Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency characterized by reduced levels of immunoglobulin (Ig)G, with or without IgA and/or IgM deficiency, and hypogammaglobulinemia. Clinical manifestations are diverse, ranging from recurrent infections to autoimmune and allergic diseases. While CVID has been rarely reported in the Korean population, particularly in adults, we report 2 adult cases of CVID comorbid with asthma and Behcet’s disease. The first case of a 53-year-old with severe allergic asthma and chronic rhinosinusitis experienced recurrent respiratory infections, stomatitis, and cystitis requiring frequent antibiotic treatment. Laboratory findings indicated a T2-high asthma phenotype, with elevated serum total IgE specific IgE to dog hair and fractional exhaled nitric oxide. Immunological evaluation revealed decreased serum IgG (including IgG1 and IgG2), along with hypogammaglobulinemia. She had been treated with regular anti-IgE antibody therapy and intravenous immunoglobulin replacement therapy (IVIGRT). The second case of a 38-year-old with Behçet’s disease and uveitis had bronchial asthma and rhinitis that were exacerbated by recurrent infections despite standard asthma therapy. Laboratory findings revealed a T2-low phenotype and a marked reduction in serum IgG (including IgG1, IgG2, and IgG4), and hypogammaglobulinemia, consistent with CVID.IVIGRT effectively reduced asthma exacerbations and infection episodes in both cases. These cases highlight the clinical heterogeneity of CVID and its potential overlap with allergic and autoimmune diseases. Immunological evaluation of underlying immunodeficiency should be considered in adult patients with asthma who present with frequent exacerbations and recurrent infections. Early diagnosis and IVIGRT can prevent complications and improve outcomes.

Background/Aims: Biliary tract cancer (BTC) is a rare malignancy with poor prognosis. We investigated genomic determinants of clinical benefit from gemcitabine, cisplatin, and nab-paclitaxel (GAP) versus gemcitabine and cisplatin (GC) in advanced BTC. Methods: Clinical and genomic data using TruSight Oncology 500 were analyzed from patients treated with GAP (N=198) or GC (N=89) as first-line therapy. Results: With a median follow-up of 33.0 months, GAP modestly improved progression-free survival (PFS) (hazard ratio [HR] 0.764; 95% confidence interval [CI] 0.591–0.989) without significant overall survival (OS) difference compared to GC. Genomic profiling revealed frequent alterations in TP53 (35.2%), KRAS (16.4%), SMAD4 (10.5%), and TNFRSF14 (10.5%), involving RTK/RAS (44.3%), TP53 (41.8%), and PI3K (20.2%) pathways. Single-gene mutations did not predict treatment benefit. However, pathway-level analysis identified PI3K pathway activation as significantly associated with inferior PFS (HR 2.148; 95% CI 1.478–3.124) and OS (HR 2.096; 95% CI 1.413–3.109) in patients receiving GAP, an effect not observed with GC. Importantly, GAP conferred clinical benefit only in patients without PI3K pathway activation, while no survival advantage was seen in those with such alterations (Pinteraction=0.023 for PFS, Pinteraction=0.003 for OS). Similar results were obtained in the independent validation cohort treated with GAP (N=103) or GC (N=64) for BTC. Conclusions Genomic profiling using next-generation sequencing identified PI3K pathway activation as key molecular determinant that differentiates patient outcomes between GAP and GC treatments in advanced BTC.

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: 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.

Background: Despite the increasing use of continuous glucose monitoring (CGM) systems, limited data exist on their perceived benefits and challenges among patients and healthcare providers. This study explored CGM-related experiences in South Korea. Methods: An anonymous online survey was conducted between January and December 2021 at four university hospitals. Respondents included patients with diabetes mellitus (DM), physicians, and DM education nurses. The survey assessed the use of CGM, its benefits, and barriers. Most devices were first-generation CGMs: FreeStyle Libre 1 (Abbott Diabetes Care), Dexcom G6 (Dexcom Inc.), and Medtronic Guardian 3 (Medtronic MiniMed). Results: Among 1,010 patients (33.4% with type 1 DM [T1DM], 63.6% with type 2 DM [T2DM], and 3.1% others; mean age, 51.4±14.6 years), 92.7% found CGM helpful. Although 59.6% reported discomfort, 81.9% intended to continue using CGM, indicating that perceived benefits outweighed barriers. The key advantages were glucose monitoring without finger pricks (T1DM, 57.9%; T2DM, 56.2%) and maintenance of target glucose levels. Discomfort was related to discomfort during activities (53.8%), skin problems (45.0%), and pain (43.0%). Healthcare provider recommendations were associated with reduced discomfort (adjusted odds ratio, 0.36; 95% confidence interval, 0.21–0.60). Physicians (n=29) cited high costs as the main barrier (T1DM, 58.9%; T2DM, 64.8%); only 51.9% and 14.5% prescribed CGM for T1DM and T2DM, respectively. Insulin adjustment and glucose control were the main reasons for prescription, while cost (89.3%) and limited consultation time (67.9%) were barriers. DM educators (n=9) reported heavy workloads, with training and follow-up times averaging 31.7±7.5 minutes and 21.7±9.7 minutes, respectively; 77.8% of DM educators identified frequent patient inquiries as their greatest burden. Conclusion CGM provides significant clinical benefits but is limited by discomfort, costs, and educational burden. Sustained adoption requires device improvements, insurance support, and workforce expansion.

Background: This study investigates changes in infection control organizations and the roles of infection control nurses in healthcare institutions during the prolonged COVID-19 pandemic. It aims to provide evidence for preparing effective responses to future emerging infectious diseases. Methods: A survey was conducted from August 9 to 27, 2021, targeting infection control nurses working in infection control departments of general hospitals in South Korea with more than 100 beds. Results: A total of 118 (38.3%) healthcare institutions responded, and 113 questionnaires ultimately analyzed. Changes in infection control organizations observed during the COVID-19 pandemic included alterations in infection control department staffing (34.5%), work arrangements (92.0%), IT systems development (45.1%), facilities and equipment (92.9%), and systems for responding to emerging infectious diseases (99.1%). Over 50% of infection control tasks were performed either frequently or daily during the pandemic, with the most significant workload increase observed in epidemiological investigations (77.9%). The most challenging task for infection control nurses was establishing and operating epidemic management systems (82.3%). Among personal factors hindering their work, a sense of responsibility for their duties scored the highest (mean 4.73/5 points). The primary structural and environmental barrier was insufficient staffing (mean 4.36/5 points). The most critical need for improving infection control efforts was increased staffing in infection control departments and enhanced administrative support (mean 4.63/5 points). Conclusion The COVID-19 pandemic brought significant changes to infection control organizations and the roles of infection control nurses. Based on the lessons learned, healthcare institutions must systematically restructure infection control organizations and enhance the expertise of infection control nurses to respond effectively to future outbreaks of emerging infectious diseases.

Background: This study investigates changes in infection control organizations and the roles of infection control nurses in healthcare institutions during the prolonged COVID-19 pandemic. It aims to provide evidence for preparing effective responses to future emerging infectious diseases. Methods: A survey was conducted from August 9 to 27, 2021, targeting infection control nurses working in infection control departments of general hospitals in South Korea with more than 100 beds. Results: A total of 118 (38.3%) healthcare institutions responded, and 113 questionnaires ultimately analyzed. Changes in infection control organizations observed during the COVID-19 pandemic included alterations in infection control department staffing (34.5%), work arrangements (92.0%), IT systems development (45.1%), facilities and equipment (92.9%), and systems for responding to emerging infectious diseases (99.1%). Over 50% of infection control tasks were performed either frequently or daily during the pandemic, with the most significant workload increase observed in epidemiological investigations (77.9%). The most challenging task for infection control nurses was establishing and operating epidemic management systems (82.3%). Among personal factors hindering their work, a sense of responsibility for their duties scored the highest (mean 4.73/5 points). The primary structural and environmental barrier was insufficient staffing (mean 4.36/5 points). The most critical need for improving infection control efforts was increased staffing in infection control departments and enhanced administrative support (mean 4.63/5 points). Conclusion The COVID-19 pandemic brought significant changes to infection control organizations and the roles of infection control nurses. Based on the lessons learned, healthcare institutions must systematically restructure infection control organizations and enhance the expertise of infection control nurses to respond effectively to future outbreaks of emerging infectious diseases.

Background: This study investigates changes in infection control organizations and the roles of infection control nurses in healthcare institutions during the prolonged COVID-19 pandemic. It aims to provide evidence for preparing effective responses to future emerging infectious diseases. Methods: A survey was conducted from August 9 to 27, 2021, targeting infection control nurses working in infection control departments of general hospitals in South Korea with more than 100 beds. Results: A total of 118 (38.3%) healthcare institutions responded, and 113 questionnaires ultimately analyzed. Changes in infection control organizations observed during the COVID-19 pandemic included alterations in infection control department staffing (34.5%), work arrangements (92.0%), IT systems development (45.1%), facilities and equipment (92.9%), and systems for responding to emerging infectious diseases (99.1%). Over 50% of infection control tasks were performed either frequently or daily during the pandemic, with the most significant workload increase observed in epidemiological investigations (77.9%). The most challenging task for infection control nurses was establishing and operating epidemic management systems (82.3%). Among personal factors hindering their work, a sense of responsibility for their duties scored the highest (mean 4.73/5 points). The primary structural and environmental barrier was insufficient staffing (mean 4.36/5 points). The most critical need for improving infection control efforts was increased staffing in infection control departments and enhanced administrative support (mean 4.63/5 points). Conclusion The COVID-19 pandemic brought significant changes to infection control organizations and the roles of infection control nurses. Based on the lessons learned, healthcare institutions must systematically restructure infection control organizations and enhance the expertise of infection control nurses to respond effectively to future outbreaks of emerging infectious diseases.

Background: This study investigates changes in infection control organizations and the roles of infection control nurses in healthcare institutions during the prolonged COVID-19 pandemic. It aims to provide evidence for preparing effective responses to future emerging infectious diseases. Methods: A survey was conducted from August 9 to 27, 2021, targeting infection control nurses working in infection control departments of general hospitals in South Korea with more than 100 beds. Results: A total of 118 (38.3%) healthcare institutions responded, and 113 questionnaires ultimately analyzed. Changes in infection control organizations observed during the COVID-19 pandemic included alterations in infection control department staffing (34.5%), work arrangements (92.0%), IT systems development (45.1%), facilities and equipment (92.9%), and systems for responding to emerging infectious diseases (99.1%). Over 50% of infection control tasks were performed either frequently or daily during the pandemic, with the most significant workload increase observed in epidemiological investigations (77.9%). The most challenging task for infection control nurses was establishing and operating epidemic management systems (82.3%). Among personal factors hindering their work, a sense of responsibility for their duties scored the highest (mean 4.73/5 points). The primary structural and environmental barrier was insufficient staffing (mean 4.36/5 points). The most critical need for improving infection control efforts was increased staffing in infection control departments and enhanced administrative support (mean 4.63/5 points). Conclusion The COVID-19 pandemic brought significant changes to infection control organizations and the roles of infection control nurses. Based on the lessons learned, healthcare institutions must systematically restructure infection control organizations and enhance the expertise of infection control nurses to respond effectively to future outbreaks of emerging infectious diseases.