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Background Children with intestinal failure (IF) receiving long-term parenteral nutrition (PN) have altered body composition (BC), but data on BC changes from start of PN onwards are lacking. Objectives We aimed to assess growth and BC in infants after neonatal intestinal surgery necessitating PN and at risk of IF, and to explore associations with clinical parameters. Methods A prospective cohort study in infants after intestinal surgery. IF was defined as PN dependency for >60 d. SD scores (SDS) for anthropometry were calculated until 6-mo corrected age. In a subgroup, fat mass (FM) and fat-free mass (FFM) were measured with air-displacement plethysmography at 2- and 6-mo corrected age. SDS for length-adjusted FM index and FFM index were calculated. Associations between cumulative amount of PN and BC parameters were analyzed with linear mixed-effect models. Results Ninety-five neonates were included (54% male, 35% born <32 wk) and 39 infants (41%) had IF. Studied infants had compromised anthropometric parameters during follow-up. At 6-mo corrected age, they remained smaller (median weight-for-age SDS –0.9 [IQR –1.5, 0.1], P  < 0.001) than the normal population. In 57 infants, 93 BC measurements were performed. FM index SDS was lower than in healthy infants at 2- and 6-mo corrected age (–0.9 [–1.6, –0.3], P  < 0.001 and –0.7 [–1.3, 0.1], P  = 0.001, respectively), but FFM index SDS did not differ. A higher cumulative amount of PN predicted a higher FM index in female infants but lower FM index in male infants. Conclusions In this cohort of infants receiving PN after intestinal surgery, compromised anthropometrics, decreased FM, and adequate FFM were observed during the first 6 mo. Male and female infants seemed to respond differently to PN when it comes to FM index. Continuing growth monitoring after the age of 6 mo is strongly recommended, and further research should explore the benefit of incorporating ongoing BC monitoring during follow-up. [ABSTRACT FROM AUTHOR]

• 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 Intestinal failure (IF) is associated with significant morbidity and mortality, yet specific parameters that determine medium- and long-term outcomes remain ill defined. Objective The aim of this study was to determine the long-term outcomes in childhood IF and identify patient characteristics associated with clinical endpoints. Design MEDLINE and EMBASE were searched for cohorts of >10 pediatric-onset IF patients with >12 mo follow-up. Random-effects meta-analysis and meta-regression weighted by follow-up duration were used to calculate clinical outcome rates and patient factors associated with outcomes. Primary outcome was mortality rate; secondary outcomes included neurodevelopmental status, transplantation, IF-associated liver disease (IFALD), enteral autonomy, and sepsis. Results In total, 175 cohorts (9318 patients and 34,549 y follow-up) were included in the meta-analysis. Overall mortality was 5.2% per y (95% CI: 4.3, 6.0) and was associated with sepsis and IFALD on meta-regression. Mortality rate improved with time from 5.9% per y pre-2000 to 4.5% per y post-2005. Sepsis rate was also predictive of IFALD and liver failure. Enteral autonomy was associated with small bowel length but not presence of ileo-cecal valve. There was a relative lack of data on neurodevelopmental outcomes. Conclusions Sepsis is the primary modifiable factor associated with mortality and liver failure, whereas enteral autonomy correlates with small-bowel length. No clear parameters have been identified that accurately predict neurodevelopmental outcomes, and hence further research is needed. Together, our findings are helpful for parental counseling and resource planning, and support targeting reduction in sepsis. [ABSTRACT FROM AUTHOR]

Background The main cause of intestinal failure is short bowel syndrome (SBS). The management goal for children with SBS is to promote intestinal adaptation while preserving growth and development with the use of parenteral nutrition (PN). Objectives This study evaluated the intestinal absorption rate in children with SBS, focusing on the role of the remnant colon. In addition, the relation between intestinal absorption rate, citrulline concentration, and small bowel length was studied. Methods Thirty-two children with SBS on PN were included. They were divided into 3 groups according to the European Society for Clinical Nutrition and Metabolism (ESPEN) anatomical classification system: type 1 SBS (n  = 9), type 2 (n  = 13), and type 3 (n  = 10). Intestinal absorption rate was assessed by a stool balance analysis of a 3-d collection of stools. Plasma citrulline concentrations were measured and the level of PN dependency was calculated. Results The total energy absorption rate did not differ significantly between the 3 groups: 68% (61–79%) for type 1, 60% (40–77%) for type 2, and 60% (40–77%) for type 3 (P  = 0.45). Children with type 2 or 3 SBS had significantly shorter small bowel length than children with type 1: 28 cm (19–36 cm) and 16 cm (2–29 cm), respectively, compared with 60 cm (45–78 cm) (P  = 0.04). Plasma citrulline concentrations were lower in type 3 SBS but not significantly different: 15 µmol/L (11–25 µmol/L) in type 1, 14 µmol/L (7–21 µmol/L) in type 2, and 9 µmol/L (6–14 µmol/L) in type 3 (P  = 0.141). A multivariate analysis confirmed the role of the remnant colon in providing additional energy absorption. Conclusion This study demonstrated the importance of the colon as a salvage organ in children with SBS. Plasma citrulline concentrations should be interpreted according to the type of SBS. Efforts should focus on conservative surgery, early re-establishment of a colon in continuity, and preserving the intestinal microbiota. [ABSTRACT FROM AUTHOR]

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]

Cycling total parenteral nutrition (TPN) limits hepatic dysfunction in adults and children with intestinal failure. Due to immature glucose homeostasis, this method is infrequently utilized in the neonatal population. Herein, we describe a successful case of cycled TPN in a neonate with complicated gastroschisis and short bowel syndrome. Within three weeks the patient had a normalized direct bilirubin and did not require pharmacologic intervention. There were two episodes of asymptomatic hypoglycemia associated with intestinal obstruction. [ABSTRACT FROM AUTHOR]

Background & Aims: Acute mesenteric ischemia (AMI) is an emergency with a high mortality rate; survivors have high rates of intestinal failure. We performed a prospective study to assess a multidisciplinary and multimodal management approach, focused on intestinal viability. Methods: In an Intestinal Stroke Center, we developed a multimodal management strategy involving gastroenterologists, vascular and abdominal surgeons, radiologists, and intensive care specialists; it was tested in a pilot study on 18 consecutive patients with occlusive AMI, admitted to a tertiary center from July 2009 to November 2011. Patients with left ischemic colitis, nonocclusive AMI, chronic mesenteric ischemia, and other emergencies were excluded. Patients received specific medical management: revascularization of viable small bowel and/or resection of nonviable small bowel; 12 patients received arterial revascularization. We evaluated the percentages of patients who survived for 30 days or 2 years, the number with permanent intestinal failure, and morbidity. Lengths and rates of intestinal resection were compared with or without revascularization, and in patients with early or late-stage disease. Results: Patients were followed up for a mean of 497 days (range, 7–2085 d); 95% survived for 30 days, 89% survived for 2 years, and 28% had morbidities within 30 days. Intestinal resection was necessary for 7 cases (39%), with mean lengths of intestinal resection of 30 cm and 207 cm, with or without revascularization, respectively (P = .03). Among patients with early or late-stage AMI, rates of resection were 18% and 71%, respectively (P = .049). Patients with early stage disease had shorter lengths of intestinal resection than those with late-stage disease (7 vs 94 cm; P = .02), and spent less time in intensive care (2.5 vs 49.8; P = .02). Conclusions: A multidisciplinary and multimodal management approach might increase survival of patients with AMI and prevent intestinal failure. [ABSTRACT FROM AUTHOR]