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Case Report
Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome
Hye Jin Chang, Hwa Young Kim, Jae Hong Choi, Hyun Jin Choi, Jae Sung Ko, Il Soo Ha, Hae Il Cheong, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2014;57(2):96-99.   Published online February 24, 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have...

Original Article
Intravenous fluid prescription practices among pediatric residents in Korea
Jiwon M. Lee, Younghwa Jung, Se Eun Lee, Jun Ho Lee, Kee Hyuck Kim, Ja Wook Koo, Young Seo Park, Hae Il Cheong, Il-Soo Ha, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2013;56(7):282-285.   Published online July 19, 2013
Purpose

Recent studies have established the association between hypotonic fluids administration and hospital-acquired hyponatremia in children. The present paper investigated the pattern of current practice in intravenous fluid prescription among Korean pediatric residents, to underscore the need for updated education.

Methods

A survey-based analysis was carried out. Pediatric residents at six university hospitals in Korea completed a survey consisting of four questions. Each...

Case Report
A case of pseudohypoaldosteronism type 1 with a mutation in the mineralocorticoid receptor gene
Se Eun Lee, Yun Hye Jung, Kyoung Hee Han, Hyun Kyung Lee, Hee Gyung Kang, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2011;54(2):90-93.   Published online February 28, 2011

Pseudohypoaldosteronism type 1 (PHA1) is a rare form of mineralocorticoid resistance characterized in newborns by salt wasting with dehydration, hyperkalemia and failure to thrive. This disease is heterogeneous in etiology and includes autosomal dominant PHA1 owing to mutations of the NR3C2 gene encoding the mineralocorticoid receptor, autosomal recessive PHA1 due to mutations of the epithelial sodium channel (ENaC) gene, and...

Renal transplantation in a patient with Bartter syndrome and glomerulosclerosis
Se Eun Lee, Kyoung Hee Han, Yun Hye Jung, Hyun Kyung Lee, Hee Gyung Kang, Kyung Chul Moon, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2011;54(1):36-39.   Published online January 31, 2011

Bartter syndrome (BS) is a clinically and genetically heterogeneous inherited renal tube disorder characterized by renal salt wasting, hypokalemic metabolic alkalosis and normotensive hyperreninemic hyperaldosteronism. There have been several case reports of BS complicated by focal segmental glomerulosclerosis (FSGS). Here, we have reported the case of a BS patient who developed FSGS and subsequent end-stage renal disease (ESRD) and provided...

A case of Bartter syndrome type I with atypical presentations
Eun Hye Lee, Ju Sun Heo, Hyun Kyung Lee, Kyung Hee Han, Hee Gyung Kang, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2010;53(8):809-813.   Published online August 31, 2010

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset...

Original Article
Polymorphisms of the NR3C1 gene in Korean children with nephrotic syndrome
Hee Yeon Cho, Hyun Jin Choi, So Hee Lee, Hyun Kyung Lee, Hee Kyung Kang, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2009;52(11):1260-1266.   Published online November 15, 2009
Purpose : Idiopathic nephrotic syndrome (NS) can be clinically classified as steroid-sensitive and steroid-resistant. The detailed mechanism of glucocorticoid action in NS is currently unknown. Methods : In this study, we investigated 3 known single nucleotide polymorphisms (SNPs) (ER22/23EK, N363S, and BclI) of the glucocorticoid receptor gene (the NR3C1 gene) in 190 children with NS using polymerase chain reaction-restriction fragment...
Risk Factors of Urinary Tract Infections Due to Extended-spectrum β-lactamase Producing Escherichia coli in Children
Nam Hee Kim, Jin A Lee, Yun Kyung Kim, Eun Hwa Choi, Il Soo Ha, Hoan Jong Lee, Yong Choi
Clin Exp Pediatr. 2004;47(2):164-169.   Published online February 15, 2004
Purpose : This study was performed to identify risk factors for urinary tract infection(UTI) by extended-spectrum β-lactamase(ESBL)-producing Escherichia coli in children. Methods : We analyzed 125 episodes of UTI which were developed in 112 children at the Seoul National University Children's Hospital from July to December 2002. Medical records of 74 episodes of UTI caused by E. coli were reviewed in...
Urinary Tract Infections in Febrile Infants under Three Months of Age
Byung Wook Eun, Yoo Mi Chung, Hee Gyung Kang, Il Soo Ha, Hae Il Cheong, Hoan Jong Lee, Yong Choi
Clin Exp Pediatr. 2003;46(3):265-270.   Published online March 15, 2003
Purpose : To characterize the infants under 3 months of age with urinary tract infections(UTIs), and especially patients with bacteremia or meningitis Methods : Hospital records of all the infants under 3 months of age discharged from our hospital for 69 consecutive months with the diagnosis of initial episode of UTI were reviewed. UTI was defined when patients had fever with...
Case Report
A Case of Systemic Capillary Leak Syndrome in a Child
Taeg Young Lee, Yoo Mi Chung, Hee Gyung Kang, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 2002;45(10):1292-1297.   Published online October 15, 2002
Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in...
Three Cases of Urine Abnormalities Associated with Ketogenic Diet
Hye Won Hahn, Ki Jung Kim, Il Soo Ha, Hae Il Jung, Yong Seung Hwang, Yong Choi
Clin Exp Pediatr. 2001;44(6):709-713.   Published online June 15, 2001
Ketogenic diet is a high-fat, low carbohydrate, low protein diet used in treatment of pediatric epilepsy since the 1920s. Currently it is used primarily to treat refractory childhood epilepsy. Few serious complications caused by ketogenic diet have been reported. Short-term complications include dehydration, hypoglycemia, vomiting, diarrhea, and refusal to eat. Long-term complications include kidney stones, recurrent infections, metabolic derangement, hypercholesterolemia,...
Original Article
Factors Associated with Renal Failure in Children with Primary Vesicoureteral Reflux
Kyung Mi Park, Kyung Hoon Paek, Jae Hong Min, Jung Su Kim, Il Soo Ha, Kwang Myung Kim, Hae Il Cheong, Hwang Choi, Yong Choi
Clin Exp Pediatr. 1999;42(7):959-965.   Published online July 15, 1999
Purpose : The pathophysiologic mechanisms and risk factors of renal functional deterioration are still controversial. We analyzed the factors associated with renal failure in children with primary vesicoureteral reflux. Methods : The medical records of 166 children who were diagnosed with primary vesicoureteral reflux from Jan. 1985 to Dec. 1996 in the Department of Pediatrics, Seoul National University Children's Hospital...
Renal Problems in Wilson Disease
Il Soo Ha, Jun Ho Lee, Yeon Ho Choe, Hae Il Cheong, Sei Won Yang, Young Yull Koh, Jeong Kee Seo, Yong Choi
Clin Exp Pediatr. 1999;42(6):817-824.   Published online June 15, 1999
Purpose : Wilson disease is a hereditary metabolic disorder characterized by an accumulation of copper in tissues which sometimes results in renal problems. This study was performed to evaluate the prevalences of the renal problems in Wilson disease and related risk factors. Methods : In 31 patients with Wilson disease, we looked for renal problems by history taking, physical examination...
Case Report
Two Cases of Oculocerebrorenal Syndrome of Lowe
So Won Kim, Young Suk Yu, In-One Kim, Hae Il Cheong, Yong Seung Hwang, Yong Choi
Clin Exp Pediatr. 1999;42(3):419-423.   Published online March 15, 1999
Oculocerebrorenal syndrome of Lowe(OCRL) is a rare X-linked disorder characterized by congenital cataract(oculo-), hypotonia, developmental delay, cognitive impairment(cerebro-), renal tubular dysfunction(renal), and growth retardation. Recently, the defective gene, OCRL-1 gene encoding [PtdIns(4,5)P2] 5-phosphatase, was cloned with mutations identified in patients. Although there have been about 200 cases of OCRL reported in English literature, only three reports have been published in...
Original Article
Longterm Follow-up of Autosomal Recessive Polycystic Kidney Disease
June Huh, Il Soo Ha, Hae Il Cheong, Jeong Kee Seo, Yong Choi
Clin Exp Pediatr. 1998;41(12):1693-1699.   Published online December 15, 1998
Purpose : This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease(ARPKD). Methods : The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. Results : The average age at diagnosis was 3 8/12 years(4months-7 3/12 years)...
Long-term Follow-up Study of Children with Minimal Change Nephrotic Syndrome
Jung Sue Kim, Hae Il Cheong, Hyun Soon Lee, Yong Choi
Clin Exp Pediatr. 1998;41(12):1675-1684.   Published online December 15, 1998
Purpose : Most of childhood MCNS has a long disease course with frequent relapses. This study was designed to analyze the long-term clinical course of childhood MCNS, focusing at relapsing pattern, treatment response and complications. Mothods : The medical records of 137 children with biopsy-proven MCNS observed during 1976 ti 1996 were analyzed retrospectively. They were classified as initial responders(111 patients,...
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