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Background & Aims: Acid-base disturbances are common in short bowel (SB) patients due to increased intestinal bicarbonate loss. However, the resulting systemic acid load has not been quantified. Base excess is used to monitor metabolic acid-base disturbances but inadequately reflects the acid load. Our aim was to investigate the systemic acid/base load in SB-patients to obtain quantitative estimates to guide the composition of parenteral support., Methods: We calculated total acid load in SB patients by summing 24-h urinary net acid excretion (NAE) and the provision of base equivalents in parenteral support. We then compared differences among anatomical SB-types: jejunostomy (SB-J), jejunocolostomy (SB-JC), and jejunoileostomy (SB-JIC). 47 urine samples from 34 SB patients were analyzed for bicarbonate (HCO 3 - ), ammonium (NH 4 + ), and titratable acid (TA) concentrations. NAE was calculated as (TA + NH 4 + ) - HCO 3 - . Mixed-effects repeated-measures models were used to statistically examine differences between SB-types and associations with parenteral nutrition and NAE. A healthy cohort served as control., Results: In comparison to SB-J, SB-JC patients had a 4.1 mmoL/l lower base excess (95% CI: -6.3 to -1.8) and an 84.5 mmol/day higher total acid load (CI: 41.3 to 127.7). There were no significant differences between SB-JIC and SB-J regarding base excess, NAE, or total acid load. Higher amounts of infused acetate, sodium, and chloride, but not the acetate/chloride ratio, were associated with lower NAE and higher base excess., Conclusions: Due to increased colonic bicarbonate loss, patients with SB-JC have a ∼4.4-fold higher acid load than healthy controls. The ion transport mechanisms mediating this bicarbonate loss from the remaining colon need further experimental investigation. NAE could be a useful tool to adjust base infusion in SB., Competing Interests: Conflicts of interest The authors have declared any potential conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). There are no conflicts of interest competing with the content of this article., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Necrotizing enterocolitis (NEC) is a potentially devastating condition that preferentially affects premature and low birth weight infants, with approximately half requiring acute surgical intervention. Surgical consult should be considered early on, and deterioration despite maximal medical therapy or the finding of pneumoperitoneum are the strongest indications for emergent surgical intervention. There is no clear consensus on the optimal surgical approach between peritoneal drainage and laparotomy; the best course of action likely depends on the infant's comorbidities, hemodynamic status, size, disease involvement, and available resources. Patients who develop surgical NEC are at a significant risk for morbidity and mortality, with long-term complications including short bowel syndrome, growth failure, and neurodevelopmental impairment. Further research into strategies that optimize outcomes following surgery for NEC in the neonatal intensive care unit and long-term are paramount., (© 2018 Published by Elsevier Ltd.)

Short bowel syndrome is a potentially devastating morbidity for the very low birth weight infant and family with a high risk for mortality. Prevention of injury to the intestine is the ideal, but, if and when the problem arises, it is important to have a systematic approach to manage nutrition, use pharmaceutical strategies and tools to maximize the outcome potential. Safely maximizing parenteral nutrition support by providing adequate macronutrients and micronutrients while minimizing its hepatotoxic effects is the initial postoperative strategy. As the infant stabilizes and starts to recover from that initial injury and/or surgery, a slow and closely monitored enteral nutrition approach should be initiated. Enteral feeds can be complemented with medications and supplements emerging as valuable clinical tools. Engaging a multidisciplinary team of neonatologists, gastroenterologists, pharmacists, skilled clinical nutrition support staff including registered dietitians and nutrition support nurses will facilitate optimizing each and every infant's long term result. Promoting intestinal rehabilitation and adaptation through evidence-based practice where it is found, and ongoing pursuit of research in this rare and devastating disease, is paramount in achieving optimal outcomes., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Abdominal wall transplantation (AWTX) has revolutionized difficult abdominal closure after intestinal transplantation (ITX). More important, the skin of the transplanted abdominal wall (AW) may serve as an immunological tool for differential diagnosis of bowel dysfunction after transplant. Between August 2008 and October 2014, 29 small bowel transplantations were performed in 28 patients (16 male, 12 female; aged 41 ± 13 years). Two groups were identified: the solid organ transplant (SOT) group (n = 15; 12 ITX and 3 modified multivisceral transplantation [MMVTX]) and the SOT-AWTX group (n = 14; 12 ITX and 2 MMVTX), with the latter including one ITX-AWTX retransplantation. Two doses of alemtuzumab were used for induction (30 mg, 6 and 24 h after reperfusion), and tacrolimus (trough levels 8-12 ng/mL) was used for maintenance immunosuppression. Patient survival was similar in both groups (67% vs. 61%); however, the SOT-AWTX group showed faster posttransplant recovery, better intestinal graft survival (79% vs. 60%), a lower intestinal rejection rate (7% vs. 27%) and a lower rate of misdiagnoses in which viral infection was mistaken and treated as rejection (14% vs. 33%). The skin component of the AW may serve as an immune modulator and sentinel marker for immunological activity in the host. This can be a vital tool for timely prevention of intestinal graft rejection and, more important, avoidance of overimmunosuppression in cases of bowel dysfunction not related to graft rejection., (© Copyright 2015 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Short bowel syndrome (SBS) is a serious condition with considerable morbidity and mortality. When treatment with parenteral nutrition fails and life-threatening complications occur, autologous intestinal reconstruction (AIR) should be considered before intestinal transplantation (ITx). Single or combined ITx should be reserved for patients with severe liver disease and as last resort in the treatment of SBS. Longitudinal intestinal lengthening and tailoring (LILT) has proven its value in AIR, but its availability depends on the expertise of the surgeons. Serial transverse enteroplasty (STEP) has similar success rates as LILT and fewer patients progress to ITx. STEP is also applicable at small bowel dilatation in ultra-short bowel syndrome. The scope may be widened when duodenal dilatation can be treated as well. Spiral intestinal lengthening and tailoring (SILT) is a promising alternative. More research is needed to confirm these findings. Therefore we suggest an international data registry for all intestinal lengthening procedures., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

The ultimate goal in the treatment of short bowel syndrome is to wean patients off parenteral nutrition, by promoting intestinal adaptation. Intestinal adaptation is the natural compensatory process that occurs after small bowel resection. Stimulating the remaining bowel with enteral nutrition can enhance this process. Additionally, medication can be used to either reduce factors that complicate the adaptation process or to stimulate intestinal adaptation, such as antisecretory drugs and several growth factors. The aim of this review was to provide an overview of the best nutritional strategies and medication that best promote intestinal adaptation., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background & Aims: Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF., Methods: 118 consecutive patients referred to our institution were assessed by a multidisciplinary team and four different categories were defined retrospectively based on their clinical course: Group 1: patients with reversible intestinal failure; group 2: patients unsuitable for ITx, group 3: patients listed for ITx; group 4: patients stable under PN. Analysis involved comparison between groups for nutritional status, central venous catheter (CVC) related complications, liver disease, and outcome after transplantation by using non parametric tests, Mann-Whitney tests, Kruskal-Wallis, Wilcoxon signed rank tests and chi square distribution for percentage., Results: 118 children (72 boys) with a median age of 15 months at referral (2 months-16 years) were assessed. Etiology of IF was short bowel syndrome [n = 47], intractable diarrhea of infancy [n = 37], total intestinal aganglionosis [n = 18], and chronic intestinal pseudoobstruction [n = 17]. Most patients (89.8%) were totally PN dependent, with 48 children (40.7%) on home-PN prior to admission. Nutritional status was poor with a median body weight at -1.5 z-score (ranges: -5 to +2.5) and median length at -2.0 z-score (ranges: -5.5 to +2.3). The mean number of CVC inserted per patient was 5.2 (range 1-20) and the mean number of CRS per patient was 5.5 (median: 5; range 0-12) Fifty-five patients (46.6%) had thrombosis of ≥2 main venous axis. At admission 34.7% of patients had elevated bilirubin (≥50 μmol/l), and 19.5% had platelets <100,000/ml, and 15% had both. Liver biopsy performed in 79 children was normal (n = 4), or showed F1 or F2 fibrosis (n = 29), bridging fibrosis F3 (n = 20), or cirrhosis (n = 26). Group 1 included 10 children finally weaned from PN (7-years survival: 100%). Group 2 included 12 children with severe liver disease and associated disorders unsuitable for transplantation (7-years survival: 16.6%). Group 3 included 66 patients (56%) who were listed for small bowel or liver-small bowel transplantation, 62/66 have been transplanted (7 years survival: 74.6%). Factors influencing outcome after liver-ITx were body weight (p < .004), length (p < .001), pre-Tx bilirubin plasma level (p < .001) and thrombosis (p < .01) for isolated ITx, Group 4 included 30 children (25.4%) with irreversible IF considered as potential candidates for isolated ITx. Four children were lost from follow up and 3 died within 2 years (survival 88.5%). Among potential candidates, the following parameters improved significantly during the first 12 months of follow up: Body weight (p.0001), length (p < .0001) and bilirubin (p < .0001)., Conclusions: many patients had a poor nutritional status with severe complications especially liver disease. PN related complications were the most relevant indication for ITx, but also a negative predictor for outcome. Early patient referral for Tx-assessment might help to identify and separate children with irreversible IF from children with transient IF or uncomplicated long-term PN, allowing to adapt a patient-based treatment strategy including or not ITx., (Copyright © 2014 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Advances in the medical and surgical treatments of intestinal failure have led to a decrease in the number of transplants over the past decade. In 2012, 152 candidates were added to the intestinal transplant waiting list, a new low. Of these, 64 were listed for intestine-liver transplant and 88 for intestinal transplant alone or with an organ other than liver. Historically, the most common organ transplanted with the intestine was the liver; this practice decreased substantially from a peak of 52.9% in 2007 to 30.0% in 2012. Short-gut syndrome, which encompasses a large group of diagnoses, is the most common etiology of intestinal failure. The pretransplant mortality rate decreased dramatically over time for all age groups, from 51.0 per 100 wait-list years in 1998-1999 to 6.7 for patients listed in 2010-2012. Numbers of intestinal and intestine-liver transplants steadily decreased from 198 in 2007 to 106 in 2012. By age, intestinal transplant recipients have changed substantially; the number of adult recipients now approximately equals the number of pediatric recipients. Graft survival has improved over the past decade. Graft failure in the first 90 days after transplant occurred in 15.7% of 2011-2012 intestinal transplant recipients, compared with 21% in 2001-2002., (© Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Kidney transplantation is the treatment of choice for end-stage renal disease whereas indications for intestinal transplantation are currently restricted to patients with irreversible small bowel failure and severe complications of total parenteral nutrition (mostly shortage and infection of venous accesses, major electrolyte disturbances and liver failure). Enteric hyperoxaluria is secondary to certain intestinal diseases like intestinal resections, chronic inflammatory bowel disease and other malabsorption syndromes and can lead to end-stage renal disease requiring kidney transplantation. We report two patients suffering from renal failure due to enteric hyperoxaluria (secondary to extensive intestinal resection) in whom we elected to replace not only the kidney but also the intestine to prevent recurrence of hyperoxaluria in the transplanted kidney., (© Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Introduction: Management of chronic radiation enteritis is often controversial, particularly due to the risk of short bowel syndrome., Methods: One hundred and seven chronic radiation enteritis patients with short bowel syndrome were studied retrospectively between 1980 and 2009. Survival and home parenteral nutrition dependence rates were evaluated with univariate and multivariate analysis., Results: The survival probabilities were 93%, 67% and 44.5% at 1, 5 and 10 years, respectively. On multivariate analysis, survival was significantly decreased with residual neoplastic disease (HR=0.21 [0.11-0.38], p<0.001), an American Society of Anesthesiologists score >3 (HR=0.38 [0.20-0.73], p=0.004) and an age of chronic radiation enteritis diagnosis >60 years (HR=0.45 [0.22-0.89], p=0.02). The actuarial home parenteral nutrition dependence probabilities were 66%, 55% and 43% at 1, 2 and 3 years, respectively. On multivariate analysis, this dependence was significantly decreased when there was a residual small bowel length >100 cm (HR=0.35 [0.18-0.68], p=0.002), adaptive hyperphagia (HR=0.39 [0.17-0.87], p=0.02) and the absence of a definitive stoma (HR=0.48 [0.27-0.84], p=0.01)., Conclusion: The survival of patients with diffuse chronic radiation enteritis after extensive intestinal resection was good and was mainly influenced by underlying comorbidities. Almost two-thirds of patients were able to be weaned off home parenteral nutrition., (Copyright © 2012 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Background: Venous access is crucial in intestinal transplantation, but a thrombosed venous system may prevent the use of central veins of the upper body. The incidence of venous thrombosis and the necessity to perform alternative vascular access (AVA) in intestinal transplant recipients have not been fully investigated., Methods: Records of adult patients who underwent intestinal transplantation between January 1, 2001, and December 31, 2009, were reviewed. Contrast venography was performed as pre-transplantation screening. Vascular accesses at the transplantation were categorized as I (percutaneous line via the upper body veins), II (percutaneous line via the lower body veins), and III (vascular accesses secured surgically, with interventional radiology, or using non-venous sites). Categories II and III were defined as AVA. Risk factors for central venous thrombosis and those for requiring AVA were analysed, respectively., Results: Among 173 patients, central venous obstruction or stenosis (<50% of normal diameter) was found in 82% (141 patients). AVA was required in 4.6% (eight patients: four in each category II and III). Large-bore infusion lines were placed via the femoral arteries in all category III patients without complications. Existing inferior vena cava filter and hypercoagulable states were identified as the risk factors for the use of AVA, but not for central venous thrombosis. Outcomes of patients who underwent AVA were similar to those of patients without AVA., Conclusions: The majority of adult patients undergoing intestinal transplantation had at least one central venous stenosis or obstruction. The recipient outcomes were comparable when either standard vascular access or AVA was used for transplantation.

Neonatal short bowel syndrome is a disease with a high morbidity and mortality. The management of these patients is complex and requires a multidisciplinary approach. Recent advances in medical and surgical treatment options have improved outcomes. The following review highlights salient points in the management of this challenging patient population., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

The optimal enteral feeding regimen in children with short-bowel syndrome (SBS) is debated by clinicians. The purpose of this article is to present an overview of published data on feeding strategies in children with SBS. A structured literature search (years 1966 through 2007) was done to identify human studies in children directly addressing nutrition (or specified nutrients) in relation to SBS. Eight relevant studies retrieved were graded by seven experts according to the Scottish Intercollegiate Guidelines Network criteria. This grading system is based on the study design and methodological quality of individual studies. Recommendations were made based on the outcome according to the Scottish Intercollegiate Guidelines Network if appropriate and on expert opinion otherwise. The most important recommendations are: Enteral nutrition should be initiated as soon as possible after bowel resection to promote intestinal adaptation. Enteral nutrition should be administered in a continuous fashion. Breast milk or standard polymeric formula (depending on the child's age) is recommended as preferred type of nutrition. Bottle-feeding (small volumes) should be started as soon as possible in neonates to stimulate the suck and swallow reflexes. Solid food can be introduced at the age of 4 to 6 months (corrected for gestational age if necessary) to stimulate oral motor activity and to avoid feeding aversion behavior. The team of experts concluded that high-quality research on the preferred types of enteral and oral nutrition in children with SBS is scarce. Multicenter prospective studies on the effects of feeding strategies on bowel adaptation, fecal production, linear growth, and clinical outcome are required to find the optimal feeding regimen in children with SBS., (Copyright 2010 American Dietetic Association. Published by Elsevier Inc. All rights reserved.)

Our recommendation at this time is that surgical bowel lengthening be considered in any chronically PN-dependent patient when there is substantial bowel dilation, regardless of remnant bowel length. Timing is determined when maximal adaptation has been achieved or when the rate of progression in enteral calories is slow and hampered by bacterial overgrowth. Currently, it seems premature to recommend primary STEP in all patients in whom surgical lengthening is considered, but it is certainly technically easier than the Bianchi procedure. These procedures are clearly indicated in patients experiencing life-threatening complications of PN, but careful selection of patients without evidence of hepatic decompensation is important. Patients with advanced liver disease are poor candidates for lengthening and should be referred for intestinal transplantation instead.

Background: Patients with short bowel syndrome may require combined liver and intestinal transplantation due to total parenteral nutrition(TPN)-related liver damage. We report combined liver and intestinal allotransplantation as a non-composite technique in a patient in China., Methods: During the operation, a 380 cm long intestine was transplanted with systemic drainage and aortic inflow, while the liver graft was placed in a piggyback fashion. Warm ischemic time of the donor graft was 2 minutes and 30 seconds, and cold ischemic time for intestinal and the liver graft was 6 hours and 40 minutes and 8 hours and 7 minutes, respectively. Immunosuppressants used after operation included tacrolimus, methylprednisolone, mycophenolate mofetil and Zenapax., Results: The recipient recovered with no evidence of rejection and was kept well on tube feeding. Eventually, he died of massive hemorrhage of the thoracic cavity on day 210 after transplantation., Conclusion: The non-composite combined liver and intestinal allotransplantation is superior to composite technique in adult patients, particularly those who have had abdominal infection or repeated abdominal operations.

Background & Aims: The aim of this study was to determine serum dipeptidyl peptidase IV (DPP IV) levels in a population of short bowel syndrome (SBS) patients, who had achieved intestinal adaptation., Methods: DDP IV reference values were determined in a healthy population (n=47). The SBS study group consisted of 30 patients (17 men, 13 women; 53.2+/-13.2 years). The criteria for inclusion were a remnant small bowel <200cm with or without colon resection. The time interval between resection and DPP IV measurement was >24 months except in 4 patients, in which it was 13-24 months (total mean: 64.7+/-47.3 months). Nutritional support was exclusively oral in 17 patients, oral plus cyclic TPN in 12 and TPN alone in one patient., Results: The reference range for DPP IV was 10-23U/l (mean: 16.01+/-3.2). In the group of SBS patients, mean serum DPP IV was 14.02+/-3.6U/l and mean body mass index 22.07+/-4.1kg/m(2). Eleven patients (36.6%) had hyperphagia and mean DPP IV in this group was 15.2+/-4.9U/l., Conclusions: Measurement of DPP IV levels in this limited series of SBS patients who had reached intestinal adaptation showed normal levels and did not provide additional clinical information. Further analysis in the earlier postoperative period will determine whether this enzyme has a role as an indicator of evolution in these patients.

Methemoglobinemia, an uncommon hemoglobinopathy, affects oxygen transport, causing tissue hypoxia. In the perioperative period, methemoglobinemia is often overlooked as a cause of low oxygen saturation, often mistaken for the more common causes of hypoxia, such as atelectasis, pulmonary edema, or pulmonary embolus, among other causes of respiratory failure. Most cases of methemoglobinemia in the perioperative period are precipitated by local anesthetics, especially benzocaine. Dapsone, a sulfone antibiotic, is used for prophylaxis against Pneumocystis carinii pneumonia in immunosuppressed patients. It is commonly used in organ transplant patients, especially those intolerant to sulfa. With the expansion of organ transplantation and improved long-term survival of these patients, presentation of status post organ transplant patients for surgery will inevitably increase. We report a case of dapsone-induced methemoglobinemia observed in a status post small bowel transplant patient in the postanesthesia care unit.

The surgical treatment of short-bowel syndrome has been traditionally based on the correction of mechanical obstruction, which is responsible for bacterial overgrowth syndrome, or on intestinal expansion procedures. Since the introduction of clinical intestinal transplantation by Lillehei in 1964, there have been remarkable advances in the immunosuppressive regimens to control rejection and in preservation techniques, monitoring and critical care. Newer and more powerful immunosuppressants have helped to transform intestinal transplantation into a clinical reality-transplantation can now be a life-saving procedure for patients with intestinal failure. It is currently indicated in the event of life-threatening complications of an underlying disease or from total parenteral nutrition (TPN). Rehabilitation in successful cases is excellent.

Diarrhoea, malabsorption and malnutrition characterize the short-bowel syndrome. Following the initial intestinal resection, complications such as fistulas and intra-abdominal abscesses may occur, but these usually resolve with appropriate surgical care. All residual intestine should be placed in continuity before serious attempts at rehabilitation with oral feedings are initiated. Small hourly oral feedings composed of food items high in complex carbohydrate and low in fat are started when appropriate and the diet is gradually increased as intestinal adaptation occurs. The goal during this process is to prevent diarrhoea and allow the formation of semiformed stools. With time, parenteral nutrition (PN) can be reduced, and the time required depends on both length of residual bowel and the particular anatomy involved-for example, the presence or absence of the colon. A programme of optimal diet plus growth hormone (0.1 mg/kg) and oral glutamine (30 g/day) enhances the adaptive process and allows many patients independence from PN. However, those with extremely short segments of jejuno-ileum (<50 cm) and no colon have excessive fluid and electrolyte losses, and intestinal transplantation may be the only therapy which allows such patients to be independent of PN.

Background: Intestinal transplantation is used in patients with short-bowel syndrome after repeated resections for Crohn's disease. We report the apparent clinical recurrence of Crohn's disease in a transplanted intestine., Methods and Findings: The patient, a 33-year-old Hispanic woman, underwent small-bowel transplantation in December, 1994. Immunosuppression with tacrolimus, methylprednisolone, bone-marrow infusions, and OKT3 was given. In July, 1995, the patient had recurrent abdominal symptoms. The histological diagnosis of Crohn's disease was established by the independent interpretations of four experienced gastrointestinal histopathologists., Interpretation: The prompt appearance of this autoimmune disorder (within 6 months of transplantation), despite massive immunosuppression may provide important insights into the nature of Crohn's disease and of the recurrence of autoimmune disease during immunosuppression.

A patient with the short-gut syndrome and antithrombin III deficiency underwent small bowel and liver grafting a year ago. Transient, mild graft-versus-host disease and intestinal rejection occurred within 2 months of grafting and were easily managed. Parenteral nutrition was discontinued 8 weeks after surgery. The patient has maintained normal nutritional indices while on an unrestricted oral diet. Small-bowel/liver grafting is feasible for patients with the short-gut syndrome and associated liver disorders. Further experience is needed to determine the specific risks, benefits, and general applicability of this procedure.

The effect of diet on jejunostomy output of fluid, fat, sodium, potassium, calcium, magnesium, zinc, and copper was studied in five metabolically stable, home parenteral nutrition patients. Three isocaloric diets were compared; one low in fat (30% kcal) but high in complex carbohydrate (55% kcal), and two high in fat (60% kcal) but low in carbohydrate (25% kcal). The polyunsaturated/saturated fatty acid ratios of the two high fat diets were 1:4 and 1:1. Although increasing the percentage of fat in the diet increased the amount of steatorrhea, altering the polyunsaturated/saturated fatty acid ratio had no clearly beneficial effect on the amount of fat absorbed. Neither the amount of fat, nor the type of fat, had any consistent influence on jejunostomy volume. The sodium and potassium concentration of the jejunostomy fluid stayed remarkably constant and hence net monovalent cation losses reflected jejunostomy volume rather than the fatcarbohydrate content of the diet eaten. The most consistent effect of the high fat diet was a marked increase in ostomy losses of divalent cations; calcium, magnesium, zinc, and copper. Most of the time a net divalent cation secretion on the high fat diet was converted into a net absorption on the low fat, high carbohydrate diet. Altering the polyunsaturated/saturated fatty acid ratio had no consistent effect on divalent cation losses. In conclusion, the proportion of fat versus carbohydrate calories does not appear to influence ostomy volume or monovalent cation loss in extreme short bowel, end jejunostomy patients; however, a high fat intake causes a significant net secretion of divalent cations.