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Original Article
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
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...
A case of Menkes disease with unusual hepatomegaly
Go Un Jeong, Anna Cho, Hee Hwang, Yong Seung Hwang, Ki Joong Kim, Jong Hee Chae, Jeong Kee Seo
Clin Exp Pediatr. 2008;51(5):538-541.   Published online May 15, 2008
Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective- tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four- month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma,...
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
A case of hereditary pancreatitis with a N29I mutation in the cationic trypsinogen gene
Jee Youn Shin, You Jeong Kim, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(10):1067-1072.   Published online October 15, 2006
Purpose : The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. Methods : We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death...
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...
Helicobacter pylori reinfection rate by a 13C-urea breath test and endoscopic biopsy tests in Korean children
Jeong Ok Shim, Jeong Kee Seo
Clin Exp Pediatr. 2006;49(3):268-272.   Published online March 15, 2006
Purpose : The reinfection rate of H. pylori reported before 13C-urea breath test(13C-UBT) era was higher than that of the post 13C-UBT era. Children are usually reluctant to receive invasive endoscopic evaluation for the reinfection of H. pylori, particularly when they are asymptomatic. The aim of the study is to discover the reinfection rate by different diagnostic tests, and to...
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...
Review Article
Helicobacter pylori infection and abdominal pain in children
Jeong Kee Seo
Clin Exp Pediatr. 2006;49(2):136-143.   Published online February 15, 2006
The relationship between H. pylori(Hp) infection and recurrent abdominal pain(RAP) in children is not clear. Current data in the literature regarding a causal relationship between Hp infection and childhood RAP are conflicting. However, meta-analysis and most of the recently published studies have not supported an association between Hp infection and an increased prevalence of abdominal pain. Most published studies have...
Case Report
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...
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...
Henoch-Scholein Purpura Presenting with Acute Abdominal Pain Preceding Skin Rash : Review of 23 Cases
Ju Young Chang, Yong Joo Kim, Kyo Sun Kim, Hee-Ju Kim, Jeong Kee Seo
Clin Exp Pediatr. 2003;46(6):576-584.   Published online June 15, 2003
Purpose : For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. Methods : The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. Results : The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median...
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...
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...
Clinical Profiles and Anatomic Classification of Intestinal Malrotation
Jung A Kim, Jae Seong Ko, Jeong Kee Seo, Kwi Won Park, Woo Sun Kim, In One Kim
Clin Exp Pediatr. 1999;42(4):526-534.   Published online April 15, 1999
Purpose : Intestinal malrotation is a developmental anomaly and may be complicated by volvulus and intestinal necrosis. We performed anatomic classification and compared clinical profiles to understand their relationship. Methods : Twenty-eight children were diagnosed as intestinal malrotation at Seoul National University Children's Hospital between Jan. 1980 to Dec. 1995. The patients were classified into 4 groups by age at...
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)...
Case Report
A Case of Intestinal Amebiasis with Protein Losing Enteropathy
Chan Young Pak, Hee Taeg Kim, Soo Young Choi, Yun Jong Kang, Yeon Chung Chung, Jin Keun Ghang, Jeong Kee Seo
Clin Exp Pediatr. 1997;40(10):1458-1464.   Published online October 15, 1997
Amebiasis is an infectious disease caused by Entameba histolytica. Amebiasis remains an extremely important consideration in the differential diagnosis of diarrhea, especially when there is associated bleeding. It is imperative that appropriate studies to establish or exclude the diagnosis of amebiasis be carried out in all patients who present with a clinical and sigmoidoscopic picture of colitis, and that patients treated with metronidazole for amebiasis...
A Case of Ischemic Enteritis
Ran Lee, Jeong Kee Seo, Kwi won Park, Jong Je Kim
Clin Exp Pediatr. 1997;40(2):254-259.   Published online February 15, 1997
Ischemic enteritis is caused by embolism or thrombosis of superior mesenteric artery and nonocclusive ischemia. Mesenteric venous thrombosis, drugs, and vasculitis are less frequent etiologic factors. In children, occlusion of microcirculation by fibrin thrombi initiated by endotoxemia may be an etiology. Severe abdominal pain, vomiting, and diarrhea with evidence of gross or microscopic bleeding are common presenting symptoms. Angiography may be...
Original Article
Caustic Esophagitis and Gastrofiberoscopy in Children
Hyeon Jeong Kim, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(11):1556-1564.   Published online November 15, 1996
Purpose : There have been many accidental ingestions of strong acid or alkali in early childhood. If the patient survives the acute effects of caustic ingestion, the reparative response can result in esophageal and gastric stenosis. However there have been few endoscopic studies on caustic esophagitis in children. The aim of this study was to review the contribution of the endoscopy to the diagnosis...
The Diagnostic Value of Liver Biopsy in Children with Fever of Unknown Origin and Hepatosplenomegaly
Sue Mee Park, Yeon Ho Choi, Jong Jae Kim, Hoan Jong Lee, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(11):1544-1555.   Published online November 15, 1996
Purpose : Although many strides have been made in the radiological and laboratory diagnosis, the liver biopsy is still considered an important tool for the diagnosis of liver disease. We report our experience that histologic investigation of the liver was essential searching for the etiologic diagnosis in eight children with fever of unknowm origin and hepatosplenomegaly, who's diagnosis were not documented by other diagnostic...
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