1.Novel Variant of FDXR as a Molecular Etiology of Postlingual Post-synaptic Auditory Neuropathy Spectrum Disorder via Mitochondrial Dysfunction: Reiteration of the Correlation between Genotype and Cochlear Implantation Outcomes
Bong Jik KIM ; Yujin KIM ; Ju Ang KIM ; Jin Hee HAN ; Min Young KIM ; Hee Kyung YANG ; Chae-Seo RHEE ; Young Cheol KANG ; Chun-Hyung KIM ; Byung Yoon CHOI
Clinical and Experimental Otorhinolaryngology 2024;17(3):206-216
Objectives:
. FDXR encodes mitochondrial ferredoxin reductase, which is associated with auditory neuropathy spectrum disorder (ANSD) and optic atrophy. To date, only two studies have described FDXR-related hearing loss. The auditory rehabilitation outcomes of this disease entity have not been investigated, and the pathophysiological mechanisms remain incompletely understood. Here we report a hearing-impaired individual with co-segregation of the FDXR variant and post-synaptic type ANSD, who underwent cochlear implantation (CI) with favorable outcomes. We suggest a possible pathophysiological mechanism of adult-onset ANSD involving mitochondrial dysfunction.
Methods:
. A 35-year-old woman was ascertained to have ANSD. Exome sequencing identified the genetic cause of hearing loss, and a functional study measuring mitochondrial activity was performed to provide molecular evidence of pathophysiology. Expression of FDXR in the mouse cochlea was evaluated by immunohistochemistry. Intraoperatively, electrically evoked compound action potential (ECAP) responses were measured, and the mapping parameters were adjusted accordingly. Audiological outcomes were monitored for over 1 year.
Results:
. In lymphoblastoid cell lines (LCLs) carrying a novel FDXR variant, decreased ATP levels, reduced mitochondrial membrane potential, and increased reactive oxygen species levels were observed compared to control LCLs. These dysfunctions were restored by administering mitochondria isolated from umbilical cord mesenchymal stem cells, confirming the pathogenic potential of this variant via mitochondrial dysfunction. Partial ECAP responses during CI and FDXR expression in the mouse cochlea indicate that FDXR-related ANSD is post-synaptic. As a result of increasing the pulse width during mapping, the patient’s CI outcomes showed significant improvement over 1-year post-CI.
Conclusion
. A novel FDXR variant associated with mitochondrial dysfunction and post-synaptic ANSD was first identified in a Korean individual. Additionally, 1-year post-CI outcomes were reported for the first time in the literature. Excellent audiologic results were obtained, and our results reiterate the correlation between genotype and CI outcomes in ANSD.
2.Early Postoperative Benefits in Receptive and Expressive Language Development After Cochlear Implantation Under 9 Months of Age in Comparison to Implantation at Later Ages
Seung Jae LEE ; Heonjeong OH ; Kyu Ha SHIN ; Sung-Min PARK ; Yun Kyeong KIM ; Do Hyun JUNG ; Jiyeon YANG ; Yejun CHUN ; Min Young KIM ; Jin Hee HAN ; Ju Ang KIM ; Ngoc-Trinh TRAN ; Bong Jik KIM ; Byung Yoon CHOI
Clinical and Experimental Otorhinolaryngology 2024;17(1):46-55
Objectives:
. The recent expansion of eligibility for cochlear implantation (CI) by the U.S. Food and Drug Administration (FDA) to include infants as young as 9 months has reignited debates concerning the clinically appropriate cut-off age for pediatric CI. Our study compared the early postoperative trajectories of receptive and expressive language development in children who received CI before 9 months of age with those who received it between 9 and 12 months. This study involved a unique pediatric cohort with documented etiology, where the timing of CI was based on objective criteria and efforts were made to minimize the influence of parental socioeconomic status.
Methods:
. A retrospective review of 98 pediatric implantees recruited at a tertiary referral center was conducted. The timing of CI was based on auditory and language criteria focused on the extent of delay corresponding to the bottom 1st percentile of language development among age-matched controls, with patients categorized into very early (CI at <9 months), early (CI at 9–12 months) and delayed (CI at 12–18 months) CI groups. Postoperative receptive/expressive language development was assessed using the Sequenced Language Scale for Infants receptive and expressive standardized scores and percentiles.
Results:
. Only the very early CI group showed significant improvements in receptive language starting at 3 months post-CI, aligning with normal-hearing peers by 9 months and maintaining this level until age 2 years. During this period (<2 years), all improvements were more pronounced in receptive language than in expressive language.
Conclusion
. CI before 9 months of age significantly improved receptive language development compared to later CI, with improvements sustained at least up to the age of 2. This study supports the consideration of earlier CI, beyond pediatric Food and Drug Administration labeling criteria (>9 months), in children with profound deafness who have a clear deafness etiology and language development delays (<1st percentile).
3.A Novel Retractable Robotic Device for Colorectal Endoscopic Submucosal Dissection
Sang Hyun KIM ; Chanwoo KIM ; Bora KEUM ; Junghyun IM ; Seonghyeon WON ; Byung Gon KIM ; Kyungnam KIM ; Taebin KWON ; Daehie HONG ; Han Jo JEON ; Hyuk Soon CHOI ; Eun Sun KIM ; Yoon Tae JEEN ; Hoon Jai CHUN ; Joo Ha HWANG
Gut and Liver 2024;18(4):377-385
Background/Aims:
Appropriate tissue tension and clear visibility of the dissection area using traction are essential for effective and safe endoscopic submucosal dissection (ESD). In this study, we developed a retractable robot-assisted traction device and evaluated its performance in colorectal ESD.
Methods:
An experienced endoscopist performed ESD 18 times on an ex vivo porcine colon using the robot and 18 times using the conventional method. The outcome measures were procedure time, dissection speed, procedure-related adverse events, and blind dissection rate.
Results:
Thirty-six colonic lesions were resected from ex vivo porcine colon samples. The total procedure time was significantly shorter in robot-assisted ESD (RESD) than in conventional ESD (CESD) (20.1±4.1 minutes vs 34.3±8.3 minutes, p<0.05). The submucosal dissection speed was significantly faster in the RESD group than in the CESD group (36.8±9.2 mm 2 /min vs 18.1±4.7 mm 2 /min, p<0.05). The blind dissection rate was also significantly lower in the RESD group (12.8%±3.4% vs 35.1%±3.9%, p<0.05). In an in vivo porcine feasibility study, the robotic device was attached to a colonoscope and successfully inserted into the proximal colon without damaging the colonic wall, and ESD was successfully performed.
Conclusions
The dissection speed and safety profile improved significantly with the retractable RESD. Thus, our robotic device has the potential to provide simple, effective, and safe multidirectional traction during colonic ESD.
4.Transradial Versus Transfemoral Access for Bifurcation Percutaneous Coronary Intervention Using SecondGeneration Drug-Eluting Stent
Jung-Hee LEE ; Young Jin YOUN ; Ho Sung JEON ; Jun-Won LEE ; Sung Gyun AHN ; Junghan YOON ; Hyeon-Cheol GWON ; Young Bin SONG ; Ki Hong CHOI ; Hyo-Soo KIM ; Woo Jung CHUN ; Seung-Ho HUR ; Chang-Wook NAM ; Yun-Kyeong CHO ; Seung Hwan HAN ; Seung-Woon RHA ; In-Ho CHAE ; Jin-Ok JEONG ; Jung Ho HEO ; Do-Sun LIM ; Jong-Seon PARK ; Myeong-Ki HONG ; Joon-Hyung DOH ; Kwang Soo CHA ; Doo-Il KIM ; Sang Yeub LEE ; Kiyuk CHANG ; Byung-Hee HWANG ; So-Yeon CHOI ; Myung Ho JEONG ; Hyun-Jong LEE
Journal of Korean Medical Science 2024;39(10):e111-
Background:
The benefits of transradial access (TRA) over transfemoral access (TFA) for bifurcation percutaneous coronary intervention (PCI) are uncertain because of the limited availability of device selection. This study aimed to compare the procedural differences and the in-hospital and long-term outcomes of TRA and TFA for bifurcation PCI using secondgeneration drug-eluting stents (DESs).
Methods:
Based on data from the Coronary Bifurcation Stenting Registry III, a retrospective registry of 2,648 patients undergoing bifurcation PCI with second-generation DES from 21 centers in South Korea, patients were categorized into the TRA group (n = 1,507) or the TFA group (n = 1,141). After propensity score matching (PSM), procedural differences, in-hospital outcomes, and device-oriented composite outcomes (DOCOs; a composite of cardiac death, target vessel-related myocardial infarction, and target lesion revascularization) were compared between the two groups (772 matched patients each group).
Results:
Despite well-balanced baseline clinical and lesion characteristics after PSM, the use of the two-stent strategy (14.2% vs. 23.7%, P = 0.001) and the incidence of in-hospital adverse outcomes, primarily driven by access site complications (2.2% vs. 4.4%, P = 0.015), were significantly lower in the TRA group than in the TFA group. At the 5-year follow-up, the incidence of DOCOs was similar between the groups (6.3% vs. 7.1%, P = 0.639).
Conclusion
The findings suggested that TRA may be safer than TFA for bifurcation PCI using second-generation DESs. Despite differences in treatment strategy, TRA was associated with similar long-term clinical outcomes as those of TFA. Therefore, TRA might be the preferred access for bifurcation PCI using second-generation DES.
5.Trends of First-Line Targeted Therapy in Korean Patients With Metastatic Clear Cell Renal Cell Carcinoma: Sunitinib Versus Pazopanib, a Multicenter Study
Minsu CHOI ; Teak Jun SHIN ; Byung Hoon KIM ; Chun Il KIM ; Kyung Seop LEE ; Seock Hwan CHOI ; Hyun Tae KIM ; Tae-Hwan KIM ; Tae Gyun KWON ; Young Hwii KO ; Yoon Soo HAH ; Jae-Shin PARK ; Se Yun KWON
Korean Journal of Urological Oncology 2022;20(2):115-122
Purpose:
There have been few reports on comparison between sunitinib and pazopanib as first-line targeted therapy in Korean metastatic clear cell renal cell carcinoma (ccRCC). We sought to analyze the treatment trends of metastatic ccRCC by comparing the effects and adverse events of sunitinib and pazopanib.
Materials and Methods:
Data of 357 metastatic RCC patients who received the sunitinib or pazopanib as the first-line targeted therapy from the Daegyeong Oncology Study Group database was obtained and analyzed. Among these patients, patients who only clear cell type was confirmed after needle biopsy or nephrectomy were included, and patients who underwent target therapy for less than 3 months were excluded.
Results:
Of 251 patients who met the inclusion criteria, sunitinib and pazopanib group were identified in 156 (62%) and 95 patients (38%), respectively. Pazopanib group was older (66 years vs. 61 years, p=0.001) and more symptomatic (65% vs. 52%, p=0.037) and had more patients with Karnofsky performance status <80 (20% vs. 11%, p=0.048) and fewer number of organ metastases (p=0.004) compared to sunitinib group. There was no significant difference in disease control rate (88.5% vs. 87.3%, p=0.744), the median progression-free survival (19 months vs. 15 months, p=0.444) and overall survival (25 months vs. 19 months, p=0.721) between sunitinib and pazopanib. The most common grade 3/4 adverse events with sunitinib and pazopanib were anemia (5%) and hand-foot syndrome (3%), respectively. There was no significant difference between sunitinib and pazopanib in number of patients who experienced grade 3/4 adverse events (15% vs. 11%, p=0.275). However, there were more patients who discontinued treatment due to only adverse events in sunitinib group compared to pazopanib group (12% vs. 3%, p=0.020).
Conclusions
In Korean metastatic ccRCC, pazopanib tended to be used in patients with poorer health status compared to sunitinib. Sunitinib and pazopanib had no significant difference in treatment effect and survival, but pazopanib had more tolerable adverse events.
6.Triple Therapy-Based on Tegoprazan, a New Potassium-Competitive Acid Blocker, for First-Line Treatment of Helicobacter pylori Infection: A Randomized, Double-Blind, Phase III, Clinical Trial
Yoon Jin CHOI ; Yong Chan LEE ; Jung Mogg KIM ; Jin Il KIM ; Jeong Seop MOON ; Yun Jeong LIM ; Gwang Ho BAIK ; Byoung Kwan SON ; Hang Lak LEE ; Kyoung Oh KIM ; Nayoung KIM ; Kwang Hyun KO ; Hye-Kyung JUNG ; Ki-Nam SHIM ; Hoon Jai CHUN ; Byung-Wook KIM ; Hyuk LEE ; Jie-Hyun KIM ; Hyunsoo CHUNG ; Sang Gyun KIM ; Jae Young JANG
Gut and Liver 2022;16(4):535-546
Background/Aims:
We examined the efficacy and safety of tegoprazan as a part of first-line triple therapy for Helicobacter pylori eradication.
Methods:
A randomized, double-blind, controlled, multicenter study was performed to evaluate whether tegoprazan (50 mg)-based triple therapy (TPZ) was noninferior to lansoprazole (30 mg)-based triple therapy (LPZ) (with amoxicillin 1 g and clarithromycin 500 mg; all administered twice daily for 7 days) for treating H. pylori. The primary endpoint was the H. pylori eradication rate. Subgroup analyses were performed according to the cytochrome P450 (CYP) 2C19 genotype, the minimum inhibitory concentration (MIC) of amoxicillin and clarithromycin, and underlying gastric diseases.
Results:
In total, 350 H. pylori-positive patients were randomly allocated to the TPZ or LPZ group. The H. pylori eradication rates in the TPZ and LPZ groups were 62.86% (110/175) and 60.57% (106/175) in an intention-to-treat analysis and 69.33% (104/150) and 67.33% (101/150) in a per-protocol analysis (non-inferiority test, p=0.009 and p=0.013), respectively. Subgroup analyses according to MICs or CYP2C19 did not show remarkable differences in eradication rate. Both first-line triple therapies were well-tolerated with no notable differences.
Conclusions
TPZ is as effective as proton pump inhibitor-based triple therapy and is as safe as first-line H. pylori eradication therapy but does not overcome the clarithromycin resistance of H. pylori in Korea
7.Changes of The Epidemiologic Competences after Introductory Course of The Korea - Field Epidemiologist Training Program(K-FETP) in Epidemiologic Intelligence Servise(EIS) Officers
Eun-Young KIM ; Moo-Sik LEE ; Tae-Jun LEE ; Kwan LEE ; Hae-Sung NAM ; Ju-Hyoung LEE ; Hong-Bin KIM ; Byung-Chul CHUN ; Sang-Won LEE ; Dong-Han LEE ; Hee-Jung KIM ; Sung-Whe KWON ; Na-Bi YOON ; Moon-Chul SHIN ; Mee-Jee LIM
Journal of Agricultural Medicine & Community Health 2022;47(2):78-89
목적: 이 연구는 2019학년도 역학조사관 입문교육 과정에 참여한 29명의 수습과정생에게 참여형 자기주도 학습 역학조사관 연수 프로그램(FETP)의 효과와 만족도 등 역량 변화를 분석해 그 결과를 향후 과정 개발의 참고 자료로 활용하고자 하였다. 방법: 교육 프로그램의 만족도와 교육 후 모듈에 대한 역량 변화를 평가하는 연구가 수행되었다. 만족도와 역량의 차이 비교는 크루스칼 왈리스 검정(Kruskal-Wallis test)를 실시하였고, 역량의 차이는 윌콕슨 부호순위검정(Wilcoxon signed rank test)에 의해 이루어 졌다. 결과: 2019년 FETP에 참여한 역학조사관 중 여성은 48.3% 였으며, 40세 미만은 9.4% 였다. 역학조사관 입문교육과정 모듈(역학조사, 보건통계 및 정보통계, 감염병 국가 체계, 감염병 질환 감시 체계, 진단 및 실험실 검사, 생물 안전 및 관리, 주요 감염성질환 관리와 조사, 커뮤니케이션, 협동과 리더십, 일반과정)별 만족도는 실무적 도움, 전문성, 기능, 태도 등에서 4점(5점 만점)을 초과하였고, 전체 4.2±0.21(5점 만점)점으로 높은 수준이였다, 모듈의 교육훈련 전후 평균 점수는 2.25±0.91, 3.68±0.63점 등으로 유의한 향상이 있었으며, 모든 모듈 및 하위 주제들도 유의한 향상이 있었다(p<0.001). 그 중에서 현장역학조사 경험이 가장 높은 변화가 있었고, 표본 수집과 실무가 가장 낮은 역량 변화가 있었다. 결론: 2019년 진행된 입문교육 과정은 수료 후 학생들의 역량은 개선되었고, 만족도는 높은 편이었다. 참여형 자기주도학습의 촉진은 역량을 향상시킬 뿐만 아니라 보건 종사자들의 자신감을 높일 수 있었다.
8.Guidelines for Cancer Care during the COVID-19 Pandemic in South Korea
Jii Bum LEE ; Minkyu JUNG ; June Hyuk KIM ; Bo Hyun KIM ; Yeol KIM ; Young Seok KIM ; Byung Chang KIM ; Jin KIM ; Sung Ho MOON ; Keon-Uk PARK ; Meerim PARK ; Hyeon Jin PARK ; Sung Hoon SIM ; Hong Man YOON ; Soo Jung LEE ; Eunyoung LEE ; June Young CHUN ; Youn Kyung CHUNG ; So-Youn JUNG ; Jinsoo CHUNG ; Eun Sook LEE ; Hyun Cheol CHUNG ; Tak YUN ; Sun Young RHA
Cancer Research and Treatment 2021;53(2):323-329
At the end of 2019, the cause of pneumonia outbreaks in Wuhan, China, was identified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In February 2020, the World Health Organization named the disease cause by SARS-CoV-2 as coronavirus disease 2019 (COVID-19). In response to the pandemic, the Korean Cancer Association formed the COVID-19 task force to develop practice guidelines. This special article introduces the clinical practice guidelines for cancer patients which will help oncologists best manage cancer patients during the COVID-19 pandemic.
9.2021 Clinical Practice Guidelines for Diabetes Mellitus in Korea
Kyu Yeon HUR ; Min Kyong MOON ; Jong Suk PARK ; Soo-Kyung KIM ; Seung-Hwan LEE ; Jae-Seung YUN ; Jong Ha BAEK ; Junghyun NOH ; Byung-Wan LEE ; Tae Jung OH ; Suk CHON ; Ye Seul YANG ; Jang Won SON ; Jong Han CHOI ; Kee Ho SONG ; Nam Hoon KIM ; Sang Yong KIM ; Jin Wha KIM ; Sang Youl RHEE ; You-Bin LEE ; Sang-Man JIN ; Jae Hyeon KIM ; Chong Hwa KIM ; Dae Jung KIM ; SungWan CHUN ; Eun-Jung RHEE ; Hyun Min KIM ; Hyun Jung KIM ; Donghyun JEE ; Jae Hyun KIM ; Won Seok CHOI ; Eun-Young LEE ; Kun-Ho YOON ; Seung-Hyun KO ;
Diabetes & Metabolism Journal 2021;45(4):461-481
The Committee of Clinical Practice Guidelines of the Korean Diabetes Association (KDA) updated the previous clinical practice guidelines for Korean adults with diabetes and prediabetes and published the seventh edition in May 2021. We performed a comprehensive systematic review of recent clinical trials and evidence that could be applicable in real-world practice and suitable for the Korean population. The guideline is provided for all healthcare providers including physicians, diabetes experts, and certified diabetes educators across the country who manage patients with diabetes or the individuals at the risk of developing diabetes mellitus. The recommendations for screening diabetes and glucose-lowering agents have been revised and updated. New sections for continuous glucose monitoring, insulin pump use, and non-alcoholic fatty liver disease in patients with diabetes mellitus have been added. The KDA recommends active vaccination for coronavirus disease 2019 in patients with diabetes during the pandemic. An abridgement that contains practical information for patient education and systematic management in the clinic was published separately.
10.Guidelines for Cancer Care during the COVID-19 Pandemic in South Korea
Jii Bum LEE ; Minkyu JUNG ; June Hyuk KIM ; Bo Hyun KIM ; Yeol KIM ; Young Seok KIM ; Byung Chang KIM ; Jin KIM ; Sung Ho MOON ; Keon-Uk PARK ; Meerim PARK ; Hyeon Jin PARK ; Sung Hoon SIM ; Hong Man YOON ; Soo Jung LEE ; Eunyoung LEE ; June Young CHUN ; Youn Kyung CHUNG ; So-Youn JUNG ; Jinsoo CHUNG ; Eun Sook LEE ; Hyun Cheol CHUNG ; Tak YUN ; Sun Young RHA
Cancer Research and Treatment 2021;53(2):323-329
At the end of 2019, the cause of pneumonia outbreaks in Wuhan, China, was identified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In February 2020, the World Health Organization named the disease cause by SARS-CoV-2 as coronavirus disease 2019 (COVID-19). In response to the pandemic, the Korean Cancer Association formed the COVID-19 task force to develop practice guidelines. This special article introduces the clinical practice guidelines for cancer patients which will help oncologists best manage cancer patients during the COVID-19 pandemic.

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