Background: Thiazolidinediones (TZDs) have been associated with various safety concerns including weight gain, bladder cancer, and congestive heart failure (CHF). This study evaluated the efficacy and safety of lobeglitazone, a novel TZD in patients with type 2 diabetes mellitus (T2DM) in real practice. Methods: In this non-interventional, multi-center, retrospective, and observational study conducted at 15 tertiary or secondary referral hospitals in Korea, a total of 2,228 patients with T2DM who received lobeglitazone 0.5 mg for more than 1 year were enrolled. Results: Overall adverse events (AEs) occurred in 381 patients (17.10%) including edema in 1.97% (n=44). Cerebrovascular and cardiovascular diseases were identified in 0.81% (n=18) and 0.81% (n=18), respectively. One case of CHF was reported as an AE. Edema occurred in 1.97% (n=44) of patients. Hypoglycemia occurred in 2.47% (n=55) of patients. Fracture occurred in 1.17% (n=26) of all patients. Lobeglitazone significantly decreased HbA1c level, resulting in a mean treatment difference of -1.05%± 1.35% (P<0.001), and decreased total cholesterol, triglyceride, and low-density lipoprotein cholesterol. However, it increased high-density lipoprotein cholesterol, regardless of statin administration. The patients who received lobeglitazone 0.5 mg showed an apparent reduction in glycosylated hemoglobin (HbA1c) from baseline during the first 6 months of treatment. The HbA1c levels remained stable between months 6 and 42. Conclusion Lobeglitazone has long-term safety profile, good glycemic-lowering effect and long-term durability of glycemic control in real-world clinical settings.

Background/Aims: Chronic enteropathy associated with SLCO2A1 gene (CEAS), an inherited disease characterized by nonspecific intestinal ulcers, has emerged in the Japanese population via loss-of-function mutations in the SLCO2A1 gene. We aimed to investigate the clinical and genetic characteristics of Korean patients diagnosed with CEAS. Methods: From July 2018 to July 2021, we performed Sanger sequencing of the SLCO2A1 gene in 46 patients with chronic intestinal ulcers. CEAS was confirmed based on known SLCO2A1 mutations. We summarized the clinical characteristics of patients with confirmed CEAS. Results: Fourteen out of 46 patients (30.4%) had genetically confirmed CEAS, and two SLCO2A1variants were detected (splicing site variant c.940+1G>A and nonsense mutation [p.R603X] in SLCO2A1). Twelve patients (85.7%) were females and the median age at diagnosis of CEAS was 44.5 years. All patients presented with abdominal pain, and 13 patients (92.9%) presented with anemia (median hemoglobin, 9.6 g/dL). Ten patients (71.4%) had hypoalbuminemia (median, 2.7 g/dL). The most commonly involved site was the ileum (13/14, 92.9%). Manifestations of primary hypertrophic osteoarthropathy (PHO), such as digital clubbing, pachydermia, and periostosis were observed in five patients (28.6%) and two male patients and one female patient satisfied all major PHO diagnostic criteria. Conclusions The clinical and genetic characteristics of Korean patients with confirmed CEAS were similar to those reported in the literature. CEAS should be considered in the differential diagnosis for patients with unexplained chronic nonspecific ulcers of the small intestine.

The outbreak of human toxoplasmosis can be attributed to ingestion of food contaminated with Toxoplasma gondii. Toxoplasmosis recently increased in domestic and stray dogs and cats. It prompted studies on the zoonotic infectious diseases transmitted via these animals. Sero- and antigen prevalences of T. gondii in dogs and cats were surveyed using ELISA and PCR, and B1 gene phylogeny was analyzed in this study. Toxoplasmosis antibodies were measured on sera of 403 stray cats, 947 stray dogs, 909 domestic cats, and 2,412 domestic dogs collected at nationwide regions, Korea from 2017 to 2019. In addition, whole blood, feces, and tissue samples were also collected from stray cats (1,392), stray dogs (686), domestic cats (3,040), and domestic dogs (1,974), and T. gondii-specific B1 gene PCR was performed. Antibody prevalence of stray cats, stray dogs, domestic cats, and domestic dogs were 14.1%, 5.6%, 2.3%, and 0.04%, respectively. Antigen prevalence of these animals was 0.5%, 0.2%, 0.1%, and 0.4%, respectively. Stray cats revealed the highest infection rate of toxoplasmosis, followed by stray dogs, domestic cats, and domestic dogs. B1 gene positives were 5 of stray cats, and identified to high/moderate pathogenic Type I/III group. These findings enforce that preventive hygienic measure should be strengthened at One Health level in dogs and cats, domestic and stray, to minimize human toxoplasmosis infections.

The Committee of Clinical Practice Guidelines of the Korean Diabetes Association revised and updated the 6th Clinical Practice Guidelines in 2019. Targets of glycemic, blood pressure, and lipid control in type 2 diabetes mellitus (T2DM) were updated. The obese and overweight population is increasing steadily in Korea, and half of the Koreans with diabetes are obese. Evidence-based recommendations for weight-loss therapy for obesity management as treatment for hyperglycemia in T2DM were provided. In addition, evidence from large clinical studies assessing cardiovascular outcomes following the use of sodium-glucose cotransporter-2 inhibitors and glucagon-like peptide 1 receptor agonists in patients with T2DM were incorporated into the recommendations.
Blood Pressure ; Diabetes Mellitus, Type 2 ; Diagnosis ; Glucagon-Like Peptide 1 ; Humans ; Hyperglycemia ; Korea ; Obesity ; Overweight

Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.
Female* ; Heart Defects, Congenital ; Humans ; Hypertension, Pulmonary* ; Pulmonary Artery* ; Pulmonary Surgical Procedures ; Transplants

BACKGROUND AND OBJECTIVES: Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. SUBJECTS AND METHODS: The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. RESULTS: The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. CONCLUSIONS: Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms.
Age of Onset ; Aneurysm ; Catheters ; Coronary Aneurysm* ; Coronary Stenosis ; Coronary Vessels ; Follow-Up Studies ; Humans ; Male ; Medical Records ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Infarction ; Myocardial Ischemia ; Prognosis* ; Retrospective Studies

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.
Arrhythmias, Cardiac ; Arteriovenous Fistula ; Biliary Atresia ; Follow-Up Studies ; Heart ; Heart Diseases ; Heterotaxy Syndrome ; Humans ; Isomerism* ; Medical Records ; Mortality ; Prognosis ; Survival Rate

OBJECTIVE: To investigate the effects of hippotherapy on psychosocial and emotional parameters in children with cerebral palsy (CP) and their caregivers. METHODS: Eight children with CP were recruited (three males and five females; mean age, 7.3 years; Gross Motor Function Classification System levels 1-3). Hippotherapy sessions were conducted for 30 minutes once weekly for 10 consecutive weeks in an indoor riding arena. The Gross Motor Function Measure (GMFM), Pediatric Balance Scale (PBS), and the Korean version of the Modified Barthel Index were evaluated. All children were evaluated by the Children's Depression Inventory, Trait Anxiety Inventory for Children, State Anxiety Inventory for Children, Rosenberg Self Esteem Scale, and the Korean-Satisfaction with Life Scale (K-SWLS). Their caregivers were evaluated with the Beck Depression Inventory, the Beck Anxiety Inventory, and the K-SWLS. We assessed children and their caregivers with the same parameters immediately after hippotherapy. RESULTS: Significant improvements on the GMFM, dimension E in the GMFM, and the PBS were observed after hippotherapy compared with the baseline assessment (p<0.05). However, no improvements were detected in the psychosocial or emotional parameters in children with CP or their caregivers. None of the participants showed any adverse effects or accidents during the 10 weeks hippotherapy program. CONCLUSIONS: Hippotherapy was safe and effectively improved gross motor and balance domains in children with CP. However, no improvements were observed in psychosocial or emotional parameters.
Anxiety ; Caregivers* ; Cerebral Palsy* ; Child* ; Classification ; Depression ; Equine-Assisted Therapy* ; Female ; Humans ; Male ; Pilot Projects* ; Self Concept

PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. METHODS: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. RESULTS: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. CONCLUSION: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.
Connective Tissue ; Diagnosis ; Early Diagnosis ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Humans ; Infant, Newborn ; Korea ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Mortality ; Parturition ; Prognosis ; Retrospective Studies ; Sequence Analysis, DNA ; Tertiary Care Centers* ; Tricuspid Valve Insufficiency ; Tricuspid Valve Prolapse

BACKGROUND AND OBJECTIVES: Cardiac catheterization is used to diagnose structural heart disease (SHD) and perform transcatheter treatment. This study aimed to evaluate complications of cardiac catheterization and the associated risk factors in a tertiary center over 10 years. SUBJECTS AND METHODS: Total 2071 cardiac catheterizations performed at the Seoul National University Children's Hospital from January 2004 to December 2013 were included in this retrospective study. RESULTS: The overall complication, severe complication, and mortality rates were 16.2%, 1.15%, and 0.19%, respectively. The factors that significantly increased the risk of overall and severe complications were anticoagulant use before procedure (odds ratio [OR] 1.83, p=0.012 and OR 6.45, p<0.001, respectively), prothrombin time (OR 2.30, p<0.001 and OR 5.99, p<0.001, respectively), general anesthesia use during procedure (OR 1.84, p=0.014 and OR 5.31, p=0.015, respectively), and total procedure time (OR 1.01, p<0.001 and OR 1.02, p<0.001, respectively). Low body weight (OR 0.99, p=0.003), severe SHD (OR 1.37, p=0.012), repetitive procedures (OR 1.7, p=0.009), and total fluoroscopy time (OR 1.01, p=0.005) significantly increased the overall complication risk. High activated partial thromboplastin time (OR 1.04, p=0.001), intensive care unit admission state (OR 14.03, p<0.001), and concomitant electrophysiological study during procedure (OR 3.41, p=0.016) significantly increased severe complication risk. CONCLUSION: Currently, the use of cardiac catheterization in SHD is increasing and becoming more complex; this could cause complications despite the preventive efforts. Careful patient selection for therapeutic catheterization and improved technique and management during the peri-procedural period are required to reduce complications.
Anesthesia, General ; Body Weight ; Cardiac Catheterization* ; Cardiac Catheters* ; Catheterization ; Catheters ; Fluoroscopy ; Heart Diseases* ; Heart* ; Intensive Care Units ; Mortality ; Partial Thromboplastin Time ; Patient Selection ; Prothrombin Time ; Retrospective Studies ; Risk Factors ; Seoul