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Case Report
A Case of Wernicke's Encephalopathy which Occurred During Chemotherapy in a Child with Acute Myeloblastic Leukemia
Sang-Nam Bae, Bu-Jin Kim, Seong-Shik Park, Sang-Ook Nam, Young-Tak Lim, Hak-Jin Kim
Clin Exp Pediatr. 2001;44(11):1320-1325.   Published online November 15, 2001
Wernicke,s encephalopathy(WE), a neurological disorder caused by thiamine deficiency, is characterized by the triad of ocular symptoms, ataxia, and mental confusion. More than 90% of the cases are observed in chronic alcoholics. Other conditions less frequently associated with WE are anorexia nervosa, prolonged parenteral nutrition, hemodialysis, uremia, hyperemesis gravidarum, gastroplasty for morbid obesity, and acquired immunodeficiency syndrome(AIDS). One of the...
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...
Two Cases of Disseminated Intravascular Coagulation Due to Status Epilepticus with High Fever
Song Heui Shin, Kyung-Rye Moon, Eun-Young Kim, Young-Il Rho
Clin Exp Pediatr. 2001;44(9):1062-1065.   Published online September 15, 2001
Status epilepticus has been occasionally reported as a cause of acute disseminated intravascular coagulation(DIC), but little is known regarding the pathogenesis of this uncommon association. The occurrence of DIC in status epilepticus may be related to widespread endothelial damage secondary to seizure-induced hyperpyrexia or status epilepticus due to high fever. We experienced two cases of status epilepticus accompanying fever in which laboratory data demonstrated...
Malignant Ectomesenchymoma in a Two-Month-Old Boy
Ji Young Hwang, Ji Hyoun Seo, Jeum-Su Kim, Young-Suk Kim, Sue-Jin Lee, Chan-Hoo Park, Yoo Kyung Kim, Sun Hoo Park, Jung Hee Lee, In Oak Ahn, Ki Hyun Chung, Byung-Kiu Park
Clin Exp Pediatr. 2001;44(8):959-964.   Published online August 15, 2001
Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The...
Original Article
The Usefulness of Magnetic Resonance Cholangiography in the Diagnosis of Biliary Atresia
Seok Joo Han, Ki Sup Chung, Myung-Joon Kim, Airi Han, Eui Ho Hwang
Clin Exp Pediatr. 2001;44(8):899-907.   Published online August 15, 2001
Purpose : To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods : Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was...
M-mode Echocardiographic Abnormalities in Infants of Diabetic Mothers
Myung Chul Hyun
Clin Exp Pediatr. 2001;44(8):881-885.   Published online August 15, 2001
Purpose : The purpose of this study was to evaluate M-mode echocardiographic abnormalities in infants of relatively well controlled diabetic mothers and to determine the timing of spontaneous regression. Methods : Echocardiographic study was prospectively carried out in 9 neonates born to diabetic mothers(infants of diabetic mothers, IDM) and 6 newborn infants(control) matched for weight and age. And the study were...
Case Report
A Case of Aseptic Meningitis Following Administration of Intravenous Immunoglobulin in A Patient with Idiopathic Thrombocytopenic Purpura
Su Jin Kim, Ji Eun Kim, Hei Won Hwang, Ji Sun Mok, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi, Woo Taek Kim
Clin Exp Pediatr. 2001;44(7):823-826.   Published online July 15, 2001
Intravenous immunoglobulin(IVIG) has been widely used to treat idiopathic thrombocytopenic purpura in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. This report is on an 11 year-old boy with ITP who suffered from aseptic meningitis following the administration of IVIG. He was given 1 g/kg of IVIG for 2 days, and on the fourth day...
Original Article
The Changes of Biochemical Bone Markers in Pediatric Epileptic Children Who Received Long-term Antiepileptic Drugs and Usefullness Thereof
Eun Kyung Choi, Kyung Hee Kim, Sun Jun Kim
Clin Exp Pediatr. 2001;44(7):778-785.   Published online July 15, 2001
Purpose : Long-term administration of anticonvulsants for pediatric epileptic patients may cause changes of bone metabolism. This study was performed to evaluate the influence of antiepileptics on the bone metabolism and growth in pediatric patients who received antiepileptics. Methods for early detection of adverse bone metabolism were also evaluated. Methods : Forty-eight epileptic patients were enrolled in this study....
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....
Original Article
A Clinical Study for Risk Factors of Cystic Periventricular Leukomalacia
Ji Eun Kim, Su Jin Kim, Woo Taek Kim
Clin Exp Pediatr. 2001;44(6):627-634.   Published online June 15, 2001
Purpose : It is very important to study the individual risk factors which cause periventricular leukomalacia(PVL) and those which affect the process of cystic formation from liquefied periventricular white matter, in order to prevent and permit early diagnosis of cystic PVL that contributes to mortality and neurologic sequelaes in preterm infants. Therefore, the present study was designed to assess the...
Case Report
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...
Original Article
Surgically Treated Intractable Child Epilepsy with Focal Cortical Dysplasia : Clinical and Electroencephalographic Findings
So Hee Chung, Ho Young Lee, Mun Hyang Lee, Hye Kyung Yoon, Bo Kyung Kim, Seung Chyul Hong
Clin Exp Pediatr. 2001;44(5):552-561.   Published online May 15, 2001
Purpose : Widespread use of MRI now gives us increased insights into the different expressions of cortical malformations. We carried out this study to characterize the clinical and EEG features of focal cortical dysplasia(FCD) which also caused intractable epilepsy requiring surgical treatment. Methods : A retrospective analysis was conducted in 8 children. The history of seizures, imaging studies, electroencephalographic findings,...
Neuro-developmental Assessment of Patients with Transposition of the Great Arteries by Localized 1H-Magnetic Resonance Spectroscopy
Ki-Soo Kim, Jee-Yeon Min, So Young Yoon, Young Hwue Kim, Jae Kon Ko, In Sook Park, Ki-Soo Kim, Dong Man Seo, Sang-Tae Kim, Jung Hee Lee, In Kyung Sung
Clin Exp Pediatr. 2001;44(5):533-544.   Published online May 15, 2001
Purpose : The purpose of this study was to evaluate the hemodynamic effect of transposition of the great arteries(TGA) on neuro-development by measuring the cerebral metabolites before and 1 year after open heart surgery(OHS) by localized in vivo 1H-magnetic resonance spectroscopy(1H- MRS) along with Bayley scales of infants development II(BSID II). Methods : Full-term newborns(N=13) with TGA and intact ventricular septum...
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...
The Relationship between Change of Cardiac Function and Systemic Oxygen Consumption according to the Dose of Dopamine in the Rabbit
Hee Ju Park, Seok Ho Yoon, Bong Seok Choi, Jung Hwoi Cho, Ku Tae Kim, Sun Young K, Sang Ook Nam, Bok Luel Lee
Clin Exp Pediatr. 2001;44(2):177-184.   Published online February 15, 2001
Purpose : We'd like to know the relationship between the changes of cardiac function and systemic O2 consumption according to the increasing dose of dopamine. Methods : Ten rabbits(from 2kg to 2.8kg) were used in this experiment. Anesthesia was induced with intraperitoneal pentobarbital sodium(35mg/kg) and tracheostomy was done. It was maintained by ventilation with a mixture of 1-3% halothane and 67-69%...
Study for Balloon Aortic Valvuloplasty in Congenital Aortic Stenosis
In Seung Park, Do Jun Cho, Mi Young Han, Jae Young Lee, Soo Jin Kim, Mee-Hye Oh, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2001;44(2):167-176.   Published online February 15, 2001
Purpose : The aims of this study are to investigate the results of balloon aortic valvuloplasty (BAV) in congenital aortic stenosis(CAS) and, especially, to compare the results between BAV performed before two months of age(Group A) and BAV after two month of age(Group B). Methods : From January 1993 to June 2000, 14 patients who were diagnosed as CAS were treated...
Clinical Features of Hemolytic Uremic Syndrome in Children
Ju Eun Lee, Su Yung Kim
Clin Exp Pediatr. 2000;43(11):1473-1479.   Published online November 15, 2000
Purpose : Hemolytic uremic syndrome(HUS) is the most common cause of renal failure in childhood in western countries, but it has been a relatively rare disease in Korea. To analyze the clinical and epidemiologic characteristics of HUS in Korea, we reporte our experience. Methods : We reviewed retrospectively the medical records of 9 patients who were diagnosed the hemolytic uremic syndrome...
Short-term Effects of Inhaled Corticosteroid Following Early Systemic Corticosteroid Administration in Premature Babies with Respiratory Distress Syndrome : A Preliminary Report
Hyeon-Soo Lee
Clin Exp Pediatr. 2000;43(11):1440-1445.   Published online November 15, 2000
Purpose : To asses the short-term effects of the topical corticosteroid, budesonide administered by metered dose inhaler(MDI) and spacer(Aerochamber MV 15) following systemic corticosteroid in premature babies with respiratory distress syndrome at birth. Methods : 19 premature babies <1800gm were randomly assigned to receive steroids(n=9) or not (n=10). The steroid group(n=9, GA[gestational age]=29.6¡¾2.9 weeks, BW[birth weight]=1.29¡¾0.35kg) received systemic dexamethasone for 48...
Epidemiologic Characteristics of T Serotyping in Relation to the Outbreak of Erythromycin Resistant Streptococcus pyogenes
Sung-Ho Cha, Kyu Jam Hwang, Young Hee Lee
Clin Exp Pediatr. 2000;43(10):1323-1329.   Published online October 15, 2000
Purpose : The identification of antigenic specificity of Streptococcus pyogenes using T serotyping is important to understand biologic characteristics of microorganisms. We would like to disover the association of the occurrence of predominant T type, with possible outbreak of erythromycin resistant Streptococcus pyogenes in this country, which has been documented since the late 1990s. Methods : Throat swab cultures were taken...
Case Report
A Case of Sporadic Caffey`s Disease(Infantile Cortical Hyperostosis) Without Mandibular Involvement
Byoung Lae Oh, Seung Woo Paik, Wan Seob Kim
Clin Exp Pediatr. 2000;43(9):1294-1299.   Published online September 15, 2000
Caffey`s disease(infantile cortical hyperostosis) is an uncommon benign and self limited disorder of unknown etiology, affecting the skeleton and its contiguous fasciae and muscles. Its occurrence in isolated cases or in multiple members in families suggests the existence of two different forms, namely a sporadic form and a familial form. The tibia is the predominant bone known to be affected...
Original Article
A Clinical Study of Acute Symptomatic Seizures in Children
Kyeong Eun Lee, Won Seop Kim
Clin Exp Pediatr. 2000;43(9):1254-1262.   Published online September 15, 2000
Purpose : To determine the incidence, etiology, sex, age, seizure type and prognosis of acute symptomatic seizures in children and evaluate the hypothesis that acute symptomatic status epilepticus( SE) is associated with an increased risk of subsequent unprovoked seizure compared with the risk of acute symptomatic seizure without SE. Methods : Five hundred and sixty-eight convulsive children visited the Pediatric Department of Chungbuk National University...
Case Report
A Case of Relapsed Acute Promyleocytic Leukemia Induced Remission with Arsenic Trioxide(As2O3)
Jin Hye Park, Heung Sik Kim, Chin Moo Kang
Clin Exp Pediatr. 2000;43(8):1132-1136.   Published online August 15, 2000
Acute promyelocytic leukemia(APL) is a unique entity in the spectrum of acute myelogenous leukemia. It has several characteristic features, including distinctive morphology, chromosomal translocation, t(15:17), disseminated intravascular coagulation and effect on retinoic acid. Retinoic acid which is a derivative of vitamin A induces differentation of APL cells in vitro and in vivo, but its cessation induces relapse of APL. Arsenic...
Two Cases of Left Ventricular Tumors Complicating Subaortic Stenosis in Newborn Infants
Ji Seon Kang, Mi Jung Hwang, Tae Hyoung Cho, Byung Ju Kim, Young Jong Woo, Jae Sook Ma
Clin Exp Pediatr. 2000;43(8):1122-1126.   Published online August 15, 2000
The most common obstruction of left ventricular outflow in childhood is produced either by a fibrous ring below the aortic valve, or by localized or diffuse muscular hypertrophy of the interventricular septum. Cardiac tumors causing subaortic stenosis in the newborn infant are extremely rare. This report describes two cases of subaortic stenosis caused by a tumor of the left ventricle...
A Case of Korean Patient with Nonketotic Hyperglycinemia; Diagnosed Based on CSF Amino Acid Analysis and Magnetic Resonance Spectroscopy
Kie-Young Park, Ai-Rhan Kim, Ki-Soo Kim, Soo-Young Pi, Tae-Sung Ko, Jung-Hee Lee, Han-Wook Yoo
Clin Exp Pediatr. 2000;43(7):993-999.   Published online July 15, 2000
Nonketotic hyperglycinemia is an extremely rare congenital metabolic disorder, which is caused by the lack of a glycine cleavage system. The onset of hyperglycinemic symptom is during the neonatal or early infant period. Progressing grave neuromotor dysfunction is one of the main symptoms. They include myoclonic seizure, hiccup, apnea, decreased deep tendon reflex, lethargy and coma. The prognosis is mostly very poor. Furthermore, there...
Two Cases of Thin Basement Membrane Nephropathy presented with Minimal Change Nephrotic Syndrome
Young Mee Seo, Jae Gul Chung, En Sil Yu, Jin Yeong Jeong, Young Seo Park
Clin Exp Pediatr. 2000;43(7):978-982.   Published online July 15, 2000
Thin basement membrane nephropathy(TBMN) is defined histologically as follows : 1) By light microscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement membrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport`s syndrome and systemic diseases that may affect the...
Original Article
A Study of Clinical Features of Acute Tumor Lysis Syndrome in Children
Ick-Jin Song, Young-Tak Lim
Clin Exp Pediatr. 2000;43(7):952-958.   Published online July 15, 2000
Purpose : Tumor lysis syndrome is characterized by hyperuricemia, hyperphosphatemia, hyperkalemia and hypocalcemia due to destruction of tumor cells. The purpose of this study is to estimate in children the incidence, onset time, differences in outcomes between hemodialysis and conservative therapy, and predictive factor of tumor lysis syndrome before treatment with chemotherapy. Methods : Subjects were 108 children who had received...
Natural History and Clinical Feature of Multicystic Dysplastic Kidney detected with Prenatal Ultrasonography
Young Lim Shin, Hye Sung Won, Jong Hyun Yoon, Young Seo Park
Clin Exp Pediatr. 2000;43(7):926-932.   Published online July 15, 2000
Purpose : Multicystic dysplastic kidney(MCDK) is characterized by the presence of multiple cysts of varying sizes. It is associated with ureteral or pelviureteral atresia, and a non-functioning kidney. The purpose of this study was to examine the clinical feature and the natural history of MCDK. Methods : A study was performed in 36 children with prenatal diagnosis of MCDK from 1989 to 1999. All...
Usefulness of Plasma Atrial Natriuretic Peptide Concentration in the Diagnosis of Patent Ductus Arteriosus in Preterm Infants
Byung Min Choi, Jae Kyun Yoon, Hyun Hee Lee, Hae Won Cheon, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 2000;43(7):897-904.   Published online July 15, 2000
Purpose : Diagnosis of a hemodynamically significant patent ductus arteriosus(PDA) that should be treated is difficult to determine by clinical and echocardiographic examinations. The aim of this study was to clarify the interrelationship of plasma atrial natriuretic peptide(ANP) concentrations and clinical signs or echocardiographic signs of PDA in preterm infants and use plasma ANP concentrations as a diagnostic indicator of a hemodynamically significant PDA. Methods...
Case Report
The Management of Three Infants with Critical Valvular Aortic Stenosis by Transcarotid Balloon Aortic Balvuloplasty
Kie-Young Park, Young-Hwue Kim, Ji-Yeon Min, Jae-Kon Ko, In-Sook Park, Chang-Yee Hong, Tae-Jin Yun, Dong-Man Seo
Clin Exp Pediatr. 2000;43(6):820-827.   Published online June 15, 2000
Critical valvular aortic stenosis(AS) is a life threatening congenital heart anomaly in neonates or infants. The main cause of death is rapidly progressing left ventricular failure. The treatment goal of critical AS is to save left ventricular function early. Before the 1980s, the preferred treatment was surgical valvotomy, however, that operative method had a high mortality rate and risk of...