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C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report

Hyun H, Kang HG, Cho U, Ha IS, Cheong HI

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli....
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Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease: Results from the KNOW-Ped CKD

Kang NR, Ahn YH, Park E, Lee KH, Baek HS, Kim SH, Cho H, Cho MH, Shin JI, Lee JH, Cheong HI, Kang HG, Park YS, Ha IS, Moon DS, Han KH

Background: Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the...
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Renal Syndromic Hearing Loss Is Common in Childhood-onset Chronic Kidney Disease

Kim JH, Lee DH, Lee Bj, Lim SH, Ahn YH, Kang HG, Ha IS, Cheong HI

Background: Hearing loss (HL) in children may adversely affect their development. HL is more prevalent in patients with chronic kidney disease (CKD) than in the general population. This study evaluated the...
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Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment

Kim MS, Lim SH, Kim JH, Ha IS, Cheong HI, Kang HG

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare and life-threatening disorder. Typical HUS is often caused by Shiga toxin-positive Escherichia coli, while aHUS is caused by dysregulation of the...
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Gorham-Stout Syndrome with Focal Segmental Glomerulosclerosis: A Case Report

Kim JH, Kim YS, Lim SH, Ahn YH, Ko JM, Suh DI, Lee KB, Moon KC, Ha IS, Cheong HI, Kang HG

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age...
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A Pediatric Case of Long-term Untreated Distal Renal Tubular Acidosis

Kedsatha P, Shin HY, Choi Y, Cheong HI, Cho TJ, Yi E, Maisai M

Distal renal tubular acidosis (dRTA) is a rare renal tubular disorder characterized by normal anion gap metabolic acidosis, hypokalemia, and high urine pH. It can be inherited or acquired. In...
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A Premature Baby with Severe Oligohydramnios and Hypotension: a Case Report of Renal Tubular Dysgenesis

Min J, Cho MH, Bae SP, Shin SH, Ha IS, Cheong HI, Kang HG

Renal tubular dysgenesis (RTD) is a rare fatal disorder in which there is poor development of proximal tubules, leading to oligohydramnios and the Potter sequences. RTD occurs secondary to renin-angiotensin...
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Severe Acute Kidney Injury with Familial Renal Hypouricemia Confirmed by Genotyping of SLC22A12

Cho CM, Cheong HI, Lee JW

Idiopathic renal hypouricemia is a hereditary disease characterized by abnormally high renal uric acid clearance. A defect in the SLC22A12 genes, which encodes the renal uric acid transporter, URAT1, is...
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Pseudohypoaldosteronism Type 1 with a NovelMutation in the NR3C2 Gene: A Case Report

Kim YM, Choi IS, Cheong HI, Kim CJ, Yang EM

Pseudohypoaldosteronism type 1 (PHA1) is a rare salt-wasting disorder caused by resistance to mineralocorticoid action. PHA1 is of two types with different levels of disease severity and phenotype as follows: systemic type...
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Genetic tests in children with steroid-resistant nephrotic syndrome

Cheong HI

Steroid-resistant nephrotic syndrome (SRNS) is a common cause of chronic kidney disease in children, and a considerable number of patients progress to end-stage renal disease. SRNS is a highly heterogeneous...
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Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Lee H, Kang E, Kang HG, Kim YH, Kim JS, Kim HJ, Moon KC, Ban TH, Oh SW, Jo SK, Cho H, Choi BS, Hong J, Cheong HI, Oh D

Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A...
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Erratum: Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

Hyun H, Park E, Cho M, Min SI, Ha J, Kang HJ, Shin HY, Ha IS, Cheong HI, Ahn YH, Kang HG

The authors regret that there were errors in (Text/Table 1). This notice corrects on page 4 and page 5.
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Primary Hyperoxaluria in Korean Pediatric Patients

Choe Y, Lee JM, Kim JH, Cho MH, Kim SH, Lee JH, Park YS, Kang HG, Ha IS, Cheong HI

BACKGROUND: Primary hyperoxaluria (PH), a rare inborn error of glyoxylate meta bolism causing overproduction of oxalate, is classified into three genetic subgroups: type 1–3 (PH1–PH3) caused by AGXT, GRHPR ,...
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Acute kidney injury associated with Yersinia pseudotuberculosis infection: Forgotten but not gone

Kim YK, Cho MH, Hyun HS, Park E, Ha IS, Cheong HI, Kang HG

BACKGROUND: Yersinia pseudotuberculosis is known to cause fever, gastroenteritis, or acute kidney injury (AKI). There have been several Y. pseudotuberculosis infection outbreaks to date associated with ingestion of contaminated food...
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Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

Hyun H, Park E, Cho M, Min SI, Ha J, Kang HJ, Shin HY, Ha IS, Cheong HI, Ahn YH, Kang HG

BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is one of the major complications of organ transplantation, especially in children with Epstein-Barr virus (EBV) viremia (EV). We performed a retrospective study to evaluate...
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Postinfectious Glomerulonephritis Associated with Pneumococcus and Influenza A Virus Infection in a Child: a Case Report and Literature Review

Huh H, Lee JK, Yun KW, Kang HG, Cheong HI

Postinfectious glomerulonephritis (PIGN) is most commonly caused by Streptococcus pyogenes in children, but PIGN associated with other pathogens has been described in the literature. A previously healthy 6-year-old boy was...
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A Pediatric Case of a D-Penicillamine Induced ANCA-associated Vasculitis Manifesting a Pulmonary-Renal Syndrome

Kang S, Cho MH, Hyun H, Kim JH, Ko JS, Kang HG, Cheong HI, Kim WS, Moon KC, Ha IS

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced...
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Genetic diagnosis of Alport syndrome

Cheong HI

No abstract available.
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Erratum: Correction of Error in Result: Urological Problems in Patients with Menkes Disease

Kim MY, Kim JH, Cho MH, Choi YH, Kim SH, Im YJ, Park K, Kang HG, Chae JH, Cheong HI

The authors regret that there was an important error in the results in Table 1; the ATP7A mutations detected in Patients 2 and 14 were incorrectly noted.
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Reninoma: a rare cause of curable hypertension

Kim JH, Kim JH, Cho MH, Park E, Hyun HS, Ahn YH, Kang HG, Moon KC, Ha IS, Cheong HI

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...
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