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Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain. This was a national multicentre and prospective study of paediatric patients with intestinal failure (IF) treated with teduglutide for at least 3 months. The data included demographic characteristics, medical background, anthropometric data, laboratory assessments, adverse events, and parenteral nutrition (PN) requirements. Treatment response was defined as a > 20% reduction in the PN requirement. The data were collected from the Research Electronic Data Capture (REDCap) database. Thirty-one patients from seven centres were included; the median age at the beginning of the treatment was 2.3 (interquartile range (IQR) 1.4–4.4) years; and 65% of the patients were males. The most frequent cause of IF was SBS (94%). The most common cause of SBS was necrotizing enterocolitis (35%). The median residual bowel length was 29 (IQR 12–40) cm. The median duration of teduglutide therapy was 19 (IQR 12–36) months, with 23 patients (74%) treated for > 1 year and 9 treated for > 3 years. The response to treatment was analysed in 30 patients. Twenty-four patients (80%) had a reduction in their weekly PN energy > 20% and 23 patients (77%) had a reduction in their weekly PN volume > 20%. Among the responders, 9 patients (29%) were weaned off PN, with a median treatment duration of 6 (IQR 4.5–22) months. The only statistically significant finding demonstrated an association between a > 20% reduction in the weekly PN volume and a younger age at the start of treatment (p = 0.028). Conclusions: Teduglutide seems to be an effective and safe treatment for paediatric patients with IF. Some patients require a prolonged duration of treatment to achieve enteral autonomy. Starting treatment with teduglutide at a young age is associated with a higher response rate. What is Known: • Glucagon-like peptide-2 (GLP-2) plays a crucial role in the regulation of intestinal adaptation in short bowel syndrome (SBS). Teduglutide is a GLP-2 analog that reduces the need for parenteral support in patients with SBS. • Data about long-term therapy with teduglutide in children in real life are still scarce. What is New: • Most pediatric patients with SBS respond in a satisfactory manner to teduglutide treatment. The occurrence of long-term adverse effects is exceptional. • Starting treatment with the drug at a young age is associated with a greater response rate. [ABSTRACT FROM AUTHOR]

The role of long‐term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long‐term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro‐intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut–brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro‐gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify – and hopefully reduce the potential for harm associated with – the use of long‐term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life‐threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time‐limited period to achieve nutritional safety, while the wider multi‐disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition. [ABSTRACT FROM AUTHOR]

Background: The primary objective of this study is to comparatively assess the safety of nasogastric (NG) feeding versus nasojejunal (NJ) feeding in patients with acute pancreatitis (AP), with a special focus on the initiation of these feeding methods within the first 48 h of hospital admission. Methods: Studies were identified through a systematic search in PubMed, EMbase, Cochrane Central Register of Controlled Trials, and Web of Science. Four studies involving 217 patients were included. This systematic review assesses the safety and efficacy of nasogastric versus nasojejunal feeding initiated within 48 h post-admission in moderate/severe acute pancreatitis, with a specific focus on the timing of initiation and patient age as influential factors. Results: The results showed that the mortality rates were similar between NG and NJ feeding groups (RR 0.86, 95% CI 0.42 to 1.77, P = 0.68). Significant differences were observed in the incidence of diarrhea (RR 2.75, 95% CI 1.21 to 6.25, P = 0.02) and pain (RR 2.91, 95% CI 1.50 to 5.64, P = 0.002) in the NG group. The NG group also showed a higher probability of infection (6.67% vs. 3.33%, P = 0.027) and a higher frequency of multiple organ failures. Subgroup analysis for early intervention (within 48 h) showed a higher risk of diarrhea in the NG group (RR 2.80, P = 0.02). No significant differences were found in the need for surgical intervention, parenteral nutrition, or success rates of feeding procedures. Conclusion: This meta-analysis highlights the importance of considering the method and timing of nutritional support in acute pancreatitis. While NG feeding within 48 h of admission increases the risk of certain complications such as diarrhea and infection, it does not significantly impact mortality or the need for surgical intervention. [ABSTRACT FROM AUTHOR]

Intra‐abdominal desmoid tumors are a rare and complex clinical problem. These tumors are locally invasive, and surgical ablation represents the mainstay of treatment. When localized at the root of the mesentery, their resection may require extensive excision of the intestine resulting in intestinal failure and life‐long total parenteral nutrition. Intestinal transplantation, either autotransplantation or allotransplantation, has been used as a viable option to treat this group of patients. Herein, we describe a series of 4 patients with unresectable intra‐abdominal desmoid tumor who underwent cadaveric isolated intestinal and ascending colon transplantation. [ABSTRACT FROM AUTHOR]

Background: Malrotation and volvulus classically present with bilious vomiting. It is more common earlier in life, but there are other causes of bile‐stained vomiting. This leads some clinicians to 'watch and wait'. In the presence of a volvulus, this is potentially a fatal decision. It is not clear from the literature if there is a safe time window in which children can be observed in the hope of avoiding transfers or radiological investigations. Aim: To determine whether time to identification and management of midgut volvulus correlated with morbidity and mortality; and whether there were patterns to transition of care. Methods: Multicentre, retrospective review of all children with malrotation ± volvulus at two tertiary children's hospitals in Brisbane from 2000 to 2012. Data collected included age at presentation, timing between symptom onset and presentation, radiological findings, and definitive surgical management. Outcomes included patient length of stay (LOS), total parenteral nutrition (TPN) duration, re‐operations and death. Results: There were 96 cases of malrotation identified, with 23 excluded (elective operation, insufficient data). Neonates made up 66% of included cases. Only 14% of cases were over 12 months old. Bilious vomiting or bile‐stained aspirates were the presenting symptoms in 71% (52). Overall mortality was 5.56%. Time from symptom onset to presentation or management was not significantly associated with morbidity or mortality. More than half (53%, 39/73) of patients received total parenteral nutrition; 20/39 for more than 10 days. Neonates and infants had a significantly higher rate of TPN compared with older children (P < 0.001). Those requiring TPN post‐operatively had a significantly higher mortality compared with those who did not (P = 0.02). Time from symptom onset to presentation or definitive management was not significantly associated with LOS, TPN duration, or need for re‐operation. Conclusion: Malrotation remains a time‐critical diagnosis to secure and treat. Even a short duration of symptoms can be associated with high morbidity or mortality. There is no place for 'watch and wait' for such patients, and malrotation/volvulus should be emergently actively excluded with contrast studies. [ABSTRACT FROM AUTHOR]

Background: Nutritional administration in acute pancreatitis (AP) management has sparked widespread discussion, yet contradictory mortality results across meta‐analyses necessitate clarification. The optimal nutritional route in AP remains uncertain. Therefore, this study aimed to compare mortality among nutritional administration routes in patients with AP using consistency model. Methods: This study searched four major databases for relevant randomized controlled trials (RCTs). Two authors independently extracted and checked data and quality. Network meta‐analysis was conducted for estimating risk ratios (RRs) with 95% confidence interval (CI) based on random‐effects model. Subgroup analyses accounted for AP severity and nutrition support initiation. Results: A meticulous search yielded 1185 references, with 30 records meeting inclusion criteria from 27 RCTs (n = 1594). Pooled analyses showed the mortality risk reduction associated with nasogastric (NG) (RR = 0.34; 95%CI: 0.16–0.73) and nasojejunal (NJ) feeding (RR = 0.46; 95%CI: 0.25–0.84) in comparison to nil per os. Similarly, NG (RR = 0.45; 95%CI: 0.24–0.83) and NJ (RR = 0.60; 95%CI: 0.40–0.90) feeding also showed lower mortality risk than total parenteral nutrition. Subgroup analyses, stratified by severity, supported these findings. Notably, the timing of nutritional support initiation emerged as a significant factor, with NJ feeding demonstrating notable mortality reduction within 24 and 48 h, particularly in severe cases. Conclusion: For severe AP, both NG and NJ feeding appear optimal, with variations in initiation timings. NG feeding does not appear to merit recommendation within the initial 24 h, whereas NJ feeding is advisable within the corresponding timeframe following admission. These findings offer valuable insights for optimizing nutritional interventions in AP. [ABSTRACT FROM AUTHOR]

Hyperemesis gravidarum has a reported incidence of approximately 0.3–3% of pregnancies. Without treatment, refractory hyperemesis gravidarum can result in dehydration, electrolyte deficiencies, and severe nutritional deficiencies, resulting in significant maternal morbidity. The overall goals of inpatient management of refractory hyperemesis gravidarum are the resumption of oral intake to an adequate level to maintain hydration and nutrition, including the ability to tolerate oral pharmacotherapy. Patients initially are stabilized with rehydration and electrolyte repletion. There are numerous pharmacotherapeutics available that can be administered intravenously to control symptoms when oral intake is not an option. However, despite maximizing typical antiemetics, there will be cases refractory to these medications, and alternative pharmacotherapeutics and nutrition-support modalities must be considered. Mirtazapine, olanzapine, corticosteroids, and gabapentin are examples of alternative pharmacotherapeutics, and enteral and parenteral nutrition are alternative therapies that can be used when oral intake is not tolerated for prolonged time periods with ongoing weight loss. In refractory cases of hyperemesis gravidarum, the risks and benefits of these alternative forms of management must be considered, along with the risks of undertreated hyperemesis gravidarum and the overall effect of hyperemesis gravidarum on patients’ quality of life. [ABSTRACT FROM AUTHOR]

Background & Aims: The intestinal rehabilitation program (IRP) is a specialized approach to managing patients with intestinal failure (IF). The goal of IRP is to reduce the patient's dependence on parenteral nutrition by optimizing nutrition intake while minimizing the risk of complications and providing individualized medical and surgical treatment. We aimed to provide a thorough overview of our extensive history in adult IRP. Methods: We reviewed the medical records of adults with IF treated at our center's IRP over the past two decades. We collected data on demographic and clinical results, such as the causes of IF, the current status of the remaining bowel, nutritional support, and complications or mortality related to IF or prolonged parenteral nutrition. Results: We analyzed a total of 47 adult patients with a median follow-up of 6.7 years. The most common cause of IF was massive bowel resection due to mesenteric vessel thrombosis (38.3%). Twenty-eight patients underwent rehabilitative surgery, including 12 intestinal transplants. The 5-year survival rate was 81.9% with 13 patients who expired due to sepsis, liver failure, or complication after transplantation. Of the remaining 34 patients, 18 were successfully weaned off from parenteral nutrition. Conclusion: Our results of IRP over two decades suggest that the individualized and multidisciplinary program for adult IF is a promising approach for improving patient outcomes and achieving nutritional autonomy. [ABSTRACT FROM AUTHOR]

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]

Introduction: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. Objectives: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. Methods: The review is based on the authors' experience supported by an in‐depth review of the published literature. Results: The history of parenteral nutrition, including out‐patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. Discussion: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant‐free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life‐saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear. [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]

Background: Short bowel syndrome (SBS) is one of the most frequent causes of intestinal failure, needing parenteral nutrition to maintain an energy-protein and water-electrolyte balance. At the Hospital Infantil de México Federico Gómez (HIMFG), the formation of two stomas is a technique used for intestinal rehabilitation, where the use of residue through the bypass technique (BT) helps to maintain gastrointestinal functionality, water-electrolyte, and nutritional stability. This study aimed to describe the technique of using intestinal residue through BT as a treatment strategy in intestinal rehabilitation and its effect on the biochemical and nutritional status of pediatric patients with SBS. Methods: An analytical and retrospective cross-sectional study was performed in patients hospitalized at HIMFG with SBS who underwent BT during their hospital stay between 2019 and 2020 and then followed up for 8 weeks. Results: A total of 10 patients were included in this study, with a mean age of 24 months; 50% were female. BT was able to reduce the inflammatory process in the liver caused by the continuous use of parenteral nutrition; enteral caloric intake increased from 25.32 kcal/kg/day to 72.94 kcal/kg/day, but it was insufficient to improve their nutritional status. Conclusions: BT is a safe and effective alternative in intestinal rehabilitation in patients with SBS to stimulate trophism and intestinal functionality, allowing a progression of enteral feeding and a decrease in the hepatic inflammatory process that occurs in these patients with prolonged parenteral nutrition. [ABSTRACT FROM AUTHOR]

Purpose: The incidence and risk factors of catheter-related bloodstream infections (CRBSI) in patients with intestinal failure (IF) have not been established, partly because catheter management methods vary from different facilities. This study aimed to identify the risk factors and incidence rate of CRBSIs in patients with IF who were given prophylactic treatment. Methods: Sixteen patients with IF who required home parenteral nutrition were enrolled in this study. Prophylactic management of CRBSI included monthly ethanol lock therapy and standardized infection prevention education. The outcomes included the incidence and risk factors of CRBSI. Results: The median incidence rate of CRBSI was 1.2 per 1000 catheter days. Univariate analysis showed that the risk of developing CRBSI was significantly associated with short bowel syndrome (< 30 cm) (p = 0.016). Other relevant findings included a significant negative correlation between serum albumin and CRBSI rate (r = − 0.505, p = 0.046), and past history of mixed bacterial infections was significantly associated with increased CRBSI rate (p = 0.013). Conclusion: CRBSIs can still develop despite undergoing prophylactic management. Risk factors for CRBSI include the residual intestinal length, nutritional status, and susceptibility to certain microorganisms. [ABSTRACT FROM AUTHOR]

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare motility disorder with high mortality rate described by Berdon in 1976. We present the first case of Berdon’s syndrome (heterozygous pathogenic variant in the ACTG2 gene) reported in Romania, a female newborn admitted in NICU “Marie S. Curie” Emergency Children’s Hospital Bucharest for intestinal obstruction after birth. Total parenteral nutrition, ileostomy, gastrostomy, clean intermittent bladder catheterisation, evaluation for multivisceral transplantation were performed. She was discharged from our NICU ward at the age of 4 years and 2 months with home total parenteral nutrition administered by her mother in sterile condition, clean intermittent catheterisation for bladder evacuation performed by her mother, monitored monthly for about three years, with normal cardio-respiratory function, no signs of thrombosis, she maintained relatively low platelet count without positive blood culture, good liver and renal function test. Normal neurological and psychomotor development according to age. Her course was complicated by multiorgan failure with death ensuing at the age of 7 years and 10 months. [ABSTRACT FROM AUTHOR]

Introduction: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung's disease. Here, we report the results of the Japanese ITx registry. Methods: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. Results: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. Conclusion: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good. [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]

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare motility disorder with high mortality rate described by Berdon in 1976. We present the first case of Berdon’s syndrome (heterozygous pathogenic variant in the ACTG2 gene) reported in Romania, a female newborn admitted in NICU “Marie S. Curie” Emergency Children’s Hospital Bucharest for intestinal obstruction after birth. Total parenteral nutrition, ileostomy, gastrostomy, clean intermittent bladder catheterisation, evaluation for multivisceral transplantation were performed. She was discharged from our NICU ward at the age of 4 years and 2 months with home total parenteral nutrition administered by her mother in sterile condition, clean intermittent catheterisation for bladder evacuation performed by her mother, monitored monthly for about three years, with normal cardio-respiratory function, no signs of thrombosis, she maintained relatively low platelet count without positive blood culture, good liver and renal function test. Normal neurological and psychomotor development according to age. Her course was complicated by multiorgan failure with death ensuing at the age of 7 years and 10 months. [ABSTRACT FROM AUTHOR]

Background: teduglutide is an agonist of glucagon-related peptide (aGLP2) effective as a treatment for patients with short bowel syndrome (SBS), an entity that affects quality of life, usually requires home parenteral nutrition (HPN) and generates significant health costs. The objective of the present narrative review was to assess the real-life experience reported with teduglutide. Methods and results: in real life, one meta-analysis and studies published with 440 patients indicate that Teduglutide is effective after the period of intestinal adaptation after surgery, reducing the need for HPN and in some cases even allowing it to be suspended. The response is heterogeneous, increasing progressively up to 2 years after the start of treatment and reaching 82 % in some series. The presence of colon in continuity is a negative predictor of early response, but a positive predictive factor for the withdrawal of HPN. The most common side effects are gastrointestinal in the early stages of treatment. There are late complications related to the stoma or the occurrence of colon polyps, although the frequency of the latter is very low. In adults, data on improved quality of life and cost-effectiveness are scarce. Conclusions: teduglutide is effective and safe and data from pivotal trials for the treatment of patients with SBS are confirmed in real life and can reduce or even stop HPN in some cases. Although it seems cost-effective, more studies are needed to identify those patients with the greatest benefit. [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]

Introduction: Short bowel syndrome (SBS) is a debilitating condition associated with significant morbidity and mortality. Historically, SBS patients require indefinite parenteral nutrition (PN) and endure lifelong nutritional challenges. The purpose of this study was to review the outcomes, specifically nutritional independence, of a multidisciplinary nutrition service. Methods: A retrospective analysis of SBS patients followed by our surgical nutrition service was performed. Patients without 1-year follow-up were excluded. Demographics and nutritional parameters were collected at 4 intervals: initial presentation, 1-year, 2-year, and 5-year follow-up. Short bowel syndrome anatomical subtypes identified through operative reports were characterized as end jejunostomy, jejunocolonic, or jejuno-ileocolonic with ileo-cecal valve intact. Intestinal failure was defined by the requirement of PN, while intestinal insufficiency was defined by enteral support requirement. Clinical outcomes examined included mortality, fistula closure, and nutritional independence. Results: The study cohort comprised 89 patients, 50 of whom had ≤ 100 cm intestinal length. Mean age was 57 ± 17y, 55 (62%) were female, and median initial intestinal length was 77 [60-120] cm. Short bowel syndrome was complicated by fistulas in 47 (53%) of patients. Overall mortality was 13%, and 67 (75%) were liberated from PN. A total of 58 (65%) underwent operative intervention and fistula closure was achieved in 37 of 47 (79%) patients. Conclusions: Short bowel syndrome patients can experience significant benefit under treatment by a multidisciplinary nutrition service. By incorporating surgical intervention, the majority of patients previously relegated to lifelong PN have the opportunity to become nutritionally independent within 5 years. [ABSTRACT FROM AUTHOR]

Background and Objectives: Home parenteral nutrition (HPN) is a life sustaining therapy for patients with chronic intestinal failure. Reported outcomes for Asian HPN patients are scarce. We aim to review the clinical outcomes of adult and paediatric HPN patients in our cohort which caters for 95% of Singaporean HPN patients. Methods and Study Design: This is a retrospective review of HPN patients from an adult (2002-2017) and paediatric cohort (2011-2017) from the largest tertiary PN centres in Singapore. Patient demographics and clinical outcomes were reviewed. Results: There were 41 adult and 8 paediatric HPN patients. Mean age was 53.0(±15.1) (adults) and 8(±1.8) years-old (paediatrics). Mean duration of HPN was 2.6(±3.5) and 3.5(±2.5) years. Leading indications for adult HPN were short bowel syndrome (SBS) (n=19,46.3%), mechanical obstruction (n=9,22.0%), and gastrointestinal dysmotility disorders (GID) (n=5,12.2%). Thirteen adult (31.7%) patients had underlying malignancy, with seven (17.3%) receiving palliative HPN. Indications for HPN amongst paediatric patients was GID (n=5,62.5%) and SBS (n=3,37.5%). Central line-associated bloodstream infection (CLABSI)/1000catheter-days was 1.0(±2.1) and 1.8(±1.3). Catheter associated venous thrombosis (CAVT)/1000catheter-days was 0.1(±0.4) and 0.7(±0.8). Biochemical Intestinal Failure Associated Liver Disease (IFALD) was found in 21.9% and 87.5%. For adults, median overall survival was 90-months (4.3,175.7,95%CI), with actuarial survival of 70.7%(1-year) and 39.0%(5-years). Median survival for adult patients with malignancy was 6-months (4.2,7.7,95%CI), actuarial survival of 85.7%(3-months) and 30.7%(1-year). One adult patient died from PN related complications. No paediatric deaths were noted. Conclusions: Whilst patient numbers were modest, we report comparable complication and survival rates to other international centres in both our adult and paediatric cohorts. [ABSTRACT FROM AUTHOR]

To describe the impact of nutritional interventions on the outcomes of children with short bowel syndrome (SBS). This was a retrospective descriptive observational review where data were obtained from the patient's medical records. Children with SBS between the ages of 0 and 24 months who obtained this diagnosis between January 2005 and December 2015 at a tertiary paediatric hospital in Cape Town were investigated. There were 46 patients (62% male, 38% female) included in the study. The median duration of parenteral nutrition (PN) support was one month (0.6, 2.2 months), after which 83% of patients were weaned from PN. Enteral nutrition was commenced in 96% of patients, with the majority (n = 36; 82%) starting on day six (±6; range 1–29 days) postoperatively and 80% of patients attaining full feeds at median 1.2 months (0.2, 36 months). Patients displayed a mean weight and length gain of 15 g/day (±4; range 19–92 g) and 2 cm/month (±1.4; range 0.25–4 cm) respectively. The main complications were PN-associated cholestasis (n = 17), fat malabsorption (n = 13) and vitamin D deficiency/insufficiency (n = 5). This study showed that early initiation of PN support was attained, and that most patients were able to achieve enteral autonomy. [ABSTRACT FROM AUTHOR]

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]

Background: Short bowel syndrome (SBS) is a leading cause of intestinal failure resulting in parenteral nutrition (PN) dependence and nutritional deficiencies. Long-term PN use is associated with the development of sepsis and intestinal failure-associated liver disease. Achieving enteral autonomy is the optimal way to prevent these complications. In SBS, the decreased intestinal length, bile acid deficiency, and rapid transit time contribute to fat malabsorption and continued PN dependence. We propose the use of an immobilized lipase cartridge (ILC; RELiZORB) that connects in-line with enteral feed tubing sets and is designed to breakdown the majority of fats provided in enteral nutrition (EN). Preclinical studies have demonstrated both improved fat and fat-soluble vitamin absorption with ILC use in a porcine model of SBS. To evaluate the clinical applicability of these findings, we designed a phase 3, open labeled, single center, clinical trial to determine the safety, tolerability, and efficacy of the RELiZORB enzyme cartridge when used daily with EN for 90 days. Methods: The patient population will include PN dependent children with SBS, aged 2–18 years. The primary outcome is the change in PN calories from baseline, assessed weekly throughout the study. Changes in growth Z-scores, 72-hour fecal fat and coefficient of fat absorption, plasma fatty acids and fat-soluble vitamins will also be evaluated. Assessment of change in continuous outcomes will be made using the area under the curve, expressed as a percent change relative to baseline, calculated over study day 7 to 90 (AUC7-90). The incidence of adverse events will be monitored and summarized by system organ class. Discussion: If successful, RELiZORB may offer a safe alternative to reducing PN dependence and achieving enteral autonomy in pediatric intestinal failure. These results would be clinically significant given the clear association between long-term PN use and complications in SBS. Trial registration: ClinicalTrials.gov NCT03530852; registered on May 21st, 2018, last update posted on September 14th, 2022. [ABSTRACT FROM AUTHOR]

The article discusses research which examined the safety and feasibility of daytime-restricted parenteral feeding and its association with oral intake or nutritional supplementation timing in children following hematopoietic stem cell transplant (HSCT). The study evaluated weight change, glucose levels and time to tolerate oral feeding among the patients. Outcomes include hyperglycemia, increased risks of infection, intensive care hospitalization and nonrelapse mortality in HSCT recipients.

Introduction The optimal timing of delivery for pregnancies complicated by prenatally diagnosed gastroschisis remains controversial. Therefore, the aim of this study was to find whether elective or expectant delivery is associated with improved neonatal outcome. Materials and Methods MEDLINE and Embase databases were searched for studies up to 2021 that reported timing of delivery for prenatally diagnosed gastroschisis. A systematic review and meta-analysis were then performed in group 1: moderately preterm (gestational age [GA]: 34–35 weeks) elective delivery versus expectant management after GA 34–35 weeks; and group 2: near-term (GA: 36–37 weeks) elective delivery versus expectant management after GA 36–37 weeks. The following clinical outcomes were evaluated: length of stay (LOS), total parenteral nutrition (TPN) days, bowel morbidity (atresia, perforation, and volvulus), sepsis, time of first feeding, short gut syndrome and respirator days, and mortality. Results Two randomized controlled trials (RCT)s and eight retrospective cohort studies were included, comprising 629 participants. Moderately preterm elective delivery failed to improve clinical outcomes. However, near-term elective delivery significantly reduced bowel morbidity (7.4 vs. 15.4%, relative risk = 0.37; 95% confidence interval [CI]: 0.18, 0.74; p = 0.005; I2 = 0%) and TPN days (mean difference =−13.44 days; 95% CI: −26.68, −0.20; p = 0.05; I2 = 45%) compared to expectant delivery. The mean LOS was 39.2 days after near-term delivery and 48.7 days in the expectant group (p = 0.06). Conclusion Based on the data analyzed, near-term elective delivery (GA 36–37 weeks) appears to be the optimal timing for delivery of pregnancies complicated by gastroschisis as it is associated with less bowel morbidity and shorter TPN days. However, more RCTs are necessary to better validate these findings. [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.

OBJECTIVE: The goal of our research was to determine the impact of clinical nutrition in the form of home parenteral nutrition (HPN) in patients with nutritional disorders, most often caused by diseases of the digestive tract, with the risk of developing malnutrition. PATIENTS AND METHODS: We retrospectively evaluated 39 patients from the Gastroenterology Clinic and the Home Parenteral Nutrition Center of the University Hospital Bratislava, whose nutritional status was evaluated based on the determination of the body mass index (BMI), the completed nutritional risk screening (NRS) questionnaire and the determination of performance status. Subsequently, after fulfilling the criteria for HPN, the initiation of parenteral nutrition (PN) followed, implemented in a domestic environment for the following two years as HPN. During this period, we did a monthly check-up of the objective condition and laboratory parameters of the enrolled patients, which were the basis for adjusting the nutritional treatment. We also evaluated the occurrence of infectious and thrombotic complications clinically and on the basis of laboratory parameters focused on culture and hemocoagulation examination. After two years, we performed control exit examinations, which we compared with the entrance examinations and statistically evaluated the success of the treatment. We evaluated the obtained data using standard statistical methods. RESULTS: During HPN, there was a statistically significant elevation of the individual monitored values ​​(BMI, absolute lymphocytes count, cholesterol, cholinesterase, total proteins, albumins), which clearly proves correctly indicated and managed HPN. We recorded vein thrombosis in v. subclavia and v. jugularis in 6 (15 %) patients. Subsequent catheter extraction was necessary after unsuccessful catheter insertion. In 13 (33 %) patients, tunneled catheter replacement was required due to infection. The mortality rate in our group was 8 % (3 patients). These were female patients aged 39, 42, and 66 years. The cause of death in all of these patients was the underlying diagnosis (oncohematological disease, systemic connective tissue disease, and repeated resections of the digestive tract for inflammatory GIT disease with the development of severe malnutrition). We recorded a positive effect of applied HPN in all three patients until death. We did not register any factors that would have a relevant influence on the success of administered HPN. CONCLUSION: Based on our results, we can conclude that the patients included in the HPN were correctly indicated, and all of them, based on the monitored parameters (regardless of gender, age, initial diagnosis, or BMI value), benefited from the applied treatment, which was correctly chosen based on their individual needs. Our results clearly document the irreplaceable role of HPN in the management of patients with nutritional intake disorders leading to the development of malnutrition. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a chronic disease whose natural history requires a changing array of management strategies over time. Chief amongst these is the chronic use of parenteral nutrition (PN) to ensure adequate nutritional intake. With time and appropriate management, approximately half of all SBS patients will successfully regain a functional, baseline level of intrinsic bowel function that will allow for them to achieve PN independence. However, the other half of SBS patients will progress into chronic intestinal failure which warrants a change in therapy to include more aggressive medical and potentially surgical measures. This review examines the evolving treatment strategies involved in the management of SBS as well as intestinal failure. [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]

Malignant bowel obstruction (MBO) is one of the most severe complications in patients with advanced ovarian cancer, with an estimated incidence up to 50%. Its presence is related to poor prognosis and a life expectancy measured in weeks for inoperable cases. Symptoms are usually difficult to manage and often require hospitalization, which carries a high burden on patients, caregivers and the healthcare system. Management is complex and requires a multidisciplinary approach to improve clinical outcomes. Patients with inoperable MBO are treated medically with analgesics, antiemetics, steroids and antisecretory agents. Parenteral nutrition and gut decompression with nasogastric tube, venting gastrostomy or stenting may be used as supportive therapy. Treatment decision-making is challenging and often based on clinical expertise and local policies, with lack of high-quality evidence to optimally standardize management. The present review summarizes current literature on inoperable bowel obstruction in ovarian cancer, focusing on epidemiology, prognostic factors, clinical outcomes, medical management, multidisciplinary interventions and quality of life. [ABSTRACT FROM AUTHOR]

Introduction:  The aims of the study are to systematically assess and critically appraise the evidence concerning two surgical techniques to lengthen the bowel in children with short bowel syndrome (SBS), namely, the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP), and to identify patient characteristics associated with a favorable outcome.Materials and Methods:  MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till December 2019. No language restriction was used.Results:  In all, 2,390 articles were found, of which 40 were included, discussing 782 patients. The median age of the patients at the primary bowel lengthening procedure was 16 months (range: 1-84 months). Meta-analysis could not be performed due to the incomparability of the groups, due to heterogeneous definitions and outcome reporting. After STEP, 46% of patients weaned off parenteral nutrition (PN) versus 52% after LILT. Mortality was 7% for STEP and 26% for LILT. Patient characteristics predictive for success (weaning or survival) were discussed in nine studies showing differing results. Quality of reporting was considered poor to fair.Conclusion:  LILT and STEP are both valuable treatment strategies used in the management of pediatric SBS. However, currently it is not possible to advise surgeons on accurate patient selection and to predict the result of either intervention. Homogenous, prospective, outcome reporting is necessary, for which an international network is needed. [ABSTRACT FROM AUTHOR]

Short bowel syndrome can occur after extensive intestinal resection, causing intestinal insufficiency or intestinal failure, which requires long‐term parenteral nutrition. Glucagon‐like peptide‐2 (GLP‐2) pharmacotherapy is now clinically used to reduce the disease burden of intestinal failure. However, many patients still cannot be weaned off from parenteral nutrition completely. The novel dual GLP‐1 and GLP‐2 receptor agonist dapiglutide has previously been shown to be highly effective in a preclinical murine short bowel model. Here, we studied the effects of dapiglutide on intestinal epithelial barrier function. In the jejunum, dapiglutide increased claudin‐7 expression and tightened the paracellular tight junction leak pathway. At the same time, dapiglutide promoted paracellular tight junction cation size selectivity in the jejunum. This was paralleled by extension of the cation selective tight junction proteins claudin‐2 and claudin‐10b and preserved claudin‐15 expression and localization along the crypt–villus axis in the jejunum. In the colon, no barrier effects from dapiglutide were observed. In the colon, dapiglutide attenuated the short bowel–associated, compensatorily increased epithelial sodium channel activity, likely secondary, by improved volume status. Future studies are needed to address the intestinal adaptation of the colon. [ABSTRACT FROM AUTHOR]

Objective: To observe the effects of enteral nutrition on nutritional status, intestinal mucosal barrier function and intestinal flora in patients with active ulcerative colitis（UC） with malnutrition. Methods: 120 patients with active UC with malnutrition who were treated in Shanghai Changzheng Hospital from August 2019 to September 2021 were selected, they were randomly divided into control group（60 cases, treated with total parenteral nutrition） and study group（60 cases, treated with enteral nutrition）. The total clinical effective rate of the two groups 3 weeks after treatment were observed, the nutritional status, intestinal mucosal barrier function index and intestinal flora of the two groups before and 3 weeks after treatment were compared, and the incidence of adverse reactions during the treatment of the two groups were recorded. Results: The total clinical effective rate of the study group（90.00%） was higher than that of the control group（68.33%）（P<0.05）. The levels of D-lactic acid（D-LA）, diamine oxidase（DAO） and endotoxin（ET） in the study group were lower than those in the control group at 3 weeks after treatment（P<0.05）. The levels of serum albumin and hemoglobin in the study group were higher than those in the control group at 3 weeks after treatment（P<0.05）. The number of Lactobacillus and Bifidobacterium in the study group was higher than that in the control group, while the number of Escherichia coli was lower than that in the control group at 3 weeks after treatment（P<0.05）. There was no significant difference in the incidence of adverse reactions between the two groups（P>0.05）. Conclusion: Enteral nutrition can promote the improvement of nutritional status and intestinal mucosal barrier function, regulate the structure of intestinal flora, which is safe and effective. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a rare disorder characterized by severe intestinal dysfunction leading to malabsorption of macronutrients and micronutrients that often results in permanent need of parenteral nutrition support. Patients can develop SBS because of massive intestinal resection or loss of intestinal function and consequently experience significant morbidity and increased healthcare utilization. The remaining anatomy and length of bowel after intestinal resection have important prognostic and therapeutic implications. Because patients with SBS constitute a heterogenous group, management is complex and multifaceted, involving nutrition support, fluid and electrolyte management, and pharmacologic therapies in particular to control diarrhea. Surgical interventions including intestinal transplantation may be considered in selected individuals. Successful care of these patients is best accomplished by a multidisciplinary team that is experienced in the management of this syndrome. [ABSTRACT FROM AUTHOR]

Background: Nutritional support posthematopoietic stem cell transplantation (HSCT) with total parenteral nutrition (TPN) or nasogastric tube feeding (NGT) in pediatric patients is associated with benefits and risks. Methods: We retrospectively reviewed the indication of TPN use in our pediatric HSCT patients and its impact on survival and possible related complications. Results: A total of 228 HSCTs were performed during the study period. TPN was used in 144 patients (63.2%) for a median of 14 days, while 8.8% had NGT feeding and 28% were able to tolerate oral feeding. Severe mucositis was seen in 104 TPN patients (72.2%) in comparison with 22 patients (26.2%) who were on Enteral Nutrition (EN) (p = <.001). Sinusoidal obstruction syndrome (SOS) was seen in 19 (13.2%) patients who had TPN compared to none in the patients who received EN (p =.001). The majority of patients who had SOS received myeloablative conditioning (MAC) therapy for hemoglobinopathy. Acute graft‐versus‐host disease (aGVHD) was seen in 24.8% of TPN patients and 9.1% of non‐TPN patients (p =.01). However, there were no statistically significant differences in chronic GVHD, bacteremia, and patients' survival between both groups. Conclusions: TPN is commonly used after pediatric HSCT in cases of severe mucositis. NGT feeding was found to be the least used nutritional support method. SOS and aGVHD were associated more frequently in TPN patients compared to EN patients. This suggests the possible disadvantages of TPN and importance of SOS preventative measures in high‐risk patients. [ABSTRACT FROM AUTHOR]

Intestinal transplantation is the standard treatment for patients with intestinal failure with severe complications due to parenteral nutrition; however, rejection leads to graft failure in approximately half of both adult and pediatric recipients within 5 years of transplantation. Although intensive immunosuppressive therapy is used in an attempt to reduce this risk, commonly used treatment strategies are generally practice- and/or expert-based, as head-to-head comparisons are lacking. In this ever-developing field, biologicals designed to prevent or treat rejection are used increasingly, with both infliximab and vedolizumab showing potential in the treatment of acute cellular rejection in individual cases and in relatively small patient cohorts. To help advance progress in clinical care, we review the current use of biologicals in intestinal transplantation, and we provide future perspectives to guide this progress. [ABSTRACT FROM AUTHOR]

Parenteral nutrition-associated liver disease (PNALD) refers to a spectrum of conditions that can develop cholestasis, steatosis, fibrosis, and cirrhosis in the setting of parenteral nutrition (PN) use. Patient risk factors include short bowel syndrome, bacterial overgrowth and translocation, disturbance of hepatobiliary circulation, and lack of enteral feeding. A growing body of evidence suggests an intricate linkage between the gut microbiota and the pathogenesis of PNALD. In this review, we highlight current knowledge on the taxonomic and functional changes in the gut microbiota that might serve as noninvasive biomarkers. We also discuss the function of microbial metabolites and associated signaling pathways in the pathogenesis of PNALD. By providing the perspectives of microbiota-host interactions in PNALD for basic and translational research and summarizing current limitations of microbiota-based approaches, this review paves the path for developing novel and precise microbiota-based therapies in PNALD. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to analyze the risk factors, causes, and management of intestinal obstruction after pediatric liver transplantation. Methods: Retrospective analysis was performed on pediatric liver transplantation recipients from January 1st 2013 to December 31st 2019 at Organ Transplant Center, Tianjin First Central Hospital. The cases of intestinal obstruction were analyzed. Results: A total of 1034 pediatric liver transplantations were performed during the study period, 66 intestinal obstructions were diagnosed in 61 recipients. Three recipients suffered intestinal obstructions twice, and one recipient suffered three times. Forty of the 66 cases were treated with non‐surgical treatment, including fasting, gastrointestinal decompression, purgation, enema, and parenteral nutrition. Surgical intervention was performed in 26 cases. Diaphragmatic hernia, intestinal inflammatory stenosis, PTLD, and intestine perforation are essential causes of intestinal obstruction in pediatric liver transplant recipients. Diaphragmatic hernia was independent risk factors for intestinal obstruction. The 1‐, 2‐ and 3‐year survival rate of the recipients with or without intestinal obstruction were 98.4%, 96.5%, 96.5% and 95.3%, 94.4%, 94.0%, respectively, without significant difference. Conclusions: Most cases of intestinal obstruction after liver transplantation in children can be remitted by non‐surgical treatment, but there are still some cases need to be treated by surgery. Both measures are related to ideal outcomes, intestinal obstruction does not increase the mortality rate in pediatric liver transplantation. [ABSTRACT FROM AUTHOR]

Invasive disease due to Malassezia pachydermatis infection is uncommon but increasingly recognized in children, especially neonates on parenteral nutrition or immunocompromised children. We describe a case of Malassezia pachydermatis fungemia in a demographically distinct patient and discuss the workup and current strategies for managing this infection in the setting of a central venous catheter. [ABSTRACT FROM AUTHOR]

BACKGROUND: To analyze prenatal ultrasound (US) markers to predict treatment and adverse neonatal outcome in fetal gastroschisis. METHODS: It was conducted a retrospective single-center study considering all pregnancies with isolated gastroschisis that were treated in our department between 2008 and 2020. 17 US markers were analyzed. Moreover, the association between prenatal ultrasound signs and neonatal outcomes was analyzed: need of bowel resection, techniques of reduction, type of closure, adverse neonatal outcomes, time to full enteral feeding, length of total parenteral nutrition and length of hospitalization. RESULTS: The analysis included 21 cases. We found significant associations between intestinal dilation (≥10 mm) appeared before 30 weeks of gestation and the need of bowel resection (p = 0.001), the length of total parenteral nutrition (p = 0,0013) and the length of hospitalization (p = 0,0017). Intrauterine growth restriction (IUGR) is a risk factor for serial reduction (p = 0,035). There were no signs significantly associated with the type of closure. Hyperbilirubinemia is related with gestational age (GA) at the diagnosis of intra-abdominal bowel dilation (IABD) (p = 0.0376) and maximum IABD (p = 0.05). All newborns with sepsis had echogenic loops in uterus (p = 0.026). The relation between the GA at delivery and the GA at the extra-abdominal bowel dilation (EABD)≥10 mm was r = 0.70. CONCLUSION: We showed the significant role of the early presence of bowel dilation in predicting intestinal resection and adverse outcomes. All IUGR fetuses needed staged reduction through the silo-bag technique. The echogenic bowel was related to neonatal sepsis, while IABD was associated with hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this review was to analyze the potential effect of the nutritional support (oral supplementation/counseling, tube feeding, parenteral nutrition) combined with chemotherapy on long-term survival of cancer patients.Methods: Using various electronic databases, we retrieved all English language papers on the combination nutritional support and chemotherapy and including data on long-term survival.Results: 29 trials (4 non-RCT) were retrieved. No significant benefit was observed except for a longer survival in a few selected subgroups of patients, depending on the tumor stage and compliance with the nutritional support. However, no study included survival as primary endpoint or was adequately powered for this purpose, and patients were seldom affected by malnutrition, a condition which would have made them extremely vulnerable and unsafe during an intensive chemotherapy with significant gastrointestinal toxicity. Finally, nutritional regimens were often inappropriate as regards quality and quantity of nutrients or too similar in the two arms of the trial to expect a result.Conclusion: From the review of these trials, it appears clear that there is absence of evidence more than evidence of absence of effect of the supplemental nutritional support. This poor scenario should not discourage future large multicenter trials to assess the potential of an early versus a delayed support in mildly malnourished patients. From the practical point of view, a nutritional supplementation should be considered when severe malnutrition caused by the disease or following repeated oncologic treatments can make the patients poorly compliant with further chemotherapy cycles, as recommended by the international guidelines. [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]

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. [ABSTRACT FROM AUTHOR]

Aim: Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants.Material: There are approximately 110 000 children born in the Czech Republic each year. A neonatal screening programme from 2005 to 2020 found that 320 neonates had PKU, including 30 premature neonates with a birth weight of less than 2500 g.Results: This study focused on three neonates who were born with ELBWs of 720, 740 and 950 g, respectively. Phenylalanine levels normalised in ELBW neonates with PKU within 1 week of the introduction of low-phenylalanine parenteral or enteral nutrition. The tolerance to phenylalanine was very high (70-110 mg/kg) in the first months of life, due to a rapid weight gain, but significantly decreased during infancy.Conclusion: Extremely low birthweight neonates with PKU need special dietary management. Regular assessments of phenylalanine are necessary during the first weeks of life to allow prompt dietary adjustments that reflect rapid weight gain and transitory high tolerance to phenylalanine. [ABSTRACT FROM AUTHOR]

Intestinal transplantation is a therapeutic treatment option for patients with irreversible intestinal failure. The presence of donor-specific antibodies (DSAs) has been associated with increased antibody-mediated rejection and allograft loss for recipients of all the solid organ transplants. This case report describes the posttransplant course in the first year of a patient who received a T-cell and B-cell flow cross-match (FXM) and complement-dependent cytotoxicity cross-match positive intestinal transplant in the presence of several class I and class II DSAs who underwent a "temporary desensitization" using the donor spleen. The temporary donor splenic transplant removed several class I and II DSAs as demonstrated by the negative subsequent T-cell FXM, the decreased mean channel shift of the positive B-cell FXM with a significant decrease in DSA mean florescence intensity post temporary splenic transplant. The patient experienced an isolated incidence of acute rejection, which responded to therapy. He had no infectious or cancerous sequelae from the immunosuppression modalities. He was able to discontinue total parenteral nutrition and gained weight after the procedure. Long-term effects are not able to be determined from this approach; hence, further research is warranted to better evaluate the real efficacy of this strategy. [ABSTRACT FROM AUTHOR]

Background: While operational tolerance has been previously described in isolated intestinal transplant, reports of this phenomenon in combined liver‐intestine transplant are lacking. Case Description: We detail a unique case of a patient who received a composite allograft including liver, pancreas, and small bowel due to short gut syndrome secondary to gastroschisis complicated by volvulus. The indication for transplantation was permanent dependence on total parenteral nutrition, end‐stage liver disease, recurrent sepsis, and persistent stomal variceal hemorrhage. The patient developed severe graft‐versus‐host disease with grade 3 skin involvement, ophthalmic, and pulmonary involvement with 53% donor T‐cell chimerism. She required aggressive therapy including high‐dose methylprednisolone, rituximab, cyclophosphamide, and alemtuzumab. Due to infection concerns following depletion of her lymphocytes, immunosuppression was discontinued with close surveillance of her allograft. Nearly 10 years later, the patient has continued off all immunosuppression without evidence of rejection or graft dysfunction and demonstrates immunocompetence with normal functional immune assays and development of appropriate live vaccination titers. Conclusion: This report of operational tolerance following pediatric composite liver‐pancreas‐intestine transplantation provides evidence that the complex immunologic balance in intestinal transplantation may on rare occasions favor immunosuppression reduction or even discontinuation. Future trials of immunosuppression minimization in this population may be warranted. [ABSTRACT FROM AUTHOR]

Background & Aims: Tufting enteropathy (TE) is a rare congenital disorder often caused by mutations in the gene encoding epithelial cell adhesion molecule (EpCam). The disease leads to diarrhoea, intestinal failure and dependence on total parenteral nutrition (TPN). These patients often have liver impairments, but the pathology and mechanism of the damage are not well understood. We evaluated liver biopsies from TE patients to understand the pathophysiology. Methods: We identified three patients with TE who underwent liver biopsy. Two normal controls and 45 patients on TPN secondary to short gut syndrome were selected for comparison (five were age‐ and TPN duration matched to the TE patients). Results: We found that all TE patients showed a complete loss of EpCam expression in enterocytes and biliary epithelial cells, while the normal and TPN groups show basolateral expression. Histologically TE patients showed ductopenia, which was not seen in control groups. E‐cadherin and β‐catenin are normally located along the lateral membrane of biliary epithelial cells. However, they were relocated to the apical membrane in TE patients, indicating a defect in the apical‐basal polarity of cholangiocytes. We examined hepatic reparative cells and found near absence of hepatic progenitor cells and intermediate hepatobiliary cells with mild reactive ductular cells in TE patients. Conclusion: Our findings show that TE is associated with disrupted polarity of cholangiocyte and ductopenia. We demonstrate for the first time a role of EpCam in the maintenance of integrity of biliary epithelium. We also provided evidence for a disrupted development of hepatic reparative cells. [ABSTRACT FROM AUTHOR]