Ji Hyun Kim

Very-early-onset autosomal dominant polycystic kidney disease coexisting with congenital adrenal hyperplasia in a newborn: a case report

C. Kim, Hwa Young Kim, Young Hwa Jung, Chang Won Choi et autres

This case report describes a newborn diagnosed with very-early-onset autosomal dominant polycystic kidney disease (VEO-ADPKD) and congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The patient presented with prenatally detected kidney cysts and exhibited clinical and laboratory features consistent with salt-wasting, including …

kr (code pays fourni par la source)

Patient-derived human induced pluripotent stem cell podocytes uncover endoplasmic reticulum and oxidative stress-mediated dysfunction in X-linked Alport syndrome

Yoo‐Jin Shin, Xianying Fang, Hanbi Lee, Do-hyun Na et autres

Background: X-linked Alport syndrome (XLAS) is an inherited nephropathy caused by pathogenic COL4A5 variants that lead to podocyte dysfunction and progressive kidney failure. However, the involvement of endoplasmic reticulum (ER) stress and oxidative stress in the development of XLAS remains insufficiently understood. …

kr (code pays fourni par la source)

Clinical practice recommendations for the management of Alport syndrome: a joint statement of the Korean Society of Nephrology and the Korean Society of Pediatric Nephrology

Ji Hyun Kim, Young‐Hwan Hwang, Eunjeong Kang, Hee Gyung Kang et autres

BACKGROUND: Alport syndrome (AS) is a hereditary kidney disorder characterized by progressive hematuria, proteinuria, and kidney failure, frequently accompanied by hearing loss and ocular abnormalities. Although blockade of the renin-angiotensin system (RAS) is established as the first-line therapy, additional pharmacological interventions have …

kr, pr (code pays fourni par la source)

Clinical characteristics and genetic analyses of Korean children with Dent disease

Eun Mi Yang, Yo Han Ahn, Ji Hyun Kim, Ji Yeon Song et autres

Dent disease is a hereditary kidney tubular disorder caused by pathogenic variants in either the CLCN5 (Dent disease 1) or OCRL1 (Dent disease 2) genes. As a rare genetic disorder, Dent disease often presents with variable clinical manifestations, leading to frequent misdiagnosis …

kr (code pays fourni par la source)

Epidemiological characteristics and treatment patterns of Alport syndrome in Korea

Min Ji Park, Ji Hyun Kim, Keum Hwa Lee, Min Hyun Cho et autres

Background: Alport syndrome is the second most common inherited kidney disease, yet many patients remain undiagnosed until advanced kidney failure or receive inappropriate immunosuppressive therapy. Genetic testing indicates it is more common than recognized, but most epidemiological studies have been regional, lacking …

kr (code pays fourni par la source)

Clinical outcomes among children with primary nephrogenic diabetes insipidus

Seon Hee Lim, Jin‐Soon Suh, Ji Hyun Kim, Seung Jae Lee et autres

ABSTRACT Background Primary nephrogenic diabetes insipidus (NDI) is a rare inherited disorder with limited data on long-term outcomes. This study assessed longitudinal outcomes with primary NDI. Methods This multicenter retrospective study included 63 patients with primary NDI. Growth rates and estimated glomerular …

kr, Éthiopie (code pays fourni par la source)

Father’s Parenting and Child Self-Control: A Comparison Between the Netherlands and South Korea

Yugyun Kim, Patricia Bravo, Jennifer S. Richards, Ji Hyun Kim et autres

Parents from different cultures may employ different parenting behaviors to support children's development. It remains unclear whether the associations of parental warmth and control with self-control abilities vary across cultures. Moreover, little is known about how these associations may differ for father' …

nl, qa (code pays fourni par la source)

Analysis of Pharmacokinetics and Comparison Between One-point Versus Two-point Sampling for Therapeutic Drug Monitoring of Vancomycin in Children

Seonghae Yoon, Seunghwan Baek, Jae‐Yong Chung, Kyunghoon Lee et autres

BACKGROUND: Vancomycin therapeutic drug monitoring (TDM) is essential for optimizing efficacy and minimizing toxicity, particularly in acute kidney injury (AKI). However, recent guidelines favor area under the curve (AUC)-guided dosing over traditional trough monitoring to improve dosing. This study aimed to compare …

kr, Éthiopie, us (code pays fourni par la source)

Comparison of estimated and measured GFR in pediatric CKD patients transitioning from adolescence to adulthood: results from KNOW-PedCKD

Seon Hee Lim, Eujin Park, Kyung Hee Han, Ji Yeon Song et autres

kr, lv (code pays fourni par la source)

Development of a prediction tool for kidney function decline in children with chronic kidney disease

S. J. Moon, Junhyug Noh, Hee Gyung Kang, Keum Hwa Lee et autres

Background: A paucity of literature exists on the development of predictive tools for the decline of kidney function in pediatric chronic kidney disease (CKD). The objective of this study is to develop and internally validate a tool for the short-term prediction of …

kr (code pays fourni par la source)

External validation of the international immunoglobulin A nephropathy (IgAN) prediction tool in Korean children with IgAN

Peong Gang Park, Jayoun Kim, Ji Hyun Kim, Yo Han Ahn et autres

Background: The International IgA nephropathy (IgAN) Prediction Tool was recently updated to predict the risk of a 30% decline in estimated glomerular filtration rate (eGFR) or kidney failure in children with IgAN. We aimed to evaluate the clinical performance of this tool …

kr, Éthiopie (code pays fourni par la source)

IP15-34 STEM CELL-DERIVED TROPHIC FACTOR DELIVERY TO TREAT ACUTE KIDNEY INJURY

Hyung Eun Yim, Kim Ds, Hyun Cheol Chung, Brian Shing et autres