Global prevalence of short bowel syndrome-associated intestinal failure in adults and children: A targeted literature review and analysis.

Short bowel syndrome-associated intestinal failure (SBS-IF) is a rare disease caused by loss of function of the intestinal surface area and the subsequent inability to maintain nutrient and fluid balance on a normal diet, which results in the need for parenteral nutrition (PN) and/or intravenous fluids. There is a scarcity of literature regarding the prevalence of SBS-IF and challenges in estimating prevalence. A targeted literature review was conducted to generate prevalence estimates for SBS-IF, primarily defined by the requirement for home PN (HPN), in adults and children across multiple geographies. Country-specific estimates of HPN prevalence and the proportion of HPN cases associated with SBS were obtained from the literature and used to inform SBS-IF prevalence estimates. Adults were defined as those aged ≥18 years and children as those aged 0-17 years, except in Japan, where adults were those aged ≥15 years and children were aged 0-14 years. In total, 15 studies were included and were used to estimate and extrapolate prevalence across 61 countries for the years 2020-2030. The estimated prevalences of diagnosed SBS-IF in the general population in 2024 ranged from 0.12 to 2.74 per 100,000 in adults and 0.09 to 1.67 per 100,000 in children. Prevalence estimates were generally lower in countries with a lower average income. This study provides up-to-date insights into the overall global and country-by-country prevalence of SBS-IF and in defined cohorts of adults and children, addressing important gaps in the current literature.