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Original Article
Oncology
Posttransplantation lymphoproliferative disorder after pediatric solid organ transplantation: experiences of 20 years in a single center
Hyung Joo Jeong, Yo Han Ahn, Eujin Park, Youngrok Choi, Nam-Joon Yi, Jae Sung Ko, Sang Il Min, Jong Won Ha, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Clin Exp Pediatr. 2017;60(3):86-93.   Published online March 27, 2017
Purpose

To evaluate the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after solid organ transplantation (SOT) in children.

Methods

We retrospectively reviewed the medical records of 18 patients with PTLD who underwent liver (LT) or kidney transplantation (KT) between January 1995 and December 2014 in Seoul National University Children's Hospital.

Results

Eighteen patients (3.9% of pediatric SOTs; LT:KT, 11:7; male to female, 9:9) were diagnosed...

Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants
Ahlee Kim, Jin Soo Moon, Hye Ran Yang, Ju Young Chang, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2015;58(1):28-32.   Published online January 31, 2015
Purpose

Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order...

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...

Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis
Jeana Hong, Mi Kyoung Song, Jae Sung Ko, Gyeong Hoon Kang, Woo Sun Kim, Jeong Kee Seo
Clin Exp Pediatr. 2009;52(4):504-507.   Published online April 15, 2009
Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of...
Original Article
Usefulness of early endoscopy for predicting the development of stricture after corrosive esophagitis in children
Ji Yong Park, Jee Youn Shin, Hye Ran Yang, Jae Sung Ko, Woo Sun Kim, Jeong Kee Seo
Clin Exp Pediatr. 2009;52(4):446-452.   Published online April 15, 2009
Purpose : This study was performed to demonstrate the usefulness of early endoscopy for predicting the development of stricture following corrosive ingestion in children. Methods : We conducted a retrospective study on 34 children who were brought to Seoul National University Childrens Hospital and Seoul National University Bundang Hospital for corrosive ingestion from 1989 to 2007. Results : The corrosive burns were...
Case Report
A case of simultaneously identified glycogen storage disease and mucopolysaccharidosis
Ju Young Lee, Jeong Ok Shim, Hye Ran Yang, Ju Young Chang, Choong Ho Shin, Jae Sung Ko, Jeong Kee Seo, Woo Sun Kim, Gyeong Hoon Kang, Jeong Han Song, Jong Won Kim
Clin Exp Pediatr. 2008;51(6):650-654.   Published online June 15, 2008
Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which...
Erratum
Assessment of modifiable lifestyle factors for obese children and adolescents through questionnaires
Jeong Wan Seo, Ji A Jung, Hye Sook Park, Jae Sung Ko, Yong Joo Kim, Jae Young Kim, Eell Ryoo, Sun Hwan Bae, Jae Geon Sim, Hye Ran Yang, Byung Ho Choe, Ky Young Cho
Clin Exp Pediatr. 2008;51(6):576-583.   Published online June 15, 2008
Purpose : The identification of specific behaviors conducive to overeating or inactivity is the cornerstone of obesity management. The Committee on Nutrition of the Korean Pediatric Society developed parent and self-reporting questionnaires about eating behavior and physical activity in 2006. The aim of this study was to evaluate the usefulness of the questionnaires in assessing modifiable lifestyle factors related to...
Review Article
The etiologies of neonatal cholestasis
Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2007;50(9):835-840.   Published online September 15, 2007
Any infant noted to be jaundiced at 2 weeks of age should be evaluated for cholestasis with measurement of total and direct serum bilirubin. With the insight into the clinical phenotype and the genotypephenotype correlations, it is now possible to evaluate more precisely the neonate who presents with conjugated hyperbilirubinemia. Testing should be performed for the specific treatable causes of...
Case Report
A case of hereditary pancreatitis with a N29I mutation in the cationic trypsinogen gene
Jee Youn Shin, Dae Sung Oh, Jeong Min Rheu, Jeong Ok Shim, Ji Sook Park, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(10):1111-1115.   Published online October 15, 2006
Hereditary pancreatitis is an autosomal dominant disease characterized by recurrent episodes of pancreatitis, often beginning in childhood, with a positive family history involving at least two other affected family members with no known other precipitating factors. Most forms of hereditary pancreatitis are caused by one of two common mutations, i.e., R122H in exon 3 and N29I in exon 2 of...
A case of post-operative chylous ascites after a splenorenal shunt operation in a child with congenital hepatic fibrosis
Jong Hyung Yoon, Hye Ran Yang, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(10):1106-1110.   Published online October 15, 2006
Chylous ascites is a rare condition caused by various diseases and conditions that interfere with the abdominal or retroperitoneal lymphatics, and uncommonly it can manifest as a post-operative complication after abdominal, retroperitoneal or mediastinal surgery. Chylous ascites can be diagnosed by a high triglyceride content in ascites. The authors experienced a 5-year-old girl with congenital hepatic fibrosis who presented with...
Original Article
Intravenous immunoglobulin for severe gastrointestinal manifestation of Henoch-Sch nlein purpura refractory to corticosteroid therapy
Hye Ran Yang, Won Jung Choi, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(7):784-789.   Published online July 15, 2006
Purpose : Henoch-Schönlein purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods...
Jagged1 mutation analysis in Alagille syndrome patients
Jae Sung Ko, Hye Ran Yang, Kyung Mo Kim, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(5):519-522.   Published online May 15, 2006
Purpose : Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has been found mutated in Alagille syndrome. The aim of the study was to investigate the mutation analysis of JAG1 gene in Korean patients with Alagille syndrome. Methods : Genomic DNA...
Case Report
Gastric neurofibroma in von Recklinghausen disease : a cause of upper gastrointestinal bleeding
Bo Sang Kwon, Jeong Ok Shim, Jeong Kee Seo, Hye Ran Yang, Jae Sung Ko, Seong Eun Jung, Woo Sun Kim, Gyeong Hoon Kang
Clin Exp Pediatr. 2006;49(2):203-207.   Published online February 15, 2006
Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive...
Development of Crohn disease in patients with myelodysplastic syndrome : report of two children
Jeong Ok Shim, Jeong Kee Seo, Hye Ran Yang, Jae Sung Ko, Hee Young Shin, Hyo Seop Ahn, Woo Sun Kim, Gyeong Hoon Kang
Clin Exp Pediatr. 2006;49(1):107-111.   Published online January 15, 2006
Crohn disease (CD) is rare, but the incidence of CD has been increasing over the past ten years. We found two cases of CD, associated with myelodysplastic syndrome (MDS), for the first time in children. In the first patient, MDS was diagnosed at three years of age and CD developed later at eight years of age. The patient presented with...
Original Article
Clinical Findings of Genotypes in Korean Patients with Glycogen Storage Disease Type Ia
Jae Sung Ko, Hye Ran Yang, Jong Won Kim, Jeong Kee Seo
Clin Exp Pediatr. 2005;48(8):877-880.   Published online August 15, 2005
Purpose : Glycogen storage disease type Ia(GSD Ia) is an autosomal recessive disorder caused by the deficiency of glucose-6-phosphatase(G6Pase). The aim of the study was to investigate the spectrum of G6Pase gene mutations and relationship between genotype and clinical findings in Korean patients with GSD Ia. Methods : Genomic DNA was extracted from peripheral leukocytes of 20 patients with GSD...
Medical Lecture Course
Mechanisms of Action of Probiotics
Jae Sung Ko
Clin Exp Pediatr. 2005;48(7):691-695.   Published online July 15, 2005
There is scientific evidence that administration of probiotics is effective in the treatment of acute infectious diarrhea in children and the prevention of antibiotic associated diarrhea and nosocomial/ community acquired diarrhea. Probiotics prevent relapse of recurrent pouchitis and decrease the initial onset of pouchitis in ulcerative colitis. Probiotic organisms suppress growth of pathogens as well as their epithelial attachment and/or...
Original Article
Correlation of Body Mass Index, Body Fat Distribution, Aminotranferases and Computed Tomography in Obese Children with Fatty Liver
So Eun Park, Hye Ran Yang, Ju Young Chang, Jae Sung Ko, Jeong Kee Seo, Whal Lee, Woo Sun Kim
Clin Exp Pediatr. 2005;48(3):276-283.   Published online March 15, 2005
Purpose : Visceral fat accumulation plays a major role in metabolic complications of obesity. It is known that nonalcoholic fatty liver in obese adults is associated with visceral fat accumulation. Body mass index(BMI) is used as the index of obesity in children. The aim of this study is to evaluate the correlation of BMI and visceral adipose tissue(VAT), and the...
A Follow-up Survey of Mothers' Antenatal Breast-feeding Plans
Hae Soon Kim, Jeong Wan Seo, Yong Joo Kim, Kee Hyoung Lee, Jae Young Kim, Jae Sung Ko, Sun Hwan Bae, Hye Sook Park
Clin Exp Pediatr. 2003;46(7):635-641.   Published online July 15, 2003
Purpose : To investigate the success rate and factors that influence breast-feeding among women having antenatal breast-feeding plans. Methods : Questionnaires included items on success in breast-feeding, reasons for failure and perinatal factors. It was done by telephone calls to 152 randomly selected women having antenatal breast-feeding plans at 4 months after delivery. The questionnaires were analysed by bivariate χ2-...
A Survey on the Understanding of Breast-feeding in Pregnant Woman
Jeong Wan Seo, Yong Joo Kim, Kee Hyoung Lee, Jae Young Kim, Jay G Sim, Hae Soon Kim, Jae Sung Ko, Sun Hwan Bae, Hye Sook Park, Beom Soo Park
Clin Exp Pediatr. 2002;45(5):575-587.   Published online May 15, 2002
Purpose : To investigate the understanding of breast-feeding in pregnant woman and the proper way of encouraging breast-feeding. Methods : Each questionnaire included items about demographic characteristics and the understanding of breast-feeding. The questionnaires were filled up by pregnant women visiting obstetric clinics in Seoul and its vicinities, Busan, Choongjoo and Chungjoo from July 2001 to August 2001. One thousand, two...
Clinical Lecture
Hepatitis in Children
Jae Sung Ko
Clin Exp Pediatr. 2002;45(5):553-559.   Published online May 15, 2002
Original Article
Therapeutic Efficacy of Cefotaxime as an Empirical Antibiotic on Ascending Cholangitis after Kasai Operation for Biliary Atresia
Dong Ho Kim, Byong Sop Lee, Yun Kyung Kim, Jae Sung Ko, Hoan Jong Lee, Jeong Kee Seo
Clin Exp Pediatr. 2002;45(4):474-481.   Published online April 15, 2002
Purpose : Ascending cholangitis is the most common complication after Kasai operations. The aim of this study is to evaluate the therapeutic efficacy of cefotaxime as an empirical antibiotic on ascending cholangitis after Kasai operations. Methods : Thirty-nine episodes of cholangitis in twenty-nine children who underwent Kasai operations at Seoul National University Children's Hospital from January 1991 to December 2000 were...
Case Report
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...
Original Article
A Study on the Disease Course and Prognosis of Cyclic Vomiting Syndrome
Hye Ran Yang, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2001;44(10):1141-1156.   Published online October 15, 2001
Purpose : To describe clinical features of cyclic vomiting syndrome(CVS), managements during episodes, prophylactic drugs as long term therapy and to investigate the disease course and prognostic factors of CVS. Methods : Thirty two children who fulfilled the diagnostic criteria of CVS and who presented to Seoul National University Children's Hospital from March 1989 to December 2000, were included. Data were...
Long-term Clinical Follow-up of Korean Children with Wilson Disease; Twenty Years` Experience
Jin Soo Moon, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2001;44(2):127-138.   Published online February 15, 2001
Purpose : To investigate the clinical features of long-term follow-up, and the treatment responses in Korean children with Wilson disease(WD). Methods : The clinical data from ninety-two children with WD, diagnosed at Seoul National University Hospital from 1976 to 1999, were reviewed. Mean duration of follow-up was 5¡¾4.5 years. We analysed the patient's records based on the clinical manifestations, laboratory findings,...
Congenital Chloride Diarrhea in 5 Korean Infants
Dong Hun Rhie, Sun Hwan Bae, Ji Eun Choi, Bo Young Yun, Dong Woo Son, Choong Ho Shin, Kyung Hee Park, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2000;43(11):1465-1472.   Published online November 15, 2000
Purpose : Congenital chloride diarrhea(CLD) is an autosomal recessive disease characterized by life-long watery diarrhea of prenatal onset with high fecal Cl－ concentration. Recent studies have revealed that the protein product of the down-regulated in adenoma(DRA) gene is an intestinal anion transporter molecule and causes CLD when mutated. We investigated the clinical characteristics of CLD in Korean infants in order...
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...
A Clinical Observation on Lupus Nephritis in Children
Kang Mo Ahn, Jae Sung Ko, Hye Won Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Hee Joo Kim
Clin Exp Pediatr. 1994;37(6):842-849.   Published online June 15, 1994
We reviewed the clinical features, histrologic patterns and clinical courses of 30 children with lupus nephritis retrospectively, and the results were summerized as follows; 1) The male to female ratio was 1:2.8, and the mean age at the onset was 108/12 years. 2) The clinical symptoms were diverse, and malaise, weight loss, anorexia, fever and malar rash were the most frequent findings. 3)...
Nephrotic Syndrome under 2 Years of Age
Jae Sung Ko, Kang Mo Ahn, Hye Won Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1993;36(10):1395-1401.   Published online October 15, 1993
There had been total 20 patients with early onset (4 months~2 years) primary nephrotic syndrome in the Deparment of Pediatrics, Seoul National University Children's Hospital, during the period from March 1987 to February 1993. We analysed clinical courses, response to treatment, pathological findings and prognosis of the patients And the results were as follows; 1) The initial responders to steroid treatment...