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Original Article
Outcomes of chronic dialysis in Korean children with respect to survival rates and causes of death
Hye Jin Chang, Kyoung Hee Han, Min Hyun Cho, Young Seo Park, Hee Gyung Kang, Hae Il Cheong, Il Soo Ha
Clin Exp Pediatr. 2014;57(3):135-139.   Published online March 31, 2014
Purpose

Adult Korean patients on chronic dialysis have a 9-year survival rate of 50%, with cardiovascular problems being the most significant cause of death. The 2011 annual report of the North American Pediatric Renal Trials and Collaborative Studies group reported 3-year survival rates of 93.4% and relatively poorer survival in younger patients.

Methods

In this study, we have reviewed data from Korean Pediatric...

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
Validation study of the Dinamap ProCare 200 upper arm blood pressure monitor in children and adolescents
Chong Guk Lee, Hyang Mi Park, Hye Jung Shin, Jin Soo Moon, Yeong Mi Hong, Nam Soo Kim, Il Soo Ha, Myeong Jin Chang, Kyeong Won Oh
Clin Exp Pediatr. 2011;54(11):463-469.   Published online November 30, 2011
Purpose

To validate the Dinamap ProCare 200 blood pressure (BP) monitor against a mercury sphygmomanometer in children 7 to 18 years old in accordance with the 2010 International Protocol of European Society of Hypertension (ESH-IP2) and the British Hypertension Society (BHS) protocol.

Methods

Forty-five children were recruited for the study. A validation procedure was performed following the protocol based on the ESH-IP2 and...

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...
Review Article
Peritoneal dialysis in children and adolescents
Il Soo Ha
Clin Exp Pediatr. 2009;52(10):1069-1074.   Published online October 15, 2009
Peritoneal dialysis is a preferred modality of replacement therapy in children and adolescents with end-stage renal disease waiting for kidney transplantation. Recent development of pediatric swan-neck catheters with cuffs, novel dialysis solutions, and cyclers for automated peritoneal dialysis enabled more flexible prescriptions of dialysis with less complication, and improved patients' activities as well as the dialysis adequacy. Principles and practical...
Original Article
Effect of renin inhibition on an experimental glomerulonephritis - a preliminary report
Ju Hyung Kang, Jae Kyung Huh, Young Sook Lee, Ji Young Han, Il Soo Ha
Clin Exp Pediatr. 2009;52(8):938-943.   Published online August 15, 2009

Purpose:We performed this study in order to investigate the effect of direct renin inhibition on an experimental animal model with nephrotoxic serum nephritis and tried to give useful information for clinical research and renin inhibitor treatment. Methods:Thirty BALB/c 6-week-old male mice were divided into 4 groups: control group (CO, n=5), control-treatment group with aliskiren (CT, n=5), disease group (DO, n=10), and disease treatment...
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...
A Case of Hereditary Fructose Intolerance
Eun Kyeong Kang, Hye Ran Yang, Jeong Kee Seo, Sun Hoan Bai, Joo Young Jeong, Jae Sung Ko, Il Soo Ha, Jeong Han Song, Kyeong Ae Wi, Yoon Sook Shin
Clin Exp Pediatr. 2002;45(1):120-124.   Published online January 15, 2002
Hereditary fructose intolerance(HFI) is an autosomal recessive disease caused by catalytic deficiency of aldolase B in which affected homozygotes develop hypoglycemia and abdominal symptoms after taking foods containing fructose. Chronic exposure to fructose may lead to progressive hepatic injury, renal injury, growth retardation, and ultimately to liver and kidney failure. Herein, we report a case of HFI with presentation of episodic vomiting, diarrhea, cold...
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,...
Medical Lecture Course
Renal Fibrosis and Transforming Growth Factor-β1
Il Soo Ha
Clin Exp Pediatr. 2001;44(3):285-291.   Published online March 15, 2001
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...
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)...
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...
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....
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...
A Case of Neonatal Cholestasis with Arthrogryposis Multiplex Congenita and Renal Tubular Insufficiency(ARC Syndrome)
Hi Soo Rhee, Soon Young Kim, Nam Sun Baik, Il Soo Ha, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(1):126-130.   Published online January 15, 1996
We report a case of ARC syndrome with arthrogryposis multiplex congenita, renal tubular insufficiency and cholestasis. The Patient presented in the early neonatal period with micrognathia, low set ears, high arched palate, multiple joint contracture, conjugated hyperbilirubinemia and failure to thrive. He died at the age of 1 month despite medical therapy. Findings of renal tubular insufficiency included persistent renal...
Original Article
Acute Hemorrhagic Cystitis(AHC) in Children -Etiology and Clinical Characteristics-
Jin Won Pyo, Eun Hwa Choi, Jin Young Park, Hoan Jong Lee, Hae Il Cheong, Il Soo Ha, Yong Choi, Kwang Myung Kim, Hwang Choi, Je Geun Chi
Clin Exp Pediatr. 1995;38(2):207-215.   Published online February 15, 1995
Purpose : AHC characterized by sudden onset of gross hematuria, dysuria and frequency oc curs in children and young adults as a self-limited disease that should be differentiated from se rious renal disorders. We have performed this study to establish the cause and characterize the clinical features of this illness in Korean children. Methods : 19 cases collected prospectively for 30...
Case Report
Recurrent Hematuria due to Renal Hypouricemia
Kyu Young Kim, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1995;38(1):129-132.   Published online January 15, 1995
A marked low concentration of serum uric acid(0.7-1.2mg/dl) was detected in a 14-year-old boy with recurrent episodes of gross hematuria. The hypouricemia accompanied with a markedly increased urinary clearance of uric acid (32.6-56.0ml/min), which was only minimally changed after both the administration of pyrazinamide, and inhibitor of the renal tubular secretion of uric acid, and the administration of probenecid, and...
Original Article
Pheochromocytoma in Children
Tae Sue Ha, Jae Hong Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Hyung Ro Moon, Gui Won Park
Clin Exp Pediatr. 1994;37(7):961-968.   Published online July 15, 1994
We Reviewed 10 hypertensive children with pheochromocytoma retrospectively and the following results were obtained. 1) Out of 10 patients, 7 were male and 3 female. Age ranged from 5.5 years to 13.8 years and their median age was 9.9 years. 2) They complained of sweating, lethargy, headache, or chest pain and so on, Hypertension were noticed in all patients. Heart murmurs were...
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