• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.424-430
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• 2006

Purpose : Juvenile rheumatoid arthritis(JRA) is one of the most common rheumatic diseases of childhood and is an important cause of short- and long-term disability. The purpose of this study was to determine the disease course and outcome in childhood patients with JRA. Methods : Fifty nine patients with JRA who were diagnosed and treated in the Department of Pediatrics, Asan Medical Center from August 1990 to November 2004 were enrolled in this study. Sex, age, type, affected joints, extra-articular manifestations, laboratory and radiologic findings, treatments, and outcomes of JRA patients were reviewed retrospectively. Results : Among JRA patients, 32.2 percent had pauciarticular type, 30.5 percent had polyarticular type and 37.3 percent had systemic type. The ratio of boys to girls was 1.7 : 1 and the mean age at onset was $9.3{\pm}3.7$(1.3-15.9) years. The most commonly affected joints were knee, ankle and wrist. The extra-articular manifestations observed were fever, rash, myalgia and lymph node enlargement, etc. The main laboratory findings observed were leukocytosis, anemia, thrombocytosis, elevated ESR, and elevated CRP. Rheumatoid factor and antinuclear antibody(ANA) were positive in 5.3 percent and 18.0 percent. Nonsteroid anti-inflammatory drugs(NSAID) were used most frequently and methotrexate with or without steroids was added in 27.1 percent of patients unresponsive to NSAID. 88.1 percent of patients were cured without functional disability and only one patient was in functional status IV. One patient, who had pulmonary involvement, died. Conclusion : Our results showed an even distribution in type of onset, male predominance, older age of onset, low incidence of iridocyclitis, and low positivity of ANA in JRA patients; this differs from occidental data. This study may suggest regional differences and variability in disease groups of JRA among different racies, but further multi-center trials and large scale epidemiological studies are needed to confirm our conclusion.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.401-409
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• 2006

Purpose : Human metapneumovirus(hMPV) is a respiratory viral pathogen that causes a wide spectrum of illnesses, ranging from asymptomatic infection to severe bronchiolitis. The virus has been identified world widely, but so far it has not been published in Korea. Methods : We obtained clinical samples by nasopharyngeal aspiration from 218 children hospitalized due to acute lower respiratory tract infections at Soonchunhyang University Hospital in Cheonan from October, 2004 to April, 2005. We designed specific primers from conserved region of fusion glycoprotein of hMPV. Total RNA was extracted and RT-PCR was performed, and single specific 423 bp product was obtained. The PCR product was confirmed to be fusion glycoprotein RNA by sequencing. Results : We detected hMPV in 15(6.9 percent) of the 218 hospitalized children. The infected children comprised nine boys and six girls; their mean age was 2.8 years(5 mo-12 yrs) and they were diagnosed with pneumonia(60 percent), bronchiolitis(33.3 percent), croup(6.6 percent). The number of cases of detected hMPV in Korea increased dramatically during the period from March to May 2005. Conclusion : hMPV is circulating in Korean children and is associated with respiratory tract infection. Additional studies are required to define the epidemiology and the extent of diseases in the general population caused by hMPV.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.368-374
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• 2006

Purpose : The prevalence of obesity in children is increasing rapidly. Epidemiologic studies suggest that obesity induced atherosclerosis may start in childhood. We investigated whether obese children show early abnormalities of the arterial wall and endothelial dysfunction. Methods : Thirty-eight obese children(14-16 years old of age, male, body mass index $29.40{\pm}3.18kg/m^2$) and forty-five age and sex-matched healthy control children(body mass index $18.43{\pm}1.01kg/m^2$) were enrolled. Their carotid artery intima-media thickness(IMT) and brachial artery flowmediated dilation(FMD) response were measured by high-quality ultrasound system, and compliance, distensibility, stiffness index, incremental elastic modulus and wall stress were calculated by equation. In addition, we looked at the relations between these arterial features and metabolic cardiovascular risk factors. Results : The obese children had significantly increased IMT($0.52{\pm}0.09mm$ vs $0.40{\pm}0.07mm$, P< 0.001) and markedly impaired FMD($7.35{\pm}7.78$ percent vs $20.34{\pm}16.81$ percent, P<0.001) than the healthy controls. But the compliance and distensibility were lower, and the stiffness index, incremental elastic modules and wall stress were higher in the obese group than the control group, but not statistically significantly. Body mass index was highly associated with increased IMT(r=0.612, P<0.001) and reduced FMD(r=-0.414, P<0.001). Conclusion : We showed the deleterious effect of child obesity on both early functional and structural atherosclerotic markers. The ultrasonic findings will be used for screening and follow up markers to identify high-risk patients among obese children.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.417-423
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• 2006

Purpose : The objectives of this study were to assess ventricular function by tissue Doppler imaging in children who were receiving chemotherapy or who had received chemotherapy, and to apply repeated tissue Doppler imaging to make an early assessment in cardiac toxicity studies. Methods : This study was conducted on 23 oncology patients on-treatment or off-treatment from April 2005 to July 2005 at Dongsan Medical Center, Keimyung University. All patients(group 1) were divided into two groups, fractional shortening(FS) over 29 percent(group 2) and FS under 28 percent (group 3) in the first category. These same patients were also divided into the following groups : group treated with anthracyclin(group 4) and group treated without anthracyclin(group 5). Deceleration time(DT), isovolumic relaxation time(IVRT), FS, peak early diastolic(E), and peak late diastolic (A) velocity of transmitral flow were measured by M-mode and pulsed wave Doppler. Systolic(Sm), peak early diastolic(Em), and peak late diastolic(Am) velocity in apical 4-chamber and 2-chamber views were measured by tissue Doppler imaging. The author calculated a modified Tei index, E/A, E/Em ratio by using measured values. Results : Twenty three patients were enrolled : 12 boys and 11 girls. The average age of patients was 8 years and 4 months. Thirteen out of 23 patients were in the group treated with anthracyclin (group 4) and 6 had FS under 28 percent(group 3). E/Em ratio showed a significant difference between group 1 and control group($6.46{\pm}1.85$ vs $7.06{\pm}1.64$, P<0.05). Other parameters had no difference statistically. Conclusion : This study showed that the change of cardiac function developed earlier in diastolic function than in systolic function, as E/Em ratio reflecting the mean LV diastolic pressure showed a significant difference between the control group and chemotherapy groups. Echocardiography using tissue Doppler imaging is a non-invasive, comfortable and reliable method for post-chemotherapy follow up.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.431-438
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• 2006

Purpose : Insulin-like growth factor binding protein(IGFBP)-3 has been known as a tumor suppressor gene, and its anti-tumor function was divided into insulin-like growth factor(IGF)-dependent and IGF-independent mechanism. In IGF-independent mechanism, IGFBP-3 directly interacts with a cell without binding of IGFs, becoming an interesting object in oncology. Several studies demonstrate that one of the well-known tumor suppressor genes, p53, induces directly IGFBP-3 transcription, and the increment of IGFBP-3 expression induces apoptosis of many cancer cells. Recently, the anti-tumor mechanisms of IGFBP-3 have been reported, but post-translational modification of IGFBP-3 and its anti-tumor mechanism are not well known. In this study, we examined whether p53 regulated the glycosylation of IGFBP-3, and analysed the meaning of IGFBP-3 glycosylation related to the apoptosis of cancer cell. Methods : The p53-mutated status of MDA-MB-231 human breast cancer cells was used in this experiment. The expression and glycosylation of IGFBP-3 were tested by Western blot analysis after infection of adenovirus mediated Ad/p53 and/or Ad/IGFBP-3. Results : Ad/p53 infected cells resulted in growth retardation and the induced apoptosis. p53 induced direct expression and glycosylation of IGFBP-3. The increase of glcosylated IGFBP-3 was able to promote cellular apoptosis, and the glycosylation of IGFBP-3 was more activated by the double treatment of Ad/p53 and Ad/IGFBP-3. Conclusion : From this study, the anti-tumor activity of IGFBP-3 was shown to improve the stabilization of IGFBP-3 through the increment of glycosylation of IGFBP-3 by p53. This result suggests that the combined gene therapy of p53 and IGFBP-3 may appropriate treatment of cancer.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.381-387
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• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.331-338
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• 2002

Purpose : To investigate the feature of the range of measles in 2000, we studied epidemics of the measles in western Kyungnam District. Methods : Among 489 patients under 15 years of age who visited or were admitted to in our hospital from January to December in 2000, we selected 344 patients whose measles IgM antibody was positive. We investigated age, monthly incidence, clinical features, vaccination history, and measles IgG antibody. Results : Patients under 12 months of age occupied the largest proportion(36.9%). Patients firstly occurred in May and geometrically increased from October. Fever, cough and rashes were observed in all patients but conjunctivitis in 54.9%, Koplik spot in 23.8%. The first vaccination ratio was 41.8%. Compared with the number of patients and the first vaccination ratio, the first vaccination ratio was the lowest under 12 months of age. After that age group, the number of patients was decreased as the first vaccination ratio was increased. Among 152 patients sampled for measles IgG antibody, 35 patients had received the first vaccination and 6 patients had received the second vaccination. Among 35 patients who received the first vaccination, 22(62.9%) patients were negative of measles IgG antibody. Among 6 patients who received the second vaccination, 3(50%) patients were negative. Conclusions : Measles patients under 12 months still dominated. Therefore, routine vaccination of single measles vaccine, is currently done at 6 month when measles are prevalent, should be considered. High first vaccination failure suggests problems of vaccines itself, transport and storage rather than vaccination methods. Therefore a thorough investigation should be made.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.370-375
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• 2002

Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods : Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA-matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. Results : Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. Conclusion : HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.363-369
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• 2003

Purpose : Pharmacologic provocation test of growth hormone(GH) is a non-physiologic method and has several limitations for diagnosing growth hormone(GH) deficiency. Spontaneous GH release studies could be important in understanding the pathophysiology of children with poor growth but normal responses to GH provocation tests. Also, the relationship between nocturnal GH secretions and sleep patterns in short stature children is poorly understood. The aim of this study is to determine whether there are differences in sleep patterns and nocturnal GH secretory profiles between idiopathic short stature children and a normal stature group. Methods : Spontaneous nocturnal GH secretions and sleep patterns were evaluated in 12 prepubertal idiopathic short stature children with normal responses to provocation tests and 9 normal stature controls. Blood samples were taken every 30 minutes from 22:00-06:30 and sleep patterns were analyzed by polysomnography. Results : The mean GH level during sleep was significantly lower in short stature children than in controls. The peak GH level after sleep, coincident with the first slow wave sleep, was lower in the short stature group. The slow wave sleep times of short stature children were decreased compared with those of normal subjects. Conclusion : These results suggest that overnight serial GH sampling is helpful to identify short stature children with subnormal GH secretions, and sleep structure differences may be associated with decreased overnight GH secretions in short stature children.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.351-357
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• 2003

Purpose : To raise awareness of the clinical importance of, and the need for proper management of acute focal bacterial nephritis(AFBN), we analyzed 22 AFBN patients and 22 other upper urinary tract infection patients by use of comparative studies. Methods : From January 2000 to May 2002, 22 AFBN patients aged from 1 month to 12 months were selected. As a control group, 22 UTI patients with no radiologic abnormalities were selected and matched by age and sex. Results : The incidence of AFBN was more common in boys than in girls. Since both groups had similar symptoms, it was difficult to diagnose AFBN by clinical presentations alone. ESR and CRP were significantly higher in AFBN patients. The most common causative organism was E. coli in both groups. On the sonographic findings, the most lesions were seen on the upper lobe of the kidney; more frequently, on left kidney. The lesions showed globular or wedge-shaped increased echogenecity. $^{99m}Tc-DMSA$ scan showed the complete coincidence of the location, size and shape in all cases compared to the findings of renal sonography. Conclusion : The roles of renal sonography and DMSA scan were very important, and ultrasonography was an excellent initial tool in diagnosing AFBN. Since the degree of infection in AFBN is more severe than other urinary tract infections and evollution into a renal abscess is possible, early diagnosis and appropriate antibiotics therapy is essential.