Intestinal failure in children receiving home parenteral nutrition: A Korean multicenter retrospective study

초록

Background Intestinal failure (IF) is a rare condition in which the intestine cannot maintain adequate nutrient absorption to support maintenance and/or growth. Data on pediatric home parenteral nutrition (HPN) in Korea are limited. We investigated the clinical characteristics of children with IF receiving HPN.Methods A nationwide multicenter retrospective study was conducted across 10 tertiary hospitals in Korea from April 2023 to June 2024. Patients who were receiving HPN and had initiated parenteral nutrition (PN) in a hospital at <= 18 years of age were included. Clinical characteristics, including catheter-related bloodstream infection (CRBSI), intestinal failure-associated liver disease (IFALD), micronutrient status, and anthropometric Z-scores, were analyzed at enrollment and compared with values at HPN initiation. Results A total of 51 patients (median age: 8.4 years) were enrolled. The most common etiologies were chronic intestinal pseudo-obstruction (CIPO, 51%) and short bowel syndrome (39%). The median ages at initiation of PN and HPN were 0.8 and 2.5 years, respectively, and the median duration of PN at study enrollment was 7.7 years. Vitamin D deficiency was the most frequent micronutrient deficiency. From PN initiation to enrollment, 70.6% experienced at least one CRBSI episode (1.57/1000 catheter days), 17.6% had catheter thrombosis, and 41.2% had IFALD. Growth outcomes demonstrated persistent growth failure. A PN dependency ratio >= 75% was observed in 52.9% of patients at enrollment, indicating limited enteral autonomy. Conclusion This first nationwide Korean cohort study of pediatric IF reveals CIPO as the leading cause. Despite long-term HPN, patients exhibited high PN dependency and persistent growth failure.

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