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.

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.

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.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Objective: To identify the prevalence and factors associated with T-score discordance between the spine and hip, as well as between the paretic and non-paretic hips in hemiplegic stroke patients, this study investigated bone mineral density (BMD) patterns. Bone loss predominantly affects the paretic hip after a stroke, and typical clinical assessments using dual-energy X-ray absorptiometry (DXA) that scan the lumbar spine (LS) and a single hip may overlook an osteoporosis diagnosis. This oversight could potentially lead to suboptimal treatment for stroke patients. Methods: This study was a multicenter retrospective analysis of 540 patients admitted for stroke rehabilitation between October 2014 and February 2022, who underwent DXA of LS and bilateral hips. Results: The prevalence rates of concordance, low LS discordance, and low hip discordance between the LS and hips were 48.2%, 12.2%, and 39.6%, respectively. The discordance rate between bilateral hips was 17.0%. The paretic side had significantly lower total hip T-scores than the non-paretic side (p<0.001). Notably low paretic hip discordance was more prevalent during the chronic phase. DXA scans of the LS and both hips revealed a 0.7%–0.9% higher major discordance compared to LS and single hip DXA scans. The multivariate analysis revealed a significant correlation between a low paretic hip discordance and cognitive impairment (adjusted odds ratio 0.071, 95% confidence interval 0.931–1.003, p<0.05). Conclusion Since stroke survivors are at high risk for hip fractures, comprehensive BMD assessments, which include LS and bilateral hips, should be considered for post-stroke osteoporosis care to enhance diagnostic accuracy and timely treatment.

The submandibular displacement of a mandibular third molar residual root presents major challenges to oral and maxillofacial surgeons due to the proximity to critical anatomical structures such as the lingual nerve and sublingual artery. Preoperative imaging can approximate the location of the residual tooth root; however, accurately determining its exact position is difficult because of the dynamic nature of the mandible and the difficulty of realtime synchronization of imaging. This study presents the successful extraction of a residual mandibular third molar root in a 67-year-old female patient achieved using a magnetic field-based navigation system. The sublingually-displaced residual root was localized using the navigation system, marked using a virtual implant placement, and positioned by a hand piece using synchronized real-time sensor data. The root was successfully removed with a minimally-invasive approach. No complications occurred postoperatively, and follow-up showed no major issues. Due to the small size of the marker, ease of calibration, and independence from visual obstacles, magnetic field-based navigation systems are a promising tool for the removal of residual roots displaced into adjacent soft tissue.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Objective: To identify the prevalence and factors associated with T-score discordance between the spine and hip, as well as between the paretic and non-paretic hips in hemiplegic stroke patients, this study investigated bone mineral density (BMD) patterns. Bone loss predominantly affects the paretic hip after a stroke, and typical clinical assessments using dual-energy X-ray absorptiometry (DXA) that scan the lumbar spine (LS) and a single hip may overlook an osteoporosis diagnosis. This oversight could potentially lead to suboptimal treatment for stroke patients. Methods: This study was a multicenter retrospective analysis of 540 patients admitted for stroke rehabilitation between October 2014 and February 2022, who underwent DXA of LS and bilateral hips. Results: The prevalence rates of concordance, low LS discordance, and low hip discordance between the LS and hips were 48.2%, 12.2%, and 39.6%, respectively. The discordance rate between bilateral hips was 17.0%. The paretic side had significantly lower total hip T-scores than the non-paretic side (p<0.001). Notably low paretic hip discordance was more prevalent during the chronic phase. DXA scans of the LS and both hips revealed a 0.7%–0.9% higher major discordance compared to LS and single hip DXA scans. The multivariate analysis revealed a significant correlation between a low paretic hip discordance and cognitive impairment (adjusted odds ratio 0.071, 95% confidence interval 0.931–1.003, p<0.05). Conclusion Since stroke survivors are at high risk for hip fractures, comprehensive BMD assessments, which include LS and bilateral hips, should be considered for post-stroke osteoporosis care to enhance diagnostic accuracy and timely treatment.

The submandibular displacement of a mandibular third molar residual root presents major challenges to oral and maxillofacial surgeons due to the proximity to critical anatomical structures such as the lingual nerve and sublingual artery. Preoperative imaging can approximate the location of the residual tooth root; however, accurately determining its exact position is difficult because of the dynamic nature of the mandible and the difficulty of realtime synchronization of imaging. This study presents the successful extraction of a residual mandibular third molar root in a 67-year-old female patient achieved using a magnetic field-based navigation system. The sublingually-displaced residual root was localized using the navigation system, marked using a virtual implant placement, and positioned by a hand piece using synchronized real-time sensor data. The root was successfully removed with a minimally-invasive approach. No complications occurred postoperatively, and follow-up showed no major issues. Due to the small size of the marker, ease of calibration, and independence from visual obstacles, magnetic field-based navigation systems are a promising tool for the removal of residual roots displaced into adjacent soft tissue.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.