Objectives: . FDXR encodes mitochondrial ferredoxin reductase, which is associated with auditory neuropathy spectrum disorder (ANSD) and optic atrophy. To date, only two studies have described FDXR-related hearing loss. The auditory rehabilitation outcomes of this disease entity have not been investigated, and the pathophysiological mechanisms remain incompletely understood. Here we report a hearing-impaired individual with co-segregation of the FDXR variant and post-synaptic type ANSD, who underwent cochlear implantation (CI) with favorable outcomes. We suggest a possible pathophysiological mechanism of adult-onset ANSD involving mitochondrial dysfunction. Methods: . A 35-year-old woman was ascertained to have ANSD. Exome sequencing identified the genetic cause of hearing loss, and a functional study measuring mitochondrial activity was performed to provide molecular evidence of pathophysiology. Expression of FDXR in the mouse cochlea was evaluated by immunohistochemistry. Intraoperatively, electrically evoked compound action potential (ECAP) responses were measured, and the mapping parameters were adjusted accordingly. Audiological outcomes were monitored for over 1 year. Results: . In lymphoblastoid cell lines (LCLs) carrying a novel FDXR variant, decreased ATP levels, reduced mitochondrial membrane potential, and increased reactive oxygen species levels were observed compared to control LCLs. These dysfunctions were restored by administering mitochondria isolated from umbilical cord mesenchymal stem cells, confirming the pathogenic potential of this variant via mitochondrial dysfunction. Partial ECAP responses during CI and FDXR expression in the mouse cochlea indicate that FDXR-related ANSD is post-synaptic. As a result of increasing the pulse width during mapping, the patient’s CI outcomes showed significant improvement over 1-year post-CI. Conclusion . A novel FDXR variant associated with mitochondrial dysfunction and post-synaptic ANSD was first identified in a Korean individual. Additionally, 1-year post-CI outcomes were reported for the first time in the literature. Excellent audiologic results were obtained, and our results reiterate the correlation between genotype and CI outcomes in ANSD.

The correct identification of filamentous fungi is challenging. We evaluated the performance of the VITEK MS v3.0 system (bioMérieux, Marcy-l’Étoile, France) for the identification of a wide spectrum of clinically relevant filamentous fungi using a Korean collection. Strains that were added to the upgraded v3.2 database were additionally identified by the VITEK MS v3.2 system. Of the 105 tested isolates, including 37 Aspergillus (nine species), 41 dermatophytes (seven species), and 27 other molds (17 species), 43 (41.0%) showed “no identification” or “multiple species identification” results at the initial VITEK MS testing; these isolates were retested using the same method. Compared with sequence-based identification, the correct identification rate using VITEK MS for Aspergillus, dermatophytes, other molds, and total mold isolates was 67.6%, 56.1%, 48.1%, and 58.1% at the initial testing and 94.6%, 78.0%, 55.6%, and 78.1% with retesting, respectively. Following retesting, 19 (18.1%) and two (1.9%) isolates showed “no identification” and “misidentification” results, respectively. VITEK MS reliably identified various filamentous fungi recovered in Korea, with a very low rate of misidentification

Allergic rhinitis (AR) is one of the most common allergic diseases characterized by stuffy nose, rhinorrhea, sneezing, and itching. Researchers have indicated an increase in the prevalence of AR and younger-age onset during the last few decades. The increasing burden of AR has caused many researchers to investigate time trends of the prevalence of AR and to identify its risk factors. The most commonly used epidemiological studies are cross-sectional ones such as the International Study of Asthma and Allergies in Childhood study and big data from National Health Insurance Service or National Health and Nutrition Examination Survey. However, these studies have many limitations including recall bias, selection bias, and deficit of objective evaluation. Furthermore, crosssectional studies cannot reflect new risk factors associated with the development of AR. New epidemiological studies will be needed to cover genetic factors, environmental changes, microbiomes, and lifestyles that are known to be risk factors for AR. Further studies will be needed to determine the prevalence, natural history, and risk factors of AR in order to advance our understanding of the pathophysiology, prevention, and management of comorbidities of AR.

BACKGROUND: Resting heart rate (RHR) is considered as a strong predictor of total mortality and hospitalization due to heart failure in hypertension patients. Bisoprolol fumarate, a second-generation beta-adrenoreceptor blockers (β-blocker) is commonly prescribed drug to manage hypertension. The present study was to retrospectively evaluate changes in the average RHR and its association with cardiovascular outcomes in bisoprolol-treated coronary artery disease (CAD) patients from the CAD treated with bisoprolol (BISO-CAD) study who had comorbid hypertension. METHODS: We performed ad-hoc analysis for hypertension sub-group of the BISO-CAD study (n = 866), which was a phase IV, multination, multi-center, single-arm, observational study carried out from October 2011 to July 2015 across China, South Korea, and Vietnam. Multivariate regression analysis was used to identify factors associated with incidence of composite cardiac clinical outcome (CCCO), the results were presented as adjusted odds ratio (OR) along with 95% confidence interval (CI) and adjusted P value. RESULTS: A total of 681 patients (mean age: 64.77 ± 10.33 years) with hypertension from BISO-CAD study were included in the analysis. Bisoprolol improved CCCOs in CAD patients with comorbid hypertension, with RHR <65 and <70 beats/min compared with RHR ≥65 and ≥75 beats/min, respectively, in the efficacy analysis (EA) set. In addition, it lowered RHR in both intent-to-treat (ITT) and EA groups after 6, 12, and 18 months of treatment. Further, RHR 70 to 74 beats/min resulted in significantly higher risk of CCCOs EA set of patients (adjusted OR: 4.34; 95% CI: 1.19-15.89; P = 0.03). Also, events of hospitalization due to acute coronary syndrome were higher when RHR 69 to 74 beats/min compared to RHR <69 beats/min in ITT patients. CONCLUSION Bisoprolol can effectively reduce RHR in Asian CAD patients with comorbid hypertension and hence, improve CCCO without affecting their blood pressure.

Background: The current Korean Blood Inventory Monitoring System (BMS) has several drawbacks. It does not provide real-time data and only monitors 211 of the more than 2500 institutes that performed blood transfusions.This survey study investigated the status of BMS use and the preparation status of the data input system of medical institutions to help in the revision of BMS in preparation for the full surveillance of BMS. Methods: A survey questionnaire was given to 200 hospitals participating in the current BMS, along with another set of questionnaires to 150 non-participating hospitals. The questionnaire consisted of the method of data registry to BMS, the current status of electronic medical records (EMR) and order communication system (OCS), perception, and readiness of adaptation of an open application programming interface system (API). Results: Two hundred and one BMS participating hospitals responded to the survey. Approximately 75% entered data with a comma-separated value (CSV) or Excel spreadsheet (xls) files, and approximately half had an in-house developed EMR and OCS. The majority showed enthusiasm for the introduction to an open API. Among the non-participating hospitals, 138 responded. Approximately 70% counted the blood inventory daily, but approximately half did not use electronic methods for this process. The response to the introduction to an open API was positive, but the readiness for a prompt introduction was low at 15.9%. Conclusion These results will help revise the current BMS. On the other hand, full surveillance of BMS is anticipated to be hindered by the ready state of each medical institute. Moreover, the encouragement of participation would require supportive government administrative measures.

Background: The current Korean Blood Inventory Monitoring System (BMS) has several drawbacks. It does not provide real-time data and only monitors 211 of the more than 2500 institutes that performed blood transfusions.This survey study investigated the status of BMS use and the preparation status of the data input system of medical institutions to help in the revision of BMS in preparation for the full surveillance of BMS. Methods: A survey questionnaire was given to 200 hospitals participating in the current BMS, along with another set of questionnaires to 150 non-participating hospitals. The questionnaire consisted of the method of data registry to BMS, the current status of electronic medical records (EMR) and order communication system (OCS), perception, and readiness of adaptation of an open application programming interface system (API). Results: Two hundred and one BMS participating hospitals responded to the survey. Approximately 75% entered data with a comma-separated value (CSV) or Excel spreadsheet (xls) files, and approximately half had an in-house developed EMR and OCS. The majority showed enthusiasm for the introduction to an open API. Among the non-participating hospitals, 138 responded. Approximately 70% counted the blood inventory daily, but approximately half did not use electronic methods for this process. The response to the introduction to an open API was positive, but the readiness for a prompt introduction was low at 15.9%. Conclusion These results will help revise the current BMS. On the other hand, full surveillance of BMS is anticipated to be hindered by the ready state of each medical institute. Moreover, the encouragement of participation would require supportive government administrative measures.

No abstract available.
Asia* ; Blood Transfusion* ; Humans

BACKGROUND AND OBJECTIVES: There are no data comparing clinical outcomes of complex percutaneous coronary intervention (PCI) between biodegradable polymer-biolimus-eluting stents (BP-BES) and durable polymer-everolimus-eluting stents (DP-EES). We sought to evaluate the safety and efficacy of BP-BES compared with DP-EES in patients undergoing complex PCI. METHODS: Patients enrolled in the SMART-DESK registry were stratified into 2 categories based on the complexity of PCI. Complex PCI was defined as having at least one of the following features: unprotected left main lesion, ≥2 lesions treated, total stent length >40 mm, minimal stent diameter ≤2.5 mm, or bifurcation as target lesion. The primary outcome was target lesion failure (TLF), defined as a composite of cardiac death, target vessel-related myocardial infarction (TV-MI), or target lesion revascularization (TLR) at 2 years of follow-up. RESULTS: Of 1,999 patients, 1,145 (57.3%) underwent complex PCI: 521 patients were treated with BP-BES and 624 with DP-EES. In propensity-score matching analysis (481 pairs), the risks of TLF (3.8% vs. 5.2%, adjusted hazard ratio [HR], 0.578; 95% confidence interval [CI], 0.246–1.359; p=0.209), cardiac death (2.5% vs. 2.5%, adjusted HR, 0.787; 95% CI, 0.244–2.539; p=0.689), TV-MI (0.5% vs. 0.4%, adjusted HR, 1.128; 95% CI, 0.157–8.093; p=0.905), and TLR (1.1% vs. 2.9%, adjusted HR, 0.390; 95% CI, 0.139–1.095; p=0.074) did not differ between 2 stent groups after complex PCI. CONCLUSIONS: Clinical outcomes of BP-BES were comparable to those of DP-EES at 2 years after complex PCI. Our data suggest that use of BP-BES is acceptable, even for complex PCI.
Coronary Artery Disease ; Death ; Drug-Eluting Stents ; Follow-Up Studies ; Humans ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Stents

No abstract available.
Escherichia ; Hemolytic-Uremic Syndrome

No abstract available.
Antibodies ; Hemodilution ; Humans ; Korea