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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]

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]