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Purpose: The short- and long-term efficacy, safety, and pharmacokinetics of teduglutide were analyzed in adult Japanese patients with short bowel syndrome and intestinal failure (SBS-IF). Methods: Patients received teduglutide 0.05 mg/kg/day in clinical trials (TED-C14-004, SHP633-306, and extension SHP633-307). Data were analyzed at 24 weeks and an interim data cut-off of 4.5 years. Results: The parenteral support (PS) volume decreased by ≥ 20% for 9/18 patients at 24 weeks and in all 11 patients by data cut-off in SHP633-307. The mean (standard deviation) PS volume decreased from baseline at 24 weeks in TED-C14-004 (−30.1 ± 25.9%) and SHP633-306 (−25.6 ± 25.5%), and at data cut-off in SHP633-307 (−57.08 ± 28.49%). Teduglutide was absorbed quickly. The adverse events were consistent with the underlying disease and known adverse drug reactions. Anti-teduglutide antibody titers declined with long-term treatment. Conclusions: In Japanese adults with SBS-IF, teduglutide treatment was associated with clinically meaningful reductions in PS requirements, similar to findings in prior international studies. No new safety concerns specific to the Japanese SBS-IF patient population were identified with short- or long-term teduglutide treatment. Anti-teduglutide antibody titers disappeared in most Japanese adults with long-term treatment. These results constitute the longest evaluation of teduglutide treatment within clinical trials reported to date. [ABSTRACT FROM AUTHOR]

Purpose: Short bowel syndrome (SBS) with intestinal failure (SBS-IF) requires long-term parenteral nutrition (PN). This study investigated the real-world etiologies of SBS, treatment patterns, and PN-related outcomes among adult patients with SBS-IF in Japan. Methods: This retrospective, observational cohort study was based on data from April, 2008 to January, 2020 from one of the largest hospital-based claim databases in Japan. Analyzed patients were aged ≥ 16 years, had received continuous PN for ≥ 6 months, and had SBS or undergone SBS-related surgery with a diagnosis of a causative disease. The primary endpoint was PN weaning. Results: We analyzed data for 393 patients. The most frequent causes of SBS-IF were ileus (31.8%), Crohn's disease (20.1%), and mesenteric ischemia (16.0%). Of 144/393 (36.6%) patients who were weaned off their PN, 48 (33.3%) were subsequently restarted on PN. Of 276/393 (70.2%) patients whose PN was initiated in hospital, 156 (56.5%) transitioned to home management. The mean duration of initial PN was 450.4 and 675.5 days for patients who were able or unable to be weaned off PN, respectively. Sepsis (67.4%), catheter-related bloodstream infections (49.1%), and liver disorders (45.0%) were the most reported PN-related complications. Conclusions: Most patients with SBS-IF in Japan could not be weaned off PN and suffered life-threatening complications. [ABSTRACT FROM AUTHOR]

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life. [ABSTRACT FROM AUTHOR]

Crohn's disease (CD) was the cause of SBS-IF in 72.7% of the patients in the CT in comparison to 20.1% of the patients in the RW study. To the Editor, Mizushima et al. recently published remarkable explorative data on the causes, treatment patterns and outcomes of short bowel syndrome with intestinal failure (SBS-IF) in Japanese adult patients [[1]]. Evaluation of economic burden with biologic treatments in Crohn's disease patients: a mirror image study using an insurance database in Japan. [Extracted from the article]

Purpose: Short-bowel syndrome (SBS) is associated with high morbidity and mortality. We conducted this study to establish the predictors of survival and weaning off parenteral nutrition (PN). Methods: We reviewed the medical records of 16 SBS infants treated at our institution within a 30-year period. SBS was defined as a residual small-bowel length (RSBL) of <75 cm. Loss of the ileocecal valve (ICV), cholestasis (D-Bil >2.0 mg/dl), enterostomy, and RSBL were all evaluated. Kaplan-Meier analysis was used to analyze the predictors. Results: The mean RSBL was 34.9 ± 22.9 cm. Six patients died (37.5%) and nine patients were weaned off PN (56.3%). Significant differences were observed in cholestasis ( p < 0.03), enterostomy ( p < 0.01), an absolute RSBL of <30 cm ( p < 0.04), and a percentage of expected RSBL of <10% ( p < 0.04) as survival predictors. Significant differences were also observed for cholestasis ( p < 0.01), loss of the ICV ( p < 0.04), an absolute RSBL of <20 cm ( p < 0.01), and a percentage of expected RSBL of <10% ( p < 0.03) as predictors of weaning off PN. Conclusion: These predictors may help us select the optimal treatments for pediatric patients with SBS. [ABSTRACT FROM AUTHOR]

Purpose: Patients with Crohn's disease (CD) often need home parenteral nutrition for short bowel syndrome (SBS), as a result of frequent surgery and extended bowel resections. We conducted this study to evaluate the influence of the patient's condition on the occurrence of SBS in CD. Methods: The subjects of this study were 721 patients, who underwent bowel resections for CD at Hyogo College of Medicine between September 1974 and September 2010. The collective total of bowel resection operations was 1286. The possible risk factors for SBS were analyzed by univariate and multivariate logistic regression analyses to determine their predictive significance. Results: SBS was diagnosed in 24 patients. A stepwise logistic regression model revealed that penetrating type (odds ratio 14.9, p = 0.02), remaining small intestine <200 cm (odds ratio 141.4, p < 0.01), ostomy creation (odds ratio 7.5, p = 0.03), and post-total colectomy (odds ratio 17.6, p < 0.01) were independent risk factors for SBS. Conclusion: These results indicate that the length of remaining small bowel is closely related to SBS in patients who have undergone surgery for CD. At least 200 cm of small bowel should be preserved to prevent SBS when total colectomy and ostomy creation are performed for CD. [ABSTRACT FROM AUTHOR]

Chronic ischemic enteritis can cause intestinal strictures, but extensive resection of the small intestine may leave patients with short bowel syndrome. Thus, the importance of preserving diseased small bowel is now recognized. We report a case of successful side-to-side isoperistaltic strictureplasty (SSIS), performed to prevent short bowel syndrome, in a patient with ischemic enteritis caused by strangulated intestinal obstruction. SSIS is useful for preserving the intestinal absorptive function in patients with a long narrowed bowel loop caused by ischemic change. To our knowledge, this is the first report of the successful treatment of a long stricture resulting from ischemic enteritis, achieved by performing SSIS. [ABSTRACT FROM AUTHOR]