1.Long-Term Evaluation of Cannabidiol in Pediatric Drug-Resistant Epilepsy: A Real-Time Single-Center Retrospective Study
Jong Ho CHA ; Hyeryung KIM ; Young Ho KIM ; Seungbok LEE ; Soo Yeon KIM ; Byung Chan LIM ; Jong-Hee CHAE ; Ki Joong KIM ; Woo Joong KIM
Annals of Child Neurology 2026;34(1):66-74
Purpose:
Cannabidiol (CBD) is a promising treatment option for pediatric drug-resistant epilepsy (DRE). The aim of this study was to assess the tolerability and safety of CBD in a single-center retrospective cohort based on real-world clinical experience.
Methods:
This study included 71 pediatric patients (median age, 8.9 years; interquartile range [IQR], 6.2 to 14.0) with Lennox–Gastaut syndrome who received purified CBD (Epidiolex, GW Pharmaceuticals) between March 2019 and July 2024. All patients had previously failed treatment with more than five anti-seizure medications (ASMs). Responder rate (≥50% seizure frequency reduction), retention rate, adverse effects (AEs), and predictors of favorable treatment response were analyzed over a median follow-up of 21.3 months (IQR, 2.8 to 38.5).
Results:
The initial responder rate during the first 3 months was 45.1%, which increased to 70.8% at 18 months and 63.0% at 24 months. The retention rate at 24 months was 52.4% (33/71). Seven patients (9.9%) achieved seizure freedom beyond 24 months of CBD therapy, and five of these patients were able to reduce their concomitant ASM burden. AEs were observed in 39.4% (28/71) of patients, with the most frequent being somnolence (20 cases) and increased seizure frequency (six cases); 92.9% of AEs occurred within the first 3 months of treatment. No serious AEs requiring treatment discontinuation were identified.
Conclusion
In this real-world study, CBD demonstrated potential as an adjunctive therapy with manageable AEs. These findings highlight that CBD can reduce seizure frequency while maintaining tolerability in pediatric patients with DRE.
2.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
3.Impact of portal/superior mesenteric vein abutment angle on prognosis in pancreatic cancer: a single-center retrospective cohort study
Hye Jeong JEONG ; DanHui HEO ; Soo Yeun LIM ; Hyeong Seok KIM ; Hochang CHAE ; So Jeong YOON ; Sang Hyun SHIN ; In Woong HAN ; Jin Seok HEO ; Ji Hye MIN ; Hongbeom KIM
Annals of Surgical Treatment and Research 2025;108(4):231-239
Purpose:
Pancreatic cancer has a poor prognosis; however, the implementation of neoadjuvant treatment enables borderline resectable cases to undergo curative resection and improves the overall survival rate. Attempts have been made to expand the eligibility criteria for neoadjuvant treatment, even in resectable cases. Some studies have suggested a correlation between vein abutment and poor prognosis or that the abutment angle may affect prognosis. This study investigated the anatomical factors affecting the vessel abutment angle and its prognostic value in pancreatic cancer.
Methods:
Patients with pancreatic ductal adenocarcinoma who underwent surgery between 2012 and 2017 were included in this study. Patients who underwent neoadjuvant treatment were excluded. Data from only the intent-to-treat pancreaticoduodenectomy group were included in the analysis. Clinicopathological characteristics; preoperative factors such as CA 19-9, preoperative biliary drainage, American Society of Anesthesiologists physical status classification, portal vein/superior mesenteric vein contact angle measured via CT scan; and intraoperative factors were collected for analysis.
Results:
A total of 365 patients were included in this study, and the abutment group included 92 patients (25.2%). The abutment and no-contact groups did not show any significant differences in terms of the overall survival or diseasefree survival rate. Among the abutment groups, patients with less than 90° and 90°–180° did not show any significant differences. In the multivariate analysis, the only preoperative factor that had a prognostic effect was CA 19-9, a biological factor.
Conclusion
When there is no vessel invasion in the abutment group, upfront surgery should be considered because the angle does not affect the overall prognosis.
4.Impact of portal/superior mesenteric vein abutment angle on prognosis in pancreatic cancer: a single-center retrospective cohort study
Hye Jeong JEONG ; DanHui HEO ; Soo Yeun LIM ; Hyeong Seok KIM ; Hochang CHAE ; So Jeong YOON ; Sang Hyun SHIN ; In Woong HAN ; Jin Seok HEO ; Ji Hye MIN ; Hongbeom KIM
Annals of Surgical Treatment and Research 2025;108(4):231-239
Purpose:
Pancreatic cancer has a poor prognosis; however, the implementation of neoadjuvant treatment enables borderline resectable cases to undergo curative resection and improves the overall survival rate. Attempts have been made to expand the eligibility criteria for neoadjuvant treatment, even in resectable cases. Some studies have suggested a correlation between vein abutment and poor prognosis or that the abutment angle may affect prognosis. This study investigated the anatomical factors affecting the vessel abutment angle and its prognostic value in pancreatic cancer.
Methods:
Patients with pancreatic ductal adenocarcinoma who underwent surgery between 2012 and 2017 were included in this study. Patients who underwent neoadjuvant treatment were excluded. Data from only the intent-to-treat pancreaticoduodenectomy group were included in the analysis. Clinicopathological characteristics; preoperative factors such as CA 19-9, preoperative biliary drainage, American Society of Anesthesiologists physical status classification, portal vein/superior mesenteric vein contact angle measured via CT scan; and intraoperative factors were collected for analysis.
Results:
A total of 365 patients were included in this study, and the abutment group included 92 patients (25.2%). The abutment and no-contact groups did not show any significant differences in terms of the overall survival or diseasefree survival rate. Among the abutment groups, patients with less than 90° and 90°–180° did not show any significant differences. In the multivariate analysis, the only preoperative factor that had a prognostic effect was CA 19-9, a biological factor.
Conclusion
When there is no vessel invasion in the abutment group, upfront surgery should be considered because the angle does not affect the overall prognosis.
5.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
6.Impact of portal/superior mesenteric vein abutment angle on prognosis in pancreatic cancer: a single-center retrospective cohort study
Hye Jeong JEONG ; DanHui HEO ; Soo Yeun LIM ; Hyeong Seok KIM ; Hochang CHAE ; So Jeong YOON ; Sang Hyun SHIN ; In Woong HAN ; Jin Seok HEO ; Ji Hye MIN ; Hongbeom KIM
Annals of Surgical Treatment and Research 2025;108(4):231-239
Purpose:
Pancreatic cancer has a poor prognosis; however, the implementation of neoadjuvant treatment enables borderline resectable cases to undergo curative resection and improves the overall survival rate. Attempts have been made to expand the eligibility criteria for neoadjuvant treatment, even in resectable cases. Some studies have suggested a correlation between vein abutment and poor prognosis or that the abutment angle may affect prognosis. This study investigated the anatomical factors affecting the vessel abutment angle and its prognostic value in pancreatic cancer.
Methods:
Patients with pancreatic ductal adenocarcinoma who underwent surgery between 2012 and 2017 were included in this study. Patients who underwent neoadjuvant treatment were excluded. Data from only the intent-to-treat pancreaticoduodenectomy group were included in the analysis. Clinicopathological characteristics; preoperative factors such as CA 19-9, preoperative biliary drainage, American Society of Anesthesiologists physical status classification, portal vein/superior mesenteric vein contact angle measured via CT scan; and intraoperative factors were collected for analysis.
Results:
A total of 365 patients were included in this study, and the abutment group included 92 patients (25.2%). The abutment and no-contact groups did not show any significant differences in terms of the overall survival or diseasefree survival rate. Among the abutment groups, patients with less than 90° and 90°–180° did not show any significant differences. In the multivariate analysis, the only preoperative factor that had a prognostic effect was CA 19-9, a biological factor.
Conclusion
When there is no vessel invasion in the abutment group, upfront surgery should be considered because the angle does not affect the overall prognosis.
7.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
8.Evidence‑based Korean guidelines for the clinical management of multiple myeloma: addressing 12 key clinical questions
Sung‑Hoon JUNG ; Youngil KOH ; Min Kyoung KIM ; Jin Seok KIM ; Joon Ho MOON ; Chang‑Ki MIN ; Dok Hyun YOON ; Sung‑Soo YOON ; Je‑Jung LEE ; Chae Moon HONG ; Ka‑Won KANG ; Jihyun KWON ; Kyoung Ha KIM ; Dae Sik KIM ; Sung Yong KIM ; Sung‑Hyun KIM ; Yu Ri KIM ; Young Rok DO ; Yeung‑Chul MUN ; Sung‑Soo PARK ; Young Hoon PARK ; Ho Jin SHIN ; Hyeon‑Seok EOM ; Sang Eun YOON ; Sang Mee HWANG ; Won Sik LEE ; Myung‑won LEE ; Jun Ho YI ; Ji Yun LEE ; Ji Hyun LEE ; Ho Sup LEE ; Sung‑Nam LIM ; Jihyang LIM ; Ho‑Young YHIM ; Yoon Hwan CHANG ; Jae‑Cheol JO ; Jinhyun CHO ; Hyungwoo CHO ; Yoon Seok CHOI ; Hee jeong CHO ; Ari AHN ; Jong Han CHOI ; Hyun Jung KIM ; Kihyun KIM
Blood Research 2025;60():9-
Multiple myeloma (MM), a hematological malignancy, is characterized by malignant plasma cell proliferation in the bone marrow. Recent treatment advances have significantly improved patient outcomes associated with MM.In this study, we aimed to develop comprehensive, evidence-based guidelines for the diagnosis, prognosis, and treat‑ ment of MM. We identified 12 key clinical questions essential for MM management, guiding the extensive literature review and meta-analysis of the study. Our guidelines provide evidence-based recommendations by integrating patient preferences with survey data. These recommendations include current and emerging diagnostic tools, thera‑ peutic agents, and treatment strategies. By prioritizing a patient-centered approach and rigorous data analysis, these guidelines were developed to enhance MM management, both in Korea and globally.
9.Mortality of very low birth weight infants by neonatal intensive care unit workload and regional group status
Sung-Hoon CHUNG ; Chae Young KIM ; Yong-Sung CHOI ; Myung Hee LEE ; Jae Woo LIM ; Byong Sop LEE ; Ki-Soo KIM ;
Clinical and Experimental Pediatrics 2024;67(11):619-627
Background:
Very low birth weight infants (VLBWIs) continue to face high mortality risk influenced by the care quality of neonatal intensive care units (NICUs). Under-standing the impact of workload and regional differences on these rates is crucial for improving outcomes.Purpose: This study aimed to assess how the structural and staffing attributes of NICUs influence the mortality rates of VLBWIs, emphasizing the significance of the availability of medical personnel and the regional distribution of care facilities.
Methods:
Data from 69 Korean NICUs collected by the Korean Neonatal Network between January 2015 and December 2016 were retrospectively analyzed. The NICUs were classified by various parameters: capacity (small, medium, large), nurse-to-bed ratio (1–4), and regional location (A, B, C). Pediatrician staffing was also analyzed and NICUs categorized by beds per pediatrician into low (≤10), medium (11–15), and high (≥16). The NICUs were classified by mortality rates into high-performance (1st and 2nd quartiles) and low-performance (3rd and 4th quartiles). Demographic, perinatal, and neonatal outcomes were analyzed using multivariate logistic regression to explore the association between NICU characteristics and mortality rates.
Results:
This study included 4,745 VLBWIs (mean gestational age, 28.4 weeks; mean birth weight, 1,088 g; 55.4% male) and found significant variations in survival rates across NICUs linked to performance and staffing levels. High-performing NICUs, often with lower bed-to-staff ratios and advanced care levels, had higher survival rates. Notably, NICUs with 2 rather than 1 neonatologist were associated with reduced mortality rates. The study also underscored regional disparities, with NICUs in certain areas showing less favorable survival rates.
Conclusion
Adequate NICU staffing and proper facility location are key to lowering the number of VLBWI deaths. Enhancing staffing and regional healthcare equity is crucial for improving the survival of this population.
10.The First Case of Congenital Nephrogenic Diabetes Insipidus Caused by AVPR2 Disruption Because of 4q25 Insertional Translocation
Boram KIM ; Yo Han AHN ; Jae Hyeon PARK ; Han Sol LIM ; Seung Won CHAE ; Jee-Soo LEE ; Hee Gyung KANG ; Man Jin KIM ; Moon-Woo SEONG
Annals of Laboratory Medicine 2024;44(3):303-305

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