Purpose: To investigate the clinical characteristics of South Korean patients with pericentral retinitis pigmentosa (RP) and to identify clinical biomarkers associated with rapid visual acuity decline based on baseline factors. Methods: This retrospective study included 59 eyes of 31 patients diagnosed with pericentral RP. Comprehensive ophthalmological examinations and genetic sequencing were conducted to assess the baseline characteristics. For biomarker analysis, eyes were categorized into two groups based on the annual rate of change in visual acuity. The clinical findings of the two groups were evaluated to identify the biomarkers associated with rapid loss of visual acuity. Results: Patients with pericentral RP in this study exhibited a mean best-corrected visual acuity of 0.17 ± 0.23 in logarithm of the minimum angle of resolution. The visual field test showed annular or semicircular scotoma with relatively preserved periphery and 27 eyes (45.8%) exhibited no macular complications in optical coherence tomography. Genetic analysis identified genes associated with previous typical and pericentral RP studies but also highlighted that many genetic causes of pericentral RP remain unidentified. Of the 55 eyes for which the rate of visual acuity change could be estimated, 18 exhibited an annual decline of ≥10%, whereas 37 showed an annual decline of <10%. Male sex and prolonged b-wave latency on dark-adapted 0.01 electroretinogram correlated with rapid visual acuity decline in the multivariate analysis. Conclusions South Korean patients with pericentral RP exhibited a milder phenotype compared to typical RP patients reported in previous studies. Genetic analysis revealed heterogeneity, with mutations in some genes commonly associated with milder forms of RP. Male sex and prolonged b-wave latency on dark-adapted 0.01 electroretinogram were significant biomarkers for predicting rapid visual acuity decline. Monitoring initial b-wave latency is important for predicting visual decline, particularly in male patients with pericentral RP.

Objective: To compare a deep learning (DL)-accelerated non-enhanced abbreviated MRI (AMRI DL) protocol with standard AMRI (AMRI STD) of the liver in terms of image quality and malignant focal lesion detection. Materials and Methods: This retrospective study included 155 consecutive patients (110 male; mean age 62.4 ± 11 years) from two sites who underwent standard liver MRI and additional AMRIDL sequences, specifically DL-accelerated single-shot fast-spin echo (SSFSE DL) and DL-accelerated diffusion-weighted imaging (DWIDL). Additional MRI phantom experiments assessed signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient (ADC) values. Three reviewers evaluated AMRIDL and AMRI STD protocols for image quality using a five-point Likert scale and identified malignant hepatic lesions. Image quality scores and per-lesion sensitivities were compared between AMRIDL and AMRI STD using the Wilcoxon signed-rank test and logistic regression with generalized estimating equations, respectively. Results: Phantom experiments demonstrated comparable SNR and higher CNR for SSFSE DL compared to SSFSE STD, with similar ADC values for DWIDL and DWI STD. Among the 155 patients, 130 (83.9%) had chronic liver disease or a history of intra- or extrahepatic malignancy. Of 104 malignant focal lesions in 64 patients, 58 (55.8%) were hepatocellular carcinomas (HCCs), 38 (36.5%) were metastases, four (3.8%) were cholangiocarcinomas, and four (3.8%) were lymphomas. The pooled per-lesion sensitivity across three readers was 97.6% for AMRIDL, comparable to 97.6% for AMRI STD. Compared with AMRI STD, AMRIDL demonstrated superior image quality regarding structural sharpness, artifacts, and noise (all P < 0.001) and reduced the average scan time by approximately 50% (2 min 29 sec vs. 4 min 11 sec). In patients with chronic liver disease, AMRIDL achieved a 96.6% per-lesion sensitivity for HCC detection, similar to 96.5% for AMRI STD (P > 0.05). Conclusion The AMRIDL protocol offers comparable sensitivity for detecting malignant focal lesions, including HCC while significantly enhancing image quality and reducing scan time by approximately 50% compared to AMRI STD.

Purpose: This study aimed to characterize the clinical patterns and severity of brain injury in neonates who survived extracorporeal membrane oxygenation (ECMO) therapy for acute respiratory failure during the neonatal period, to evaluate their short-term neurodevelopmental outcomes, and to identify the factors associated with these outcomes. Methods: We retrospectively reviewed the medical records of neonates who survived ECMO between 2018 and 2024. Based on brain magnetic resonance imaging (MRI) findings, the patients were classified into two groups: no/mild and moderate/severe brain injury. Neurodevelopmental outcomes were assessed at 12–40 months of age using the Bayley Scale of Infant Development II/III and/or the Korean Developmental Screening Test. Results: Among the 19 neonates included in the study, 18 (94.7%) showed varying degrees of brain injury on MRI (mild: 12, moderate: 1, severe: 5). Neonates with moderate/severe brain injury had significantly longer durations of ECMO support and extended durations of mechanical ventilation and were more likely to receive continuous renal replacement therapy than those with no or mild injury. Developmental delay was identified in 36.8% of survivors and was significantly associated with prolonged mechanical ventilation, longer neonatal intensive care unit stays, and a higher incidence of seizures. Conclusion Brain injury is frequently observed on MRI in neonates treated with ECMO. However, its direct association with adverse neurodevelopmental outcomes is not definitive. Since MRI findings alone cannot predict developmental outcomes, clinical and environmental factors should be integrated into prognostic assessments.

Objective: To compare a deep learning (DL)-accelerated non-enhanced abbreviated MRI (AMRI DL) protocol with standard AMRI (AMRI STD) of the liver in terms of image quality and malignant focal lesion detection. Materials and Methods: This retrospective study included 155 consecutive patients (110 male; mean age 62.4 ± 11 years) from two sites who underwent standard liver MRI and additional AMRIDL sequences, specifically DL-accelerated single-shot fast-spin echo (SSFSE DL) and DL-accelerated diffusion-weighted imaging (DWIDL). Additional MRI phantom experiments assessed signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and apparent diffusion coefficient (ADC) values. Three reviewers evaluated AMRIDL and AMRI STD protocols for image quality using a five-point Likert scale and identified malignant hepatic lesions. Image quality scores and per-lesion sensitivities were compared between AMRIDL and AMRI STD using the Wilcoxon signed-rank test and logistic regression with generalized estimating equations, respectively. Results: Phantom experiments demonstrated comparable SNR and higher CNR for SSFSE DL compared to SSFSE STD, with similar ADC values for DWIDL and DWI STD. Among the 155 patients, 130 (83.9%) had chronic liver disease or a history of intra- or extrahepatic malignancy. Of 104 malignant focal lesions in 64 patients, 58 (55.8%) were hepatocellular carcinomas (HCCs), 38 (36.5%) were metastases, four (3.8%) were cholangiocarcinomas, and four (3.8%) were lymphomas. The pooled per-lesion sensitivity across three readers was 97.6% for AMRIDL, comparable to 97.6% for AMRI STD. Compared with AMRI STD, AMRIDL demonstrated superior image quality regarding structural sharpness, artifacts, and noise (all P < 0.001) and reduced the average scan time by approximately 50% (2 min 29 sec vs. 4 min 11 sec). In patients with chronic liver disease, AMRIDL achieved a 96.6% per-lesion sensitivity for HCC detection, similar to 96.5% for AMRI STD (P > 0.05). Conclusion The AMRIDL protocol offers comparable sensitivity for detecting malignant focal lesions, including HCC while significantly enhancing image quality and reducing scan time by approximately 50% compared to AMRI STD.

Purpose: This study aimed to characterize the clinical patterns and severity of brain injury in neonates who survived extracorporeal membrane oxygenation (ECMO) therapy for acute respiratory failure during the neonatal period, to evaluate their short-term neurodevelopmental outcomes, and to identify the factors associated with these outcomes. Methods: We retrospectively reviewed the medical records of neonates who survived ECMO between 2018 and 2024. Based on brain magnetic resonance imaging (MRI) findings, the patients were classified into two groups: no/mild and moderate/severe brain injury. Neurodevelopmental outcomes were assessed at 12–40 months of age using the Bayley Scale of Infant Development II/III and/or the Korean Developmental Screening Test. Results: Among the 19 neonates included in the study, 18 (94.7%) showed varying degrees of brain injury on MRI (mild: 12, moderate: 1, severe: 5). Neonates with moderate/severe brain injury had significantly longer durations of ECMO support and extended durations of mechanical ventilation and were more likely to receive continuous renal replacement therapy than those with no or mild injury. Developmental delay was identified in 36.8% of survivors and was significantly associated with prolonged mechanical ventilation, longer neonatal intensive care unit stays, and a higher incidence of seizures. Conclusion Brain injury is frequently observed on MRI in neonates treated with ECMO. However, its direct association with adverse neurodevelopmental outcomes is not definitive. Since MRI findings alone cannot predict developmental outcomes, clinical and environmental factors should be integrated into prognostic assessments.

Background: The ionic mechanism underlying Brugada syndrome (BrS) arises from an imbalance in transient outward current flow between the epicardium and endocardium.Previous studies report that artemisinin, originally derived from a Chinese herb for antimalarial use, inhibits the Ito current in canines. In a prior study, we showed the antiarrhythmic effects of artemisinin in BrS wedge preparation models. However, quinidine remains a well-established antiarrhythmic agent for treating BrS. Therefore, this study aims to investigate the efficacy of combining artemisinin with low-dose quinidine in suppressing ventricular tachyarrhythmia (VTA) in experimental canine BrS models. Methods: Transmural pseudo-electrocardiogram and epicardial/endocardial action potential (AP) were recorded from coronary-perfused canine right ventricular wedge preparation. To mimic the BrS model, acetylcholine (3 μM), calcium channel blocker verapamil (1 μM), and Ito agonist NS5806 (6–10 μM) were administered until VTA was induced. Subsequently, lowdose quinidine (1–2 μM) combined with artemisinin (100 μM) was perfused to mitigate VTA.Key parameters, including AP duration, J wave area, notch index, and T wave dispersion, were measured. Results: After administering the provocation agents, all sample models exhibited prominent J waves and VTA. Artemisinin alone (100–150 μM) suppressed VTA and restored the AP dome in all three preparations. Its infusion resulted in reductions in the J wave area and epicardial notch index. Consequently, low-dose quinidine (1–2 μM) did not fully restore the AP dome in all six sample models. However, when combined with additional artemisinin (100 μM), lowdose quinidine effectively suppressed VTA in all six models and restored the AP dome while also decreasing the J wave area and epicardial notch index. Conclusion Low-dose quinidine alone fails to fully alleviate VTA in the BrS wedge model.However, its combination with artemisinin effectively suppresses VTA. Artemisinin may reduce the therapeutic dose of quinidine, potentially minimizing its associated adverse effects.

Background: The ionic mechanism underlying Brugada syndrome (BrS) arises from an imbalance in transient outward current flow between the epicardium and endocardium.Previous studies report that artemisinin, originally derived from a Chinese herb for antimalarial use, inhibits the Ito current in canines. In a prior study, we showed the antiarrhythmic effects of artemisinin in BrS wedge preparation models. However, quinidine remains a well-established antiarrhythmic agent for treating BrS. Therefore, this study aims to investigate the efficacy of combining artemisinin with low-dose quinidine in suppressing ventricular tachyarrhythmia (VTA) in experimental canine BrS models. Methods: Transmural pseudo-electrocardiogram and epicardial/endocardial action potential (AP) were recorded from coronary-perfused canine right ventricular wedge preparation. To mimic the BrS model, acetylcholine (3 μM), calcium channel blocker verapamil (1 μM), and Ito agonist NS5806 (6–10 μM) were administered until VTA was induced. Subsequently, lowdose quinidine (1–2 μM) combined with artemisinin (100 μM) was perfused to mitigate VTA.Key parameters, including AP duration, J wave area, notch index, and T wave dispersion, were measured. Results: After administering the provocation agents, all sample models exhibited prominent J waves and VTA. Artemisinin alone (100–150 μM) suppressed VTA and restored the AP dome in all three preparations. Its infusion resulted in reductions in the J wave area and epicardial notch index. Consequently, low-dose quinidine (1–2 μM) did not fully restore the AP dome in all six sample models. However, when combined with additional artemisinin (100 μM), lowdose quinidine effectively suppressed VTA in all six models and restored the AP dome while also decreasing the J wave area and epicardial notch index. Conclusion Low-dose quinidine alone fails to fully alleviate VTA in the BrS wedge model.However, its combination with artemisinin effectively suppresses VTA. Artemisinin may reduce the therapeutic dose of quinidine, potentially minimizing its associated adverse effects.

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.