1.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
2.Robot-assisted ureteral reconstruction for managing kidney transplant patients with ureteric complications
Dongho SHIN ; San KANG ; Seung Ah RHEW ; Chang Eil YOON ; Hyong Woo MOON ; Yong Hyun PARK ; Hyuk Jin CHO
Investigative and Clinical Urology 2025;66(1):18-26
Purpose:
To evaluate the feasibility of robot-assisted ureteral reconstruction as a minimally invasive alternative to open surgery for managing ureteric complications in transplanted kidneys.
Materials and Methods:
From January 2020 to December 2023, robot-assisted ureteral reconstruction was performed on fifteen kidney transplant patients with vesicoureteral reflux (VUR) or ureteral stricture who had previously failed endoscopic treatments.
Results:
Twelve females and three males, with a mean age of 48.6±6.6 years, were included in the study. Nine patients (60.0%) underwent surgery due to VUR (grade III or higher) of the transplanted kidney, and six patients (40.0%) had transplanted ureteral strictures. Postoperative voiding cystourethrogram (VCUG) was performed at 3.2±1.6 months. Seven patients (77.8%) became VUR-free, while two patients (22.2%) had VUR regression from grade IV to I. All six patients who underwent reconstruction due to anastomosis site stricture became stenosis-free without the need for an indwelling ureteral catheter. In cases where the ureter was too short for reimplantation, a Boari flap or end-to-end anastomosis with the native ureter was performed. The mean hospital stay was 5.9±4.5 days. The urethral catheter was removed after 15.1±5.4 days, and the ureteral catheter was removed after 4.9±1.5 weeks. The mean follow-up period was 23.9±6.8 months, with no additional interventions required after surgery. No complications above Clavien-Dindo grade I were recorded.
Conclusions
Robotic ureteral reconstruction is technically feasible and offers an effective, minimally invasive treatment for ureteric complications in kidney transplant patients, serving as an alternative to open surgery.
3.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
4.Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy
Soo-Hyun KIM ; Yunjung CHOI ; Eun Kyoung OH ; Ichizo NISHINO ; Shigeaki SUZUKI ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Hyunjin KIM ; Young-Min LIM ; Seol-Hee BAEK ; Je-Young SHIN ; Hung Youl SEOK ; Seung-Ah LEE ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2025;21(1):31-39
Background:
and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods:
We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined.
Results:
The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies.
Conclusions
This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.
5.2024 Korean Society of Myocardial Infarction/National Evidence-Based Healthcare Collaborating Agency Guideline for the Pharmacotherapy of Acute Coronary Syndromes
Hyun Kuk KIM ; Seungeun RYOO ; Seung Hun LEE ; Doyeon HWANG ; Ki Hong CHOI ; Jungeun PARK ; Hyeon-Jeong LEE ; Chang-Hwan YOON ; Jang Hoon LEE ; Joo-Yong HAHN ; Young Joon HONG ; Jin Yong HWANG ; Myung Ho JEONG ; Dong Ah PARK ; Chang-Wook NAM ; Weon KIM
Korean Circulation Journal 2024;54(12):767-793
Many countries have published clinical practice guidelines for appropriate clinical decisions, optimal treatment, and improved clinical outcomes in patients with acute coronary syndrome. Developing guidelines that are specifically tailored to the Korean environment is crucial, considering the treatment system, available medications and medical devices, racial differences, and level of language communication. In 2017, the Korean Society of Myocardial Infarction established a guideline development committee. However, at that time, it was not feasible to develop guidelines, owing to the lack of knowledge and experience in guideline development and the absence of methodology experts. In 2022, the National EvidenceBased Healthcare Collaborating Agency collaborated with a relevant academic association to develop internationally reliable guidelines, with strict adherence to the methodology for evidence-based guideline development. The first Korean acute coronary syndrome guideline starts from the 9 key questions for pharmacotherapy.
6.2024 Korean Society of Myocardial Infarction/National Evidence-Based Healthcare Collaborating Agency Guideline for the Pharmacotherapy of Acute Coronary Syndromes
Hyun Kuk KIM ; Seungeun RYOO ; Seung Hun LEE ; Doyeon HWANG ; Ki Hong CHOI ; Jungeun PARK ; Hyeon-Jeong LEE ; Chang-Hwan YOON ; Jang Hoon LEE ; Joo-Yong HAHN ; Young Joon HONG ; Jin Yong HWANG ; Myung Ho JEONG ; Dong Ah PARK ; Chang-Wook NAM ; Weon KIM
Korean Circulation Journal 2024;54(12):767-793
Many countries have published clinical practice guidelines for appropriate clinical decisions, optimal treatment, and improved clinical outcomes in patients with acute coronary syndrome. Developing guidelines that are specifically tailored to the Korean environment is crucial, considering the treatment system, available medications and medical devices, racial differences, and level of language communication. In 2017, the Korean Society of Myocardial Infarction established a guideline development committee. However, at that time, it was not feasible to develop guidelines, owing to the lack of knowledge and experience in guideline development and the absence of methodology experts. In 2022, the National EvidenceBased Healthcare Collaborating Agency collaborated with a relevant academic association to develop internationally reliable guidelines, with strict adherence to the methodology for evidence-based guideline development. The first Korean acute coronary syndrome guideline starts from the 9 key questions for pharmacotherapy.
7.Successful Treatment of Relapsed Disseminated Juvenile Xanthogranuloma with Central Nervous System Involvement
Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM ; Pyeong Hwa KIM ; Ah Young JUNG ; Kyung-Nam KOH
Clinical Pediatric Hematology-Oncology 2024;31(2):47-51
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.
8.Breast Cancer Statistics in Korea, 2021
Chihwan David CHA ; Chan Sub PARK ; Hee-Chul SHIN ; Jaihong HAN ; Jung Eun CHOI ; Joo Heung KIM ; Kyu-Won JUNG ; Sae Byul LEE ; Sang Eun NAM ; Tae In YOON ; Young-Joon KANG ; Zisun KIM ; So-Youn JUNG ; Hyun-Ah KIM ;
Journal of Breast Cancer 2024;27(6):351-361
The Korean Breast Cancer Society (KBCS) has collected nationwide registry data on clinicopathologic characteristics and treatment since 1996. This study aimed to analyze the clinical characteristics of breast cancer in Korea and assess changes in breast cancer statistics for 2021 using data from the KBCS registry and the Korean Central Cancer Registry. In 2021, 34,628 women were newly diagnosed with breast cancer. The median age of women diagnosed with breast cancer was 53.4 years, with the highest incidence occurring in the 40–49 age group. The most common molecular subtype was hormone receptor-positive and human epidermal growth factor receptor 2 (HER2)-negative, accounting for 69.1% of cases, while HER2-positive subtypes comprised 19.3%. During the coronavirus disease 2019 pandemic, the national breast cancer screening rate declined. However, the incidence of early-stage breast cancer (stages 0 and I) continued to increase, accounting for 65.6% of newly diagnosed cases in 2021. Our results showed that the overall survival rate for patients with breast cancer has improved, primarily due to a rise in early-stage diagnoses and advancements in treatment.
9.Successful Treatment of Relapsed Disseminated Juvenile Xanthogranuloma with Central Nervous System Involvement
Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM ; Pyeong Hwa KIM ; Ah Young JUNG ; Kyung-Nam KOH
Clinical Pediatric Hematology-Oncology 2024;31(2):47-51
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.
10.Successful Treatment of Relapsed Disseminated Juvenile Xanthogranuloma with Central Nervous System Involvement
Su Hyun YOON ; Sung Han KANG ; Hyery KIM ; Ho Joon IM ; Pyeong Hwa KIM ; Ah Young JUNG ; Kyung-Nam KOH
Clinical Pediatric Hematology-Oncology 2024;31(2):47-51
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

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