Idiopathic inflammatory myopathies (IIMs) are heterogeneous immune-mediated muscle disorders with variable extramuscular manifestations and outcomes. Myositis-specific auto-antibodies (MSAs) have transformed the IIM field by enabling a serology-informed taxonomy based on distinct clinical phenotypes, prognostic trajectories, and therapeutic responses. Anti-Jo-1 and other antisynthetase antibodies characterize interstitial lung disease (ILD)-prone forms of disease; anti-MDA5 identifies patients at risk of rapidly progressive ILD and early mortality; anti-TIF1-γ and anti-NXP2 are indicative of cancer-associated myositis; anti-SRP and anti-HMGCR delineate necrotizing myopathies; and anti-cN1A supports the diagnosis of inclusion-body myositis. Beyond diagnosis, MSA profiles provide prognostic information, including about survival, relapse risk, and organ-specific complications, and they are increasingly being used to guide treatment decisions and predict biologic responsiveness. This review summarizes the diagnostic, prognostic, and therapeutic implications of MSAs across the IIM spectrum and highlight their growing importance in clinical practice for risk stratification and patient management.

Background/Aims: Patients with postural tachycardia syndrome (POTS) commonly experience gastrointestinal (GI) symptoms. We aim to assess the feasibility and preliminary efficacy data for DA-9701, a prokinetic agent targeting 5-hy-droxytryptamine 1A, 5-hydroxytryptamine 4, and dopamine D 2 receptors, in patients with POTS. Methods: In a randomized, double-blind, placebo-controlled, single-center crossover trial, patients with POTS were given either 30 mg of DA-9701 or a placebo 3 times daily for eight weeks in a 1:1 ratio. After a 4-week washout, patients received the alternate treatment for another 8 weeks. The primary endpoint focused on assessing the change in GI symptoms (total Nepean Dyspepsia Index-Korean version [NDI-K] symptom score) from baseline over the 8 week-treatment period. Endpoints were assessed in all enrolled and randomized patients (intention-to-treat), and in those who completed the trial (per-protocol analysis). Results: Between January 2022 and August 2023, 24 patients were randomized (n = 12 per group), with 3 discontinuing after randomization. DA-9701 did not significantly improve primary endpoints for total NDI-K symptom scores in either the intention-to-treat (least-squares means, –13.9 vs. –9.5, P = 0.326) or per-protocol analyses (–17.2 vs –12.0, P = 0.242).Notably, a trend toward improvement in specific GI symptoms, such as upper abdominal pain, was observed in both intention-to-treat (–0.6 vs 0.7; P = 0.066) and per-protocol analyses (–0.9 vs 0.6; P = 0.045). No serious adverse events were observed. Conclusion DA-9701 did not improve GI symptoms in this crossover trial; however, its potential effect on specific GI symptoms merits further investigation.

As the population ages, the incidence of osteoporotic vertebral compression fractures (OVCF) continues to rise, leading to an increased use of cement augmentation procedures. Consequently, clinicians are more frequently encountering patients with cement-augmented vertebrae who require additional spinal instrumentation. However, pedicle screw insertion into previously cement-augmented vertebral bodies remains technically challenging. This study aimed to describe a simple and reproducible technique for a safe and effective insertion of a pedicle screw into cement-augmented vertebral bodies. Ten patients with a history of cement augmentation for OVCF, who subsequently developed severe kyphotic deformity or degenerative spinal disease requiring posterior instrumentation, were treated using this technique. Pedicle screws were successfully inserted into all cement-augmented vertebrae without any intraoperative complications, including drill tip breakage, cement dislodgement, or anterior wall violation. In conclusion, pedicle screw insertion into cementaugmented vertebral bodies can be performed safely and reliably. This method may simplify a procedure that has traditionally been regarded as technically demanding.

Purpose: Current guidelines recommend vaccination at least 2 weeks before chemotherapy initiation to optimize the immune response despite limited evidence. Our previous study indicated no differences in short-term immune response for the 13-valent pneumococcal conjugate vaccine (PCV13) according to the vaccination timing. This study aims to investigate the long-term efficacy of PCV13 and clinical factors associated with the respective antibody response. Materials and Methods: Patients with gastric or colorectal cancer who received adjuvant chemotherapy were enrolled and divided into two groups: vaccinated 2 weeks before chemotherapy (arm A) and vaccinated concurrently with chemotherapy (arm B). Serum samples were collected before vaccination and in one month, 3 years, and 5 years. Immune responses were measured using enzyme-linked immunosorbent assay and multiplex opsonophagocytosis assay. Results: Including 63 patients, both groups showed an initial increase in the geometric mean titers of opsonophagocytic activity and the geometric mean concentrations of serotype-specific IgG levels after one month, followed by a decline at 3 and 5 years, particularly for serotypes 1, 14, 18C, and 19A. Despite the decline, global protection was maintained for 5 years, although global response decreased. The two arms did not show significant differences in immunogenicity nor in factors such as vaccination timing, age, cancer type, or chemotherapy regimen. Conclusion Vaccination timing is not a significant factor for the immunogenicity of PCV13 in cancer patients undergoing adjuvant chemotherapy. Global protection against pneumococcal infection was sustained for > 5 years, and global response remained in over half of patients.

Hepatocellular carcinoma (HCC) is a major global health burden and is often diagnosed at an advanced stage, when curative treatment options are limited. Current surveillance strategies, including ultrasonography and serum alpha-fetoprotein measurement, lack sufficient sensitivity and specificity, highlighting the urgent need for improved biomarkers. MicroRNAs (miRNAs), small noncoding RNAs that regulate gene expression at the posttranscriptional level, have emerged as key regulators of hepatocarcinogenesis. Their dysregulation contributes to tumor growth, resistance to cell death, and metastasis, with some miRNAs serving as oncogenes and others as tumor suppressors. Circulating miRNAs have emerged as promising diagnostic biomarkers because of their stability in body fluids and tissue-specific expression patterns. In addition, they provide valuable prognostic information by reflecting tumor behavior and response to treatment. This review summarizes the roles of miRNAs in HCC pathogenesis and discusses their potential clinical applications in HCC diagnosis and prognosis.

We report a genetically confirmed case of adult-onset Alexander disease (AOAD) presenting with progressive dysarthria and palatal tremor. Brain magnetic resonance imaging revealed medullary atrophy and olivary hyperintensities. A pathogenic variant of the glial fibrillary acidic protein gene (p.R416W) was identified. The palatal tremor indicated involvement of the Guillain-Mollaret triangle. This case underscores the diagnostic value of bulbar signs and neuroimaging in AOAD and highlights the importance of integrating clinical, radiological, and genetic findings for early definitive diagnosis.

Background: Recombinant human follicle-stimulating hormone (rhFSH) is commonly used to treat female infertility, but its short half-life necessitates multiple doses. Even corifollitropin alfa, with an extended half-life, requires supplementary injections of rhFSH after 7 days. This study aimed to develop and evaluate a long-acting follicle-stimulating hormone (FSH) formulation using anti-serum albumin Fab-associated (SAFA) technology to avoid additional injections and enhance ovarian function. Methods: SAFA-FSH was synthesized using a Chinese hamster ovary expression system. Its biological efficacy was confirmed through assays measuring its ability to stimulate cyclic adenosine monophosphate (cAMP) production, estradiol synthesis, and the expression of human cytochrome P450 family 19 subfamily A member 1 (hCYP19α1) and human steroidogenic acute regulatory protein (hSTAR) in human ovarian granulosa (KGN) cells. To evaluate the effects of SAFA-FSH, we compared its impact on serum estradiol levels and ovarian weight increase with that of rhFSH in Sprague-Dawley (SD) rats using the modified Steelman-Pohley test. Results: The results indicated that SAFA-FSH induces cAMP synthesis in KGN cells and upregulates the expression of hCYP19α1 and hSTAR in a dose-dependent manner. Female SD rats, aged 21 days, receiving daily subcutaneous human chorionic gonadotropin injections for 5 days exhibited a significant increase in serum estradiol levels and ovarian weight when administered SAFA-FSH on the first day or when given nine injections of rhFSH over 5 days. Notably, the group receiving SAFA-FSH on the first and third days demonstrated an even greater rise in serum estradiol levels and ovarian weight. Conclusion These findings suggest that SAFA-FSH presents a promising alternative to current rhFSH treatments for female infertility. However, further research is essential to thoroughly assess its safety and efficacy in clinical contexts.

OBJECTIVES: Low handgrip strength (HGS) in children and adolescents might be associated with the risk of metabolic syndrome (MetS) and insulin resistance. This study prospectively evaluated the association between HGS in childhood and MetS in adolescence. METHODS: Based on data from the Ewha Birth and Growth Study, this study analyzed HGS at ages 7 to 9 and metabolic indices at ages 13 to 15. In total, 219 participants were analyzed. The risk of MetS was evaluated using the continuous metabolic syndrome score (cMetS), and insulin resistance was assessed using fasting blood insulin and homeostasis model assessment of insulin resistance (HOMA-IR). Relative HGS in childhood was determined by dividing HGS by body weight and categorized as sex-specific quartiles. RESULTS: This study found an inverse association between relative HGS levels in childhood and MetS and insulin resistance in adolescence. For each 1-group increase in relative HGS quartiles, cMetS (standarard [Std] β=-0.64, p<0.01), HOMA-IR (Std β=-0.21, p<0.01), and fasting blood insulin (Std β=-0.21, p<0.01) all decreased on average. These associations remained significant even after adjusting for confounding factors. CONCLUSIONS Our study showed a prospective association between HGS in childhood and the risk of MetS and insulin resistance in adolescence. It provides significant epidemiological evidence, emphasizing the importance of efforts to increase muscle strength from a young age to mitigate the risk of MetS and insulin resistance in adolescence.

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.