1.Connective tissue disease is associated with the risk of posterior reversible encephalopathy syndrome following lung transplantation in Korea
Tae Jung KIM ; Hyun Joo LEE ; Samina PARK ; Sang-Bae KO ; Soo-Hyun PARK ; Seung Hwan YOON ; Kwon Joong NA ; In Kyu PARK ; Chang Hyun KANG ; Young Tae KIM ; Sun Mi CHOI ; Jimyung PARK ; Joong-Yub KIM ; Hong Yeul LEE
Acute and Critical Care 2025;40(1):79-86
Background:
Posterior reversible encephalopathy syndrome (PRES) is a rare complication of lung transplantation with poorly understood risk factors and clinical characteristics. This study aimed to examine the occurrence, risk factors, and clinical data of patients who developed PRES following lung transplantation.
Methods:
A retrospective analysis was conducted on 147 patients who underwent lung transplantation between February 2013 and December 2023. The patients were diagnosed with PRES based on the clinical symptoms and radiological findings. We compared the baseline characteristics and clinical information, including primary lung diseases and immunosuppressive therapy related to lung transplantation operations, between the PRES and non-PRES groups.
Results:
PRES manifested in 7.5% (n=11) of the patients who underwent lung transplantation, with a median onset of 15 days after operation. Seizures were identified as the predominant clinical manifestation (81.8%, n=9) in the group diagnosed with PRES. All patients diagnosed with PRES recovered fully. Patients with PRES were significantly associated with connective tissue disease-associated interstitial lung disease (45.5% vs. 18.4%, P=0.019, odds ratio=9.808; 95% CI, 1.064–90.386; P=0.044). Nonetheless, no significant variance was observed in the type of immunotherapy, such as the use of calcineurin inhibitors, blood pressure, or acute renal failure subsequent to lung transplantation.
Conclusions
PRES typically manifests shortly after lung transplantation, with seizures being the predominant initial symptom. The presence of preexisting connective tissue disease as the primary lung disease represents a significant risk factor for PRES following lung transplantation.
2.A survey of anesthesiologists aged 60 years and older in Korea: current status, challenges, and future strategies
Won Uk KOH ; Shamin Ara SULTANA ; Jong Ho KIM ; So Young LIM ; Sang woo KIM ; Sung Mi HWANG ; Youngsuk KWON ; Jae Jun LEE ; Hong Seuk YANG
Anesthesia and Pain Medicine 2025;20(1):86-97
Background:
As the global population ages, medical professionals are also aging. This study investigates the status of Korean anesthesiologists over the age of 60.
Methods:
Anesthesiologists aged 60 and older, residing and working in Korea, were invited to participate in this study via e-mail from February to March 2021 and by mail from June to August 2021. The survey consisted of 40 questions covering 10 topics, including health status, residence, work style, and economic status. Depending on the type of question, answers were ranked on a scale of 1, 2, and 3, with the most preferred response being selected.
Results:
A total of 63 responses were received, resulting in a response rate of 26.5%. Among the respondents, 56 were currently practicing as anesthesiologists and reported satisfaction with their clinical practice and life status. On average, they treated 24 patients per day without experiencing significant discomfort or inconvenience in their roles as senior physicians. Twenty-four respondents acknowledged physical discomfort related to aging, and nine expressed cautions regarding age-related changes. Fifty-two respondents indicated that reeducation for advanced medical practice as anesthesiologists was necessary.
Conclusions
Senior anesthesiologists in Korea are primarily working in secondary and tertiary hospitals and are satisfied with their current life status. A comprehensive evaluation of reeducation programs for advanced clinical practice and retirement strategies for senior anesthesiologists is needed to address the growing number of aging physicians in the workforce.
3.Carrier Frequency and Prevalence of Citrin Deficiency in East Asians and Koreans Based on Comprehensive Analysis of Pathogenic SLC25A13 Variants
Mi-Ae JANG ; Won Young HEO ; Jong Kwon LEE ; Jong-Won KIM ; Sang-Mi KIM ; Ja-Hyun JANG ; Hyung-Doo PARK
Annals of Laboratory Medicine 2025;45(5):530-538
Background:
Citrin deficiency is an autosomal recessive disorder caused by pathogenic variants in SLC25A13, presenting with various age-dependent clinical phenotypes and a broad spectrum of severity. However, few studies have examined the frequency and prevalence of citrin deficiency. We aimed to analyze the carrier frequency and disease prevalence in East Asian populations and Koreans.
Methods:
We comprehensively reviewed the literature and conducted a cross-sectional study to analyze genomic databases, including the Genome Aggregation Database (gnomAD), Korean Variant Archive (KOVA), and Tohoku Medical Megabank Organization (ToMMo), to identify pathogenic SLC25A13 variants in East Asian populations. A founder 3-kilobase (kb) insertion in intron 16 of SLC25A13 was investigated using whole-genome sequencing data from 681 Koreans with the Linux grep command.
Results:
Twenty-three pathogenic SLC25A13 variants were identified, with c.852_855del being the most common. Analysis of data from 17,501 East Asian individuals in the gnomAD and ToMMo databases revealed a carrier frequency of 1 in 62 people. Analysis of data from 7,214 individuals in the gnomAD and KOVA databases revealed a carrier frequency of 1 in 86, corresponding to an estimated disease prevalence of 1 in 29,502.c.1177+1G > A was identified as the most prevalent pathogenic variant in Koreans. The 3 kb insertion in intron 16 was detected in three out of 681 individuals, indicating a carrier frequency of 1 in 228.
Conclusions
The high carrier frequency of citrin deficiency in East Asians highlights the need for enhanced genetic screening and counseling, particularly in Korea, providing a valuable reference for future studies on genetic diversity and pathogenic variants in this population.
4.Mutation-Driven Immune Microenvironments in Non-Small Cell Lung Cancer: Unrevealing Patterns through Cluster Analysis
Youngtaek KIM ; Joon Yeon HWANG ; Kwangmin NA ; Dong Kwon KIM ; Seul LEE ; Seong-san KANG ; Sujeong BAEK ; Seung Min YANG ; Mi Hyun KIM ; Heekyung HAN ; Seong Su JEONG ; Chai Young LEE ; Yu Jin HAN ; Jie-Ohn SOHN ; Sang-Kyu YE ; Kyoung-Ho PYO
Yonsei Medical Journal 2024;65(12):683-694
Purpose:
We aimed to comprehensively analyze the immune cell and stromal components of tumor microenvironment at the single-cell level and identify tumor heterogeneity among the major top-derived oncogene mutations in non-small cell lung cancer (NSCLC) using single-cell RNA sequencing (scRNA-seq) data.
Materials and Methods:
The scRNA-seq dataset utilized in this study comprised 64369 primary tumor tissue cells from 21 NSCLC patients, focusing on mutations in EGFR, ALK, BRAF, KRAS, TP53, and the wild-type.
Results:
Tumor immune microenvironment (TIM) analysis revealed differential immune responses across NSCLC mutation subtypes. TIM analysis revealed different immune responses across the mutation subtypes. Two mutation clusters emerged: KRAS, TP53, and EGFR+TP53 mutations (MC1); and EGFR, BRAF, and ALK mutations (MC2). MC1 showed higher tertiary lymphoid structures signature scores and enriched populations of C2-T-IL7R, C3-T/NK-CXCL4, C9-T/NK-NKG, and C1-B-MS4A1 clusters than cluster 2. Conversely, MC2 cells exhibited higher expression levels of TNF, IL1B, and chemokines linked to alternative immune pathways. Remarkably, co-occurring EGFR and TP53 mutations were grouped as MC1. EGFR+TP53 mutations showed upregulation of peptide synthesis and higher synthetic processes, as well as differences in myeloid and T/NK cells compared to EGFR mutations. In T/NK cells, EGFR+TP53 mutations showed a higher expression of features related to cell activity and differentiation, whereas EGFR mutations showed the opposite.
Conclusion
Our research indicates a close association between mutation types and tumor microenvironment in NSCLC, offering insights into personalized approaches for cancer diagnosis and treatment.
5.Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience
Joowon LEE ; Bo Sang KWON ; Mi Kyoung SONG ; Sang-Yun LEE ; Jung Min KO ; Gi Beom KIM ; Eun Jung BAE
Korean Circulation Journal 2024;54(12):853-864
Background and Objectives:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVTcausative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.
Methods:
We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated.
Results:
RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028).Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02–0.38;p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23–179.95; p<0.001).
Conclusions
Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.
6.Acute tolerance to rocuronium -A case report-
Jong Ho KIM ; Sang joon PARK ; Youngsuk KWON ; Sung Mi HWANG ; Hong Seuk YANG
Anesthesia and Pain Medicine 2024;19(4):333-338
A booster dose can result in a similar reaction to the initial dose. Neuromuscular blocking agents (NMBAs) can produce a comparable reaction in the absence of specific pathophysiologic alterations. Case: An initial dose of rocuronium 40 mg was given to a male patient (50 years old, height 168 cm, weight 54 kg, body mass index 19.13 kg/m2 ) for anesthesia. The onset was usual, but the duration was brief. Two booster doses were administered at 20 min intervals, but recovery came quickly. So, acute tolerance was suspected. Muscle function was restored to greater than train-of-four ratio 0.75 while spontaneous aided breathing was maintained without the need of further NMBAs. Following the operation, sugammadex (1.85 mg/kg) was provided to prevent residual neuromuscular inhibition. Conclusions: Anesthetists must be able to suspect acute tolerance to NMBAs in patients with no unique medical history and have a plan to resolve it.
7.Perioperative nutritional practice of surgeons in Korea: a survey study
Ji-Hyeon PARK ; Mi Ran JUNG ; Sang Hyun KIM ; Hongbeom KIM ; Gyeongsil LEE ; Jae-Seok MIN ; Heung-Kwon OH ; Jung Hoon BAE ; Yoona CHUNG ; Dong-Seok HAN ; Seung Wan RYU ;
Annals of Clinical Nutrition and Metabolism 2024;16(3):134-148
Purpose:
Enhanced recovery after surgery (ERAS) protocols advocate reduced fasting and early nutrition to improve recovery in surgical patients. However, data on ERAS implementation among Korean surgeons performing major abdominal surgeries remain sparse.
Methods:
A survey conducted by the External Relation Committee of the Korean Society of Surgical Metabolism and Nutrition assessed perioperative nutritional practices among 389 Korean general surgeons from February to September 2023. The survey covered preoperative fasting, carbohydrate drinks, nasogastric tube use, postoperative dietary progression, parenteral nutrition (PN), and oral supplements, yielding 551 responses stratified by specialty.
Results:
More than 80% of respondents practiced “midnight NPO (Nil Per Os)” fasting, often at the anesthesiology department’s request, while 70%–80% reported no use of preoperative carbohydrate drinks. Most surgeons began dietary progression with water on postoperative day one, advancing to a liquid or soft diet by day two. PN was routinely prescribed by 49% of respondents, with a common dosage of 1,000–1,500 kcal/d. Oral supplements were selectively provided, with 21% of surgeons prescribing them universally.
Conclusion
The results reveal significant variability in perioperative nutrition practices across Korean surgical specialties, with many adhering to traditional practices despite ERAS guidelines. These findings highlight a need for standardized guidelines in Korea to optimize perioperative nutritional support and improve patient recovery outcomes following major abdominal surgeries.
8.Acute tolerance to rocuronium -A case report-
Jong Ho KIM ; Sang joon PARK ; Youngsuk KWON ; Sung Mi HWANG ; Hong Seuk YANG
Anesthesia and Pain Medicine 2024;19(4):333-338
A booster dose can result in a similar reaction to the initial dose. Neuromuscular blocking agents (NMBAs) can produce a comparable reaction in the absence of specific pathophysiologic alterations. Case: An initial dose of rocuronium 40 mg was given to a male patient (50 years old, height 168 cm, weight 54 kg, body mass index 19.13 kg/m2 ) for anesthesia. The onset was usual, but the duration was brief. Two booster doses were administered at 20 min intervals, but recovery came quickly. So, acute tolerance was suspected. Muscle function was restored to greater than train-of-four ratio 0.75 while spontaneous aided breathing was maintained without the need of further NMBAs. Following the operation, sugammadex (1.85 mg/kg) was provided to prevent residual neuromuscular inhibition. Conclusions: Anesthetists must be able to suspect acute tolerance to NMBAs in patients with no unique medical history and have a plan to resolve it.
9.Perioperative nutritional practice of surgeons in Korea: a survey study
Ji-Hyeon PARK ; Mi Ran JUNG ; Sang Hyun KIM ; Hongbeom KIM ; Gyeongsil LEE ; Jae-Seok MIN ; Heung-Kwon OH ; Jung Hoon BAE ; Yoona CHUNG ; Dong-Seok HAN ; Seung Wan RYU ;
Annals of Clinical Nutrition and Metabolism 2024;16(3):134-148
Purpose:
Enhanced recovery after surgery (ERAS) protocols advocate reduced fasting and early nutrition to improve recovery in surgical patients. However, data on ERAS implementation among Korean surgeons performing major abdominal surgeries remain sparse.
Methods:
A survey conducted by the External Relation Committee of the Korean Society of Surgical Metabolism and Nutrition assessed perioperative nutritional practices among 389 Korean general surgeons from February to September 2023. The survey covered preoperative fasting, carbohydrate drinks, nasogastric tube use, postoperative dietary progression, parenteral nutrition (PN), and oral supplements, yielding 551 responses stratified by specialty.
Results:
More than 80% of respondents practiced “midnight NPO (Nil Per Os)” fasting, often at the anesthesiology department’s request, while 70%–80% reported no use of preoperative carbohydrate drinks. Most surgeons began dietary progression with water on postoperative day one, advancing to a liquid or soft diet by day two. PN was routinely prescribed by 49% of respondents, with a common dosage of 1,000–1,500 kcal/d. Oral supplements were selectively provided, with 21% of surgeons prescribing them universally.
Conclusion
The results reveal significant variability in perioperative nutrition practices across Korean surgical specialties, with many adhering to traditional practices despite ERAS guidelines. These findings highlight a need for standardized guidelines in Korea to optimize perioperative nutritional support and improve patient recovery outcomes following major abdominal surgeries.
10.Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience
Joowon LEE ; Bo Sang KWON ; Mi Kyoung SONG ; Sang-Yun LEE ; Jung Min KO ; Gi Beom KIM ; Eun Jung BAE
Korean Circulation Journal 2024;54(12):853-864
Background and Objectives:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVTcausative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.
Methods:
We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated.
Results:
RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028).Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02–0.38;p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23–179.95; p<0.001).
Conclusions
Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.

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