1.Congenital Contractures of the Limbs and Face, Hypotonia, and Developmental Delay (CLIFAHDD) Associated with a De Novo Missense Variant in NALCN: The First Korean Case Report
Yoon Hee JO ; Yoo Jung LEE ; Juhyun KONG ; Yun-Jin LEE ; Sang Ook NAM ; Young Mi KIM
Annals of Child Neurology 2026;34(1):108-108
2.Revision of Nutrition Quotient for Korean preschool children: NQ-P 2021
Hyo-Jeong HWANG ; Ki Nam KIM ; Young-Suk LIM ; Ji-Yun HWANG ; Hye-Young KIM ; Jung-Sug LEE
Journal of Nutrition and Health 2026;59(1):45-62
Purpose:
This study was conducted to revise the Nutrition Quotient for Preschool children (NQ-P), a tool for evaluating the overall dietary quality and eating behavior among Korean preschool-aged children.
Methods:
At the first stage, 20 items for assessing dietary behavior were selected based on the previous NQ-P checklist, incorporating the results of the Seventh Korea National Health and Nutrition Examination Survey, national nutrition policies, dietary guidelines, and recent literature reviews. A pilot study was conducted with 100 preschool children residing in Seoul and Gyeonggi Province, using an 18-item checklist. After exploratory factor analysis and expert consultation, 15 items were finalized for use in nationwide survey checklists. The nationwide survey was conducted with a stratified sample of 800 preschool children across six metropolitan cities. Confirmatory factor analysis was used to revise the NQ-P 2021 and make it suitable for the structural equation model.
Results:
The NQ-P 2021 was developed using 15 checklist items organized into three factors:balance (7 items), moderation (4 items), and practice (4 items). The standardized path coefficients were used as item weights to determine the nutrition quotients. The weights of balance, moderation, and practice were 0.40, 0.15, and 0.45, respectively. In the nationwide survey, the mean NQ-P 2021 score was 59.67. The average scores for balance, moderation, and practice were 51.00, 62.45, and 66.46, respectively.
Conclusion
The revised NQ-P 2021 provides a validated and practical tool for assessing the dietary quality and eating behaviors of preschool children in Korea.
3.Stress Accelerates Depressive-Like Behaviors through Increase of Notch2 Expression in N141I Mutation Presenilin-2 Transgenic Mice
Seung Sik YOO ; Sun Mi GU ; Kyung Tak NAM ; Jeong Soon CHOI ; Yong Sun LEE ; In Jun YEO ; Ji Eun YU ; Sanghyeon KIM ; Dong Won LEE ; Hyeon Joo HAM ; Ju Young CHANG ; Jaesuk YUN ; Dong Ju SON ; Sang-Bae HAN ; Jin Tae HONG
Biomolecules & Therapeutics 2026;34(3):544-555
Alzheimer’s disease (AD) is characterized by progressive cognitive deterioration and significant depression. However, the mechanisms linking depression to AD pathology remain unclear. Here, we investigated whether Notch2 signaling mediates depressionlike behaviors in presenilin-2 (PS2) N141I mutant mice, an early-onset AD model. PS2 wild-type (WT) and mutant (MT) mice aged 12-15 months were subjected to unpredictable chronic mild stress (UCMS) for 4 weeks, followed by sucrose preference, tail-hanging, and forced swimming tests. Behavioral assessments showed that UCMS exacerbated anhedonia and immobility only in PS2 MT mice. Molecular analysis revealed concomitant increases in plasma corticosterone, hippocampal γ-secretase activity, and Notch2 expression, and elevated total and phosphorylated glucocorticoid receptor levels in PS2 MT-UCMS mice. Gene expression profiling of human hippocampal datasets confirmed upregulation of NOTCH2 in Alzheimer’s disease and depression.Pharmacological inhibition of γ-secretase and Notch signaling with DAPT normalizes depressive behavior, reduces corticosterone release, attenuates GR phosphorylation, and inhibits Notch2 signaling in PS2 MT mice. These findings identify Notch2 as a pivotal mediator linking chronic stress to molecular changes associated with depression and AD, and suggest that targeting Notch2 signaling may provide therapeutic benefits for comorbid mood and neurodegenerative disorders.
4.Efficacy of Chemotherapy Following Prior PARP-Inhibitor Treatment in Patients with Ovarian Cancer
Jung Chul KIM ; Junsik PARK ; Yong Jae LEE ; Eun Ji NAM ; Sang Wun KIM ; Sung-Hoon KIM ; Young Tae KIM ; Se Ik KIM ; Jae-Weon KIM ; Byoung-Gie KIM ; Jung-Yun LEE
Cancer Research and Treatment 2026;58(1):292-299
Purpose:
Considering the current lack of consensus on post–poly(adenosine diphosphate-ribose) polymerase inhibitor (PARPi) treatment strategies, this study aimed to evaluate the efficacy of subsequent therapy and compare the outcomes of regimes in patients with recurrent ovarian cancer after PARPi treatment.
Materials and Methods:
This multi-center retrospective cohort study analyzed data on patients diagnosed with ovarian cancer between January 2012 and June 2023 who had previously used PARPi after first- to fourth-line platinum-based chemotherapy. The primary endpoint was progression-free survival (PFS), which was the interval between recurrence after using PARPi and subsequent recurrence in the case of recurrence.
Results:
Of 318 patients, 147/318 (46.2%) recurred after the PARPi maintenance. Patients were categorized into groups based on subsequent therapy except non-treated (11/147, 7.5%): platinum-based chemotherapy (89/147, 60.5%), non-platinum-based chemotherapy (21/147, 14.3%), other treatments (26/147, 17.7%), and the median PFS (mPFS) for each group were 7.3, 4.8, and 11.4 months, respectively. Among the platinum-based chemotherapy group, the gemcitabine+carboplatin regimen demonstrated a longer mPFS (10.1 months) than the other regimens (6.6 months, p=0.019). In non-platinum-based chemotherapy, no statistically significant differences were observed among the regimens. And, in the other therapy group, where the proportion of patients with oligometastasis was as high as 88.5%, no significant differences were observed among the therapies, including other modalities.
Conclusion
In the subsequent chemotherapy of recurrent ovarian cancer after platinum-based chemotherapy and PARPi, the gemcitabine+carboplatin regimen demonstrated a potential to delay recurrence more effectively compared to other therapies.
5.Validating the Korean Geriatric Assessment Tool in Elderly Multiple Myeloma Patients: A Multicenter Study
Ji Yun LEE ; Sang-A KIM ; Youngil KOH ; Ho-Young YHIM ; Gyeong-Won LEE ; Chang-Ki MIN ; Young Rok DO ; Hyo Jung KIM ; Sung Hwa BAE ; Hyeon-Seok EOM ; Sung-Hoon JUNG ; Hyunkyung PARK ; Seung-Hyun NAM ; Ji Hyun LEE ; Sung-Hyun KIM ; Hyun Jung LEE ; Young Seob PARK ; Soo-Mee BANG
Cancer Research and Treatment 2026;58(1):311-319
Purpose:
This study evaluates the Korean Cancer Study Group Geriatric Score-7 (KG-7) frailty screening tool’s effectiveness in elderly multiple myeloma (MM) patients to prevent under and overtreatment.
Materials and Methods:
This prospective pilot cohort study included 100 elderly patients aged 70 and older with newly diagnosed MM who had not undergone transplantation from August 2020 to January 2022.
Results:
The median age was 77 years, and 73.0% of patients were classified at International Staging System stages 2 or 3. Using a 5-point cutoff on the KG-7 index (non-frail, score ≥ 5; frail, score < 5), 31% were categorized as frail. After a median follow-up of 26.8 months, the 3-year overall survival rate was 73.0%. There was no statistically significant association between any frailty index and the risk of death. However, frail patients defined by the simplified frailty index (hazard ratio [HR], 2.49; 95% confidence interval [CI], 1.09 to 5.95; p=0.030) and by KG-7 (HR, 2.43; 95% CI, 1.03 to 5.86; p=0.043) had a significantly higher risk of grade 3-4 non-hematologic toxicity, whereas the International Myeloma Working Group definition did not. Over a 24-month tracking period, vulnerability as measured by KG-7 either improved or deteriorated.
Conclusion
The pilot study, which had a limited number of participants, did not demonstrate KG-7’s effectiveness in predicting survival; however, it successfully predicted severe non-hematologic toxicities. We plan to conduct larger studies in elderly MM patients to determine whether KG-7 can help tailor their treatment regimens.
6.Nationwide Trends in Coronary Artery Bypass Grafting in the Republic of Korea, 2005–2022: A Comparison with International Data
Min Ho JU ; Jun Ho LEE ; Yun Jin KIM ; Ho Jin KIM ; Ho Young HWANG ; Sang Yoon YEOM ; Hee Jung KIM ; Young-Nam YOUN ; Wook Sung KIM ; Man-Jong BAEK ; Hyun Keun CHEE ;
Journal of Chest Surgery 2026;59(1):7-16
Coronary artery bypass grafting (CABG) remains a key revascularization strategy for ischemic heart disease; however, nationwide trends in the Republic of Korea have not been thoroughly investigated. Using data from the Korean National Health Insurance Service, we analyzed adult patients who underwent isolated CABG between 2005 and 2022. We evaluated surgical volume, patient demographics, procedural strategies (off-pump vs. onpump), and outcomes. International comparisons were conducted using national cardiac surgery registry data. A total of 51,923 CABG cases were identified. Annual surgical volume declined until 2013 but gradually increased thereafter, reaching 3,717 cases in 2022. Despite this recovery, Korea’s per capita CABG rate remains among the lowest worldwide.In contrast, more than 60% of procedures were performed off-pump—the highest rate worldwide. Over time, the average patient age and prevalence of diabetes increased, whereas in-hospital mortality showed a modest decline. Compared with other countries, the Republic of Korea demonstrated a uniquely low procedural volume and a strong preference for off-pump CABG. This nationwide analysis highlights Korea’s distinctive CABG practice patterns and provides valuable insights for optimizing future clinical and policy decisions in cardiac surgical care.
7.A Comparative Study of Three National Surveys on Biliary Atresia by the Korean Association of Pediatric Surgeons
Yeon Jun JEONG ; Dayoung KO ; Hyunhee KWON ; Ki Hoon KIM ; Dae Yeon KIM ; Soo-Hong KIM ; Wontae KIM ; Hae-Young KIM ; Hyun Young KIM ; Seong Chul KIM ; Younghyun NA ; Jung-Man NAMGOONG ; So Hyun NAM ; Junbeom PARK ; Jinyoung PARK ; Tae-Jun PARK ; Jeong-Meen SEO ; Ji-Young SUL ; Joonhyuk SON ; Hyun Beak SHIN ; Joohyun SIM ; Soo Min AHN ; Hee Beom YANG ; Jung-Tak OH ; Chaeyoun OH ; Joong Kee YOUN ; Sanghoon LEE ; Ju Yeon LEE ; Kyong IHN ; Hye Kyung CHANG ; Eunyoung JUNG ; Jae Hee CHUNG ; Yu Jeong CHO ; Yun Mee CHOE ; Soo Jin Na CHOI ; Seok Joo HAN ; In Geol HO ; Ji-Won HAN
Advances in Pediatric Surgery 2025;31(2):47-58
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.
8.Comparative Analysis of National Surveys of Intestinal Atresia: A Retrospective Study by the Korean Association of Pediatric Surgeons
Jinyoung PARK ; Dayoung KO ; Eun-jung KOO ; Hyunhee KWON ; Ki Hoon KIM ; Dae Yeon KIM ; Seong Chul KIM ; Soo-Hong KIM ; Wontae KIM ; HaeYoung KIM ; Hyun-Young KIM ; So Hyun NAM ; Jung-Man NAMGOONG ; Junbeom PARK ; Taejin PARK ; Min-Jung BANG ; Jeong-Meen SEO ; Ji-Young SUL ; Joonhyuk SON ; Joohyun SIM ; Soo Min AHN ; Hee-Beom YANG ; Jung-Tak OH ; Chaeyoun OH ; Joong Kee YOUN ; Sanghoon LEE ; Ju Yeon LEE ; Kyong IHN ; Hye Kyung CHANG ; Yeon Jun JEONG ; Eunyoung JUNG ; Jae Hee CHUNG ; Min Jeong CHO ; Yun-Mee CHOE ; Seok Joo HAN ; In Geol HO ; Jeong HONG
Advances in Pediatric Surgery 2025;31(1):8-15
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.
9.Is presumed clinical stage I endometrial cancer using PET-CT and MRI accurate in predicting surgical staging?
Ki Eun SEON ; Yoori SHIN ; Jung-Yun LEE ; Eun Ji NAM ; Sunghoon KIM ; Young Tae KIM ; Sang Wun KIM
Journal of Gynecologic Oncology 2025;36(2):e25-
Objective:
To evaluate upstaging, lymph node (LN) metastasis, and recurrence in patients with presumed stage I endometrial cancer using preoperative magnetic resonance imaging (MRI) and positron emission tomography-computed tomography (PET-CT).
Methods:
Retrospective review of 422 patients with presumed clinical stage I endometrial cancer diagnosed via MRI and PET-CT (July 2014–June 2023). Surgical staging included pelvic lymph nodes (PLNs) and para-aortic lymph nodes (PALNs), classifying patients as low/ intermediate- or high-risk groups.
Results:
Post-operative upstaging rate was 14.5% (8.8% low/intermediate-risk vs. 22.8% high-risk, p<0.001). LN metastasis occurred in 5.5% of patients (2.0% low/intermediaterisk vs. 10.5% high-risk, p<0.001), with a dual imaging negative predictive value of 0.945.PLN metastasis was 4.5% (2.0% low/intermediate vs. 8.2% high-risk, p=0.003), and PALN metastasis was 2.6% (0.4% low/intermediate-risk vs. 5.8% high-risk, p=0.001). In low/ intermediate-risk group: tumors ≤2cm had 1.1% LN metastasis rate, endometrium-limited 0.8%, and ≤2cm with endometrium-limited 0.9%. Deep myometrial invasion (odds ratio [OR]=4.4; 95% confidence intervals [CIs]=1.6–12.4) and tumor size >2 cm on MRI (OR=2.9;95% CI=0.8–9.9) increased LN metastasis risk. Median 48.5-month follow-up showed an 8.1% overall recurrence rate (4.0% low/intermediate-risk vs. 14.0% high-risk, p<0.001), with 2.4% nodal recurrences (1.2% low/intermediate-risk vs. 4.1% high-risk).
Conclusion
High-risk patients had significant upstaging, LN metastasis, and recurrence rates. Even in low/intermediate-risk groups, some patients exhibited LN metastasis and nodal recurrence, underscoring the importance of comprehensive surgical staging, including PALN evaluation, for precise diagnosis and treatment.
10.Congenital Contractures of the Limbs and Face, Hypotonia, and Developmental Delay (CLIFAHDD) Associated with a De Novo Missense Variant in NALCN: The First Korean Case Report
Yoon Hee JO ; Yoo Jung LEE ; Juhyun KONG ; Yun-Jin LEE ; Sang Ook NAM ; Young Mi KIM
Annals of Child Neurology 2025;33(1):34-37

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