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.

Patients with ectodermal dysplasia often have atrophied alveolar bone and an inadequate maxillomandibular relationship owing to congenital edentulism.Accurate implant placement that can overcomes anatomical limitations and orthognathic surgery to improve the maxillomandibular relationship is necessary for creating implant-supported prosthesis for these patients. Implant placement and provisional prosthesis fabrication before orthognathic surgery can provide critical fixed reference points and ensure accuracy during orthognathic surgery.In our patient, a digital system was used to design a surgical guide that considered the predictable position of the definitive prosthesis, allowing the placement of implants to overcome anatomical limitations and the creation of fixed reference points via the delivery of a provisional prosthesis for effective orthognathic surgery. The lack of compensation during orthognathic surgery was considered in the definitive prosthesis. As a result, a prosthesis with a minimal anterior cantilever was fabricated. This study aimed to determine the appropriate sequence of multidisciplinary collaborations that would, result in the best functional and aesthetic outcomes.

Patients with ectodermal dysplasia often have atrophied alveolar bone and an inadequate maxillomandibular relationship owing to congenital edentulism.Accurate implant placement that can overcomes anatomical limitations and orthognathic surgery to improve the maxillomandibular relationship is necessary for creating implant-supported prosthesis for these patients. Implant placement and provisional prosthesis fabrication before orthognathic surgery can provide critical fixed reference points and ensure accuracy during orthognathic surgery.In our patient, a digital system was used to design a surgical guide that considered the predictable position of the definitive prosthesis, allowing the placement of implants to overcome anatomical limitations and the creation of fixed reference points via the delivery of a provisional prosthesis for effective orthognathic surgery. The lack of compensation during orthognathic surgery was considered in the definitive prosthesis. As a result, a prosthesis with a minimal anterior cantilever was fabricated. This study aimed to determine the appropriate sequence of multidisciplinary collaborations that would, result in the best functional and aesthetic outcomes.

Purpose: Pancreas transplantation (PT) is a definitive treatment for diabetes mellitus (DM), restoring endogenous insulin secretion and improving glycemic control. Despite its efficacy, PT is less common in South Korea compared to Western nations. This study aims to report the clinical outcomes of PT over 2 decades at a single center, focusing on surgical techniques, complications, and graft survival. Methods: A retrospective analysis of 69 PT recipients at Seoul National University Hospital between January 2002 and December 2023 was conducted. Data on recipient and donor demographics, surgical details, immunosuppressive regimens, and graft outcomes were collected. Graft survival was evaluated using Kaplan-Meier analysis, with subgroup comparisons using the log-rank test. Graft failure was defined as graft removal, PT re-registration, insulin dependence exceeding 0.5 units/kg/day for more than 90 days, or patient death. Results: Among the 69 recipients, 50 (72.5%) had type 1 DM, and 18 (26.1%) had type 2 DM. Simultaneous pancreaskidney (SPK) transplantations comprised 84.1% (n = 58), and pancreas-after-kidney (PAK) transplantations accounted for 10.1%. The 1-year and 5-year death-censored pancreas graft survival rates were 92.7% and 89.6%, respectively, with no significant difference between SPK and PAK (P = 0.330). Graft failure occurred in 10 patients, primarily due to pancreatitis and rejection. Donor-related factors, particularly anoxic brain injury, were significantly associated with lower graft survival (P = 0.045). Conclusion PT outcomes in this cohort align with international standards, emphasizing the importance of donor selection and tailored immunosuppression. Expanding PT indications to include selective type 2 DM patients could benefit South Korea’s PT programs with adequate resource allocation.

Patients with ectodermal dysplasia often have atrophied alveolar bone and an inadequate maxillomandibular relationship owing to congenital edentulism.Accurate implant placement that can overcomes anatomical limitations and orthognathic surgery to improve the maxillomandibular relationship is necessary for creating implant-supported prosthesis for these patients. Implant placement and provisional prosthesis fabrication before orthognathic surgery can provide critical fixed reference points and ensure accuracy during orthognathic surgery.In our patient, a digital system was used to design a surgical guide that considered the predictable position of the definitive prosthesis, allowing the placement of implants to overcome anatomical limitations and the creation of fixed reference points via the delivery of a provisional prosthesis for effective orthognathic surgery. The lack of compensation during orthognathic surgery was considered in the definitive prosthesis. As a result, a prosthesis with a minimal anterior cantilever was fabricated. This study aimed to determine the appropriate sequence of multidisciplinary collaborations that would, result in the best functional and aesthetic outcomes.

Patients with ectodermal dysplasia often have atrophied alveolar bone and an inadequate maxillomandibular relationship owing to congenital edentulism.Accurate implant placement that can overcomes anatomical limitations and orthognathic surgery to improve the maxillomandibular relationship is necessary for creating implant-supported prosthesis for these patients. Implant placement and provisional prosthesis fabrication before orthognathic surgery can provide critical fixed reference points and ensure accuracy during orthognathic surgery.In our patient, a digital system was used to design a surgical guide that considered the predictable position of the definitive prosthesis, allowing the placement of implants to overcome anatomical limitations and the creation of fixed reference points via the delivery of a provisional prosthesis for effective orthognathic surgery. The lack of compensation during orthognathic surgery was considered in the definitive prosthesis. As a result, a prosthesis with a minimal anterior cantilever was fabricated. This study aimed to determine the appropriate sequence of multidisciplinary collaborations that would, result in the best functional and aesthetic outcomes.

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.

Purpose: Pancreas transplantation (PT) is a definitive treatment for diabetes mellitus (DM), restoring endogenous insulin secretion and improving glycemic control. Despite its efficacy, PT is less common in South Korea compared to Western nations. This study aims to report the clinical outcomes of PT over 2 decades at a single center, focusing on surgical techniques, complications, and graft survival. Methods: A retrospective analysis of 69 PT recipients at Seoul National University Hospital between January 2002 and December 2023 was conducted. Data on recipient and donor demographics, surgical details, immunosuppressive regimens, and graft outcomes were collected. Graft survival was evaluated using Kaplan-Meier analysis, with subgroup comparisons using the log-rank test. Graft failure was defined as graft removal, PT re-registration, insulin dependence exceeding 0.5 units/kg/day for more than 90 days, or patient death. Results: Among the 69 recipients, 50 (72.5%) had type 1 DM, and 18 (26.1%) had type 2 DM. Simultaneous pancreaskidney (SPK) transplantations comprised 84.1% (n = 58), and pancreas-after-kidney (PAK) transplantations accounted for 10.1%. The 1-year and 5-year death-censored pancreas graft survival rates were 92.7% and 89.6%, respectively, with no significant difference between SPK and PAK (P = 0.330). Graft failure occurred in 10 patients, primarily due to pancreatitis and rejection. Donor-related factors, particularly anoxic brain injury, were significantly associated with lower graft survival (P = 0.045). Conclusion PT outcomes in this cohort align with international standards, emphasizing the importance of donor selection and tailored immunosuppression. Expanding PT indications to include selective type 2 DM patients could benefit South Korea’s PT programs with adequate resource allocation.

Purpose: Pancreas transplantation (PT) is a definitive treatment for diabetes mellitus (DM), restoring endogenous insulin secretion and improving glycemic control. Despite its efficacy, PT is less common in South Korea compared to Western nations. This study aims to report the clinical outcomes of PT over 2 decades at a single center, focusing on surgical techniques, complications, and graft survival. Methods: A retrospective analysis of 69 PT recipients at Seoul National University Hospital between January 2002 and December 2023 was conducted. Data on recipient and donor demographics, surgical details, immunosuppressive regimens, and graft outcomes were collected. Graft survival was evaluated using Kaplan-Meier analysis, with subgroup comparisons using the log-rank test. Graft failure was defined as graft removal, PT re-registration, insulin dependence exceeding 0.5 units/kg/day for more than 90 days, or patient death. Results: Among the 69 recipients, 50 (72.5%) had type 1 DM, and 18 (26.1%) had type 2 DM. Simultaneous pancreaskidney (SPK) transplantations comprised 84.1% (n = 58), and pancreas-after-kidney (PAK) transplantations accounted for 10.1%. The 1-year and 5-year death-censored pancreas graft survival rates were 92.7% and 89.6%, respectively, with no significant difference between SPK and PAK (P = 0.330). Graft failure occurred in 10 patients, primarily due to pancreatitis and rejection. Donor-related factors, particularly anoxic brain injury, were significantly associated with lower graft survival (P = 0.045). Conclusion PT outcomes in this cohort align with international standards, emphasizing the importance of donor selection and tailored immunosuppression. Expanding PT indications to include selective type 2 DM patients could benefit South Korea’s PT programs with adequate resource allocation.

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.