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Volume 49(11); Nov 2006
Review Articles
Newborn screening of inherited metabolic disease in Korea
Dong Hwan Lee
Korean J Pediatr. 2006;49(11):1125-1139.   Published online November 15, 2006
In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia & congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU & congenital hypothyroidism to increase test numbers with same...
Diagnosis of inherited metabolic disorders based on their diverse clinical features and laboratory tests
Han-Wook Yoo
Korean J Pediatr. 2006;49(11):1140-1151.   Published online November 15, 2006
Inherited metabolic disorders are individually rare but as a whole, they are nor rare. Since Archibald Garrod introduced a concept of “inborn error of metabolism” or “chemical individuality”, more than 500 diseases are currently known, affecting approximately one in 500 newborns cumulatively. They frequently manifest with acute, life-threatening crisis that require immediate specific intervention or they present with insidious diverse...
Treatment and management of patients with inherited metabolic diseases
Jin-Sung Lee
Korean J Pediatr. 2006;49(11):1152-1157.   Published online November 15, 2006
Inherited metabolic disease is rare disorders that show symptoms mainly in pediatric age and early treatment is important for preventing complications of the disease. Recent development in molecular and biochemical techniques help clinicians with proper diagnosis of patients, however, many of the disease still remain lack of effective therapeutic strategies. Better understanding on biochemical and molecular basis of pathogenesis of...
Original Articles
Diagnostic classification and clinical aspects of floppy infants in the neonatal and pediatric intensive care units
Eun Sun Kim, Kyung Eun Jung, Sang Duk Kim, Eo Kyung Kim, Jong Hee Chae, Han Suk Kim, June Dong Park, Ki Joong Kim, Beyong Il Kim, Yong Seung Hwang, Jung-Hwan Choi
Korean J Pediatr. 2006;49(11):1158-1166.   Published online November 15, 2006
Purpose : The purpose of this study is to make a diagnostic classification and discuss a diagnostic strategy of floppy infants by investigating clinical, neurological, electrophysiological, and genetic analysis of infants admitted to intensive care units with the complaint of hypotonia. Methods : A retrospective study was performed from Jan. 1993 to Dec. 2005 in neonatal and pediatric intensive care units...
Diagnostic value of various screening tests in neonatal sepsis
Hyun Gon Je, Young Mi Jeoung, Soo Jin Jeong
Korean J Pediatr. 2006;49(11):1167-1173.   Published online November 15, 2006
Purpose : To evaluate various sepsis screening tests, individually and in combination, to formulate a guideline for the diagnosis of neonatal sepsis. Methods : The study was a retrospective cohort study. It took place at the neonatal intensive care unit of the Paediatric Department, Il Sin Christian Hospital, Busan, Korea, over a period of 68 months from 1st, April, 2001 to...
The iron status and the relationship between iron deficiency and body mass index in middle school girls
Hee Youn Han, Chang Joo Hee, Hae Sun Shim, Young Jin Hong, Byong Kwan Son, Hwan Cheol Kim, Soon Ki Kim
Korean J Pediatr. 2006;49(11):1174-1179.   Published online November 15, 2006
Purpose : Obesity and iron deficiency are common nutritional problems. Obese children are known to have iron deficiencies, but one presented opposite opinion in Korea. This study investigated the prevalence of obesity and iron deficiency in Incheon and the relationship between iron deficiency and obesity. Methods : Physical measurement and hematologic examinations were done a 764 healthy female students aged 14...
Predictive value of cobalt chloride solution for hypohidrosis with topiramate
Hyuk Lee, Seung Hyun Lee, Sun Jun Kim
Korean J Pediatr. 2006;49(11):1180-1185.   Published online November 15, 2006
Purpose : The aims of this study were to verify the incidence of hypohidrosis and to determine the predictive value of noninvasive indicator test (NeurocheckTM) for sweating after administration of topiramate in newly diagnosed pediatric epileptic patients. Methods : A total of 46 epileptic patients (22 boys; 24 girls) on topiramate treatment were evaluated in this study at the Department...
Cause of enteroviral infection in children in chungnam area summer, 2005
Se Yun Jeon, Suk Joo Choi, Yong Bae Kim, Hae Seon Nam, Kwi Sung Park, Kyung Ah Baek, Joon Soo Park
Korean J Pediatr. 2006;49(11):1186-1193.   Published online November 15, 2006
Purpose : Enterovirus infection is a type of viral infection that occurs relatively frequently in children during summer. It has clinical symptoms of non-specific fever, aseptic encephalomeningitis, gastrointestinal diseases, skin rash and, hand-foot-mouth disease. However, it can also occcaisionally, result in fatal symptoms like myocarditis, epicardial inflammation, transverse myelitis, quadriplegia and etc. There have been epidemic enterovirus studies, but not...
Usefulness of modified ambu in patients who need artificial ventilation
Kee Soo Ha, Il Hong Moon, Hee Sun Lee, Dong Han Shin, So Hee Eun, Baik-Lin Eun, Young Sook Hong, Joo Won Lee
Korean J Pediatr. 2006;49(11):1194-1201.   Published online November 15, 2006
Purpose : The comatose mentality can be catastrophic, especially if the condition is severe or the duration is prolonged. Therefore, delayed diagnosis can result in a poor outcome or death. The best radiologic modality to differentiate from cerebral lesions in patients suffering from cerebral diseases is magnetic resonance imaging (MRI) rather than computed tomography (CT). Special apparatuses with metal...
Modified Tei index in patients with Kawasaki disease by tissue doppler imaging
Hee Jung Kim, Jung Hwa Cha, Young Mi Hong
Korean J Pediatr. 2006;49(11):1202-1210.   Published online November 15, 2006
Purpose : A quantitative and easily measured Doppler index of combined systolic and diastolic ventricular myocardial performance (Tei index) was recently proposed as a potentially useful predictor of global myocardial performance. However, presence of heart rate fluctuation makes it unreliable. Therefore, the modified Tei index was introduced by using tissue Doppler imaging (TDI) which enables measuring contraction and relaxation velocities...
Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation
Seon-Ju Lee, Jae-Wook Lee, Dae-Hyoung Lee, Young-Joo Kwon, Young-Shil Park, Hui Sung Hwang, Sun Young Kim, Ji Kyoung Park, Pil-Sang Jang, Min-Ho Jung, Nak-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim, Byung-Churl Lee
Korean J Pediatr. 2006;49(11):1211-1215.   Published online November 15, 2006
Purpose : In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. Methods : We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and...
Methacholine and adenosine 5'-monophosphate challenge tests in children with atopic asthma and with nonatopic asthma, and their relationships to blood eosinophil markers
Sun Hee Lee, Do Kyun Kim, Sun Hee Choi, Young Yull Koh
Korean J Pediatr. 2006;49(11):1216-1222.   Published online November 15, 2006
Purpose : Though atopic and nonatopic asthma have different clinical manifestations, bronchial hyperresponsiveness (BHR) and airway inflammations are common characteristics of them. We investigated BHR to both methacholine and adenosine 5'-monophosphate (AMP), and their relationships with blood eosinophil markers in nonatopic asthma as well as atopic asthma. Methods : We studied 116 children (82 atopics; 34 nonatopics) with mild to moderate...
Case Report
Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse
June Seung Sung, Ki Hwan Kim, Dong Gyun Han, Mi Jeong Kim, Young Kook Cho, Hae Yul Chung, Hee Jo Baek, Jae Sook Ma, Hoon Kook, Tai Ju Hwang
Korean J Pediatr. 2006;49(11):1223-1226.   Published online November 15, 2006
Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of...
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