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Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by uncontrolled terminal complement activation. Eculizumab, a monoclonal antibody C5 inhibitor was introduced in Korea in 2009 and has been the standard treatment option for PNH., Methods: This study assessed the long-term efficacy/safety of eculizumab in PNH using real-world data from the Korean Health Insurance Review and Assessment Service. Eighty patients who initiated eculizumab from 2009-2020 were enrolled., Results: At eculizumab initiation, the median age was 51.5 years, lactate dehydrogenase (LDH) 6.8 × upper limit of normal, and granulocyte clone size 93.0%. All patients had at least one PNH-related complication before eculizumab initiation, including renal failure (n = 36), smooth muscle spasm (n = 24), thromboembolism (n = 20), and pulmonary hypertension (n = 15). The median (range) duration of eculizumab treatment was 52.7 (1.0, 127.3) months (338.6 total treated patient-years). Despite high disease activity in the study population before treatment initiation, overall survival was 96.2% and LDH levels were stabilized in most patients during treatment. PNH-related complications at treatment initiation were resolved in 44.4% of patients with renal failure, 95.8% with smooth muscle spasm, 70.0% with thromboembolism, and 26.7% with pulmonary hypertension. Extravascular hemolysis occurred in 28.8% of patients (n = 23; 0.09 per patient-year) and breakthrough hemolysis in 18.8% (n = 15; 0.06 per patient-year). No treatment discontinuation cases related to eculizumab were observed., Conclusion: These data provided evidence for the long-term efficacy and safety of eculizumab in Korean PNH patients with high disease burdens., Competing Interests: Jin Seok Kim has received honoraria and consulting fees from Handok Inc. Jun Ho Jang has received research support from Alexion, AstraZeneca Rare Disease. Sung-Soo Yoon has received research funding from Chong Kun Dang Pharm, JW Pharmaceutical, Kyowa Kirin, Roche-Genentech, and Yuhan Pharmaceutical; has served on the advisory board for Amgen, Antengene, Astellas, Celgene, Janssen, Merck, Novartis, and Takeda; and has received consulting fees from Ticaros Therapeutics. BokJin Hyun, HyunSun Nam, and Eunhye Kim are employees of Handok Inc. Min Joo Kwak and Yong Kyun Won were an employee of Handok Inc. at the time of study. Jong Wook Lee has served on advisory boards and has received honoraria and research grants from Alexion, AstraZeneca Rare Disease. Deog-Yeon Jo, Seo-Yeon Ahn, Je-Hwan Lee, Sung-Hyun Kim, Chul Won Choi, Ho-Jin Shin, Min-Kyoung Kim, Jae Hoon Lee, Yeung-Chul Mun, and Jee Hyun Kong have no potential conflicts of interest to disclose., (© 2023 The Korean Academy of Medical Sciences.)

The aim of this comparative study involving pre- and post-tests was to analyze the effectiveness of patient safety educational materials developed for the Comprehensive Plans for Patient Safety in Korea (2018-2022), and to suggest how to improve patient safety literacy. A face-to-face survey interview comprising items related to general information and patient safety literacy was completed by 217 patients and their families who visited three general hospitals in Seoul and one general hospital in Gyeonggi-do for treatment between 25 October and 15 November 2019. In the interview, the patients were asked questions about whether the patient safety educational materials were "easy to understand," provided "help in safe hospitalization," and enabled patients to practice patient safety independently ("do it yourself"). The literacy of the patient safety educational materials was analyzed using a paired t -test with a p value of 0.05. The comparison between patient safety literacy on pre- and post-tests revealed that among all participants, there were significant differences in "easy to understand," "help in safe hospitalization," and "do it yourself" scores. To improve patient safety literacy, patient education materials need to optimize communication by improving patients' knowledge, skills, and attitudes for maintaining and promoting healthy living.

Background: This study aims to increase understanding of how patient and family education affects the prevention of medical errors, thereby providing basic data for developing educational contents., Methods: This descriptive study surveyed patients, families, and Patient Safety Officers to investigate the relationship between educational contents and medical error prevention. The Chi-square test and ANOVA were used to derive the results of this study. The educational contents used in this study consisted of health information (1. current medicines, 2. allergies, 3. health history, 4. previous treatments/tests and complications associated with them) and Speak Up (1. handwashing, 2. patient identification, 3. asking about medical conditions, 4. asking about test results, 5. asking about behaviour and changes in lifestyle, 6. asking about the care plan, 7. asking about medicines, and 8. asking about medicine interactions)., Results: In this study, the first criterion for choosing a hospital for treatment in Korea was 'Hospital with a famous doctor' (58.6% patient; 57.7% families). Of the patients and their families surveyed, 82.2% responded that hospitals in Korea were safe. The most common education in hospitals is 'Describe your medical condition', given to 69.0% of patients, and 'Hospitalisation orientation', given to 63.4% of families. The most important factors in preventing patient safety events were statistically significant differences among patients, family members, and Patient Safety Officers (p = 0.001). Patients and families had the highest 'Patient and family participation' (31.0% of patients; 39.4% of families) and Patient Safety Officers had the highest 'Patient safety culture' (47.8%)., Conclusions: Participants thought that educational contents developed through this study could prevent medical errors. The results of this study are expected to provide basic data for national patient safety campaigns and standardised educational content development to prevent medical errors.

Anti-GQ1b antibody syndrome encompasses Miller Fisher syndrome and its related disorders. We retrospectively identified 11 pediatric patients (5.4-18 years old) with anti-GQ1b antibody syndrome. Diagnoses of patients included acute ophthalmoparesis (n = 6), classical Miller Fisher syndrome (n = 2), Miller Fisher syndrome/Guillain-Barré syndrome (n = 1), acute ataxic neuropathy (n = 1), and pharyngeal-cervical-brachial weakness (n = 1). Nine patients (81.8%) fully recovered. Maturational change in GQ1b antigen expression and the accessibility of anti-GQ1b antibodies might be the cause of the difference of clinical manifestations in children with anti-GQ1b antibody syndrome., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Purpose: The purpose of this study was to evaluate the effects of a social support program on the stress, anxiety, guilt, and life satisfaction levels among Korean mothers of children with precocious puberty., Design and Methods: A non-equivalent control group and a non-synchronized design were used in this quasi-experiment study. Thirty-four mothers (divided into equal-sized experimental and control groups), 32-47 years of age, with children aged 6-11 years old, were recruited from a pediatric outpatient department at a national university hospital. The experimental group was involved in four sessions of a 4-week social support program. Descriptive statistics were used for demographic characteristics. Chi-square tests and t-tests were used to evaluate group differences., Results: The program significantly reduced mothers' stress and guilt and significantly increased their life satisfaction. No significant differences in changes in anxiety were reported between the groups., Conclusions: This study confirms that the social support program was a useful nursing intervention for Korean mothers of children with precocious puberty, which can be extensively applied to help mothers in similar situations., Practice Implications: The preliminary study findings may inform healthcare professionals to develop effective interventions to promote psychosocial well-being of mothers of children with precocious puberty through strengthening their social support and to further improve the quality of life of children with precocious puberty and their families., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Mucopolysaccharidosis I (MPS I) is an autosomal recessive lysosomal storage disorder caused by a lack of the lysosomal enzyme α-L-iduronidase (IDUA). To date, more than 200 IDUA mutations have been reported. However, only a few types of mutations are recurrent and the frequencies of mutations differ from country to country., Methods: We performed the IDUA mutation analysis in seven patients who were biochemically diagnosed with MPS I in the Department of Pediatrics, Samsung Medical Center, from 2009 to 2014. Here, we describe the results of the IDUA mutation analysis in seven patients with MPS I and the IDUA mutational spectrum in Korean patients with MPS I, including previous data., Results: The IDUA mutations were found in all 14 alleles of 7 patients, and 11 kinds of IDUA mutations were identified. The detected mutations were five missense mutations (p.A79V, p.L346R, p.T388K, p.P496R, and p.C577Y), two nonsense mutations (p.Y618* and p.R628*), two deletions (c.683delC and c.1591delC), one splice site mutation (c.972+1G>A), and one duplication (c.613_617dup). Among these, p.T388K, p.C577Y, c.683delC, c.1591delC, and c.972+1G>A were novel mutations that have not previously been reported. After taking everything into consideration, including IDUA mutation analysis of the previously reported 10 unrelated Korean patients with MPS I, p.L346R and c.704ins5 were most commonly found in Korean patients with MPS I. However, p.W402* and p.Q70*, which have mainly been found in Caucasian patients, were not found., Conclusion: As a result, p.L346R and c.704ins5, which were the most common in Korea, which is geographically situated midway between China and Japan, were some of the most common mutations in China and Japan, respectively. These results are especially worthy of notice.

Background: Intestinal microbiota play an important role in maintaining the homeostasis of the host immune system. To analyze the alteration of the intestinal microbial community structure in Korean Crohn's disease (CD) patients, we performed a comparative metagenomic analysis between healthy people and CD patients using fecal samples and mucosal tissues of ileocecal valve., Methods: 16S rRNA genes from fecal samples or mucosal tissues of 35 CD patients and 15 healthy controls (HC) were amplified using a universal primer set and sequenced with GS FLX Titanium. The microbial composition and diversity of each sample were analyzed with the mothur pipeline, and the association between microbial community and clinical characteristics of the patients were investigated., Results: The contribution of bacterial groups to the intestinal microbial composition differed between CD and HC, especially in fecal samples. Global structure and individual bacterial abundance of intestinal microbial community were different between feces and ileocecal tissues in HC. In CD patients with active stage, relative abundances of Gammaproteobacteria and Fusobacteria were higher in both fecal and mucosal tissue samples. Moreover, the intestinal microbial community structure was altered by anti-tumor necrosis factor (anti-TNF) treatment., Conclusions: Our 16S rRNA sequence data demonstrate intestinal dysbiosis at the community level in Korean CD patients, which is similar to alterations of the intestinal microbial community seen in the western counterparts. Clinical disease activity and anti-TNF treatment might affect the intestinal microbial community structure in CD patients.

The paper analyzes the healthcare-seeking behavior of South Korean immigrants in Toronto, Canada, and how transnationalism shapes post-migration health and health-management strategies. Built upon largely separate research areas in ethnicity and health, health geography, and transnationalism, the paper conceptualizes immigrant health as influenced by individual characteristics, the migration and resettlement experience, and place effects at both a local and a transnational scale. A mixed-method approach is used to capture insights into health status and experiences in accessing local and transnational healthcare among South Korean immigrants - a fast growing visible minority group in Canada. Statistical analysis of data from the Canadian Community Health Survey discloses patterns and trends in health and healthcare use among the Korean Canadian, overall foreign-born, and native-born populations. Focus groups reveal in-depth information on the decline of Korean immigrants' health status and the array of sociocultural, economic and geographic barriers in accessing healthcare in Canada, which gave rise to their transnational use of health resources in the home country. The transnational strategies included traveling to South Korea for medical examinations or treatment, importing medications from South Korea to Canada, and consulting health resources in South Korea by phone or email. The results provide timely knowledge on how a recent immigrant group adapts to Canada in the domain of health and adds a transnational perspective to the literature on ethnicity and health., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

An agar-degrading bacterium, designated as strain G7(T), was isolated from a coastal seawater sample from Gaya Island (Gayado in Korean), Republic of Korea. The isolated strain G7(T) is gram-negative, rod shaped, aerobic, non-motile, and non-pigmented. A similarity search based on its 16S rRNA gene sequence revealed that it shares 95.5%, 90.6%, and 90.0% similarity with the 16S rRNA gene sequences of Catenovulum agarivorans YM01(T), Algicola sagamiensis, and Bowmanella pacifica W3-3A(T), respectively. Phylogenetic analyses demonstrated that strain G7(T) formed a distinct monophyletic clade closely related to species of the family Alteromonadaceae in the Alteromonas-like Gammaproteobacteria. The G+C content of strain G7(T) was 41.12 mol%. The DNA-DNA hybridization value between strain G7(T) and the phylogenetically closest strain YM01(T) was 19.63%. The genomes of G7(T) and YM01(T) had an average ANIb value of 70.00%. The predominant isoprenoid quinone of this particular strain was ubiquinone-8, whereas that of C. agarivorans YM01(T) was menaquinone-7. The major fatty acids of strain G7(T) were Iso-C15:0(41.47%), Anteiso-C15:0(22.99%), and C16:1ω7c/iso-C15:02-OH (8.85%), which were quite different from those of YM01(T). Comparison of the phenotypic characteristics related to carbon utilization, enzyme production, and susceptibility to antibiotics also demonstrated that strain G7(T) is distinct from C. agarivorans YM01T. Based on its phenotypic, chemotaxonomic, and phylogenetic distinctiveness, strain G7(T) was considered a novel genus and species in the Gammaproteobacteria, for which the name Gayadomonas joobiniege gen. nov. sp. nov. (ATCC BAA-2321 = DSM25250(T) = KCTC23721(T)) is proposed.

Background and Aim:  The widely accepted range of upper limits of normal (ULN) alanine aminotransferase (ALT) levels (ULN < 40 U/L) was recently challenged by several reports. Both ALT and aspartate aminotransferase (AST) are commonly used as surrogate markers of liver disease, but almost all studies of aminotransferase activity were conducted on ALT. We investigated not only ULN of ALT but also AST activity and to identify factors modulating them in healthy Korean., Methods: A cross-sectional study of 411,240 registered blood donors in all nationwide blood banks belonging to the Korean Red Cross were conducted. ULN of ALT and AST was evaluated adjusting their age according to the national population census database. "Decision tree model" was used to identify the affecting factors of ALT and AST and optimal cut-off points of affecting factors., Results:  "ULN of ALT" was 34 U/L in men and 24 U/L in women and "ULN of AST" was 32 U/L in men and 26 U/L in women in the blood donor database. Decision tree analysis showed that ALT levels were mostly influenced by body mass index level and its critical two cut-off points were 23.5 kg/m2 and 25.8 kg/m2 , respectively. The most affecting factor of AST was gender., Conclusion: Upper limits of normal of ALT and AST in Koreans were lower than conventional accepted values (< 40 U/L) but higher than recently suggested values (male < 30 U/L and female < 19 U/L). Body mass index was the most determining factor for ALT and gender was the most influencing factor for AST activity., (© 2012 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd.)