• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1232-1238
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• 2005

Purpose : Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X-linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods : Four patients who were treated with Lorenzo's oil for at least 12 months were enrolled from 1996 to 2003. During treatment, changes of neurologic symptoms, brain magnetic resonance imaging(MRI) findings, and serum saturated very long-chain fatty acid(C26:0) were assessed. Results : Two patients with childhood cerebral ALD had progression of neurologic symptoms and MRI lesions during treatment. One asymptomatic patient developed childhood cerebral ALD after six year treatment of Lorenzo's oil. One "Addison only" patient remained neurologically intact after three years. During Lorenzo's oil therapy, serum C26:0 levels which had increased at diagnosis decreased in all four patients. Conclusion : Treatment with Lorenzo's oil did not prevent disease progression in childhood cerebral ALD patients who were already symptomatic. Long term follow up will be needed in asymptomatic ALD including "Addison only" to reveal the efficacy of Lorenzo's oil.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.643-647
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• 2006

Purpose : Recently, incidences of chronic cough with allergic disease have been increased. The identification of specific allergens for chronically coughing children is significant for diagnosis, proper treatment and prevention. The aim of this study was to identify significant contributors for sensitization in chronically coughing children. Methods : A total of 106 children, aged 1-6 years, visiting for chronic cough(>3 weeks), were tested for inhalant multiple allergosorbent assay(MAST), eosinophil count and total IgE. Parents completed a questionnaire on past history of atopy(infantile eczema) and bronchiolitis, family history of allergy, breastfeeding, smoking, pets and floor coverings at home. Results : Significant contributors for sensitization were : age(4-6 years), infantile eczema, positive family history of allergy and smoking by family. Conclusion : We were able to identify significant contributors for sensitization in chronically coughing children.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.484-489
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• 2003

Purpose : To understand morphologic and hemodynamic variations in patients with pulmonary atresia with intact ventricular septum(PA/IVS), and to decide the best treatment modalities, we measured right ventricular volume, inflow, and outflow valvular annulus size in these patients and compared them with those of normal newborns. Methods : Eight patients with PA/IVS diagnosed by echocardiography from January to December 2001 were enrolled in this study. Among the total eight patients, five were male and three were female. The mean age of patients was 6.9 days(1-34 days), and the mean body weight was 3,343 gm (2,970-4,000 gm). Ten fullterm newborn infants with sepsis or hyperbilirubinemia without heart disease were enrolled as a control group. Echocardiographic and Doppler studies using Acuson Aspen (7Mh probe) were recorded on super-VHS videotape and later on, with review mode. We measured volumes of right and left ventricles, aortic, pulmonic, mitral and tricuspid valvular annulus sizes using an installed program, and then these parameters were compared with those of the control group. Results : Mean Z-value of tricuspid valvular annulus in PA/IVS was $-3.69{\pm}2.80$(-8.4--0.45), and tricuspid/mitral valvular annulus size ratio $0.68{\pm}0.15$(0.43--0.84). The more the tricuspid/mitral valvular annulus size ratio, the more Z-value of tricuspid valvular annulus(P=0.003, r=0.885). Those patients who underwent pulmonary valvuloplasty(balloon or surgical) had a tendency toward larger volume of the right ventricle, more Z-value of pulmonic and tricuspid valvular annulus, and more tricuspid/mitral valvular annulus size ratio than those patients who underwent a shunt operation. Conclusion : Compared to a measurement of the volume of the right ventricle, measurements of tricuspid/mitral valvular annulus size ratio and Z-value of tricuspid valvular annulus may be easier and better parameters to decide the treatment method and to predict prognosis in PA/IVS patients.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.454-458
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• 2003

Purpose : Rotavirus is the main cause of infantile diarrheal disease worldwide. The purpose of this study is to assess the difference between clinical manifestations and feeding or delivery methods in healthy full-term neonates and those with nosocomially-acquired rotaviral infection. Methods : The study was conducted on 348 babies who were delivered in Dongguk University Kyongju Hospital from Jan 1 to June 30 in 2001. Stool specimens were collected from clinically symptomatic neonates. We studied the rate of positive stool rotazyme ELISA tests and positive symptoms. We compared the frequency of clinical manifestations according to the feeding methods [breast feeding(BF) or formula feeding(FF)] and the delivery methods[normal vaginal delivery(NVD) or Cesarean section(C/sec)]. Results : Rotavirus was detected in 152 of 348 babies. The rate of positive rotazyme ELISA test was 43.7% in healthy full-term symptomatic neonates. The most common symptom of rotaviral enteritis was mild fever; the others were watery diarrhea, abdominal distension, vomiting, delayed weight gain, irritability, poor oral intake and dehydration. Compared with FF neonates, BF neonates have lower frequency of symptoms, especially watery diarrhea, delayed weight gain and poor oral intake. The symptomatic frequency was higher in FF and NVD neonates than BF and C/sec. Conclusion : The symptoms of rotaviral enteritis were less frequent in BF or C/sec delivered neonates. BF appeared to alleviate the rotaviral enteritis but further studies are needed. The cause of the lower frequency of symptoms in C/sec delivered neonates was unknown.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1188-1193
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• 2007

Purpose : The prevalence of rickets in the world is on the rise not only in developing but also in developed countries. In Korea, breastfeeding has increased. There have been few studies on the possible association of rickets with breastfeeding. The purpose of this study was to identify the development and the clinical presentation of subclinical rickets in breastfed infants. Methods : We investigated patients who were breastfed and had hypovitaminosis D in the blood from May 2006 to April 2007, and who were diagnosed with vitamin D deficient rickets from May 2003 to April 2006. We evaluated the results of blood tests, x-rays and other relevant information in the medical record. A questionnaire that included questions on the diet of patients, the mothers activity during pregnancy and place of residence was administered. Results : Twelve patients (66%: male, 34%: female) were enrolled in this study. There were eight in the asymptomatic and four in the symptomatic group. The median age for each group was 8 months (range 4-11 month) and 5.5 months (5-8 month). All patients in the symptomatic group were breastfed until diagnosed. In the asymptomatic group, they were breastfed for four to six months, and then weaned with only thin rice soup and vegetables. Nine patients had a vitamin D concentration below 20 ng/mL and three patients had levels between 20 and 29 ng/mL. Alkaline phosphatase (ALP) and parathyroid hormone (PTH) were elevated in both groups. There were statistically significant $25-OHD_3$ levels in the blood in both groups (P=0.008). Ten of the patients (83%) also had iron deficient anemia. Conclusion : Vitamin D deficiency and subclinical rickets has been identified in Korea. However, the prevalence of this disease has not been determined. The main limitation of this study was the small number of patients and the absence of a control group.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1102-1111
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• 2008

Purpose : Resveratrol, extracted from red wine and grapes, has an anti-cancer effect, an antiinflammatory effect, and an antioxidative effect mainly in heart disease and also has neuroprotective effects in the adult animal model. No studies for neuroprotective effects during the neonatal periods have been reported. Therefore, we studied the neuroprotective effect of resveratrol on hypoxic-ischemic brain damage in neonatal rats via anti-apoptosis. Methods : Embryonic cortical neuronal cell culture of rat brain was performed using pregnant Sprague-Dawley (SD) rats at 18 days of gestation (E18) for the in vitro approach. We injured the cells with hypoxia and administered resveratrol (1, 10, and $30{\mu}g/mL$) to the cells at 30 minutes before hypoxic insults. In addition, unilateral carotid artery ligation with hypoxia was induced in 7-day-old neonatal rats for the in vivo approach. We injected resveratrol (30 mg/kg) intraperitoneally into animal models. Real-time PCR and Western blotting were performed to identify the neuroprotective effects of resveratrol through anti-apoptosis. Results : In the in vitro approach of hypoxia, the expression of Bax, caspase-3, and the ratio of Bax/Bcl-2, indicators of the level of apoptosis, were significantly increased in the hypoxia group compared to the normoxia group. In the case of the resveratrol-treated group, expression was significantly decreased compared to the hypoxia group. And the results in the in vivo approach were the same as in the in vitro approach. Conclusion : The present study demonstrates that resveratrol plays neuroprotective role in hypoxic-ischemic brain damage during neonatal periods through the mechanism of anti-apoptosis.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.736-741
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• 2008

Purpose : The incidence of thromboembolic episodes in children with nephrotic syndrome (NS) is low; however, these episodes are often severe. Moreover, both pulmonary thromboembolism (PTE) and renal vein thrombosis (RVT) rarely show clinical symptoms. This study was performed to determine the benefits of routine screening in the detection of thrombosis in childhood NS. Methods : Among 62 children with nephrotic syndrome, a total of 54 children (43 males, 11 females) were included in this study. When the patients experienced their first NS episode, we performed renal Doppler ultrasonography in order to detect RVT. To rule out the possibility of PTE, a lung perfusion scan was performed. Computed tomographic (CT) pulmonary angiography was recommended to patients who showed possible signs of PTE. All patients were evaluated for clinical signs of thrombosis, biochemical indicators of renal disease, as well as clotting and thrombotic parameters. Results : RVT or related clinical symptoms were not observed in any children. Based on the findings of the lung perfusion scans, 15 patients (27.8%) were observed with as a high probability of PTE. We were able to perform a CT pulmonary angiography only on 12 patients, and 5 patients were diagnosed with PTE (prevalence 8.1%). The serum fibrinogen level in the group with PTE was significantly higher ($776.7{\pm}382.4mg/dL$, P<0.05) than that in the group without PTE, and other parameters were not significantly different between each group. Conclusion : Further studies are required for clarifying the role of renal Doppler ultrasonography for the detection of RVT in NS. Children with NS who developed non-specific respiratory symptoms should be evaluated for the diagnosis of PTE. In the management of NS, a lung perfusion scan should be performed at the time of the initial episode of NS regardless of the pulmonary symptoms, since patients having PTE are either often asymptomatic, or present with nonspecific symptoms.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.747-753
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• 2008

Purpose : Diabetes mellitus is a major risk factor for the development of cardiovascular disease. Early atherosclerotic changes in the arterial walls begin in adolescence and the risk factors are associated with its development. To assess the usefulness of carotid artery intima-media thickness (IMT), as a marker of early atherosclerosis, we evaluated the structural and functional characteristics of the carotid artery and investigated their relationshop with the metabolic and anthropometric parameters in children and adolescents with type 1 diabetes. Methods : For this study, we enrolled 23 children with type 1 diabet and 19 age and sex-equivalent healthy children as the control group. Metabolic and anthropometric parameters such as serum lipid levels, plasma glycated hemoglobin (HbA1c), and body mass index were measured after a 12-h fasting period. The carotid artery IMT was measured by a high-quality ultrasound system, and compliance, and distensibility were calculated by an equation. Results : There were no significant differences between the 2 groups with regard to the sex ratio, age, blood pressure and serum cholesterol levels' however, HbA1c levels were significantly higher in the diabetic children ($8.5{\pm}1.8$ vs. $5.0{\pm}0.2$, P=0.001). Ultrasonographic findings showed that compared with the control group, the diabetic group had higher IMT ($0.45{\pm}0.06mm$ vs. $0.41{\pm}0.04mm$, P=0.04), but there were no significant differences in compliance and distensibility. The HbA1c (P=0.002) and high-density lipoprotein cholesterol (P=0.026) levels were independent IMT predictors in the diabetic group. Conclusion : Here, the carotid artery IMT was higher in the diabetic group, and it is correlated with atherosclerotic risk factor. Thus, carotid IMT could be evaluated as a marker of early atherosclerosis in diabetic children.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.671-689
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• 2008

The economic recession of North Korea has been prolonged, the need for humanitarian assistance for the women and children of DPRK has been raised. In March 2006, South Korean government signed MOU with World Health Organization (WHO) to financially support "Improving Women's and Children's Health in DPRK (IWCH)" project. The assistance projects through UNICEF and the non-government organizations of South Korea were also followed. IWCH project consists of three parts; nutrition, disease management, children and maternity care. The first term (2006-2007) of the project leading by WHO was finished, and the second term (2008-2010) is just begun. The projects driven by NGOs have relatively been delayed due to difficulties in negotiating on project contents and places with North Korea. Recently, however, re-modeling processes of an obstetric/gynecology hospital and a children hospital in Nampo were started. Up to recently, South Korean government has played only a limited role in the humanitarian assistance for North Korea. IWCH project is, however, a full-scale initiative driven by government based on a systematic review of need and priorities. A significant amount of budget and relatively long term (five year) project compare to the previous short term and small size programs were expected to make more meaningful achievement. Despite these positive aspects, the project remains a list of unsolved problems a lack of mutual trust, a different decision making process between South and North Korea, a lack of conflict management process, and unpredictability and complexity of international politics. In spite of such kind of political uncertainty, the health care sector will be a leading area in the process of improving relationship between South and North Korea, particularly, humanitarian assistance for women and children will play a crucial role in the process. The successful implementation of IWCH project, therefore, will contribute to provide the reference model in developing the mutually constructive relationship between South and North