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

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

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

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

Aim: Spiral Intestinal Lengthening and Tailoring (SILT) invented by our team is a new technique that offers minimal mesenteric handling and a more physiological result compared to the STEP procedure. Its feasibility has been tested in animal models and now we report the first successful human application in extreme short bowel syndrome. Materials and Methods: A 3-year-old girl suffered subtotal loss of her small bowel and ileocaecal junction as a result of midgut volvulus. Only 15 cm of jejunum remained intact. Parenteral nutrition (PN), gastrostomy feeding, controlled bowel expansion and SILT procedure were applied. Results: The length of the jejunum increased from the initial 15 to 22 cm during 12 months of PN and bowel expansion. Eleven centimeter of distended bowel was further lengthened up to 20 cm by SILT giving a total small bowel length of 31 cm. Oral and gastrostomy feedings were commenced 5 days postoperatively. There were no surgical complications 6 months after the procedure. The patient's liver function was preserved, she was weaned off PN, discharged from hospital, but remained on gastrostomy top up feeding. The net weight gain of the patient was 1,800 g 6 months after the procedure. Conclusion: SILT procedure is a safe and feasible technique for human intestinal lengthening and tailoring. [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]

Parenteral nutrition associated liver disease (PNALD) is the major source of morbidity and mortality in children with short bowel syndrome (SBS). There is emerging evidence that omega-6 fatty acids (omega6FA) within the parenteral solution play a major role in PNALD and their effects may be reversed or ameliorated by substitution with omega-3 fatty acids (omega3FA). This paper reviews the mechanisms whereby omega3FAs may influence PNALD by improving bile flow, inhibiting steatosis, and having immunomodulatory effects. The early clinical experience with omega3FAs in SBS and PNALD is briefly reviewed and the implications of such, and future directions are considered. [ABSTRACT FROM AUTHOR]