Pediatric kidney disease has a relatively lower prevalence than do other pediatric conditions and has a notably different etiology from kidney diseases observed in adults. Furthermore, the pediatric population is unique in that they experience ongoing growth and development, distinguishing them from adult patients. Consequently, pediatric patients with kidney disease require more specialized and meticulous nutritional management than do adults. To address this need and promote optimal dietary practices for pediatric patients with kidney disease, pediatric nephrologists from the Korean Society of Pediatric Nephrology and nutritionists from the Korean Society of Clinical Nutrition have collaborated to establish nutritional guidelines specifically tailored to Korean dietary patterns. These guidelines offer detailed, nutrient-specific recommendations covering energy, protein, calcium, phosphorus, and potassium consumption while providing practical, culturally relevant guidance intended to support both pediatric patients and their caregivers.

Purpose: Stem cell transplantation (SCT) has historically played a major role in the long-term remission of mantle cell lymphoma (MCL), an incurable hematological malignancy. Using data from the Korean Society of Bone and Marrow Transplantation registry, we retrospectively analyzed the role of autologous (auto) and allogeneic (allo) SCT in longterm MCL survival. Methods: This study analyzed data from 188 patients (age ≥ 19 years at the time of transplantation) who underwent a transplant for MCL from 2011 to 2020. Progression-free survival (PFS) was defined as the time from transplantation to disease progression, relapse, or death from any cause. Overall survival (OS) was defined as the time from transplan‑ tation to death from any cause or the last follow-up. Results: In total, 109 patients underwent consolidative SCT after first-line chemotherapy. The 3-year PFS and OS rates were 65.4% and 78.5%, respectively, in the auto-SCT group, and 66.7% and 71.4%, respectively, in the allo-SCT group. The PFS and OS did not differ significantly between the auto- and allo-SCT groups. As part of salvage treatment, 52 patients with relapsed or refractory disease underwent auto- or allo-SCT. Patients who underwent auto-SCT with complete remis‑ sion/partial remission status reported better outcomes. In patients with refractory status, allogeneic transplantation using human leukocyte antigen (HLA) fully matched donors was a significantly favorable factor for PFS and OS. Conclusion The long-term survival of patients who underwent consolidative transplantation was similar to that reported in previous studies. Auto-SCT may be beneficial in patients who respond to salvage therapy, whereas allo-SCT with HLA-matched donors may be an alternative for patients with refractory disease. 

Purpose: To report a nationwide survey on neuroblastoma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2020. Methods: The clinical data of pediatric patients diagnosed with and treated for neuroblastoma from 2005 to 2019 in 19 hospitals of KAPS members were collected. Survival and prognostic factor analyses were performed using the log rank test and Cox proportional hazard analysis. A p-value <0.05 was considered significant. Results: A total of 669 patients with neuroblastoma were registered for the study. The results were presented and discussed at the 36th annual meeting of the KAPS, which was held in Seoul on August 21, 2020. Conclusion This study provides information on patient demographics, prognostic outcomes, and comprehensive treatment outcomes for neuroblastoma. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of neuroblastoma.

Purpose: To report the findings of a perception survey on intestinal malrotation conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2021. Methods: The perceptions on intestinal malrotation regarding clinical decision making of the KAPS members were collected through web-based survey. Results: A total of 22 surgeons were answered for this study. The results were presented and discussed at the 37th annual meeting of KAPS, which was held in Seoul on June 18, 2021. Conclusion This study provides the clinical decisions of the KAPS members on the intestinal malrotation. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of intestinal malrotation.

Purpose: Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey. Methods: This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival. Results: The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey. Conclusion While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.

Purpose: This study aims to investigate and compare the incidence, demographic characteristics, clinical manifestations, preoperative diagnostic methods, anatomical classifications, associated anomalies, operative treatments, and postoperative outcomes of patients with intestinal atresia treated by the members of the Korean Association of Pediatric Surgeons (KAPS) through three nationwide surveys. Methods: KAPS conducted 3 national surveys in 1998, 2010, and 2024 to examine the patients diagnosed with intestinal atresia. In preparation for the survey, we developed a customized case registration form to obtain data on patient sex, birth weight, gestational age, clinical manifestations, preoperative diagnostic methods, anatomical types, associated anomalies, operative treatments, and postoperative outcomes. Authorized KAPS members completed the case registration form. Results: The first, second, and third national surveys included 218, 222, and 236 individuals diagnosed with intestinal atresia, respectively. The male-to-female ratios were 1.5:1, 1.1:1, and 1.1:1, respectively. The first, second, and third national surveys revealed that 34.3%, 43.3%, and 53.4% of patients were born before 37 weeks of gestation, respectively. Additionally, 28.7%, 32.0%, and 40.7% of patients had a birth weight under 2,500 g. In the third national survey, duodenoduodenostomy was the most common procedure, performed in 70 out of 82 patients diagnosed with duodenal atresia. Resection and anastomosis were the main surgical procedures conducted in 47 out of 54 cases of jejunal atresia and 74 out of 92 cases of ileal atresia. The mortality rates in the first, second, and third national surveys were 13.8%, 3.6%, and 1.3% respectively, with the lowest rate observed in the third national survey. Conclusion These national surveys offer valuable insights into the current state of intestinal atresia, including specific surgical interventions and postoperative outcomes in South Korea. For pediatric surgeons aiming to enhance their understanding of intestinal atresia and its treatment options, these surveys could be an indispensable resource and guide.

Pediatric kidney disease has a relatively lower prevalence than do other pediatric conditions and has a notably different etiology from kidney diseases observed in adults. Furthermore, the pediatric population is unique in that they experience ongoing growth and development, distinguishing them from adult patients. Consequently, pediatric patients with kidney disease require more specialized and meticulous nutritional management than do adults. To address this need and promote optimal dietary practices for pediatric patients with kidney disease, pediatric nephrologists from the Korean Society of Pediatric Nephrology and nutritionists from the Korean Society of Clinical Nutrition have collaborated to establish nutritional guidelines specifically tailored to Korean dietary patterns. These guidelines offer detailed, nutrient-specific recommendations covering energy, protein, calcium, phosphorus, and potassium consumption while providing practical, culturally relevant guidance intended to support both pediatric patients and their caregivers.

Background: Hospital environments, particularly shared rooms, are vulnerable to the transmission of carbapenem-resistant Enterobacterales (CRE). The incidence of CRE colonization in the Korean homeless population remains unknown. This study aimed to analyze the impact of targeted active surveillance of CRE in hospital wards following two outbreaks. Methods: This retrospective study was conducted in a homeless ward with shared rooms at a municipal hospital in Seoul. The CRE incidence was calculated from October 1, 2023, to May 31, 2024. Active surveillance was initiated on January 22, 2024. Poisson regression analysis was used to compare CRE incidence events at three months before and four months after the intervention. The risk factors for CRE colonization were also analyzed. Results: The CRE colonization rate decreased from 1.149 to 0.815 per 1,000 patient-days post-intervention; however, the change was not statistically significant (rate ratio: 0.986, 95% confidence interval (95% CI): 0.389-2.496, P=0.976). In contrast to the secondary cases, one acquired CRE case was detected after the intervention without an outbreak. The CRE colonization rate was higher in the homeless ward than in the general ward. CRE colonization was significantly associated with age (adjusted odds ratio (aOR), 1.071; 95% CI: 1.014-1.132;P=0.014), previous antibiotic exposure (aOR, 6.796; 95% CI: 1.215-38.029; P=0.029), and co-colonization with other multidrug resistant bacteria (aOR, 7.168; 95% CI: 2.224-23.096;P=0.001). Conclusion A relatively high incidence of CRE colonization was observed in the homeless ward. After active surveillance, no CRE outbreaks occurred following the implementation.

Purpose: Sirolimus has emerged as a safe and effective treatment for complicated lymphatic malformations (LMs). We aim to prove the effectiveness and safety of sirolimus as a therapeutic option for patients with complicated LMs. Methods: Fifty-eight patients with complicated LMs treated with sirolimus for at least 6 months at multicenter between July 2018 and January 2023 were enrolled. All patients were administered oral sirolimus starting at 0.8 mg/m 2 every 12 hours, with target serum concentration levels of 8–15 ng/mL. Evaluation for clinical symptoms and LMs volume on MRI were reviewed to assess treatment response and toxicities. Evaluation of disease response was divided into 3 values:complete response, partial response (significant, moderate, and modest), and progressive disease. Results: The median age at the initiation of sirolimus treatment was 6.0 years (range, 1 month–26.7 years). The median duration of treatment was 2.0 years (range, 6 months–4.4 years). The most common lesions were head and neck (25 of 58, 43.1%). Forty-six patients (79.3%) demonstrated a reduction in LMs volume on MRI or improvement of clinical symptoms including 2 complete responses. The young age group and the patients who underwent few prior therapies showed better responses. None of the patients had toxicities attributable to sirolimus with a Common Terminology Criteria for Adverse Events grade of ≥3. Conclusion Oral sirolimus treatment brought a successful outcome without severe adverse effects. It could be the firstline therapy, especially for the young age group of complicated LMs, and an additional option for refractory lesions that did not respond to conventional treatment.

Background: Hospital environments, particularly shared rooms, are vulnerable to the transmission of carbapenem-resistant Enterobacterales (CRE). The incidence of CRE colonization in the Korean homeless population remains unknown. This study aimed to analyze the impact of targeted active surveillance of CRE in hospital wards following two outbreaks. Methods: This retrospective study was conducted in a homeless ward with shared rooms at a municipal hospital in Seoul. The CRE incidence was calculated from October 1, 2023, to May 31, 2024. Active surveillance was initiated on January 22, 2024. Poisson regression analysis was used to compare CRE incidence events at three months before and four months after the intervention. The risk factors for CRE colonization were also analyzed. Results: The CRE colonization rate decreased from 1.149 to 0.815 per 1,000 patient-days post-intervention; however, the change was not statistically significant (rate ratio: 0.986, 95% confidence interval (95% CI): 0.389-2.496, P=0.976). In contrast to the secondary cases, one acquired CRE case was detected after the intervention without an outbreak. The CRE colonization rate was higher in the homeless ward than in the general ward. CRE colonization was significantly associated with age (adjusted odds ratio (aOR), 1.071; 95% CI: 1.014-1.132;P=0.014), previous antibiotic exposure (aOR, 6.796; 95% CI: 1.215-38.029; P=0.029), and co-colonization with other multidrug resistant bacteria (aOR, 7.168; 95% CI: 2.224-23.096;P=0.001). Conclusion A relatively high incidence of CRE colonization was observed in the homeless ward. After active surveillance, no CRE outbreaks occurred following the implementation.