Dayoung Ko

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.

Hwang H, et al. Ann Surg Treat Res. 2026 Mar;110(3):194-201. https://doi.org/10.4174/astr.2026.110.3.194

Purpose: Pediatric surgical patients are vulnerable to perioperative malnutrition, yet standardized nutritional care and structured nutrition support team (NST) involvement remain inconsistent across institutions. Although multidisciplinary nutritional support has gained increasing attention, data on pediatric NST practices within surgical settings in Korea are limited. This study examined the availability and composition of pediatric NSTs, perioperative nutritional practices, and barriers in hospitals performing pediatric surgery.Methods: A nationwide cross-sectional survey was conducted among tertiary and secondary hospitals that perform pediatric surgery in Korea. The questionnaire assessed hospital characteristics, the presence and composition of pediatric NSTs, perioperative nutritional screening and support practices, monitoring protocols.Results: A total of 12 hospitals participated. Although all were high-capacity institutions, only half reported having a pediatric NST. Routine preoperative nutritional screening was performed in 50% of hospitals, and validated tools such as Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP) and Pediatric Yorkhill Malnutrition Score (PYMS) were used in 41.7%. Hospitals with a pediatric NST more frequently had institutional protocols for nutritional evaluation (66.7% vs. 16.7%) and were more likely to administer central venous parenteral nutrition postoperatively (83.3% vs. 0%, P=0.015). Enhanced Recovery After Surgery protocols were implemented in only two hospitals (16.7%). Major barriers to pediatric NST operation included insufficient staffing and time constraints.Conclusion: Pediatric NSTs and standardized perioperative nutrition protocols remain underutilized in Korean surgical centers. Institutions with a pediatric NST demonstrated more structured nutritional practices. Expanding NST infrastructure and establishing standardized perioperative protocols for pediatric surgical patients may enhance the quality and consistency of nutritional care.

Duchenne muscular dystrophy (DMD) is a devastating X-linked disorder caused by dystrophin gene mutations. Despite recent advances in understanding the disease etiology and applying emerging treatment methodologies, glucocorticoid derivatives remain the only general therapeutic option that can slow disease development. However, the precise molecular mechanism of glucocorticoid action remains unclear, and there is still need for additional remedies to complement the treatment. Here, using single-nucleus RNA sequencing and spatial transcriptome analyses of human and mouse muscles, we investigated pathogenic features in patients with DMD and palliative effects of glucocorticoids. Our approach further illuminated the importance of proliferating satellite cells and revealed increased activity of a signal transduction pathway involving EZH2 in the patient cells. Subsequent administration of EZH2 inhibitors to Dmd mutant mice resulted in improved muscle phenotype through maintaining the immune-suppressing effect but overriding the muscle weakness and fibrogenic effects exerted by glucocorticoids. Our analysis reveals pathogenic mechanisms that can be readily targeted by extant therapeutic options for DMD.

Pediatric intestinal perforation is a surgical emergency that must be promptly addressed regardless of the specific cause. Here we report a case of colon perforation caused by indeterminate inflammatory bowel disease (IBD) in an autistic 13-year-old boy. Ulcerative colitis (UC) and lymphoma were first suspected but subsequently ruled out. The patient was previously hospitalized locally for 8 days due to diarrhea. He was diagnosed with UC and colon perforation in the emergency room. He then underwent subtotal colectomy with end ileostomy. Pathological examination of the colon showed multiple perforations with absence of chronic crypt change (a characteristic of UC), presence of undermining ulcers, and atypical lymphocyte infiltrations. Lymphoma was ruled out from immunohistochemistry and blood tests. Indeterminate colitis was finally suggested as the cause of perforation. Genetic analysis confirmed KBG syndrome, but no abnormalities otherwise known to be relevant to colitis. This case demonstrates that spontaneous colon perforation might occur in KBG syndrome patients suffering from severe enteritis without IBD, malignancy, or other conditions known to cause perforation, supporting the necessity of close monitoring when such patients present with severe symptoms including fever and abdominal distension without showing improvement.

Ko D, et al. Ann Surg Treat Res. 2025 Jun;108(6):390-396. https://doi.org/10.4174/astr.2025.108.6.390

The study aimed to evaluate the characteristics and operative-related factors in children who underwent fundoplication, analyze surgical outcomes categorized by disease entity and surgical indication, and identify prognostic factors for reoperation risk. A total of 109 pediatric patients who underwent fundoplication between 2008 and 2022 were retrospectively analyzed. Patients were grouped by disease entity and surgical indication. Underlying diseases, comorbidities, sex, gestational age, birth weight, preoperative symptoms, and operation-related factors were examined. Outcomes were classified as short-term and long-term adverse events. We investigated differences in clinical outcomes according to disease entity and surgical indication. Then we statistically identified preoperative predictors for the risk of reoperation. The most common disease entity was neurological impairment (n = 92). Pulmonary comorbidity (42.2%) and aspiration/regurgitation (87.2%) were the most common. Most surgeries were performed laparoscopically (86.2%). There were 12 short-term and 25 long-term adverse events, with long-term events occurred more frequently in the neurological impairment (NIP) group compared to the non-NIP group (P = 0.04). None of the factors showed a significant relationship with the risk of reoperation. Neurologically impaired children were more likely to experience long-term adverse events post-fundoplication. However, no significant predictors for reoperation risk were identified.

Jeong YC, et al. Ann Surg Treat Res. 2024 Jun;106(6):322-329. https://doi.org/10.4174/astr.2024.106.6.322