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Biomarkers of Glycosaminoglycans (GAG) accumulation in patients with mucopolysaccharidosis type VI—LeukoGAG, Corneal Opacification (COM) and Carotid Intima Media Thickening (CIMT)
Get PDFMucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal storage disorder characterized by deficient activity of arylsulfatase B enzyme (ASB) resulting in cellular accumulation of dermatan sulfate (DS) and chondroitin sulfate (CS) that leads to cell injury. Urinary glycosaminoglycans (GAG) are often used as a biomarker in MPS diseases for diagnosis and to monitor treatment efficacy. This study evaluated leukocyte GAGs (leukoGAG) and skin GAGs as alternate biomarkers representing intracellular GAG changes in patients with MPS VI and treated with enzyme replacement therapy (ERT). In addition, we evaluated corneal opacification measurements (COM) and carotid intima media thickness (CIMT) as indicators of GAG accumulation and tissue injury. The study was performed in a serial two-step design in a single center. A quantitative method to measure leukoGAG levels in leukocytes was developed in Study 1 to compare the GAG levels between MPS VI patients and a control group and to assess correlations between leukoGAG and urineGAG. Study 2 validated the leukoGAG measurement, assessed the effect of ERT infusion on leukoGAG and ASB activity in leukocytes, identified correlations between leukoGAG and other biomarkers, and assessed differences in GAG accumulation between MPS VI patients and control subjects. In Study 1, leukoCS and leukoDS levels were significantly higher in the MPS VI group than the control group (leukoCS: 37.9 ± 10.2 and 2.9 ± 1.5 μg/μg protein, respectively, p = 0.005; leukoDS: 0.26 ± 0.2 and 0.0 ± 0.0 μg/μg protein, respectively, p = 0.028) with positive correlations between leukoCS and urine CS and leukoDS and urineDS. In Study 2, leukoCS (32.0 ± 11.8 vs 6.9 ± 3.1 μg/mg protein, p = 0.005) and leukoDS (0.4 ± 0.1 and 0.2 ± 0.1 μg/mg protein, p = 0.020) were significantly higher compared with control subjects. Thus, these results highlight the potential of leukoGAG as a new biomarker representing intracellular GAG accumulation in MPS VI patients and may be valuable for patient management
Optimizing the Hospital Blood Bank Stock in Korea: A Comparative Analysis of the Uniform 5-Day Stock Index and a Novel Blood Stock Index
Get PDFBackground: Maintaining optimal blood inventory levels in hospitals is important to prevent blood shortage and wastage. We aimed to provide an efficient blood inventory management strategy for hospital blood banks nation-wide by comparing the current use of 5-day issuable stock (IS) with Lim’s IS as a novel target IS. Methods: The average and CV of daily usage (DU) were calculated from information entered into Korea’s Blood Management System by 194 participating hospitals in 2019 and 2020. Using these data, Lim’s IS was calculated by determining the simulated annual average blood shortage day nearest to 1 for each blood group in each hospital. The 5-day IS (5IS) was estimated by multiplying the average DU in 2018 by five to count the shortage days in 2019. Results: The average DU (0.3–231.3 units) and corresponding CV (0.33–7.14) in the participating hospitals were inversely proportional (r = –0.699 to –0.695). The hypothetical averages of 5IS and Lim’s IS were 27.0 ± 41.2 and 24.7 ± 20.8, respectively (P = 0.006). The shortage days for 5IS and Lim’s IS were 8.9 ± 22.7 and 1.0 ± 1.9, respectively (P < 0.001). Conclusions: While 5IS was unacceptable for universal application, Lim’s IS remained near one shortage day and is considered more efficient than 5IS. Hospitals should implement indicators that consider DU and its variations. This is the first study to introduce Lim’s IS as an indicator of optimal blood inventory, and the data are expected to provide guidance for effective blood inventory management nationwide, particularly during blood shortages
Risk Factors of Postpartum Depression Among Korean Women: An Analysis Based on the Korean Pregnancy Outcome Study (KPOS)
Get PDFBackground: Postpartum depression (PPD) can negatively affect infant well-being and child development. Although the frequency and risk factors of PPD symptoms might vary depending on the country and culture, there is limited research on these risk factors among Korean women. This study aimed to elucidate the potential risk factors of PPD throughout pregnancy to help improve PPD screening and prevention in Korean women. Methods: The pregnant women at 12 gestational weeks (GW) were enrolled from two obstetric specialized hospitals from March 2013 to November 2017. A questionnaire survey was administered at 12 GW, 24 GW, 36 GW, and 4 weeks postpartum. Depressive symptoms were assessed using the Edinburgh Postnatal Depression Scale, and PPD was defined as a score of > 10. Results: PPD was prevalent in 16.3% (410/2,512) of the participants. Depressive feeling at 12 GW and postpartum factors of stress, relationship with children, depressive feeling, fear, sadness, and neonatal intensive care unit admission of baby were significantly associated with a higher risk of PPD. Meanwhile, high postpartum quality of life and marital satisfaction at postpartum period were significantly associated with a lower risk of PPD. We developed a model for predicting PPD using factors as mentioned above and it had an area under the curve of 0.871. Conclusion: Depressive feeling at 12 GW and postpartum stress, fear, sadness, relationship with children, low quality of life, and low marital satisfaction increased the risk of PPD. A risk model that comprises significant factors can effectively predict PPD and can be helpful for its prevention and appropriate treatment
Steatotic liver disease predicts cardiovascular disease and advanced liver fibrosis: A community-dwelling cohort study with 20-year follow-up
No full textBackground: Steatotic liver disease (SLD) has emerged as new nomenclature to increase awareness and reflect the pathophysiology of the disease better. We investigated the risk of advanced fibrosis and cardiovascular disease (CVD) in SLD using data derived from a Korean prospective cohort. Methods: We defined SLD using the fatty liver index (FLI) and identified advanced fibrosis with the age-adjusted Fibrosis-4 Index. SLD was further subcategorized into metabolic dysfunction-associated SLD (MASLD), MASLD with increased alcohol intake (MetALD), and alcohol-associated liver disease (ALD). Findings: The Ansung–Ansan cohort of the Korean Genome and Epidemiology study, following 9497 participants from 2002 to 2020, included 3642 (38.3%) with MASLD, 424 (4.5%) with MetALD, and 207 (2.1%) with ALD. During the median follow-up of 17.5 years, CVD risk was higher in those with MASLD (hazard ratio [HR], 1.27; 95% confidence interval [CI], 1.12–1.45; P < 0.001), MetALD (HR, 1.88; 95% CI, 1.33–2.65; P < 0.001), and ALD (HR, 1.95; 95% CI, 1.01–3.77; P < 0.001) than in those without SLD, after adjusting for conventional risk factors. Notably, CVD risk was higher in the MetALD than in the MASLD group (P = 0.027). In the MASLD group, the number of cardiometabolic risk factors (CMRFs) correlated positively with CVD risk (HR, 1.34; 95% CI, 1.24–1.45; P < 0.001 for trend). Among the CMRFs, hypertension (HR, 1.94; 95% CI, 1.63–2.31; P < 0.001) was the predominant contributor to CVD. The MASLD (HR, 1.39; 95% CI, 1.25–1.55; P < 0.001), MetALD (HR, 1.75; 95% CI, 1.38–2.23; P < 0.001), and ALD (HR, 2.00; 95% CI, 1.30–3.07; P = 0.002) groups had a higher risk of advanced fibrosis than did the non-SLD group (P < 0.001 for trend). Interpretation: Our study provides new insight into hepatic and cardiovascular outcomes related to SLD subtypes. The risk of CVD increased in the order of no SLD, MASLD, and MetALD. The SLD subcategories, considering CMRFs and alcohol intake, outperformed traditional fatty liver categorizations in predicting CVD risk. The proposed SLD terminology could impact clinical practice, warranting further exploration of the heterogeneity of clinical outcomes among SLD subtypes
The Effect of Sex on the Remimazolam Dosage Required for Successful i-gel Supraglottic Airway Insertion with Remifentanil in Non-Paralyzed Patients: An Up-and-Down Sequential Allocation Trial
Get PDF(1) Background: The physiological and pharmacological variations between men and women are known to influence drug efficacy. The objective of this study was to determine the 50% and 95% effective doses (ED50 and ED95) of remimazolam required for i-gel supraglottic airway (ISA) insertion under remifentanil infusion without neuromuscular blocking agents (NMBAs) in both males and females. (2) Methods: Patients aged 19–65 years, scheduled for general anesthesia using ISA, were enrolled in this study. Patients were divided into two groups based on their sex. The anesthesia process began with a remifentanil infusion targeting an effect-site concentration of 3.0 ng/mL, accompanied by a remimazolam injection. The initial remimazolam dose was 0.25 mg/kg, and it was adjusted with a step size of 0.05 mg/kg based on the outcome of ISA insertion in the preceding patient. (3) Results: The ED50 of remimazolam (mean ± standard error) was 0.28 ± 0.02 mg/kg in the male group and 0.18 ± 0.02 mg/kg in the female group (p < 0.001). Additionally, ED95, which was calculated using the isotonic regression method, was significantly comparable between the male and female groups (male: 0.35 mg/kg, 95% confidence interval [CI] = 0.34–0.35; female: 0.29 mg/kg, 95% CI = 0.25–0.30). (4) Conclusions: This study showed that both the ED50 and the ED95 of remimazolam for successful ISA insertion was higher for men than that for women. Therefore, while using remimazolam alongside remifentanil infusion without NMBAs for ISA insertion, one should consider the patient’s sex for appropriate dosing
중이강 내 발생한 미만성 거대 B 세포 림프종 1예
Get PDFDiffuse large B cell lymphoma (DLBCL), being one of the major subtypes of non-Hodgikin’s lymphoma, is marked by extranodal involvement. In patients with DLBCL, the stomach and gastrointestinal tract are commonly involved, but few cases have reported the involvement with the middle ear cavity and temporal bone. The present study reports a patient diagnosed with DLBCL characterized by middle ear cavity involvement. We also reviewed other similar cases in the literature
Corrigendum to ‘Assessment of deep learning-based auto-contouring on interobserver consistency in target volume and organs-at-risk delineation for breast cancer: Implications for RTQA program in a multi-institutional study’ [The Breast 73 (2024) 103599]
Get PDFThe authors apologize for the oversight in presenting incomplete affiliations for author Jin Sung Kim. Jin Sung Kim is affiliated with the Department of Radiation Oncology, Yonsei University College of Medicine, Seoul, Republic of Korea, and Oncosoft Inc., Seoul, Republic of Korea. The authors would like to apologize for any inconvenience caused
Real-world treatment trajectories of adults with newly diagnosed asthma or COPD
Get PDFBackground There is a lack of knowledge on how patients with asthma or chronic obstructive pulmonary disease (COPD) are globally treated in the real world, especially with regard to the initial pharmacological treatment of newly diagnosed patients and the different treatment trajectories. This knowledge is important to monitor and improve clinical practice. Methods This retrospective cohort study aims to characterise treatments using data from four claims (drug dispensing) and four electronic health record (EHR; drug prescriptions) databases across six countries and three continents, encompassing 1.3 million patients with asthma or COPD. We analysed treatment trajectories at drug class level from first diagnosis and visualised these in sunburst plots. Results In four countries (USA, UK, Spain and the Netherlands), most adults with asthma initiate treatment with short-acting ß2 agonists monotherapy (20.8%-47.4% of first-line treatments). For COPD, the most frequent first-line treatment varies by country. The largest percentages of untreated patients (for asthma and COPD) were found in claims databases (14.5%-33.2% for asthma and 27.0%-52.2% for COPD) from the USA as compared with EHR databases (6.9%-15.2% for asthma and 4.4%-17.5% for COPD) from European countries. The treatment trajectories showed step-up as well as step-down in treatments. Conclusion Real-world data from claims and EHRs indicate that first-line treatments of asthma and COPD vary widely across countries. We found evidence of a stepwise approach in the pharmacological treatment of asthma and COPD, suggesting that treatments may be tailored to patients' needs
Comparative study on the age-related incidence of seborrheic keratosis and verruca plana in patients with verruca plana-like lesions
Get PDFSeborrheic keratosis (SK) is a common skin disease in the elderly. However, in cases where SK presenting as multiple skin-colored or clustered lesions can be easily misdiagnosed as verruca plana (VP), especially in the young population. This retrospective study investigated the prevalence of SK and VP in the lesions that appear clinically similar to VP according to age. We examined the pathology slides of the skin tissue and photographs of patients who were clinically suspected to have VP. A total of 503 patients were included in the study, out of which 174 patients were finally diagnosed with SK (34.6%) and 132 with VP (26.2%). The mean ages of the SK- and VP-diagnosed group were 39.3 and 35.4 years, respectively. SK had a higher prevalence among individuals older than 30 years, and relative frequency of SK should not be ignored in patients with a grouped distribution in their 20 s and 30 s. Therefore, our study suggests that multiple verrucous skin-colored to brownish plaques are also commonly diagnosed as SK in young people as well as VP, and the prevalence of SK and VP may not always depend solely on chronological aging, and the prevalence of SK among young people may be higher than commonly believed stereotypes suggest