Publications by authors named "Hye-Sun Hyun"

Article Synopsis
  • - This study examined the relationship between genotype and phenotype in X-linked Alport syndrome (XLAS) among 216 Korean patients, finding that males experience earlier onset and more severe kidney failure than females.
  • - About 60% of male patients developed kidney failure by the age of 25, with significant differences in kidney survival based on their genetic group, while only 20% of female patients experienced kidney failure by age 50.
  • - The results suggest a clear genotype-phenotype correlation in both male and female patients, highlighting the need for better understanding of XLAS in women, who tend to have a later onset of symptoms.
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Introduction: Bartter syndrome (BS) is a rare salt-wasting tubulopathy caused by mutations in genes encoding sodium, potassium, or chloride transporters of the thick ascending limb of the loop of Henle and/or the distal convoluted tubule of the kidney. BS is characterized by polyuria, failure to thrive, hypokalemia, metabolic alkalosis, hyperreninemia, and hyperaldosteronism. Potassium and/or sodium supplements, potassium-sparing diuretics, and nonsteroidal anti-inflammatory drugs can be used to treat BS.

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  • This study focused on pediatric rhabdomyolysis in South Korea, analyzing 880 cases from 23 hospitals to identify characteristics and risk factors related to acute kidney injury (AKI).
  • The most affected age group was children aged 3 to 5, with neurological disorders and infections being common causes of rhabdomyolysis, and an 11.3% incidence rate of AKI was noted among the patients.
  • Key risk factors for AKI were identified, including multiorgan failure and specific laboratory findings, which could help in early detection and intervention for at-risk pediatric patients.
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Infectious disease outbreaks such as COVID-19 and MERS pose a major threat to healthcare workers' (HCWs) physical and mental health. Studies exploring the positive changes gained from adapting to traumatic events, known as post-traumatic growth (PTG), have attracted much attention. However, it is unclear which factors or experiences lead to PTG among HCWs.

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Article Synopsis
  • Children with nephrotic syndrome (NS) are at a higher risk for acute kidney injury (AKI), and the incidence of AKI in this group is rising, prompting a nationwide study to explore this issue in hospitalized pediatric patients.
  • The study involved 363 children with NS who were hospitalized over a five-year period, revealing that 16.2% experienced AKI, predominantly due to long disease duration, low albumin levels, and treatment with methylprednisolone.
  • Most AKI cases were reversible, with a small percentage leading to chronic kidney disease; however, those with AKI had longer hospital stays compared to those without it, indicating the clinical significance of monitoring these patients closely.
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Background: Although various forms of online education are on the rise worldwide, effects of such innovative approach are yet to be validated. This study analyzes whether blended learning cardiopulmonary resuscitation (CPR) education that integrates e-learning and face-to-face education is effective in improving nursing students' knowledge, attitude, and self-efficacy.

Methods: A randomized controlled design was used.

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Article Synopsis
  • The study discusses a resurgence of kidney-related issues, specifically acute kidney injury (AKI), linked to infections previously thought to be nearly eradicated due to improved public health, often resulting from contaminated food or water.
  • Researchers collected and analyzed medical records from nine patients admitted to Seoul National University Children's Hospital in 2017, all showing symptoms like fever and gastrointestinal distress, with many having a common history of drinking mountain water.
  • The findings indicate that this infection can lead to serious complications like AKI and highlight the need for clinicians to consider this diagnosis in patients with prolonged fever, gastroenteritis, and non-resolving AKI.
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  • Extended-spectrum-beta-lactamase (ESBL)-producing bacteria are a growing cause of urinary tract infections (UTIs) in children under 2 years old, with 17.3% of cases linked to these bacteria.
  • Key risk factors for ESBL UTIs include a history of previous UTIs, recent antibiotic use, hospitalization in the past 3 months, and underlying urinary issues.
  • Relapse rates after treatment were low (<3%) for both ESBL and non-ESBL groups, suggesting that empirically using effective antibiotics may be viable, though more research is needed to support these findings.
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Article Synopsis
  • The study aimed to create and validate a tool to assess neighborhood factors that influence obesity among rural adults in Korea.
  • It involved both qualitative and quantitative research methods, including focus groups and multiple surveys with adults aged 19-65.
  • The final tool, containing 37 items and validated through factor analysis, demonstrated good reliability and consistency, making it useful for public health initiatives targeting obesity in rural areas.
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Objectives: Inflammation is a major mechanism underlying coronary heart disease (CHD) and C-reactive protein (CRP) is a marker of inflammation. When administered soon after menopause, menopausal hormone therapy (MHT) prevents CHD. This study was conducted to examine the impact of estrogen by administration route on CRP in postmenopausal Korean women using micronized progesterone (MP4) for endometrial protection.

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A retrospective review was performed to assess the risk factors and outcomes of BK virus infection and nephropathy (BKVN), an early complication in pediatric kidney allograft recipients. The study investigated the incidence, risk factors, and clinical outcomes of BK viremia and BKVN in a Korean population of pediatric patients who received renal transplantation from 2001-2015 at the Seoul National University Hospital. BKVN was defined as biopsy-proven BKVN or plasma BK viral loads >10,000 copies/mL for >3 weeks.

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Background/aims: Additional validation study was warranted to confirm the clinical significance of C score, which was recently added to the Oxford classification for immunoglobulin A nephropathy (IgAN).

Methods: We performed a multicenter retrospective cohort study in four hospitals in Korea. Patients who had biopsied glomeruli less than eight or inadequate follow-up information were excluded.

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The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma.

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Article Synopsis
  • Recent genetic studies show that Schimmelpenning-Feuerstein-Mims syndrome (SFMS), which features sebaceous nevi, is a mosaic RASopathy caused by mutations in specific genes in some patients.
  • A case report describes a girl with extensive nevus sebaceous and hypophosphatemic rickets, identified as cutaneous skeletal hypophosphatemia syndrome (CSHS), which involved a mutation in the gene found only in her skin tissue.
  • The findings suggest that more research is necessary to understand how Ras activation may contribute to skeletal issues in CSHS, likely linked to overproduction of fibroblast growth factor 23.
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Background: Multicentric carpotarsal osteolysis syndrome (MCTO) is characterized by progressive destruction and disappearance of the carpal and tarsal bones associated with nephropathy. MCTO is caused by loss-of-function mutations in the MAF bZIP transcription factor B (MAFB) gene.

Case Presentation: This report describes three unrelated patients with MAFB mutations, including two male and one female patient.

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Article Synopsis
  • Galloway-Mowat syndrome (GAMOS) is a rare genetic disorder featuring early-onset kidney issues, brain anomalies, and microcephaly, linked to mutations in KEOPS gene subunits.
  • A new case involved three siblings with a specific homozygous mutation in the TP53RK gene, leading to severe symptoms like nephrotic syndrome, microcephaly, and early death.
  • This study highlights the genetic connection of GAMOS to TP53RK mutations, marking it as only the second such report in the field.
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Background/aims: Primary distal renal tubular acidosis (dRTA) in children is a rare genetic disorder, and three causative mutated genes have been identified: SLC4A1, ATP6V1B1, and ATP6V0A4. We analyzed the prevalence and phenotypic differences of genetic mutations in children with dRTA.

Methods: A total of 17 children with dRTA were enrolled in the study.

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Objective The aim of this study was to investigate the relationship between working environment and weight control efforts among obese workers in Korea. Methods This study was based on the 2011 3rd Korean Working Conditions Survey, which was conducted on workers aged 15 years or older. A sample of 484 obese workers was included in the study.

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Objectives: This study investigates investigated the relationship between social and physical environments, and moderate to vigorous physical activity (MVPA) amongst adults in both rural and urban areas within Korea.

Methods: A sample of 128,735 adults from the 2013 Community Health Survey (CHS) was analyzed using a multilevel logistic analysis.

Results: Urban residents with higher satisfaction in public transportation satisfaction and rural residents with more access to sports parks, hiking trails, and bike cycle paths were more likely to be active.

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Background: Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children.

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Background: Gordon syndrome (GS) is a rare form of monogenic hypertension characterized by low renin hypertension, hyperkalemia, hyperchloremic metabolic acidosis, and normal glomerular filtration rate. To date, four genes causing GS have been identified as: WNK1, WNK4, CUL3, and KLHL3.

Case Presentation: We report three cases of GS in two families.

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Cystinuria is an inherited disorder characterized by defective renal reabsorption of cystine and dibasic amino acids leading to nephrolithiasis. This study was conducted to analyze the genotypes and phenotypes of pediatric patients with cystinuria. Eight children from Seoul National University Hospital and Asan Medical Center presenting with cystinuria from January 2003 to June 2016 were retrospectively analyzed.

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