Background/Aims: This nationwide cohort study aimed to evaluate (1) whether primary Sjogren’s syndrome (pSS) can contribute to the development of dementia and (2) whether the use of hydroxychloroquine (HCQ) can decrease the incidence of dementia in patients with pSS using the Health Insurance Review and Assessment database. Methods: We established a cohort between 2008 and 2020 of 20,160 patients with pSS without a history of dementia. The control group comprised sex- and age-matched individuals with no history of autoimmune disease or dementia. Cox proportional hazard analyses were performed to identify the association between pSS and dementia development. We also assessed the hazard ratio (HR) of dementia in early users of HCQ (within 180 days of the diagnosis of pSS) compared to non-users, adjusted for age, sex, and comorbidities. Results: The incidence of dementia was 0.68 (95% CI 0.64–0.72) cases per 100 person-years in pSS, and it was 0.58 (0.56–0.60) in the controls. The adjusted HR (aHR) of developing dementia was 1.16 (1.09–1.25) times greater in the pSS group than in the controls. The risk of dementia did not increase in HCQ users (aHR 1.07 [0.94–1.21]), but HCQ non-users had a 1.22 (1.12–1.33) higher risk of developing dementia than the matched controls. The use of HCQ lowered the risk of dementia in comparison with non-users in patients with pSS (aHR 0.82 [0.71–0.94]). Conclusions Our results suggest that pSS is associated with an increased risk of dementia. HCQ may prevent dementia in patients with pSS.

Background/Aims: This nationwide cohort study aimed to evaluate (1) whether primary Sjogren’s syndrome (pSS) can contribute to the development of dementia and (2) whether the use of hydroxychloroquine (HCQ) can decrease the incidence of dementia in patients with pSS using the Health Insurance Review and Assessment database. Methods: We established a cohort between 2008 and 2020 of 20,160 patients with pSS without a history of dementia. The control group comprised sex- and age-matched individuals with no history of autoimmune disease or dementia. Cox proportional hazard analyses were performed to identify the association between pSS and dementia development. We also assessed the hazard ratio (HR) of dementia in early users of HCQ (within 180 days of the diagnosis of pSS) compared to non-users, adjusted for age, sex, and comorbidities. Results: The incidence of dementia was 0.68 (95% CI 0.64–0.72) cases per 100 person-years in pSS, and it was 0.58 (0.56–0.60) in the controls. The adjusted HR (aHR) of developing dementia was 1.16 (1.09–1.25) times greater in the pSS group than in the controls. The risk of dementia did not increase in HCQ users (aHR 1.07 [0.94–1.21]), but HCQ non-users had a 1.22 (1.12–1.33) higher risk of developing dementia than the matched controls. The use of HCQ lowered the risk of dementia in comparison with non-users in patients with pSS (aHR 0.82 [0.71–0.94]). Conclusions Our results suggest that pSS is associated with an increased risk of dementia. HCQ may prevent dementia in patients with pSS.

Background/Aims: This nationwide cohort study aimed to evaluate (1) whether primary Sjogren’s syndrome (pSS) can contribute to the development of dementia and (2) whether the use of hydroxychloroquine (HCQ) can decrease the incidence of dementia in patients with pSS using the Health Insurance Review and Assessment database. Methods: We established a cohort between 2008 and 2020 of 20,160 patients with pSS without a history of dementia. The control group comprised sex- and age-matched individuals with no history of autoimmune disease or dementia. Cox proportional hazard analyses were performed to identify the association between pSS and dementia development. We also assessed the hazard ratio (HR) of dementia in early users of HCQ (within 180 days of the diagnosis of pSS) compared to non-users, adjusted for age, sex, and comorbidities. Results: The incidence of dementia was 0.68 (95% CI 0.64–0.72) cases per 100 person-years in pSS, and it was 0.58 (0.56–0.60) in the controls. The adjusted HR (aHR) of developing dementia was 1.16 (1.09–1.25) times greater in the pSS group than in the controls. The risk of dementia did not increase in HCQ users (aHR 1.07 [0.94–1.21]), but HCQ non-users had a 1.22 (1.12–1.33) higher risk of developing dementia than the matched controls. The use of HCQ lowered the risk of dementia in comparison with non-users in patients with pSS (aHR 0.82 [0.71–0.94]). Conclusions Our results suggest that pSS is associated with an increased risk of dementia. HCQ may prevent dementia in patients with pSS.

Background/Aims: This nationwide cohort study aimed to evaluate (1) whether primary Sjogren’s syndrome (pSS) can contribute to the development of dementia and (2) whether the use of hydroxychloroquine (HCQ) can decrease the incidence of dementia in patients with pSS using the Health Insurance Review and Assessment database. Methods: We established a cohort between 2008 and 2020 of 20,160 patients with pSS without a history of dementia. The control group comprised sex- and age-matched individuals with no history of autoimmune disease or dementia. Cox proportional hazard analyses were performed to identify the association between pSS and dementia development. We also assessed the hazard ratio (HR) of dementia in early users of HCQ (within 180 days of the diagnosis of pSS) compared to non-users, adjusted for age, sex, and comorbidities. Results: The incidence of dementia was 0.68 (95% CI 0.64–0.72) cases per 100 person-years in pSS, and it was 0.58 (0.56–0.60) in the controls. The adjusted HR (aHR) of developing dementia was 1.16 (1.09–1.25) times greater in the pSS group than in the controls. The risk of dementia did not increase in HCQ users (aHR 1.07 [0.94–1.21]), but HCQ non-users had a 1.22 (1.12–1.33) higher risk of developing dementia than the matched controls. The use of HCQ lowered the risk of dementia in comparison with non-users in patients with pSS (aHR 0.82 [0.71–0.94]). Conclusions Our results suggest that pSS is associated with an increased risk of dementia. HCQ may prevent dementia in patients with pSS.

Background/Aims: This nationwide cohort study aimed to evaluate (1) whether primary Sjogren’s syndrome (pSS) can contribute to the development of dementia and (2) whether the use of hydroxychloroquine (HCQ) can decrease the incidence of dementia in patients with pSS using the Health Insurance Review and Assessment database. Methods: We established a cohort between 2008 and 2020 of 20,160 patients with pSS without a history of dementia. The control group comprised sex- and age-matched individuals with no history of autoimmune disease or dementia. Cox proportional hazard analyses were performed to identify the association between pSS and dementia development. We also assessed the hazard ratio (HR) of dementia in early users of HCQ (within 180 days of the diagnosis of pSS) compared to non-users, adjusted for age, sex, and comorbidities. Results: The incidence of dementia was 0.68 (95% CI 0.64–0.72) cases per 100 person-years in pSS, and it was 0.58 (0.56–0.60) in the controls. The adjusted HR (aHR) of developing dementia was 1.16 (1.09–1.25) times greater in the pSS group than in the controls. The risk of dementia did not increase in HCQ users (aHR 1.07 [0.94–1.21]), but HCQ non-users had a 1.22 (1.12–1.33) higher risk of developing dementia than the matched controls. The use of HCQ lowered the risk of dementia in comparison with non-users in patients with pSS (aHR 0.82 [0.71–0.94]). Conclusions Our results suggest that pSS is associated with an increased risk of dementia. HCQ may prevent dementia in patients with pSS.

Background/Aims: Metabolic dysfunction-associated steatotic liver disease (MASLD) is characterized by fat accumulation in the liver. MASLD encompasses both steatosis and MASH. Since MASH can lead to cirrhosis and liver cancer, steatosis and MASH must be distinguished during patient treatment. Here, we investigate the genomes, epigenomes, and transcriptomes of MASLD patients to identify signature gene set for more accurate tracking of MASLD progression. Methods: Biopsy-tissue and blood samples from patients with 134 MASLD, comprising 60 steatosis and 74 MASH patients were performed omics analysis. SVM learning algorithm were used to calculate most predictive features. Linear regression was applied to find signature gene set that distinguish the stage of MASLD and to validate their application into independent cohort of MASLD. Results: After performing WGS, WES, WGBS, and total RNA-seq on 134 biopsy samples from confirmed MASLD patients, we provided 1,955 MASLD-associated features, out of 3,176 somatic variant callings, 58 DMRs, and 1,393 DEGs that track MASLD progression. Then, we used a SVM learning algorithm to analyze the data and select the most predictive features. Using linear regression, we identified a signature gene set capable of differentiating the various stages of MASLD and verified it in different independent cohorts of MASLD and a liver cancer cohort. Conclusions We identified a signature gene set (i.e., CAPG, HYAL3, WIPI1, TREM2, SPP1, and RNASE6) with strong potential as a panel of diagnostic genes of MASLD-associated disease.

Background: The aims of this study are to review data on 4-months age National Health Screening Program for Infants and Children (NHSPIC) using a National Health Insurance Service (NHIS) database, and to analyze the newborn hearing screening (NHS) results and related characteristics of the 4-months NHSPIC for 7 years in South Korea. Methods: We analyzed a NHIS database of infants who had participated in the 4-month age NHSPIC from 2010 to 2016. According to the results of hearing questionnaires and physical examination, we analyzed the outcomes of NHS and related infantile and socioeconomic factors. Results: Among 3,128,924 of total eligible infants in Korea between the year 2010 and 2016, 69.2% (2,164,621 infants) conducted 4-months age NHSPIC, and 94.4% (2,042,577 infants) of which performed hearing questionnaires regarding NHS. Among the total hearing examinees, premature infants accounted for 3.6%, infants who were hospitalized in the neonatal intensive care unit (NICU) for more than 5 days accounted for 5.6%, and infants with head and neck abnormalities were 0.6%. The NHS performing rate was 79.1% for total hearing examinees in 2010, but gradually increased to 88.9% in 2016. The NHS performing rate in 2016 was 93.4% for premature infants, 91.7% for NICU hospitalized babies. The mean referral rate was 0.6% for total hearing examinees, 1.4% for premature infants, and 2.3% for NICU hospitalized babies. When we analyzed the NHS performing rate and the referral rate according to the household income level, the NHS performing rate of infants in Medical Aid programs was the lowest as 65.6%, and the NHS performing rates in other five levels of NHIS was higher ranging between 85.1% to 86.0%. The referral rate of infants in the Medical Aid program (3.8%) was significantly higher than those of infants in other classes (1.10–1.25%). Conclusion The estimated overall NHS performing rate in Korea gradually increased and was 88.9% in 2016. The overall referral rate was low as 0.6%, and it was significantly different depending on the infant’s health condition and household income levels. We assume that our finding would help to establish policies managing hearing impaired children, and to develop the customized hearing care service programs considering the household economic levels.

OBJECTIVE: Cerebral hemodynamic and metabolic changes may occur during the rewarming phase of targeted temperature management in post cardiac arrest patients. Yet, studies on different rewarming rates and patient outcomes are limited. This study aimed to investigate post cardiac arrest patients who were rewarmed with different rewarming rates after 24 hours of hypothermia and the association of these rates to the neurologic outcomes.METHODS: This study retrospectively investigated post cardiac arrest patients treated with targeted temperature management and rewarmed with rewarming rates of 0.15°C/hr and 0.25°C/hr. The association of the rewarming rate with poor neurologic outcomes (cerebral performance category score, 3 to 5) was investigated.RESULTS: A total of 71 patients were analyzed (0.15°C/hr, n=36; 0.25°C/hr, n=35). In the comparison between 0.15°C/hr and 0.25°C/hr, the poor neurologic outcome did not significantly differ (24 [66.7%] vs. 25 [71.4%], respectively; P=0.66). In the multivariate analysis, the rewarming rate of 0.15°C/hr was not associated with the 1-month neurologic outcome improvement (odds ratio, 0.54; 95% confidence interval, 0.16 to 1.69; P=0.28).CONCLUSION: The rewarming rates of 0.15°C/hr and 0.25°C/hr were not associated with the neurologic outcome difference in post cardiac arrest patients.
Critical Care Outcomes ; Heart Arrest ; Hemodynamics ; Humans ; Hypothermia ; Multivariate Analysis ; Pilot Projects ; Retrospective Studies ; Rewarming

Point mutations in the human cardiac homeobox gene NKX2.5 are associated with familial atrial septal defect (ASD), atrioventricular (AV) conduction disturbance, as well as sudden cardiac death. To date, more than 60 NKX2.5 mutations have been documented, but there are no reports in Korea. We are reporting the first Korean family with ASD and AV block associated with a novel mutation in the NKX2.5 coding region. A 9-year-old boy presented with a slow and irregular pulse, and was diagnosed with secundum ASD and first degree AV block. The boy's father, who had a history of ASD correction surgery, presented with second degree AV block and atrial fibrillation. The boy's brother was also found to have secundum ASD and first degree AV block. There were two sudden deaths in the family. Genetic testing revealed a novel mutation of NKX2.5 in all affected members of the family.
Atrial Fibrillation ; Atrioventricular Block* ; Child ; Clinical Coding ; Death, Sudden ; Death, Sudden, Cardiac ; Fathers ; Genes, Homeobox ; Genetic Testing ; Heart Septal Defects, Atrial* ; Humans ; Korea ; Male ; Point Mutation ; Siblings

BACKGROUND: Hepatitis B virus (HBV) infection leads to hepatic and extrahepatic manifestations including chronic kidney disease (CKD). However, the association between HBV and CKD is not clear. This study investigated the association between chronic HBV infection and CKD in a nationwide multicenter study. METHODS: A total of 265,086 subjects who underwent health-check examinations in 33 hospitals from January 2015 to December 2015 were enrolled. HBV surface antigen (HBsAg) positive cases (n = 10,048), and age- and gender-matched HBsAg negative controls (n = 40,192) were identified. CKD was defined as a glomerular filtration rate (GFR) < 60 mL/min/1.73 m² or proteinuria as at least grade 2+ of urine protein. RESULTS: HBsAg positive cases showed a significantly higher prevalence of GFR < 60 mL/min/1.73 m² (3.3%), and proteinuria (18.9%) than that of the controls (2.6%, P < 0.001, and 14.1%, P < 0.001, respectively). In the multivariate analysis, HBsAg positivity was an independent factor associated with GFR < 60 mL/min/1.73 m² along with age, blood levels of albumin, bilirubin, anemia, and hemoglobin A1c (HbA1c). Likewise, HBsAg positivity was an independent factor for proteinuria along with age, male, blood levels of bilirubin, protein, albumin, and HbA1c. A subgroup analysis showed that HBsAg positive men but not women had a significantly increased risk for GFR < 60 mL/min/1.73 m². CONCLUSION: Chronic HBV infection was significantly associated with a GFR < 60 mL/min/1.73 m² and proteinuria (≥ 2+). Therefore, clinical concern about CKD in chronic HBV infected patients, especially in male, is warranted.
Anemia ; Antigens, Surface ; Bilirubin ; Case-Control Studies* ; Female ; Glomerular Filtration Rate ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Male ; Multivariate Analysis ; Prevalence ; Proteinuria ; Renal Insufficiency, Chronic*