. [ABSTRACT FROM AUTHOR]

Introduction:  Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.Materials and Methods:  Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994-2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).Results:  In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0-38.0); median birth weight was 2,440 g (IQR: 1,700-2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0-92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.Conclusion:  We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF. [ABSTRACT FROM AUTHOR]

Background and aims Patients on parenteral nutrition for short bowel syndrome (SBS) have a high risk of thrombotic complications and are often treated with parenteral anticoagulation. Direct oral anticoagulants are absorbed proximally in the digestive tract and may represent alternative regimens in selected SBS patients. In our pilot study, we provided pharmacokinetics parameters of dabigatran etexilate and rivaroxaban in this setting and compared peak (Cmax), trough (Ctrough) concentrations, and areas-under-the-concentration-time-curve (AUC 0 − t ) to reference values retrieved from phase I-III studies. Methods We enrolled 6 adults with a remaining small bowel length ≤ 200 cm, normal renal/hepatic function, and intact stomach. In our crossover study, patients were exposed to twice-daily dabigatran etexilate 150 mg and once-daily rivaroxaban 20 mg. Results After 5 days of dabigatran dosing, Ctrough and Cmax geometric means were 39 μg/L (90% CI: 23–66) and 88 μg/L (90% CI: 56–137), respectively; AUC 0–12 h was 958 μg ∗ h/L (90% CI: 635–1445). After 5 days of rivaroxaban dosing, Ctrough and Cmax geometric means were 9 μg/L (90% CI: 1–71) and 167 μg/L (90% CI: 102–276), respectively; AUC 0–24 h was 1720 μg ∗ h/L (90% CI: 899–3300). Absorption was negligible in one patient with ultra-short (~ 15 cm) bowel. For dabigatran, Cmax ratio was 0.57 (SD 0.33) and Ctrough ratio was 0.35 (SD 0.44). For rivaroxaban, the mean observed-to-reference ratios AUC 0–24 h and Cmax ratios were 0.73 (SD 0.32) and 0.76 (SD 0.34), respectively. Conclusions While in SBS patients there is some absorption of the oral anticoagulants dabigatran etexilate and rivaroxaban, it appears to be lower than reference values. Plasma drug levels showed significant inter-individual variability. [ABSTRACT FROM AUTHOR]

Purpose: Short-bowel syndrome (SBS) is associated with high morbidity and mortality. We conducted this study to establish the predictors of survival and weaning off parenteral nutrition (PN). Methods: We reviewed the medical records of 16 SBS infants treated at our institution within a 30-year period. SBS was defined as a residual small-bowel length (RSBL) of <75 cm. Loss of the ileocecal valve (ICV), cholestasis (D-Bil >2.0 mg/dl), enterostomy, and RSBL were all evaluated. Kaplan-Meier analysis was used to analyze the predictors. Results: The mean RSBL was 34.9 ± 22.9 cm. Six patients died (37.5%) and nine patients were weaned off PN (56.3%). Significant differences were observed in cholestasis ( p < 0.03), enterostomy ( p < 0.01), an absolute RSBL of <30 cm ( p < 0.04), and a percentage of expected RSBL of <10% ( p < 0.04) as survival predictors. Significant differences were also observed for cholestasis ( p < 0.01), loss of the ICV ( p < 0.04), an absolute RSBL of <20 cm ( p < 0.01), and a percentage of expected RSBL of <10% ( p < 0.03) as predictors of weaning off PN. Conclusion: These predictors may help us select the optimal treatments for pediatric patients with SBS. [ABSTRACT FROM AUTHOR]

Introduction: Short bowel syndrome (SBS) neonates have complex management challenges, meaning a significant health care extra cost. This is 10 years retrospective SBS study-based estimate of children and neonates in our hospital. Population estimate of the incidence and mortality rate of neonates with SBS is not so accurate because of the differences in the definition, followup and regional referral patterns. The introduction of total parenteral nutrition (TPN) led to a remarkable improvement in the survival of SBS patients, but unfortunately the most common cause of death in SBS patient's is also TPNinduced hepatic dysfunction. Even though the survival of patients with less than or equal to 40 cm of residual small bowel is now routine. The long term survival of infants with as little as 20 to 30 cm small bowel can be expected. The management goal of these patients is to reduce the duration of TPN and to maximize intestinal nutrient absorption. It is difficult to predict the duration and the type of nutritional support for patients with SBS. Some patients may require permanent parenteral nutrition on either a continuous or intermittent basis depending on the length of the residual bowel. Objective: To evaluate the direct and indirect evidence that adaptation occurs after an extensive bowel resection, to review the factors that influence adaptation and to assess the strategies used in attempts to optimize this process. Methodology: A retrospective medical record review of newborns and children with SBS treated at our hospital between 2007 and 2017. Medical records of patients studied were retrieved from our archives and analyzed. Results: a p-value less than 0.05 (p-value = 0.033) was obtained, being statistically significant proving that there is a direct correlation between the post-operation intestinal length and the duration of parenteral nutrition. Conclusion: The major predictors of weaning from PN are adjusted small bowel length and the amount of energy patient can derive from enteral feeding attempts, also as a result of new management strategies combined with a multidisciplinary team approach, majority of patients will wean from PN despite short intestinal length. [ABSTRACT FROM AUTHOR]

Parenteral nutrition (PN)-induced villus atrophy is a major cause of intestinal failure (IF) for children suffering from short bowel syndrome (SBS), but the precise mechanism remains unclear. Herein, we report a pivotal role of farnesoid X receptor (FXR) signaling and fatty acid oxidation (FAO) in PN-induced villus atrophy. A total of 14 pediatric SBS patients receiving PN were enrolled in this study. Those patients with IF showed longer PN duration and significant intestinal villus atrophy, characterized by remarkably increased enterocyte apoptosis concomitant with impaired FXR signaling and decreased FAO genes including carnitine palmitoyltransferase 1a (CPT1a). Likewise, similar changes were found in an in vivo model of neonatal Bama piglets receiving 14-day PN, including villus atrophy and particularly disturbed FAO process responding to impaired FXR signaling. Finally, in order to consolidate the role of the FXR-CPT1a axis in modulating enterocyte apoptosis, patient-derived organoids (PDOs) were used as a mini-gut model in vitro. Consequently, pharmacological inhibition of FXR by tauro-β-muricholic acid (T-βMCA) evidently suppressed CPT1a expression leading to reduced mitochondrial FAO function and inducible apoptosis. In conclusion, impaired FXR/CPT1a axis and disturbed FAO may play a pivotal role in PN-induced villus atrophy, contributing to intestinal failure in SBS patients. [ABSTRACT FROM AUTHOR]

Short bowel syndrome is a severe malabsorption disorder, and prolonged parenteral nutrition is essential for survival in some cases. Among the undesirable effects of long-term parenteral nutrition is an increase in proinflammatory cytokines. The aim of the present study was to measure the serum levels of interleukin-6, interleukin-10, tumor necrosis factor alpha, and transforming growth factor beta, in patients with short bowel syndrome on cyclic parenteral nutrition and patients who had previously received but no longer require parenteral nutrition. The study was cross-sectional and observational. Three groups were studied as follows: Parenteral nutrition group, 9 patients with short bowel syndrome that receive cyclic parenteral nutrition; Oral nutrition group, 10 patients with the same syndrome who had been weaned off parenteral nutrition for at least 1 year prior to the study; Control group, 13 healthy adults, matched for age and sex to parenteral and oral groups. The following data were collected: age, tobacco use, drug therapies, dietary intake, body weight, height, blood collection. All interleukins were significantly higher in the parenteral group compared with the control group as follows: interleukin-6: 22 ± 19 vs 1.5 ± 1.4 pg/mL, P = .0002; transforming growth factor β : 854 ± 204 vs 607 ± 280 pg/mL, P = .04; interleukin-10: 8 ± 37 vs 0.6 ± 4, P = .03; tumor necrosis factor α : 20 ± 8 vs 8 ± 4 pg/mL, P < .0001. We concluded that parenteral nutrition in short bowel syndrome patients, regardless of its duration, increases serum proinflammatory cytokines. [ABSTRACT FROM AUTHOR]

Background and Objectives: Certain patients who undergo proximal jejunum resection are unable to undergo primary anastomosis and require exteriorization of the proximal jejunum. These patients usually have major problems with short bowel due to the high output of the stoma. The output of a proximal jejunostomy contains abundant amounts of enzymes and electrolytes. Therefore, it is a feasible approach to re-infuse jejunostomy output to regain homeostasis. To evaluate the effects of proximal jejunostomy output reinfusion into the distal small bowel for patients with short bowel syndrome, and to determine whether reinfusion could avoid long-term parenteral nutrition (PN).Methods and Study Design: PN was initiated immediately after surgery. When patients started enteral nutrition, we started the proximal jejunostomy output reinfusion protocol. Proximal jejunostomy output reinfusion was performed by the patients, and continued by them after discharge. When proximal jejunostomy output reinfusion could be performed stably, PN was stopped.Results: The median length of the proximal jejunum was 20 cm and of the distal small bowel was 77.5 cm in patients who could stably receive proximal jejunostomy output reinfusion alone. Three patients did not require home PN; they only required PN during hospitalization. Four patients successfully underwent stoma takedown with intestinal anastomosis after 6-7 months without any nutritional or metabolic complications.Conclusion: Short bowel syndrome patients with an adequate length of small bowel and functional colon could avoid long-term PN by receiving reinfusion of proximal jejunostomy output into the distal small bowel. [ABSTRACT FROM AUTHOR]

Background & Aims We aimed to evaluate the value of AST to platelet ratio ( APRI) and transient elastography ( TE) as predictors of liver histopathology in children with intestinal failure ( IF). Methods Altogether 93 liver biopsies from 57 children with parenteral nutrition ( PN) duration ≥3 months were analysed. APRI measurement and TE ( n = 46) were performed at the time of biopsy. Results IF was caused by short bowel syndrome in 75% of patients. At the time of liver biopsy, PN dependent patients ( n = 42) were younger with longer PN duration compared to those weaned off PN ( n = 51) (2.2 vs. 7.6 years, P < 0.001; 26 vs. 10.5 months, P = 0.043). Elevated transaminase or bilirubin levels were found in 51%, splenomegaly in 26%, and oesophageal varices in 3.5%. Histological fibrosis was present in 61% (Metavir stage F1; 27%, F2; 26%, F3-4; 9%), cholestasis in 25% and steatosis in 22% of biopsy specimens. TE was superior to APRI in prediction of any liver histopathology (fibrosis, cholestasis or steatosis) with areas under the receiving operating curve ( AUROC) of 0.86 (95% CI 0.74-0.97) and 0.67 (95% CI 0.58-0.78) respectively. For prediction of ≥F1 and ≥F2 fibrosis, AUROC values for TE were 0.78 (95% CI 0.64-0.93) and 0.73 (95% CI 0.59-0.88), whereas APRI did not correlate with fibrosis stages. For detection of histological cholestasis, the AUROC for APRI was 0.77 (95% CI 0.64-0.89). Conclusions Both TE and APRI are promising noninvasive methods for monitoring the development of IF-related liver histopathology. TE values reflected the degree of fibrosis better while APRI detected histological cholestasis more accurately. [ABSTRACT FROM AUTHOR]

Background: institutions with multidisciplinary teams have shown improvements in patient outcomes with intestinal failure. Multidisciplinary approach allows an integral management and effective communication between families and care teams.Objective: describe the multidisciplinary management and outcome in pediatric patients with intestinal failure.Methods: retrospective study in patients 18 years old or less, with intestinal failure and Total Parenteral Nutrition (TPN) required. Simple frequencies and percentages were used for qualitative variables, and central tendency and dispersion measures were used for quantitative variables.Results: 33 patients with a median follow up of 281 days were evaluated. The median duration of the TPN was 68 days and the mean of catheter-related infections was 2.26 per patient. In 31 patients oral or enteral nutrition was provided, starting in 61.3% of cases through tube and continuous infusion. As concomitant treatment 72.7% of children received ursodeoxycholic acid, 67.7%, cholestyramine 57.6% loperamide, 48.5% antibiotics and 36.4% probiotic. The families of 24 patients were evaluated by social work professionals. Intestinal autonomy was achieved in 69.7% of cases, 72.7% of them showed an improvement in the score z of weight and showed an end albumin significantly higher than the initial (p value: 0.012).Conclusions: the management of patients with intestinal failure is a challenge for health institutions and require care based on a standardized protocol and a multidisciplinary group. [ABSTRACT FROM AUTHOR]

Pediatric intestinal failure (IF) remains to be associated with significant morbidity and mortality, the most frequent underlying etiologies being short bowel syndrome (SBS), and primary motility disorders. Management aims to assure growth and development, while preventing complications and facilitating weaning off parenteral support (PS) by fully utilizing adaptation potential of the remaining gut. Probability of survival and weaning off PS is improved by coordinated multidisciplinary intestinal rehabilitation combining individualized physiological enteral and parenteral nutrition (PN), meticulous central line care and medical management with carefully planned surgical care. Increasing evidence suggests that autologous intestinal reconstruction (AIR) surgery is effective treatment for selected short bowel patients. Bowel lengthening procedures normalize pathological adaptation-associated short bowel dilatation with potential to support intestinal absorption and liver function by various mechanisms. Although reversed small intestinal segment, designed to prolong accelerated intestinal transit, improves absorption in adult SBS, its feasibility in children remains unclear. Controlled bowel obstruction to induce dilatation followed by bowel lengthening aims to gain extra length in patients with the shortest duodenojejunal remnant. Reduced PS requirement limits the extent of complications, improving prognosis and quality of life. The great majority of children with SBS can be weaned from PS while prognosis of intractable primary motility disorders remains poor without intestinal transplantation, which serves as a salvage therapy for life-threatening complications such as liver failure, central vein thrombosis or recurrent bloodstream infections. [ABSTRACT FROM AUTHOR]

In this case study, we demonstrate the effects of different lipid emulsions on liver function in a 52-year-old woman with short bowel syndrome who was totally dependent on parenteral nutrition. Over a 13-month period after small bowel resection and jejunostomy, we followed the patient's plasma triglycerides and liver enzyme levels as well as body weight and discomfort levels. During the first 3 months when parenteral nutrition including a lipid emulsion containing 50% soybean oil/50% medium-chain triglyerides was administered daily, the patient reported feeling unwell (experiencing dizziness and palpitations) and her triglycerides and liver enzyme levels rose to 366 mg/dL and 145 U/L (alanine aminotransferase [ALT]), respectively; these levels recovered when this emulsion was discontinued. For the following 9 months, an emulsion containing 80% olive oil and 20% soybean oil was administered, and the patient's triglycerides (182 mg/dL) did not increase to abnormal levels and liver enzyme levels were only mildly elevated (109 U/L). The patient felt well and her body weight increased from 51 kg to 55 kg during this period. These results suggest that parenteral nutrition with a reduced soybean oil content may better preserve liver function in patients with short bowel syndrome. [ABSTRACT FROM AUTHOR]