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Volume 42(7); Jul 1999
Clinical Lecture
Recent Advances in Treatment of Childhood Cancers
Young-Ho Lee
J Korean Pediatr Soc. 1999;42(7):893-899.   Published online July 15, 1999
Original Articles
Characterization of Molecular Defects in Korean Families with Inherited Ornithine Transcarbamylase Deficiency and Their Genotype-Phenotype Correlations
Han-Wook Yoo
J Korean Pediatr Soc. 1999;42(7):900-910.   Published online July 15, 1999
Purpose : This study was undertaken to characterize molecular defects in Korean families with ornithine transcarbamylase(OTC) deficiency, correlate it with phenotype using in vitro expression study, and utilize it for making prenatal molecular diagnosis. Methods : To investigate molecular lesions resulting in OTC deficiency in 15 unrelated Korean families, the OTC genes of probands were amplified exon by exon and analyzed...
Antenatal Thyrotropin-Releasing Hormone to Prevent Respiratory Distress Syndrome in Preterm
Ai-Rhan Kim, Sung-Jong Park, Hye-Sun Yoon, Ki-Soo Kim, Hye-Sung Won, In-Sik Lee, Ahm Kim, Soo-Young Pi
J Korean Pediatr Soc. 1999;42(7):911-920.   Published online July 15, 1999
Purpose : The purpose of this study was to assess the efficacy of antenatal thyrotropin-releasing hormone(TRH) given to mothers at risk for preterm delivery and assess various neonatal outcomes in infants born to these mothers. Methods : Sixty-one mothers(TRH+Dexamethasone(D):30, D:31) with preterm labor at 26-34 weeks of gestational age were randomized into a study group which received 400μg of TRH at...
Inhibitory Effects of Dexamethasone on Production IL-1α and TNF-α in Peripheral Blood Mononuclear Cells
Eun Ae Park, Kyung Hyo Kim, Gyoung Hee Kim
J Korean Pediatr Soc. 1999;42(7):921-927.   Published online July 15, 1999
Purpose : IL-1α and TNF-α have a seminal role in the intial events of inflammation. An area of intense interest is the assessment of new therapeutic modalities that regulate inflammatory response in acute bacterial infection. We conducted this study to compare the inhibitory effects of dexamethasone on the secretion of proinflammatory cytokines, such as IL-lα and TNF-α from cord...
Survey of Harada Scoring of Occurrence of Coronary Aneurysm in Kawasaki Disease Compared to Current Criteria
Chang Kyu Nam, Yong Wook Kim, Ki Bok Kim
J Korean Pediatr Soc. 1999;42(7):928-935.   Published online July 15, 1999
Purpose : Gamma-globulin is effective in preventing coronary aneurysm, the primary complication of Kawasaki disease(KD). However, in order to predict high-risk cases which absolutely require γ-globulin, because of its high expenses, Harada score(HS) was introduced in Japan in 1990. We attempted to compare HS scoring with the health insurance criteria currently used in Korea. Methods : Retrospective studies were performed on...
Clinical Analysis and Comparison of Kawasaki Disease between Patients Younger than One Year of Age and Those over One Year of Age
Hee-Sun Chung, Kyung-Yil Lee, Ji-Whan Han, Sang-Won Cha, Dong-Joon Lee, Kyung-Tai Whang
J Korean Pediatr Soc. 1999;42(7):936-942.   Published online July 15, 1999
Purpose : To identify the risk factors for coronary sequelae in Kawasaki disease, we analyzed and compared the clinical features and laboratory findings of Kawasaki disease in patients younger than one year of age with those over one year of age. Methods : A retrospective chart review was conducted of all children with Kawasaki disease who were admitted to the...
Effect of Fetal Cardiac Bypass on Fetal Cardiovascular System - Fetal Lamb Study -
Jung Yun Choi, June Huh, Yong Jin Kim, Won Gon Kim, Jeong Ryul Lee, Chong Sung Kim
J Korean Pediatr Soc. 1999;42(7):943-952.   Published online July 15, 1999
Purpose : This study was aimed to evaluate the feasibility of echo-Doppler study on fetal lamb, analyse the distribution of blood flow before and after fetal cardiac bypass, and consequently assess the effect of fetal cardiac bypass on the fetal cardiovascular system. Methods : Ten fetal lambs at 120 to 150 days of gestation which underwent cardiac bypass for 30 minutes...
Effects of Cisapride on Action Potential Duration and on ATP-Sensitive K Channel in Guinea Pig Ventricular Muscles
Chan Uhng Joo, Young Jae Kim, Sung Ki Mun, Soo Wan Chae
J Korean Pediatr Soc. 1999;42(7):953-958.   Published online July 15, 1999
Purpose : Cisapride(PrepulsidⓇ) has been recently associated with long QT syndrome. It has been reported to cause Torsades de Pointes and induce early afterdepolarization in rabbit Purkinje fibers. We investigated the electrophysiological effects of cisapride on cardiac action potential duration and ATP-sensitive K channel in papillary muscles. Methods : Cardiac action potentials in guinea pig papillary muscle were recorded with microelectrodes...
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
J Korean Pediatr Soc. 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...
Changes in GFAP Immunoreactivity of Astrocytes in Rats with Reye's Syndrome Induced by Valproic Acid and the Effects of Carnitine Supplementation
Young-Yoo Kim, Kyung-Tai Whang
J Korean Pediatr Soc. 1999;42(7):966-979.   Published online July 15, 1999
Purpose : This study was designed to investigate whether valproic acid(VPA) induces Reye's syndrome by analyzing blood chemistry, carnitine(CARN) concentration, and the morphologic changes of hepatocytes. The changes of GFAP immunoreactivity of astrocytes were examined in rats with Reye's syndrome induced by VPA. And the effects of CARN supplementation on Reye's syndrome were also investigated. Methods : Ten rats were assigned...
Role of Polymorphism in HLA DQ-α and -β Chain Loci in the Pathophysiology of Autoimmune Thyroid Disease in Children with and without Turner Syndrome
Kye Shik Shim, Kyu Chul Choeh, Sei Won Yang, Sa Jun Chung, Jin Sung Lee, Kyung Tae Lee, Sung Ho Goh, Yong Sung Kim
J Korean Pediatr Soc. 1999;42(7):980-990.   Published online July 15, 1999
Purpose : About 10% of girls with Turner syndrome may have autoimmune thyroid disease(AIT), but the disease's pathophysiology has not yet been elucidated. Accordingly, this study was performed to observe whether the pathogenesis of AIT in children with Turner syndrome and without Turner syndrome correlate with special loci of DQ α and β chain in HLA. Methods : Blood samples were...
Effects of Growth Hormone Treatment on Alterations of Growth and Bone Metabolism in Rats Induced by Dexamethasone Administration
Woo Yeong Chung, Jeong Nyu Lee, Hye Kyoung Yoon
J Korean Pediatr Soc. 1999;42(7):991-1002.   Published online July 15, 1999
Purpose : The aim of this study was to investigate the alterations of growth and bone metabolism in SDR induced by dexa administration and to evaluate the effects of GH treatment in dexamethasone(dexa) induced growth and bone metabolism in SDR. Methods : Forty-five female Sprague-Dawley rats(weight 150-170gm) were divided in 3 groups: Group 1(n=15) received normal saline as control, Group 2(n=15)...
Case Reports
A Case of Ectopic CD7(+) Congenital Monocytic Leukemia
Na Yeon Kim, Dong Seok Lee, Jin Hwa Jeong, Sung Min Cho, Doo Kwun Kim, Sung Min Choi, Woo Taek Kim, Kyung Im Ha
J Korean Pediatr Soc. 1999;42(7):1003-1007.   Published online July 15, 1999
Congenital acute leukemia is a rare disorder with approximately 200 cases reported. It is defined as a childhood leukemia occurring at birth or before 1 month of age at a rate of 1%. Acute leukemias are generally classified according to morphology, cytochemistry and cell surface marker expression. Most leukemias conform to an ordered lineage-specific pattern of gene expression, but a...
A Case of Split Hand Split Foot Anomaly
Mi Do Lee, Byung Moon Ahn, Moo Young Song, Eun Ryoung Kim, Mi Wook Jin, Il Soo Kim
J Korean Pediatr Soc. 1999;42(7):1008-1013.   Published online July 15, 1999
We experienced a case of split hand split foot(SHSF) anomaly in a male neonate who had a deficiency of the middle finger, syndactyly of the 1st and 2nd finger and a deep median cleft in both hands. He also had a deep median cleft between 2nd and 3rd toe, syndactyly of the 1st and 2nd, 3rd and 4th toe without...
A Case of Kawasaki Disease Diagnosed with the Assistance of Ophthalmologic Examination
Soo-Jung Ha, Kyung-Yil Lee, Sang-Won Cha, Dong-Joon Lee, Ji-Whan Han, Kyung-Tai Whang, Ik-Soo Kim
J Korean Pediatr Soc. 1999;42(7):1014-1018.   Published online July 15, 1999
Kawasaki disease is a systemic vasculitis in which bilateral nonexudative conjunctivitis and anterior uveitis are common early findings in the course of illness. We report an eleven-year-old boy with atypical Kawasaki disease who was diagnosed with the help of an ophthalmologic examination. The patient presented with fever, epigastric pain with vomiting, erythematous rash, and severe bilateral conjunctival injection, a red...
A Childhood Case of Nephrotic Syndrome Complicated with Cerebral Venous Sinus Thrombosis Followed by Left Popliteal Artery Thrombosis
Si-Eun Lee, Sue-Jin Lee, Yang-Suk Jung, Jae-Young Lim, Chan-Hoo Park, Hyang-Ok Woo, Jin-Jong Yoo, Hee-Shang Youn
J Korean Pediatr Soc. 1999;42(7):1019-1025.   Published online July 15, 1999
We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of...
A Case of Isolated Right Ventricular Hypoplasia without Tricuspid Atresia or Pulmonary Atresia
Eui Jun Yang, Sun Young Kim, Sank Ook Nam, Hee Ju Park, Nam Hee Kwak
J Korean Pediatr Soc. 1999;42(7):1026-1030.   Published online July 15, 1999
Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was...
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