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.

Purpose: This study aimed to evaluate the long-term prognosis of patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma, including overall survival (OS), remission, and factors associated with an aggressive disease course. Materials and Methods: Medical records of 153 patients diagnosed with gastric MALT lymphoma between 2013 and 2020 were retrospectively reviewed. Patients experiencing relapse, progression, high-grade transformation, or residual diseasewere included in the aggressive group and were compared with those in the indolent group. Additionally, the endoscopic findings of Helicobacter pylori-negative patients were reviewed. Results: Patient characteristics were as follows: mean age (56.9±11.2 years), sex (male, 51.0%), H. pylori infection (positive, 79.7%), endoscopic location (distal, 89.5%), endoscopic feature (superficial, 89.5%), clinical stage (stage I, 92.8%), invasion depth by endoscopic ultrasound (mucosa, n=115, 75.7%), and bone marrow result (no involvement, n=77, 100.0%). The median follow-up period was 59 months (mean, 61; range, 36–124) and the continuous remission period (n=149) was 51 months (mean, 50; range, 3–112). The 5-year survival rate was 97.7% while the 5-year continuous remission was 88.3%. Factors associated with the patients in the aggressive group were old age, sex(male), and clinical stage II or higher. H. pylori-negative patients’ endoscopy revealed a high incidence of atrophic gastritis in the antrum. Conclusions The long-term prognosis of gastric MALT lymphoma appears indolent and is indicated by the 5-year OS and continuous remission rates. Aggressive disease courses are associated with old age, sex (male), and clinical stage II or higher, but are not related to OS.

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthralgia, skin rashes, and hyperferritinemia. The rash is usually salmon-colored, non-itchy, accompanied by fever, and disappears with an improvement of fever. However, in some cases, the rash persisted regardless of fever. Here, we present a case of AOSD with an atypical persistent rash that showed histological findings resembling those of neutrophilic urticarial dermatosis. The patient was a 60-year-old woman with high fever, arthralgia, and a persistent flagellated skin rash. Despite systemic steroid treatment, the patient developed a serious complication: macrophage activation syndrome. Since this case presented with an atypical persistent rash with histological resemblance to neutrophilic urticarial dermatosis, we report its contribution to the further study of AOSD.

Prolonged skin inflammation is caused by disrupted skin barrier resulting in chronic inflammatory diseases such as atopic dermatitis. As a potent natural product with anti-inflammatory property, Aster glehni (A. glehni) is a traditional edible herb and has been used to treat diabetes or colitis-associated colon cancer. In present study, we figured out an additional effect of A. glehni ethanol extract (AGE) in pro-inflammatory cytokines-stimulated human keratinocytes. Mixture of tumor necrosis factor-alpha (TNF-α) and interferongamma (IFN-γ) was used to induce inflammatory responses in the HaCaT keratinocytes. AGE suppressed activation of ERK mitogen-activated protein kinase, nuclear factor (NF)-κB, and signal transducer and activator of transcription 1 and 3 (STAT1 and STAT3). The treatment of AGE inhibited mRNA expressions of proinflammatory cytokines in TNF-α and IFN-γ-stimulated HaCaT cells. Also, AGE induced up-regulated expressions of skin barrier molecules like filaggrin, loricrin, or ZO-1. We evaluated the effects of AGE on protein or mRNA expression levels using western blot or qRT-PCR, respectively. Taken together, these results suggest that the treatment of AGE exerts anti-inflammatory effect on keratinocytes through suppressing inflammatory signaling pathways and up-regulating skin molecules in HaCaT keratinocytes.

Prolonged skin inflammation is caused by disrupted skin barrier resulting in chronic inflammatory diseases such as atopic dermatitis. As a potent natural product with anti-inflammatory property, Aster glehni (A. glehni) is a traditional edible herb and has been used to treat diabetes or colitis-associated colon cancer. In present study, we figured out an additional effect of A. glehni ethanol extract (AGE) in pro-inflammatory cytokines-stimulated human keratinocytes. Mixture of tumor necrosis factor-alpha (TNF-α) and interferongamma (IFN-γ) was used to induce inflammatory responses in the HaCaT keratinocytes. AGE suppressed activation of ERK mitogen-activated protein kinase, nuclear factor (NF)-κB, and signal transducer and activator of transcription 1 and 3 (STAT1 and STAT3). The treatment of AGE inhibited mRNA expressions of proinflammatory cytokines in TNF-α and IFN-γ-stimulated HaCaT cells. Also, AGE induced up-regulated expressions of skin barrier molecules like filaggrin, loricrin, or ZO-1. We evaluated the effects of AGE on protein or mRNA expression levels using western blot or qRT-PCR, respectively. Taken together, these results suggest that the treatment of AGE exerts anti-inflammatory effect on keratinocytes through suppressing inflammatory signaling pathways and up-regulating skin molecules in HaCaT keratinocytes.

Prolonged skin inflammation is caused by disrupted skin barrier resulting in chronic inflammatory diseases such as atopic dermatitis. As a potent natural product with anti-inflammatory property, Aster glehni (A. glehni) is a traditional edible herb and has been used to treat diabetes or colitis-associated colon cancer. In present study, we figured out an additional effect of A. glehni ethanol extract (AGE) in pro-inflammatory cytokines-stimulated human keratinocytes. Mixture of tumor necrosis factor-alpha (TNF-α) and interferongamma (IFN-γ) was used to induce inflammatory responses in the HaCaT keratinocytes. AGE suppressed activation of ERK mitogen-activated protein kinase, nuclear factor (NF)-κB, and signal transducer and activator of transcription 1 and 3 (STAT1 and STAT3). The treatment of AGE inhibited mRNA expressions of proinflammatory cytokines in TNF-α and IFN-γ-stimulated HaCaT cells. Also, AGE induced up-regulated expressions of skin barrier molecules like filaggrin, loricrin, or ZO-1. We evaluated the effects of AGE on protein or mRNA expression levels using western blot or qRT-PCR, respectively. Taken together, these results suggest that the treatment of AGE exerts anti-inflammatory effect on keratinocytes through suppressing inflammatory signaling pathways and up-regulating skin molecules in HaCaT keratinocytes.

Prolonged skin inflammation is caused by disrupted skin barrier resulting in chronic inflammatory diseases such as atopic dermatitis. As a potent natural product with anti-inflammatory property, Aster glehni (A. glehni) is a traditional edible herb and has been used to treat diabetes or colitis-associated colon cancer. In present study, we figured out an additional effect of A. glehni ethanol extract (AGE) in pro-inflammatory cytokines-stimulated human keratinocytes. Mixture of tumor necrosis factor-alpha (TNF-α) and interferongamma (IFN-γ) was used to induce inflammatory responses in the HaCaT keratinocytes. AGE suppressed activation of ERK mitogen-activated protein kinase, nuclear factor (NF)-κB, and signal transducer and activator of transcription 1 and 3 (STAT1 and STAT3). The treatment of AGE inhibited mRNA expressions of proinflammatory cytokines in TNF-α and IFN-γ-stimulated HaCaT cells. Also, AGE induced up-regulated expressions of skin barrier molecules like filaggrin, loricrin, or ZO-1. We evaluated the effects of AGE on protein or mRNA expression levels using western blot or qRT-PCR, respectively. Taken together, these results suggest that the treatment of AGE exerts anti-inflammatory effect on keratinocytes through suppressing inflammatory signaling pathways and up-regulating skin molecules in HaCaT keratinocytes.