A5032704413- Renal Diseases and Glomerulopathies
- Dialysis and Renal Disease Management
- Pediatric Urology and Nephrology Studies
- Complement system in diseases
- Chronic Kidney Disease and Diabetes
- Blood Pressure and Hypertension Studies
- Ion Transport and Channel Regulation
- Urinary Tract Infections Management
- Drug-Induced Adverse Reactions
- Adenosine and Purinergic Signaling
- Systemic Lupus Erythematosus Research
- Renal and related cancers
- Adolescent and Pediatric Healthcare
- Tracheal and airway disorders
- Childhood Cancer Survivors' Quality of Life
- Muscle and Compartmental Disorders
- Eosinophilic Disorders and Syndromes
- Food Allergy and Anaphylaxis Research
- Blood disorders and treatments
- Celiac Disease Research and Management
- Heart Rate Variability and Autonomic Control
- Urological Disorders and Treatments
- Iron Metabolism and Disorders
- Inflammatory mediators and NSAID effects
- Lymphadenopathy Diagnosis and Analysis
Seoul National University
2010-2025
Seoul National University Children's Hospital
2011-2025
Hyundai Pharm (South Korea)
2025
Pusan National University Yangsan Hospital
2015-2024
New Generation University College
2023-2024
Seoul National University Hospital
2023
Korean Society of Hypertension
2019-2022
Pusan National University Hospital
2007-2021
Pusan National University
2005-2021
Kyushu University
2020
The mitochondrial pool of Hsp90 and its paralogue, TRAP1, suppresses cell death reprograms energy metabolism in cancer cells; therefore, TRAP1 have been suggested as target proteins for anticancer drug development. Here, we report that the actual protein mitochondria is current inhibitors cannot effectively inactivate because their insufficient accumulation mitochondria. To develop inhibitors, determined crystal structures human complexed with inhibitors. isopropyl amine inhibitor PU-H71 was...
Ravulizumab, a long-acting complement C5 inhibitor engineered from eculizumab, allows extending maintenance dosing every 2–3 weeks to 4–8 depending on bodyweight. Here, we evaluated the efficacy and safety of ravulizumab in inhibitor-naïve children (under 18 years) with atypical hemolytic uremic syndrome. In this phase III, single-arm trial, was administered eight patients 20 kg over, four under kg. The primary endpoint complete thrombotic microangiopathy response (normalization platelet...
Rationale & ObjectiveAtypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by complement dysregulation. Ravulizumab C5 inhibitor (C5i) approved for the treatment aHUS. This analysis assessed long-term outcomes ravulizumab in adults and pediatric patients with aHUS.Study DesignThis reports 2-year data from two phase 3, single-arm studies.Setting ParticipantsOne study included C5i-naive (NCT02949128) other cohorts (C5i-naive those who switched to...
Background Although the cause of adenotonsillar hypertrophy remains unknown, some studies have shown that allergy may be a risk factor. Purpose This study determined levels allergen-specific immunoglobulin E (sIgE) in tissues children with and evaluated clinical significance local atopy tissues. Methods We measured 21 types specific serum 102 compared sensitization patterns The patients were divided into three groups—atopy, atopy, nonatopy—according to tissues, symptoms among groups...
Orphan nuclear receptor estrogen-related γ (ERRγ) regulates cell growth and tumorigenesis in various cancers. However, the clinical relevance of ERRγ to hepatocellular carcinoma (HCC) remains unclear. Here we examined significance HCC its potential as a therapeutic target. levels tissues from completely resected specimens 190 patients were immunohistochemically their association with stage pathological grade was analyzed. Small interfering RNA (siRNA)-mediated knockdown (siRNA-ERRγ) or an...
Although Hsp90 inhibitors can inhibit multiple tumorigenic pathways in cancer cells, their anticancer activity has been disappointingly modest. However, by forcing into the mitochondria with mitochondrial delivery vehicles, they were converted potent drugs targeting paralog TRAP1. Here, to improve drug accumulation without using vehicle, we increased freely available concentrations cytoplasm reducing binding of abundant cytoplasmic Hsp90. After analyzing X-ray cocrystal structures, purine...
The anti-CD20 monoclonal antibody rituximab (RTX) has been proposed as a rescue therapy for difficult-to-treat nephrotic syndrome (NS). We conducted clinical trial to evaluate the efficacy and safety of RTX in children with NS dependent on or resistant steroids calcineurin inhibitors (CNIs).A multicenter open-label was performed at 8 major pediatric nephrology centers Korea. investigation consisted randomized controlled steroid- CNI-dependent (DDNS; randomization into group control group,...
Abstract Background Atypical hemolytic uremic syndrome ( aHUS ) is a rare disease with genetic predisposition. Few studies have evaluated the in A sian population. We studied K orean pediatric cohort to delineate clinical characteristics and genotypes. Methods multicenter of 51 children was screened for mutations using targeted exome sequencing covering 46 complement related genes. Anti‐complement‐factor‐ H autoantibody (anti‐ CFH titers were measured. Multiplex ligation‐dependent probe...
Steroid-resistant nephrotic syndrome (SRNS) is one of the major causes end-stage renal disease (ESRD) in childhood and mostly associated with focal segmental glomerulosclerosis (FSGS). More than 50 monogenic SRNS or FSGS have been identified. Recently, mutation detection rate pediatric patients has reported to be approximately 30%. In this study, genotype-phenotype correlations a cohort 291 Korean SRNS/FSGS were analyzed. The overall was 43.6% (127 patients). WT1 most common causative gene...
Anaplastic thyroid cancer (ATC), a rare with poor prognosis, is associated insufficient function of the sodium iodide symporter (NIS). Estrogen-related receptor γ (ERRγ) member orphan nuclear receptors important functions in cell development and homeostasis. However, there are no reports that demonstrate whether ERRγ related to NIS function. Here, we evaluated role regulation ATC cells using GSK5182, an inverse agonist ERRγ. <b>Methods:</b> Two lines, BHT-101 CAL62, were incubated GSK5182 at...
Background: Little information exists regarding the differences in clinical and laboratory characteristics of Kikuchi-Fujimoto disease (KFD) according to age. Objective: To evaluate KFD Methods: The relevance sex, age, features, findings, courses, follow-up results were retrospectively evaluated patients diagnosed with at Pusan National University Hospital between 2010 2020. Results: Eighty (46 children 34 adults) a mean age 21.5 ± 11.8 years (range, 3-49 years) included study. Those aged...
Hypertension is becoming one of the most common health conditions in children and adolescents due to increasing childhood obesity. We aimed provide auscultatory blood pressure (BP) normative reference values for Korean non-overweight adolescents.BP measurements aged 10 18 years were performed National Health Nutrition Examination Survey (KNHANES) from 1998 2016. BP was measured using a mercury sphygmomanometer. Sex-, age- height-specific systolic (SBP) diastolic (DBP) percentiles calculated...
Blood pressure (BP) measurement using an auscultatory sphygmomanometer is recommended for diagnosing hypertension in children. As mercury sphygmomanometers (MSs) are banned owing to environmental concerns, it crucial determine the accuracy of mercury-free replace them. We analyzed these devices guide National Survey selection.
Hypertension is one of the most important complications chronic kidney disease (CKD) as it exacerbates progression in children. The aim this study to identify characteristics and factors associated with hypertension children CKD.
Abstract Background Coenzyme Q10 (CoQ10) nephropathy is a well-known cause of hereditary steroid-resistant nephrotic syndrome, primarily impacting podocytes. This study aimed to elucidate variations in individual cell-level gene expression CoQ10 using single-cell transcriptomics. Methods We conducted sequencing kidney biopsy specimen from 5-year-old boy diagnosed with caused by compound heterozygous COQ2 mutation complicated immune complex-mediated glomerulonephritis. The analysis focused on...
Pediatric hypertension (HTN) is a significant, growing health concern worldwide and also in Korea. Diagnosis, evaluation, treatment of HTN Korean children adolescents are uncertain due to limitations using the current international guidelines, since recommendations by American Academy Pediatrics (AAP) European Society Hypertension (ESH) guidelines differ. Furthermore, these for Western youth, who racially ethnically different from Koreans. In addition, reference blood pressure values all...
Abstract Poly (ADP-ribose) polymerase 1 (PARP1) inhibitors are the first successfully approved synthetic lethal cancer treatments targeting BRCA1/2-mutated breast and ovarian cancers. However, limited responsiveness among patients with BRCA1/2 mutations emergence of resistance to PARP highlight growing need for alternative drugs, either as monotherapy or in combination inhibitors. Ubiquitin-Specific Protease (USP1), a member deubiquitinating enzyme (DUB) family, plays crucial role DNA repair...
Abstract BRM and BRG (SMARCA2/4) are two mutually exclusive catalytic ATPase subunits of the BAF complexes. SMARCA4 is frequently mutated in multiple cancers, including about 10% non-small cell lung cancer (NSCLC). As a result, deficient becomes more dependent on its paralog, SMARCA2, for their survival. Consequently, selective inhibitor targeting SMARCA2 potentially effective therapeutic option SMARCA4-mutant cancers. We identified HD-10991 HD-11030 as inhibitors evaluated potency...