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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,...
Case Report
A Case of Systemic Lupus Erythematosus(SLE) with Antiphospholipid Antibodies Presented with Tendency for Severe Bleeding
Mi-Sook Chang, Myoung-A Kim, Eun-Sil Dong, Young-Min Ahn, Yong Choi
Clin Exp Pediatr. 1998;41(8):1149-1152.   Published online August 15, 1998
Original Article
A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus
Jae Hong Min, Kyung Hoon Paek, Kyung Mi Park, Jin Soon Hwang, Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Kyou Seup Han, Yong Choi
Clin Exp Pediatr. 1998;41(7):974-978.   Published online July 15, 1998
Seoul National University College of Medicine, Seoul, Korea Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus(SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly...
Type I Vitamin D Dependent Rickets
Soo Ja Hwang, Jung Soo Kim, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1998;41(7):877-882.   Published online July 15, 1998
Purpose : Vitamin D dependent rickets(VDDR) is a rare, autosomal recessively transmitted disorder characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type Ⅰ VDDR arises from primary deficiency in the renal 1α-hydroxylase that produces 1,25(OH)2D3. So patients with type I VDDR require life long administration of vitamin D. Methods : There had been 6 children(4...
Case Report
A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis
Jung-Jin Yu, Dong Kyu Jin, Hae Il Cheong, Hyun Soon Lee, Yong Choi
Clin Exp Pediatr. 1998;41(3):415-419.   Published online March 15, 1998
Diffuse mesangial sclerosis(DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis...
Original Article
The Efficacy of Fetal Ultrasonography and Postnatal Abdominal Ultrasonography for the Diagnosis of Neonatal Abdominal Mass
Hee Eun Lee, Ee Kyung Kim, Hee Seok Kim, Yun Kyoung Lee, Chan Hwu Park, Kyung Ran Park, June Dong Park, Beyong Il Kim, Woong Heum Kim, Jung Hwan Choi, Gui Won Park, Hwang Choi, Yong Choi, Chong Ku Yun
Clin Exp Pediatr. 1998;41(3):299-306.   Published online March 15, 1998
Purpose : Most neonatal abdominal masses are benign in nature, but early detection and management is important to avoid the development of complications. So, we studied the causes of neonatal abdominal masses and the efficacy of ultrasonography for early detection and diagnosis. Methods : We reviewed 36 newborn infants with abdominal masses who had been admitted to the nursery and Neonatal...
The Prevalence of A985G Mutation in Medium Chain Acyl-Coenzyme A Dehydrogenase(MCAD) Gene in Neonates Determined from Guthrie Card
Baeck Hee Lee, Hye Won Park, Moon Soo Park, Ho Jin Park, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 1997;40(12):1645-1650.   Published online December 15, 1997
Purpose : Medium chain acyl-CoA dehydrogenase(MCAD) deficiency is an autosomal recessive disoder of β oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However,...
Case Report
Three Cases of Cardiac Arrythmias Associated with Intravenous Methylprednisolone Pulse Therapy
Jun Ho Lee, Jun Huh, Myung Dong Shin, Dong Kyu Jin, Il Soo Ha, Hae Il Cheong, Chung Il Noh, Yong Choi
Clin Exp Pediatr. 1997;40(3):393-407.   Published online March 15, 1997
Methylprednisolone(MP) is administered by means of intravenous pulse therapy especially in the patients with focal segmental glomerulosclerosis(FSGS). There have been reports of its complications in a few increasing frequency. We experienced three cases of cardiaccomplications during intravenous pulsetherapy.Therewere onecaseof2¡ÆA-V block(Mobitztype I) and two cases of bradycardia and hypotension. In the former,2¡ÆA-V block in the first case deveoloped 45 hours after...
Original Article
Clinicopathological Study about Childhood Primary Nephrotic Syndrome Resistant to 4-week Daily Steroid Therapy
Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1996;39(12):1729-1735.   Published online December 15, 1996
Purpose : Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. Methods : Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87...
Cytodiagnostic Urinalysis in the Differentiation of Leukocyturias of Children with Bacterial Urinary Tract Infection (UTI) from Those of Children without UTI
Il Soo Ha, Hae Il Cheong, Young Joon Hong, Jin Q Kim, Yong Choi
Clin Exp Pediatr. 1996;39(8):1122-1129.   Published online August 15, 1996
Purpose : Although routine urinalysis can detect leukocyturia, difficulties remain in differentiating subpopulations of leukocytes and various epithelial cells in urinary sediments. This study was performed to appreciate the value of cytodiagnostic urinalysis in the discrimination of leukocyturias of bacterial urinary tract infection (UTI) from those without UTI. Methods : Urine from 12 children with UTI (UTI group) and from 11 children without UTI (non-UTI...
Case Report
A Case of Mauriac's syndrome
Se Young Kim, Choong Ho Shin, Il Soo Ha, Hae Il Cheong, Sei Won Yang, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1996;39(7):1020-1024.   Published online July 15, 1996
Mauriac's syndrome was described in the 1920s, as a triad of poorly controlled insulin dependent diabetes mellitus, profound growth retardation, and hepatomgaly. Following the wide availability of insulin and intensification of diabetic control, this entity has become quite rare. A 9-year-old female child was transferred to pediatric OPD of SNUCH because of hyperglycemia, short stature, and visual disturbance. Five years prior to admission, she...
Original Article
Primary Distal Renal Tubular Acidosis in Children
Byoung Chul Kang, Hye Won Park, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1996;39(7):987-996.   Published online July 15, 1996
Purpose : This study was designed to examine the clinical characteristics and the effect of treatment in children with primary distal renal tubular acidosis (dRTA). Methods : Medical records of 4 children diagnosed as dRTA at Seoul National University Children's Hospital were reviewed, and the clinical manifestations, laboratory findings, diagnostic criteria and effects of treatments were investigated. Results : All cases presented with growth retardation....
Studies on the Distribution of Percentiles of Skinfold Thickness and Body Mass Index among Adolescents in Seoul
Jae Sung Ko, Kang Mo Ahn, Yon Ho Choe, Eun Hwa Choi, Yong Choi, Yoon Ok An
Clin Exp Pediatr. 1996;39(6):773-779.   Published online June 15, 1996
Purpose : With development of socioeconomic status, the prevelance of adolesecnt obesity, which is a risk factor for cardiovascular disease, is increasing. Skinfold and body mass index are indirectly used measurement of obesity in clinical and epidemiologic studies. Triceps skinfold, subscapular skinfold and body mass index were measured in adolescents aged 12 to 17. Percentile values by age and sex and...
Case Report
Antiphospholipid Syndrome Presented with Pulmonary Embolism and Deep Venous Thrombosis in Child
Ran Lee, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1996;39(5):722-726.   Published online May 15, 1996
Antiphospholipid syndrome is a thrombotic disorder and the serologic marker of the syndrome is antiphospholipid antibody(lupus anticoagulant, anticardiolpin antibody, or both). In a 13-year-old girl who presented with dyspnea, pulmonary embolism and femoral vein thrombosis were demonstrated by lung scan and abdominal ultrasonography. She had 3 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia, positive antinuclear antibody, low C3...
Isolated Splenic Infarction in a Girl with Systemic Lupus Erythematosus
Kyung Ran Park, Hee Eun Lee, Yun Ae Jeon, Il Soo Ha, Hae Il Cheong, Joong Gon Kim, Yong Choi
Clin Exp Pediatr. 1996;39(4):562-566.   Published online April 15, 1996
Splenic infarction is a rare disease and usually detected as a complication of SBE, atrial fibrillation, hemoglobinopathy, or myeloproliperative disorders. Although thrombotic event, such as deep vein, cerebral or renal thrombosis, is a frequent complication of SLE, there have been only a couple of cases documenting splenic infarction complicated in SLE. This is, to our knowledge, the first case report...
Cystinuria 3 Cases
Ji Eun Choi, Bo Young Yun, Hae Won Park, Jae Hong Park, Il Sue Ha, Hae Il Jeong, Yong Choi, Hwang Choi, In Won Kim
Clin Exp Pediatr. 1995;38(2):245-251.   Published online February 15, 1995
We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed...