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Volume 45(9); Sep 2002
A Case of Duplication 9p Syndrome(Partial Trisomy 9p)
Me Gyoung Lee, Young-Nae Roh, Young-Hoon Kim, Chung Sun Baek, Me Gyoung Sin, Jin Lee, Pil-Sang Jang, Jin-Tack Kim, Kweon-Haeng Lee, Kyung-Tai Whang
Accepted January 1, 1970  
Trisomy 9p syndrome was first described by Rethore, et al in 1970 and about 150 cases have been reported. Trisomy 9p has been reported as either partial or complete. The term "duplication 9p syndrome" instead of "trisomy 9p syndrome" is used since most of the reported patients had only partial duplication rather than the whole arm duplication of 9p. Duplication...
Clinical Lecture
Childhood Brain Tumors
Thad T. Ghim
J Korean Pediatr Soc. 2002;45(9):1055-1058.   Published online September 15, 2002
Medical Lecture Course
Signal Averaged Electrocardiogram and Clinical Application
Soon Ju Lee
J Korean Pediatr Soc. 2002;45(9):1059-1064.   Published online September 15, 2002
Original Articles
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
J Korean Pediatr Soc. 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...
Changes of Serum Fatty Acid and Carnitine Levels after Administration of L-carnitine in Rats
Jae Won Lee, Young Mi Hong
J Korean Pediatr Soc. 2002;45(9):1075-1082.   Published online September 15, 2002
Purpose : Obesity is known to be associated with hypertension, dyslipidemia, and fatty liver and is thought to be associated with increased levels of free fatty acids. One of the strategies for decreasing free fatty acid levels is stimulation of hepatic lipid oxidation with L-carnitine. Carnitine is an essential cofactor for transport of long-chain fatty acid into mitochondria for oxidation....
Serum Fatty Acid and Carnitine Levels in Obese Children with Fatty Livers
Jin Bum Lee, Jae Won Lee, Young Mi Hong
J Korean Pediatr Soc. 2002;45(9):1083-1089.   Published online September 15, 2002
Purpose : The prevalence of obese children has recently increased. Obesity is known to be associated with complications such as hypertension, fatty liver, hyperlipidemia, and insulin resistance. L-carnitine is an essential cofactor for the transport of long chain fatty acids into mitochondria for β-oxidation. The purpose of this study is to measure serum free fatty acid and carnitine levels, and...
Flexible Bronchoscopic Findings and Therapeutic Effects in Atelectasis of Children
Mee Yong Shin, Jong Hee Hwang, Eun Hee Chung, Jeong Hee Moon, Ju Suk Lee, Yong Min Park, Kang Mo Ahn, Sang Il Lee
J Korean Pediatr Soc. 2002;45(9):1090-1096.   Published online September 15, 2002
Purpose : We evaluated the clinical manifestations, bronchoscopic findings and therapeutic effects of flexible fiberoptic bronchoscopy in atelectasis of children. Methods : Sixty six children who received bronchoscopy due to persistent atelectasis, acute severe atelectasis and incidental atelectasis on plain chest radiography were studied retrospectively. Results : The most common causative underlying disease was pneumonia(60.4%). Other underlying conditions were pulmonary tuberculosis, chronic...
Electrocardiographic and Echocardiographic Characterisitics of Wolff-Parkinson-White Syndrome in Preschool Children
Jeoung Min Chu, Hyun Sup Sim, Soo Chul Cho, Chan Uhng Joo
J Korean Pediatr Soc. 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...
iption Factor NF-κB Caused by Acute Hypoxia in the Heart
Chan Uhng Joo, Woo Suk Juhng, Jae Cheol Kim, Ho Keun Yi
J Korean Pediatr Soc. 2002;45(9):1106-1113.   Published online September 15, 2002
Purpose : Nuclear factor-κB(NF-κB) is now recognized as playing a potential role in programmed cell death and the adaptive response to various stress. Cellular hypoxia is a primary manifestation of many cardiovascular diseases. It seems that vascular endothelial growth factor (VEGF) and insulin like growth factor-I(IGF-I) have a function as a protective molecule in the heart against several stress including...
Abnormal Fractal Correlation of Heart Rate in Children with Neurocardiogenic Syncope
Keoung-Young Kim, Eun-Young Joo, Myung-Kul Yum, Je-Wen Oh, Chang-Ryul Kim, Nam-Su Kim, Cheol-Beom Lee, Chung-Ill Noh
J Korean Pediatr Soc. 2002;45(9):1114-1119.   Published online September 15, 2002
Purpose : The purposes of this study were to determine short- and long-term fractal correlation behavior of heart rates during daily activity in patients with neurocardiogenic syncope. Methods : Twenty five patients with histories of neurocardiogenic syncope episodes were included. Their analogue 24h ambulatory Holter electrocardiograms were analyzed. The tape was digitized and the digitized electrocardiograms were partioned into...
A Study on Central Vein Cannulation Using Audible Doppler Guidance in Children and Infants
Young Joon Ahn, Jin Young Jung, Soo-Jong Hong
J Korean Pediatr Soc. 2002;45(9):1120-1125.   Published online September 15, 2002
Purpose : Percutaneous cannulation of the central vein in children and infants may be technically difficult and can cause serious complications. There are many techniques to improve the success rate and to decrease the complications in central vein cannulation. This study was undertaken to determine whether audible Doppler guidance can help operators improve the results of central vein cannultion in...
Clinical Characteristics and Genetic Analysis of Prader-Willi Syndrome
Ji Eun Lee, Kwang Bin Moon, Jong Hee Hwang, Eun Kyung Kwon, Sun Hee Kim, Jong Won Kim, Dong Kyu Jin
J Korean Pediatr Soc. 2002;45(9):1126-1133.   Published online September 15, 2002
Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from...
Clinical Study of Childhood Accidents from a Hospital Over Ten Years with Regard to Foreign Body Aspiration
Cheol-Min Kim, Jun-Young Song, Ja Hyung Kim, Ki Soo Kim, Soo-Jong Hong
J Korean Pediatr Soc. 2002;45(9):1134-1140.   Published online September 15, 2002
Purpose : Childhood accidents have been increasing recently. Accidents rank as the leading cause of childhood mortality and morbidity. We performed this study to evaluate the causes of childhood accidents. Methods : The authors analysed retrospectively the medical records of 6,410 cases of childhood accidental injuries who visited the emergency room of Asan Medical Center from January 1990 to December...
Case Reports
A Case of Klinefelter Syndrome associated with Unilateral Multicystic Dysplastic Kidney in a Newborn Infant
Kyung A Ha, Sun Mi Chung, Eun Jin Choi, Jin Kyung Kim, Un Seok Nho, Jae Shin Park, Woo Taek Kim, Young Dae Kwon
J Korean Pediatr Soc. 2002;45(9):1141-1145.   Published online September 15, 2002
Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal...
A Case of Stevens-Johnson Syndrome Plus Vanishing Bile Duct Syndrome Associated with Ibuprofen Use
Jee Yee Choi, Su Young Kim, Soon Ok Byun, Jae Hong Park
J Korean Pediatr Soc. 2002;45(9):1146-1149.   Published online September 15, 2002
Acute vanishing bile syndrome is a cause of progressive cholestasis. It is most often drug or toxin related. It's pathogenesis is unknown. Stevens-Johnson syndorme is a well-recognized immune complex-mediated hypersensitivity reaction. It is induced by drug or infection. It has classic systemic, mucosal, and dermatologic manifestations. We report a case of a 14 years old female suffering from Stevens-Johnson syndrome...
Two Cases of Wolff-Parkinson-White Syndrome in a Family
Chan Uhng Joo, So Hee Lim, Pyung Han Hwang
J Korean Pediatr Soc. 2002;45(9):1150-1154.   Published online September 15, 2002
Wolff-Parkinson-White(WPW) syndrome is characterized by electrographic evidence of ventricular preexcitation, which predisposes to supraventicular arrhythmias. Familial occurrence of WPW syndrome is uncommon. We observed two affected siblings in a family. Five members of the family underwent 12-lead electrocardiography and echocardiography. Although known genetic abnormality of the 7q34-q36(PRKAG2) for the familial WPW syndrome was evaluated, the mutation was not detected in...
A Case of Alport Syndrome Associated with Diffuse Esophageal Leiomyomatosis
Jin Young Jung, Cheol-Min Kim, Yean Jung Lim, Ja Hyung Kim, Chong Woo You, Bo-Hwa Choi, Soo-Joung Hong, Young Seo Park
J Korean Pediatr Soc. 2002;45(9):1160-1164.   Published online September 15, 2002
University of Ulsan, Seoul, Korea We report a case of Alport syndrome associated with esophageal leiomyomatosis, presenting as recurrent pneumonia. A 5-year old girl who had a history of cataract visited the out patient clinic with a complaint of recurrent wheezing and respiratory difficulty which had started five months previously. Chest magnetic resonance image(MRI) and esophagography, checked on the suspicion of...
A Case of Satoyoshi Syndrome Presented with Progressive Muscular Spasm and Alopecia
Kyung Ran Son, Jin Hwa Kook, Byung Ju Kim, Sung Jin Kim, Jae Sook Ma
J Korean Pediatr Soc. 2002;45(9):1165-1169.   Published online September 15, 2002
Satoyoshi syndrome(generalized Komuragaeri disease) is a rare disorder of unknown cause, characterized by progressive, painful, intermittent muscle spasms and alopecia. Endocrinopathy with amenorrhea, secondary skeletal abnormalities, and diarrhea or unusual malabsorption are frequently seen. It seems that autoimmunity may play a role in its pathogenesis. We report a 13-year-old girl with characteristic manifestations of the syndrome. She was treated with...
A Case of Centronuclear Myopathy
Yun Hee Kim, Yun Hee Kim, Young Se Kwon, Young Se Kwon, Dae Hyun Lim, Dae Hyun Lim, Yong Hun Jun, Yong Hun Jun, Soon Ki Kim, Soon Ki Kim, Young Jin Hong, Young Jin Hong, Byong Kwan Son, Byong Kwan Son, Hae Seung Han, Hea Seung Han
J Korean Pediatr Soc. 2002;45(9):1170-1174.   Published online September 15, 2002
Centrinuclear myopathy, an uncommon condition, is one of the congenital myopathies. It is characterized by the presence of central nuclei of muscle cells which can be detected on electronmicroscopy. It is believed to arise as a result of maturational arrest with persistence of microtubes postnatally. We report a boy with generalized hypotonia and muscle weakness who was diagnosed as centrinuclear...
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