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.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.
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.Efficacy of Learning Disorder Treatment for Reading or Mathematics Disorders: An Open Study
Hyunju LEE ; Inhye SONG ; Woo Young KIM ; Hannah HUH ; Eun Kyoung LEE ; Jaesuk JUNG ; Cheon Seok SUH ; Hanik YOO
Journal of the Korean Academy of Child and Adolescent Psychiatry 2024;35(2):143-149
Objectives:
This study aimed to identify the effectiveness of treatment programs for children with reading (RD) or mathematics disorders (MD). Structured treatment programs were developed to improve phonological awareness and number sense among children and adolescents with RD or MD, respectively, and the effectiveness of the learning disorder treatment programs were evaluated.
Methods:
We used standardized, objective diagnostic, and evaluation tools not only to recruit participants with RD, MD, or comorbid attention deficit and hyperactivity disorder, but also to assess the effectiveness of the treatments regarding both improved core neurocognitive deficits of RD or MD and academic achievement. Forty children with RD or MD received one-on-one treatments from therapists.
Results:
In the RD group, treatment effects were observed in all subtests. In the word and paragraph reading tests, the accuracy rates and fluency improved. The results of the phonological working memory test, word–sound correspondence test, and rapid automatic naming tests also improved. In the MD group, the accuracy rate and fluency on the arithmetic test improved. An increase in the accuracy rate in the size and distance comparison tests and a decrease in the error rate in the estimation test were also observed. However, there were no improvements in reaction time in these subtests.
Conclusion
Learning disorder treatment programs that focus on improving phonological awareness or number sense in children with RD or MD improved achievement, phonological awareness, and number sense.
5.Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy
Eun Kyoung OH ; Seung-Ah LEE ; Hyun Joon LEE ; Yoon Jin CHA ; Sungjun KIM ; Hyung-Soo LEE ; Bum Chun SUH ; Ha Young SHIN ; Seung Woo KIM ; Byeol-A YOON ; Seong-il OH ; Yoo Hwan KIM ; Joong-Yang CHO ; Jeong Hee CHO ; Ki-Han KWON ; Young-Chul CHOI ; Hyung Jun PARK
Journal of Clinical Neurology 2023;19(5):460-468
Background:
and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy.
Methods:
We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy.
Results:
Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years.Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of antiHMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027).
Conclusions
Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.
6.Effect of silane incorporated adhesive or adhesive using separate silane on bonding of silica-based ceramics
Woo Kyoung SUH ; Kwang-Mahn KIM ; Jae-Sung KWON
Korean Journal of Dental Materials 2023;50(1):1-8
The purpose of this study was to evaluate the effectiveness of silane on the surface treatment of silica-based ceramic when silane is incorporated into the universal adhesive versus using silane separately from the adhesive. The materials used in this study are as follows: 1) All-Bond Universal, 2) Silane mixed with All-Bond Universal, 3) Porcelain primer (as a regular silane primer), and 4) Scoth Bond Universal (which is a commercial silane-containing universal adhesive). IPS e-Max press, a lithium disilicate, was used as the silica-based ceramic. Contact angle test was measured to evaluate hydrophobicity on a silica-based ceramic surface. Shear bond strength was tested by universal testing machine. The surfaces of the specimen were polished in order to eliminate any bias from micro mechanical retention due to an uneven surface. The results indicated that only silane applied to the silica based ceramic surface resulted in highest contact angle and shear bond strength. Using silane separately and using silane incorporated into the universal adhesive (contains hydrophobic resin) resulted in different performance on silica-based ceramic. Therefore, it was concluded that the silane should be used separately from a hydrophobic resin monomer in order to maximize its porcelain surface treatment function.
7.A simplified two-marker immunohistochemistry strategy for Lynch syndrome screening in endometrial cancer patients
Ala AIOB ; Yeo Rae KIM ; Kidong KIM ; Hyojin KIM ; Yong Beom KIM ; Duck Woo KIM ; Jae Hong NO ; Soo Hyun SEO ; Dong Hoon SUH ; Kyoung Un PARK
Obstetrics & Gynecology Science 2023;66(6):537-544
Objective:
To examine the efficacy of MSH6 and PMS2 immunohistochemistry (IHC) as a screening method for Lynch syndrome in endometrial cancer patients.
Methods:
Through multidisciplinary discussions, an institutional MSH6 and PMS2 IHC-initiated cascade test (MSH6, PMS2 IHC→microsatellite instability [MSI] assay→germline mismatch repair [MMR] gene sequencing) was developed to screen for Lynch syndrome in endometrial cancer patients. Testing was performed on a consecutive cohort of 218 newly diagnosed endometrial cancer patients who underwent surgery at a tertiary hospital in the Republic of Korea between August 2018 and December 2020. The number of MMR deficiencies (MSH6 or PMS2 loss in IHC) and results of subsequent tests (MSI assay and germline MMR gene sequencing) were examined.
Results:
MMR deficiency was detected in 52 of the 218 patients (24.0%). Among these 52 patients, 34 (65.0%) underwent MSI testing, of which 31 (91.0%) exhibited high MSI. Of the 31 patients with MSI-high status, 15 (48.0%) underwent germline MMR gene sequencing. Subsequently, Lynch syndrome was diagnosed in five patients (33.0%).
Conclusion
Lynch syndrome screening using MSH6 and PMS2 IHC-initiated cascade testing is a viable strategy in the management of endometrial cancer. A simplified strategy (MSH6 and PMS2 IHC→germline MMR gene sequencing) was proposed because most women with MMR deficiencies exhibited high MSI.
8.Changes in adhesive strength and pH of dental universal adhesive in accordance with varying proportions of 10-MDP
Woo Kyoung SUH ; Kwang-Mahn KIM ; Jae-Sung KWON
Korean Journal of Dental Materials 2023;50(4):281-292
This study investigated the physical properties of dental universal adhesive according to the varying proportions of 10-MDP, a functional monomer that is a representative component of universal adhesives. Experimental dental universal adhesives were made with 5 different proportions of 10-MDP (from 8.906 wt% to 10.500 wt%). Shear bond strength between bovine teeth and composite resin blocks were measured by applying each experimental adhesive between them. Following the shear bond strength test, fractured surfaces were observed for measurement of the fracture modes. Finally, the pH of the liquid experimental adhesives were measured, to understand results from shear bond strength. As the results, bonding strength of the experimental dental universal adhesive increased gradually with increasing amounts of 10-MDP, but showed a tendency to decrease gradually after a certain amount was exceeded. The highest adhesive strength was observed when 9.710 wt% of 10-MDP was added. In terms of pH, results showed that as the amount of 10-MDP in the experimental dental universal adhesive increased, the pH decreased from pH 2.9 to pH 2.76.Based on the results of this study, it was evident that the proportions of 10-MDP resulted in changes in adhesive strength and pH, though the optimal amount of 10-MDP would need a further investigations in the future.
9.Diagnosis and treatment of hereditary angioedema: An expert opinion
Jae-Woo JUNG ; So-Young PARK ; Sun Young YOON ; Gun-Woo KIM ; Kyoung-Hee SOHN ; Sung-Yoon KANG ; Hye Jung PARK ; Min-Kyu KANG ; Joo-Hee KIM ; Kyung Hee PARK ; Dong In SUH ; Dong Hun LEE ; Sae-Hoon KIM ; Hyouk-Soo KWON ; Hye-Ryun KANG
Allergy, Asthma & Respiratory Disease 2022;10(2):80-88
Hereditary angioedema (HAE) is a rare disease, but it severely interrupts daily life activities and can sometimes be life-threatening. Therefore, early diagnosis and prompt treatment of HAE attacks are critical. Physicians should be aware of how to diagnose and manage HAE to prepare not to miss a diagnosis when treating HAE patients. Physicians must also carry out tests to confirm the diagnosis of HAEs caused by C1 inhibitor deficiency (type 1) or C1 inhibitor dysfunction (type 2) in patients with recurrent angioedema. In addition, recent studies revealed another type of HAE which is not related to C1 inhibitor (normal C1 inhibitor HAE). Once HAE is confirmed, patients and their caregivers should be given with short-term and long-term treatment plans to relieve or prevent HAE attacks. HAE requires life-long measures, including psychological support for patients and self-management education.
10.Predicting stage ypT0–1N0 for nonradical management in patients with middle or low rectal cancer who undergo neoadjuvant chemoradiotherapy: a retrospective cohort study
Jeehye LEE ; In Jun YANG ; Jung Wook SUH ; Hong-min AHN ; Heung-Kwon OH ; Duck-Woo KIM ; Young-Hoon KIM ; Kyoung Ho LEE ; Sung-Bum KANG
Annals of Surgical Treatment and Research 2022;103(1):32-39
Purpose:
It is important to discover predictive factors that can identify rectal cancer patients who will respond well to neoadjuvant concurrent chemoradiotherapy (CCRT) to develop management strategies, preserve sphincter and avoid overtreatment. This study explored clinical factors that would predict the adequacy of nonradical management after CCRT in patients with middle or low rectal cancer.
Methods:
We retrospectively evaluated 447 patients with middle or low rectal cancer who were treated with curative surgery after neoadjuvant CCRT between January 2010 and December 2019. The good response group comprised patients with stages ypT0–1N0 on resection after CCRT; the remaining patients were included in the poor response group.
Results:
Of 447 patients (mean age, 60.37 ± 11.85 years), 108 (24.2%) had ypT0–1N0 (71.3% with ypT0N0, 4.6% with ypTisN0, and 24.1% with ypT1N0). Overall, 19 patients with cT1–2 (50.0% vs. 21.8% with cT3–4, P < 0.001), 22 with well-differentiated tumors (51.2% vs. 21.3% with moderately/poorly differentiated tumors, P < 0.001), 16 with fungating tumors (47.1% vs.22.3% with other types, P = 0.001), and 66 with anterior/posterior circumference direction (28.9% vs. 19.2% with lateral/ encircling direction, P = 0.016) had stage ypT0–1N0. On multivariable analysis, cT1–2 (P = 0.021) and well-differentiated tumor (P = 0.001) were independent predictors of ypT0–1N0. Fungating tumors were not significantly associated with ypT0– 1N0 (P = 0.054).
Conclusion
Stage cT1–2 and well differentiation are predictors of ypT0–1N0, while fungating tumors could be considered clinically meaningful, possibly identifying candidates for nonradical treatment post-CCRT.

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