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

Background and ObjectivesShort-bowel syndrome (SBS) results from the loss of a significant portion of the small intestine leading to a state of malabsorption. After an intestinal loss, there is a process of adaptation involving the Glucagon-Like Peptide-2 (GLP-2), an enteroendocrine peptide also involved in nutrient absorption. Teduglutide is a recombinant analog of GLP-2 approved in 2016 to treat selected SBS pediatric patients who are dependent on parenteral support. The present systematic review aims to evaluate the efficacy of Teduglutide in pediatric patients with SBS in reducing the need for parenteral nutrition (PN).Materials and MethodsWe performed a literature search on MEDLINE and Embase to include articles up to November 2021. We included articles that involved using Teduglutide in the SBS pediatric population to define its efficacy in reducing the need for PN. The key words used were GLP-2, teduglutide, child.ResultsFourteen studies completely fulfilled the inclusion criteria. Two hundred 23 patients were treated with Teduglutide, and the median duration of treatment was 45 weeks (IQR: 36–52.5 weeks). One-hundred and fifty-two patients were treated with 0.05 mg/Kg/d of subcutaneous Teduglutide, 38 received 0.025 mg/Kg/d and 8 received either 0.125 mg/Kg/d or 0.20 mg/Kg/d. A total of 36 patients achieved enteral autonomy (EA) after a median of 24 weeks of treatment (IQR: 24–48 weeks) and 149 patients showed a reduction in PN needs in terms of volume, calories, or hours per day. Eleven studies reported complications: gastrointestinal were the most common, with 89 cases reported in treated patients and 11 in non-treated patients.ConclusionTeduglutide appears safe and effective in reducing PN requirements and improving EA in the pediatric population. However, more studies are needed to understand its efficacy in the long term and after discontinuation and possible complications.Systematic Review Registration[https://www.crd.york.ac.uk/prospero/], identifier [CRD42022301593].

• The small intestine is an essential constituent of the digestive system and plays a major role in the absorption of nutrients. • Acute mesenteric ischemia remains an emergency case. • Failure to adapt and the inadequate nutritional supply result in Short bowel syndrome. • A minimal indispensable length is required for nutritional autonomy. • Parenteral Nutrition still presents a pertinent problem of complications, charges and impairment in quality of life. The small bowel is an essential organ for maintaining adequate nutrition. Decrease in length could be associated with malnutrition and may require that the patient receives parenteral nutritional support. We report a case of a 59-year-old man who survived32 years with a short bowel of 35 cm length without any parenteral nutrition. A 59-year-old gentleman, with a history of hypercoagulable state, presented for severe abdominal pain and obstipation of one day's duration. Upon presentation, the patient was hemodynamically unstable, with abdominal guarding and tenderness upon physical examination. Laboratory studies showed metabolic acidosis with leukocytosis and electrolyte disturbances, and an abdominal CT scan showed thickening of the sigmoid and multiple air-fluid levels. The patient was operated urgently for suspicion of mesenteric ischemia, however, he was found intraoperatively to have a very short dilated small bowel with jejunotransverse anastomosis. The necessity for surgical resection of the small bowel can arise for a sum of reasons. However, the removal of a big amount of small bowel may not be adaptive and appropriate digestion will no more be possible. In the aftermath of the resection, patients require parenteral nutrition for a certain period after which they may switch to enteral and oral nutrition, and subsequently intestinal adaptation by thickening and growth of the remaining intestinal villi. Patients with a short bowel, particularly those surgically removed, can survive even with a very short remaining bowel length, as a result of intestinal adaptation, nutrition enhancement, and elimination of parenteral nutrition. [ABSTRACT FROM AUTHOR]

Background: Weaning from parenteral support is considered indirect evidence of intestinal adaptation in patients with short bowel syndrome (SBS), but direct evidence is lacking. The objective of this study was to examine if intestinal adaptation could be demonstrated as increase in intestinal absorption of energy and wet weight over time measured by repeated metabolic balance studies (MBSs) and to examine whether adaptation was determined by the anatomy of the remnant bowel.Methods: We retrospectively analyzed data from 48 repeated MBSs performed in 13 adult patients with SBS. Results were presented graphically and interpreted. The interpatient and intrapatient heterogeneity was compared based on anatomy of the remnant bowel.Results: The number of repeated MBSs ranged from 2 to 7, and time between last intestinal resection and MBS from 5 months to 18.1 years. In 6 patients, the first MBS was performed within 2 years after last resection, but only 1 patient had repeated MBSs within this period. Nine patients had an end jejunoileostomy, and 4 patients had a jejuno-colonic or ileo-colonic anastomosis. None of the patients had jejunoileal anastomosis with a preserved ileocecal valve. Interpatient and intrapatient heterogeneity of wet weight and energy absorption was larger in patients without colon in continuity. The wet weight and energy absorption data showed no tendency toward intestinal adaptation in any anatomical group.Conclusion: We observed no signs of late-phase intestinal adaptation in this selected group of patients with SBS. Future prospective MBSs are needed to understand the time course and magnitude of intestinal adaptation. [ABSTRACT FROM AUTHOR]

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

This review aims to discuss the role of nutrition and feeding practices in necrotizing enterocolitis (NEC), NEC prevention, and its complications, including surgical treatment. A thorough PubMed search was performed with a focus on meta-analyses and randomized controlled trials when available. There are several variables in nutrition and the feeding of preterm infants with the intention of preventing necrotizing enterocolitis (NEC). Starting feeds later rather than earlier, advancing feeds slowly and continuous feeds have not been shown to prevent NEC and breast milk remains the only effective prevention strategy. The lack of medical treatment options for NEC often leads to disease progression requiring surgical resection. Following resection, intestinal adaptation occurs, during which villi lengthen and crypts deepen to increase the functional capacity of remaining bowel. The effect of macronutrients on intestinal adaptation has been extensively studied in animal models. Clinically, the length and portion of intestine that is resected may lead to patients requiring parenteral nutrition, which is also reviewed here. There remain significant gaps in knowledge surrounding many of the nutritional aspects of NEC and more research is needed to determine optimal feeding approaches to prevent NEC, particularly in infants younger than 28 weeks and

Summary Short bowel syndrome (SBS) is a rare disease, resulting from extensive resection of the small intestine. Depending on the severity of malabsorption, it will lead to intestinal failure, defined as the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, resulting in a situation where intravenous supplementation is required. The risk of developing intestinal failure is related to the remaining length of small intestine and the anatomy of the remnant bowel. SBS incidence has been estimated to range from 5 to 10 patients per year per million population. The main consequence of SBS is a marked reduction of intestinal absorption surface and its main complication is undernutrition and hydro-electrolytic abnormalities. Parenteral nutrition (PN), the major treatment of intestinal failure, has long-term complications. In case of PN dependency, treatment with trophic factors can be proposed. Glucagon-like peptide-2 (GLP-2) analogs allow significant reduction of PN dependency and improve quality of life. Rehabilitative surgery should always be proposed, with the primary goal of restoring digestive continuity. Sometimes, an additional surgical procedure, such as an antiperistaltic reversal of a small bowel segment, is performed when restoring digestive continuity in patients with insufficient length of remnant small intestine to enhance the possibility of PN withdrawal. Intestinal transplantation is proposed as a last resort. [ABSTRACT FROM AUTHOR]

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

Pediatric intestinal failure is a complex and devastating condition defined as the inability of the intestine to absorb an adequate amount of fluid and nutrients to sustain life. The primary goal of intestinal failure treatment is to achieve enteral autonomy with a customized treatment plan. Although recent improvements in intestinal failure patient care have led to significant improvements in the morbidity and mortality rate, children with intestinal failure are at risk for multiple complications such as intestinal failure associated liver disease, recurrent septic episodes, central line complications, metabolic bone disease, impaired kidney function, and failure to thrive. In this article, we review the current literature on the etiology and factors affecting prognosis of pediatric IF. [ABSTRACT FROM AUTHOR]

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

Short bowel syndrome develops when the remnant mass of functioning enterocytes following massive resections cannot support growth or maintain fluid-electrolyte balance and requires parenteral nutrition. Resection itself stimulates the intestine's inherent ability to adapt morphologically and functionally. The capacity to change is very much related to the high turnover rate of enterocytes and is mediated by several signals; these signals are mediated in large part by enteral nutrition. Early initiation of enteral feeding, close clinical monitoring, and ongoing assessment of intestinal adaptation are key to the prevention of irreversible intestinal failure. The length of the functional small bowel remnant is the most important variable affecting outcome. The major objective of intestinal rehabilitation programs is to achieve early oral nutritional autonomy while maintaining normal growth and nutrition status and minimizing total parenteral nutrition related comorbidities such as chronic progressive liver disease. Remarkable progress has been made in terms of survivability and quality of life, especially in the context of coordinated multidisciplinary programs, but much work remains to be done. [ABSTRACT FROM AUTHOR]

Abstract: Short bowel syndrome (SBS) is a reduction in functioning bowel length which is most often a result of surgical resection. Risk factors in the neonatal period include necrotising enterocolitis, small bowel atresia and gastroschisis. With increasing survival of preterm infants there is an increase in incidence. Management is dependent on the use of parenteral nutrition to maintain fluid and electrolyte homeostasis and promote growth and development with the longer term aim being to promote intestinal adaptation to achieve partial or complete enteral autonomy. In this review we discuss the incidence, aetiology, pathophysiology, medical and surgical treatments and outcome. [Copyright &y& Elsevier]

Purpose: The aim of this study is to determine if prospective determinations of citrulline could be predictive of the bowel adaptation in children with short bowel syndrome (SBS). Methods: Between March 2005 and March 2010, we prospectively included 28 SBS patients on parenteral nutrition. The citrulline and the enteral intake determinations were scheduled at the inclusion and at 6-month intervals. We assessed the correlation between citrulline and bowel length as well as enteral caloric intake, longitudinal trend of citrulline and association between patients characteristics according to the course of bowel adaptation. Results: Citrulline significantly correlated with the residual duodenum-jejunum length ( r = 0.22, P = 0.0113) and with enteral intake ( r = 0.20, P = 0.016 and r = 0.48, P = 0.0001). Baseline citrulline at the cutoff >10 μmol/L and a longitudinal increase >25% provided a weak association with bowel adaptation (likelihood ratio (LR), 2.6 and 2.4, respectively), unlike residual small bowel length ≥20 cm and the presence of >50% of the colon (LR, 10 and 6, respectively). Conclusions: Citrulline seems to be a powerful biomarker of the intestinal function, showed by the correlation with the residual duodenum-jejunum length and the enteral absorption, but not of its prospective changes during the bowel adaptation process. Future studies may be necessary to confirm this finding. [ABSTRACT FROM AUTHOR]

Introduction: Current medical management of short bowel syndrome (SBS) involves the use of lifelong parenteral nutrition (PN). Glucagon-like peptide-2 (GLP-2), an important intestinotrophic growth factor has been shown to increase intestinal absorption in SBS through augmentation of post-resection intestinal adaptation. This may lead to the reduction of PN dependence in patients with SBS. Areas covered in review: Advancing research of GLP-2 physiology has spurred the growing understanding of the diverse effects of GLP-2. The development of the degradation resistant GLP-2 analog, teduglutide (GattexTM, NPS Pharmaceuticals, Bedminster, NJ), has allowed its exploration as a therapeutic agent in a variety of clinical settings. Recent multicenter, placebocontrolled studies of GLP-2 in SBS patients demonstrate meaningful reductions in PN requirements with good safety profiles. The reparative and immunomodulatory effects of teduglutide may also be beneficial in patients with inflammatory bowel disease (IBD). Safety concerns about possible carcinogenic properties during long-term use require ongoing evaluation. Summary: GLP-2 appears to offer a novel adjuvant treatment modality for SBS. Promise for its use in other clinical settings like IBD has been shown in small pilot studies. [ABSTRACT FROM AUTHOR]

Only 50 years ago intestinal failure was considered incompatible with life. Since then, developments in parenteral nutrition, and, more recently, small intestinal transplantation, have provided new therapeutic options with the potential to offer long-term survival with a good quality of life. Current medical and surgical strategies are aimed at enhancing intestinal adaptation, improving absorption to achieve nutritional independence, and minimizing the complications of parenteral nutrition therapy. An integrated, multidisciplinary approach to the management of patients with intestinal failure, closely linked to a transplantation program to facilitate early referral, is recognized as a key factor in optimizing patient outcomes. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) occurs when the body is unable to sustain its energy and fluid requirements without support, due to loss of functional small bowel. Prolonged IF is seen after large intestinal resection and described as short bowel syndrome (SBS). The hallmark of the management is parental nutrition (PN), which is costly and may be associated with the well-recognized problems of parental nutrition associated liver disease (PNALD) and line related sepsis. Cessation of PN at the earliest possible stage is desirable but for this enteral autonomy has to be achieved first. Intestinal adaptation occurs when the remaining gut goes through morphological changes increasing its absorptive capacity. Factors such as intraluminal nutrients, gastrointestinal secretions and hormones facilitate adaptation. Enteral feeds are a potent stimulant to adaptation, and should be started as soon as the clinical situation permits. Some drugs are thought to increase intestinal adaptation. These include glutamine, growth hormone and glucagon like peptide-2, but there is a paucity of pediatric data to guide their use. In some cases surgical bowel lengthening procedures can be performed to increase the absorptive surface area. An isolated liver transplantation may be required if the liver has sustained irreversible damage but intestinal autonomy seems achievable. When prolonged PN is either unsustainable or associated with unacceptable side effects, small bowel transplantation should be considered as a treatment option. [ABSTRACT FROM AUTHOR]

This review aims to discuss the role of nutrition and feeding practices in necrotizing enterocolitis (NEC), NEC prevention, and its complications, including surgical treatment. A thorough PubMed search was performed with a focus on meta-analyses and randomized controlled trials when available. There are several variables in nutrition and the feeding of preterm infants with the intention of preventing necrotizing enterocolitis (NEC). Starting feeds later rather than earlier, advancing feeds slowly and continuous feeds have not been shown to prevent NEC and breast milk remains the only effective prevention strategy. The lack of medical treatment options for NEC often leads to disease progression requiring surgical resection. Following resection, intestinal adaptation occurs, during which villi lengthen and crypts deepen to increase the functional capacity of remaining bowel. The effect of macronutrients on intestinal adaptation has been extensively studied in animal models. Clinically, the length and portion of intestine that is resected may lead to patients requiring parenteral nutrition, which is also reviewed here. There remain significant gaps in knowledge surrounding many of the nutritional aspects of NEC and more research is needed to determine optimal feeding approaches to prevent NEC, particularly in infants younger than 28 weeks and <1000 grams. Additional research is also needed to identify biomarkers reflecting intestinal recovery following NEC diagnosis individualize when feedings should be safely resumed for each patient. [ABSTRACT FROM AUTHOR]