• Search
  • Advanced Search

Search

  • HOME
  • Search
Original Article
Electrocardiographic and Echocardiographic Characterisitics of Wolff-Parkinson-White Syndrome in Preschool Children
Jeoung Min Chu, Hyun Sup Sim, Soo Chul Cho, Chan Uhng Joo
Clin Exp Pediatr. 2002;45(9):1097-1105.   Published online September 15, 2002
Purpose : This study was conducted to estabilish the prevalence, clinical features and relationship between ECG findings and echocardiographic findings of Wolff-Parkinsion-White(WPW) syndrome in asymptomatic preschool children. Methods : An electrocardiographic screening study was performed on 77,824 preschool children in Jeonbuk province from April, 1999 to August, 2001. Patients with WPW syndrome underwent echocardiographic study. Results : Twenty three patients with WPW...
Sudden Unexpected Death in Infancy (Analysis of 34 Cases Including 13 Autopsies)
Yeo Ok Moon, Hee Kyoung Choi, Jeoung-A Her, Woo Jong Shin, Myoung-A Kim, Seong Yong Lee, Seong Hee Jang, Eun Sil Dong, Chong Jae Kim, Young Min Ahn, Je Geun Chi
Clin Exp Pediatr. 2002;45(9):1065-1074.   Published online September 15, 2002
Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical...
Case Report
Atypical Kawasaki Disease Presented with Toxic Shock Syndrome
Kyoung Yeon Lee, Jun Eun Park, Woo Sung Park
Clin Exp Pediatr. 2002;45(8):1048-1051.   Published online August 15, 2002
Toxic shock syndrome(TSS) is clinically similar to Kawasaki disease(KD) in that both of them are characterized by fever, desquamating rash and mucous membrane erythema. In contrast the main feature of TSS is hypotension, whereas the complication of KD is coronary vasculitis. We report an 8-year-old boy who fulfilled the crireria for TSS and KD. Initially he showed clinical features of...
A Case of Marshall-Smith Syndrome
Yeo Ok Moon, Woo Jong Shin, Youn Jeong Shin, Eun Sil Dong, Young Min Ahn
Clin Exp Pediatr. 2002;45(7):906-911.   Published online July 15, 2002
Marshall-Smith syndrome is characterized by a triad of facial dysmorphism, failure to thrive and accelerated osseous maturation. We report a one-month-old male infant with of this rare syndrome, with laryngeal anomalies who died at 6 months of age with pneumonia. This is the first case of Marshall-Smith syndrome in Korea.
Original Article
Renal Anomalies in Children with Turner Syndrome
Ji Young Kim, Sun Young Hong, Young Mi Park, Yong Hoon Park, Woo Yeong Chung
Clin Exp Pediatr. 2002;45(7):891-895.   Published online July 15, 2002
Purpose : The prevalence of renal anomalies in Turner syndrome(TS) has been reported to vary from 33% to 60%. The purpose of this study was to clarify the true incidence of renal malformations in Korean TS. Methods : We evaluated 33 patients with Turner syndrome diagnosed by karyotype in Inje University Busan Paik hospital and Youngnam University from January 1995....
Thymus Size and Its Relationship to Perinatal Diseases; Respiratory Distress Syndrome and Intrauterine Growth Retardation
Sun Mi Chung, Woo Taek Kim
Clin Exp Pediatr. 2002;45(7):855-861.   Published online July 15, 2002
Purpose : Thymus size can be affected by several factors and perinatal diseases can be estimated by its size. The purpose of this study was to search for a relationship between cardiothymic/ thoracic(CT/T) ratio and perinatal diseases such as neonatal respiratory distress syndrome(RDS) and intrauterine growth retardation(IUGR) by measuring the width of the cardiothymic shadow at the level of the...
Case Report
Hemophagocytic Syndrome with Kawasaki Disease and Peripheral Gangrene
Hwa Jun Yun, Ko Woon Jeon, Hwang Min Kim, Seok Won Park, Young Uh
Clin Exp Pediatr. 2002;45(5):664-668.   Published online May 15, 2002
A twenty six months-old boy developed hemophagocytic syndrome during the course of Kawasaki disease. Despite the appropriate treatment modalities for Kawasaki disease, he developed thrombocytopenia, hepatomegaly, high-grade fever, hypertriglyceridemia, peripheral gangrene, and evidence of hemophagocytosis in bone marrow biopsy. Although the course was stormy, he responded well to a combination therapy of corticosteroid and etoposide.
A Case of Rett Syndrome with MECP2 Gene Mutation
Jin Kyung Kim, Chang Seok Ki, Jong Won Kim
Clin Exp Pediatr. 2002;45(4):540-544.   Published online April 15, 2002
Rett syndrome is an X-linked dominant, progressive neurodevelopmental disorder, with a prevalence estimated to be one in 10,000-15,000 girls, which is thought to be the second most common genetic causes of mental retardation in females after Down syndrome. Patients with classic Rett syndrome show an apparently normal neonatal period, followed by developmental regression and deceleration of head growth, accompanied by...
Two Cases of Gitelman's Syndrome Diagnosed by Renal Clearance Study
Tae Hwa Kim, Seung Jun Kim, Yu Kyung Seo, Jung-Yeon Shim, Hye Lim Jung, Moon Soo Park, Dong Hyuk Kum
Clin Exp Pediatr. 2002;45(3):413-417.   Published online March 15, 2002
Gitelman's syndrome is an autosomal recessive disorder characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria that has recently been reported to be linked to thiazide-sensitive Na-Cl cotransporter gene mutation. In this study, we performed renal clearance studies to differentiate Gitelman's from Bartter's syndrome and to confirm the diagnosis in two patients clinically diagnosed with Gitelman's syndrome. Each patient was hydrated...
A Case of Partial DiGeorge Syndrome in Prematurity
Tae Jung Sung, Eun Young Ko, Dal Hyon Kim, Ji Eun Oh, Young Se Kwon, Dae Hyun Lim, Byong Kwan Son
Clin Exp Pediatr. 2002;45(3):383-389.   Published online March 15, 2002
We experienced a case of partial DiGeorge syndrome in a 35＋5 week premature female infant presented with micrognathia, fish-shaped mouth, beaked nose, nasal regurgitation, obstructive sleep apnea, velopharyngeal insufficiency and late onset hypocalcemic seizures. The chromosome 22q11 microdeletion was found by the FISH method. The lab findings showed serum calcium level of 4.4 mg/dL, ionized calcium level of 0.49 mg/dL,...
Original Article
Short-term Results of Hematopoietic Stem Cell Transplantation for Children with Myelodysplastic Syndrome
Jin Lee, Soh Yeon Kim, Bin Cho, Pil Sang Jang, Nak Gyun Chung, Hack Ki Kim
Clin Exp Pediatr. 2002;45(3):370-375.   Published online March 15, 2002
Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods :...
Case Report
A False Diagnosis of Hypoplastic Left Heart Syndrome in a Fetus
Youn Jeong Shin, Sung Hee Jang, Jung Yun Choi, Eun Sook Han
Clin Exp Pediatr. 2002;45(2):267-272.   Published online February 15, 2002
Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe...
Original Article
Clinical Study of the Correlation of Tumor Necrosis Factor Alpha and the Proteinuria of Henoch-Schönlein Nephritis and Idiopathic Nephrotic Syndrome
Dong-Ho Jeong, Jeong-Hyun Park, Hye-Cheon Jeong, Hyun-Hoe Koo, Jun-Ho Lee, Tae-Sun Ha
Clin Exp Pediatr. 2002;45(2):240-246.   Published online February 15, 2002
Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha(TNF-α) is related to intrarenal or serum concentration of TNF-α. So, we studied the relationship between the concentration of TNF-α and aggravation of glomerular damage in the Henoch-Schönlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of...
Study on the Frequency of Receptor Insensitivity to Androgen Using the Androgen-receptor Binding Assay in the Genetic Male Children with Abnormalities of Sexual Differentiation
Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2002;45(1):95-102.   Published online January 15, 2002
Purpose : The development of external genitalia in genetic male is dependent on the transcriptional regulatory activity of dihydrotestosterone(DHT)-androgen receptor complex in the genital skin. The abnormality of androgen receptor encompasses a wide range of phenotypes. We investigated the androgen receptor binding capacity of genetic males with ambiguous genitalia(grade was determined by Prader grade) for the availability as screening test. Methods : The binding capacity...
Case Report
A Case of the Use of Intravenous Immunoglobulin of Treatment for HyperIgE Syndrome
Yoon Ji Choi, Dong IL Lee, Hong Bae Kim
Clin Exp Pediatr. 2001;44(11):1301-1304.   Published online November 15, 2001
Hyper-IgE syndrome is a rare congenital immune deficiency disease characterized by severe eczema, recurrent infection of the sinopulmonary tract, cold subcutaneous abscess and high serum IgE levels. It is an autosomal dominant disease with incomplete penetrance. We examined the case of a 12 month old infant with hyperIgE syndrome since neonate. The diagnosis of hyper IgE syndrome was made because...
Fanconi-Bickel Syndrome Presented with Diabetes Mellitus and Galactosemia : Identification of a Novel Mutation in the GLUT2 Gene
You-Jeong Kim, Sun-Hee Rim, Young-Lim Shin, Han-Wook Yoo
Clin Exp Pediatr. 2001;44(10):1201-1205.   Published online October 15, 2001
Fanconi-Bickel syndrome is a rare autosomal recessive disorder of the carbohydrate metabolism recently demonstrated to be caused by mutations in GLUT2, the gene for the glucose transporter protein 2 expressed in the liver, pancreatic β islet-cells, intestine and kidney. Typical clinical and laboratory findings of Fanconi-Bickel syndrome are hepatomegaly secondary to glycogen accumulation, glucose and galactose intolerance, fasting hypoglycemia, a...
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...
Case Report
Galloway-Mowat Syndrome in Two Siblings
Hae-Sung Jung, Eun-Young Cho, Jae-Young Lim, Ji-Hyeoan Seo, Myoung-Bum Choi, Chan-Ho Park, Hang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 2001;44(9):1081-1084.   Published online September 15, 2001
We report on two Korean siblings with multiple congenital anomalies : microcephaly, gyral abnormality, minor facial anomalies, and congenital nephrotic syndrome. The first infant developed proteinuria at age 3 days. This condition appeared similar to that described by Galloway and Mowat and reviewed by Cooperstone, et al, especially the presence of abnormal gyral patterns. She died at 19 months. The second infant; the brother...
A Case of Trisomy 9 Mosaicism Mimicking Smith-Lemli-Opitz Syndrome
Su Jin Kim, Jin Hwa Jeong, Sung Min Cho
Clin Exp Pediatr. 2001;44(9):1047-1051.   Published online September 15, 2001
Trisomy 9 mosaicism is a disease characterized not only by intrauterine growth retardation and mental retardation but also congenital heart defects, musculoskeletal, genitourinary and CNS anomalies, as well as craniofacial anomalies such as microcephaly, micrognathia, narrowed temples, prominent occiput, broad-based nose with bulbous tip, low set ears, deeply set eyes, short palpebral fissure and small mouth. This syndrome was first reported back in 1973...
A Case of Vascular Leak Syndrome During Induction Chemotherapy of Acute Lymphoblastic Leukemia(T Cell Type)
Kye Wool Kang, Hwang Min Kim, Won Kyu Choi, Baek Keun Lim, Chang Hun Lee
Clin Exp Pediatr. 2001;44(9):1041-1046.   Published online September 15, 2001
Vascular leak syndrome(VLS) is characterized by endothelial damage, which causes extravasation of plasma proteins and fluid from capillaries into the extravascular space. It has been suggested that the increased vascular permeability is the result of an IL-2-induced suppression of endothelin- 1 secretion by endothelial cells, an IL-2-induced activation of the complement cascade or TNF-αrelease from IL-2-activated T-cells. A 13-year-old male patient visited our hospital...
A Case of Apert's Syndrome with Encephalocele and Hypogenesis of Corpus Callosum
Min Kyoung Kwon, Ja Yun Yu, Mi Ran Kim, Kon Hee Lee, Hae Ran Lee, Kwang Nam Kim
Clin Exp Pediatr. 2001;44(7):832-836.   Published online July 15, 2001
Apert's syndrome(acrocephalosyndactyly) is a rare congenital anomaly that is characterized by the combination of premature fusion of multiple cranial sutures primarily involving the coronal suture and severe symmetrical syndactyly of fingers and toes. Some patients of this syndrome have various abnormalities of the central nervous system. We experienced a case of Apert's syndrome with craniofacial malformations, symmetric syndactyly, occipital encephalocele...
Original Article
Radiologic Findings of Hands in Turner Syndrome
Young Sim Han, Moo Young Oh, Young Joon Lee, Woo Yeong Chung
Clin Exp Pediatr. 2001;44(7):817-822.   Published online July 15, 2001
Purposes : Radiologic findings of hands in Turner syndrome(TS) which have been reported were metacarpal sign(MS), carpal sign(CS), phalangeal sign(PS), osteopenia, and delayed bone maturation. The aim of this study is to evaluate the difference of radiologic findings in TS, idiopathic short stature(ISS), and growth hormone deficiency(GHD). Methods : Sixty girls with short stature were studied for chromosome analysis, and...
Childhood Obstructive Sleep Apnea Syndrome : Clinical and Polysomnographic Findings
Yang Jun Park, Dae Kyun Koh, Jin Hee Oh, Soo Jung Lee
Clin Exp Pediatr. 2001;44(7):786-795.   Published online July 15, 2001
Purpose : Although pediatricians may see a lot of patients with clinically suspected obstructive sleep apnea syndrome(OSAS), the actual incidence, clinical and polysomnographic manifestations of OSAS in children have not well been recognized and reported cases are rare. The objective of this study was to evaluate the efficacy of polysomnography in the diagnosis of OSAS in children and to analyze...
Case Report
49, XXXXY Syndrome with Multicystic Kidney in a Neonate
Eun Young Park, Soo Jee Moon
Clin Exp Pediatr. 2001;44(6):714-717.   Published online June 15, 2001
The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hypogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases....
Zellweger Syndrome : First Confirmed Neonatal Case in Korea
Sung Sin Kim, Yun Hwa An, Yun Sil Chang, Dong Kyu Jin, Won Soon Park, Moon Hang Lee, Jong Won Kim, Hye Kyoung Yoon, Bo Kyoung Kim
Clin Exp Pediatr. 2001;44(6):694-698.   Published online June 15, 2001
We describe below a case of Zellweger syndrome case with facial dysmorphism, profound hypotonia, and hepatomegaly. He died at the age of 2 months. Zellweger syndrome is a disease marked by the absence of hepatic and renal peroxisomes. Because peroxisomes have many vital anabolic and catabolic functions within the cell, their absence results in profound cellular dysfunction. A biochemical study...
A Case of Klippel-Feil Syndrome
Hyun Ja Kim, Kang Ho Baek, Hyang Do Ko, Man Tak Oh
Clin Exp Pediatr. 2001;44(5):597-601.   Published online May 15, 2001
Klippel-Feil syndrome(KFS) consists of short neck, low posterior hairline and restriction of motion of the neck due to fusion of cervical vertebrae. The typical disorder results from a failure of the normal segmentation of mesodermal somites during 3-8 weeks of gestation. In 1912, the first complete clinical description of this syndrome was given by Klippel and Feil. Feil reported ...
An Infant with Vertical Transmission of Human Immunodeficiency Virus Infection Detected Due to Failure to Thrive
Byung Wook Eun, Ho Kyung Lim, Yun Kyung Kim, Yuong Ho Kwak, Jung Youn Hong, He Sun Jung, Kyung Mo Kim, Hoan Jong Lee
Clin Exp Pediatr. 2001;44(5):592-596.   Published online May 15, 2001
Five to fifteen percent of patients affected by human immunodeficiency virus(HIV) are children in developing countries. In Korea, most of HIV infections in children have been transfusion-related, and cases of vertical transmission have been very rare so far and are usually suspected due to a maternal positive history. We experienced a case of vertical transmission of HIV in a 19...
A Case of Maple Syrup Urine Disease Associated with Acrodermatitis Enteropathica-like Syndrome Due to Iisoleucine Deficinecy During Diet Therapy
Ki Hyang Moon, Oh Sook Gwon, Jung Im Lee, Seong Woo Rho, Seong Sook Jeon, Son Sang Seo
Clin Exp Pediatr. 2001;44(4):469-474.   Published online April 15, 2001
Maple syrup urine disease(MSUD) is an autosomal recessive disorder involving the metabolism of the branched-chain amino acids(BCAA) such as leucine, isoleucine and valine. The disorder is due to a defect in branched-chain α-ketoacid dehydrogenase(BCKAD) and the classic form causes rapid progressive and overwhelming illness beginning in the first weeks of life, present with poor feeding, lethargy, change in muscle tone,...
Original Article
Clinical Characteristics of Williams Syndrome
Choong Ho Shin, Ho Sung Kim, Sei Won Yang, Jung Yun Choi
Clin Exp Pediatr. 2001;44(4):443-449.   Published online April 15, 2001
Purpose : Williams syndrome(WS) is an autosomal dominant disorder resulting from a submicroscopic deletion of contiguous genes on the long arm of chromosome 7. We evaluated the physical, neurodevelopmental, and behavioral characteristics of children and adults with WS confirmed by fluorescence in situ hybridization. Methods : The clinically suspicious twelve cases with cardiac anomaly were confirmed as WS with fluorescence in...
Case Report
A Case of Streptococcal Toxic Shock Like Syndrome with Pleural Effusion
Jinyoung Song, Keunha Ji, Heeseuk Kim, Jungwoo Rhoo, Dongwook Kim, Jongkook Lee
Clin Exp Pediatr. 2001;44(2):200-204.   Published online February 15, 2001
Streptococcal toxic shock like syndrome is a rapidly progressive and a fatal disease like staphylococcal toxic shock syndrome. In spite of the wide expansion and development of potent antibiotics, streptococcal infection still threatens human being. Recently we experienced a patient with toxic shock like syndrome who was suffered from fever and neck pain with rapid progression to hypotension and multiorgan...
TOPICS

Browse all articles >

ARTICLE CATEGORY

Browse all articles >

BROWSE ARTICLES
FOR CONTRIBUTORS
ABOUT
Editorial Office
Korean Pediatric Society
#1606 Seocho World Officetel, 19 Seoun-ro, Seocho-ku, Seoul 06732, Korea
Tel: +82-2-3473-7306    Fax: +82-2-3473-7307    E-mail: office@e-cep.org                

Clinical and Experimental Pediatrics is an open access journal. All articles are distributed under the terms of the Creative Commons Attribution NonCommercial License (http://creativecommons.org/licenses/by-nc/4.0/)

Copyright © 2026 by Korean Pediatric Society.      Developed in M2PI