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The Basic Data Analysis of Lupus Nephritis in Children (소아 루프스 신염에 대한 기초 조사)

  • Min Jae Hong;Paek Kyung Hoon;Park Kyung Mi;Kim Jung Sue;Ha Il Soo;Cheong Hae Il;Kim Joong Gon;Choi Yong
    • Childhood Kidney Diseases
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    • v.3 no.1
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    • pp.80-87
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    • 1999
  • Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.

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A Comparative Analysis of Clinical Features and Treatment Outcomes of Intussusception according to Age Distribution (연령에 따른 장중첩증의 임상양상 및 치료에 대한 비교분석)

  • Park, Bum Chul;Kim, Seok Young;Jung, Su Jin
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.150-156
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    • 2005
  • Purpose: We divided the children diagnosed with intususception into three different age groups and compared the clinical symptoms and treatment outcomes in order to analysis diagnosis and treatment of Intusussception. Methods: A retrospective chart review was established of 159 patients diagnosed with intussuception who had been admitted to Department of Pediatrics and General Surgery, Bundang CHA hospital from January, 2000 to May, 2004. We divided the patients into three groups, according to their age. Those who were under five months of age (group I; 21 patients), those between 5~11 months of age (group II; 61 patients), and those older than 11 months of age (group III; 77 patients). Then we compared the age, sex, seasonal distribution of occurrence, the cause, the clinical features, radiologic review, the type of intusussception, surgical methods, recurrence rate and treatment outcomes, among these three groups. Results: On comparing the clinical symptoms and signs among the three groups, the most common major clinical symptom and sign was irritabiltity in all three groups. Vomiting with irritability was statistically more common in group I (p<0.05) and bloody stool was most frequent in group III. The average time taken until a diagnosis was made after the symptom onset was, 21 hours in group I, 20 hours in group II and 22 hours in group III. Which showed no significant difference. But there was a higher rate of delayed diagnosis in group I, which took over 48 hours until the diagnosis was made (group I; 23.8%, group II; 4.9%, group III; 7.8%). Simple abdominal X-rays showed more frequent instances of intestinal obstruction in group I (p<0.05). The primary treatment done was barium enema which showed a failure rate of 52.4% in group I, 26.2% in group II and 14.3% in group III. Showing the highest failure rate in group I. Conclusion: The youngest group had vague symptoms which lead to delayed diagnosis and more frequent surgical procedures. As so, we advocate the importance of further evaluation and close observation, considering intussusception in children with symptoms of irritability and vomiting, especially in the early infant group.

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Influence of Climate Factors and PM10 on Rotaviral Infection: A Seasonal Variation Study (Rotavirus 감염의 연도별 유행시기의 변동양상 및 기후요소와 PM10과의 관계)

  • Im, Hae-Ra;Jeon, In-Sang;Tchah, Hann;Im, Jeong-Soo;Ryoo, Eell;Sun, Yong-Han;Cho, Kang-Ho;Im, Ho-Joon;Lee, Gwang-Hoon;Lee, Hak-Soo;Kang, Yune-Jeung;Noh, Yi-Gn
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.6 no.2
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    • pp.120-128
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    • 2003
  • Purpose: Recently, while the authors were experiencing that the epidemic period of rotaviral infection happened more in the early spring, we tried to find out how the outbreaks of rotaviral infection are changing in detail depending on the weather condition since it has something to do with the climate factors and PM10. Methods: Fourteen hundreds seventy nine patients who were proved to be positive to rotavirus were chosen among children less than 5 years old from January 1995 to June 2003. Among various climate factors, monthly average temperature, humidity, rainfall and PM10 were selected. Results: Rotaviral infection was most active in 2002 as 309 (20.9%) patients. It has been the spring that is the most active period of rotaviral infection since 2000. The temperature (RR=0.9423, CI=0.933424~0.951163), rainfall (RR=1.0024, CI=1.001523~1.003228) and PM10 (RR=1.0123, CI=1.009385~1.015248) were significantly associated with the monthly distribution of rotaviral infection. Conclusion: Through this study we determined that the epidemic period of rotaviral infection is changed to spring, which is different from the usual seasonal periods such as late fall or winter as reported in previous articles. As increased PM10 which could give serious influence to the human body, and changing pattern of climate factors such as monthly average temperature and rainfall have something to do with the rotaviral infection, we suppose that further study concerning this result is required in the aspects of epidemiology, biology and atmospheric science.

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Positive Rate of HBsAg in School Children in Incheon Area (인천 지역 초, 중, 고등학생의 B형 간염 바이러스 표면항원 양성률에 대한 조사 연구)

  • Chang, Ji Yeon;Jeong, Su Jin;Kim, Soon Ki;Son, Byong Kwan;Hong, Young Jin;Hong, Kwang Sun
    • Pediatric Infection and Vaccine
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    • v.10 no.2
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    • pp.153-158
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    • 2003
  • Purpose : The incidence of hepatitis B virus infection has gradually decreased since 1983 when hepatitis B vaccine was firstly produced. This study was performed to evaluate the efficacy of hepatitis B immunization. Methods : The elementary, middle and high school children in Incheon area were enrolled in this study in 1997 and 1998. Hepatitis B virus surface antigen(HBsAg) was measured using reversed passive hemagglutination(RPHA). Results : The results were as follows The positive rates of HBsAg in elementary, middle and high school children were 0.7%(337/46,861), 2.5%(381/15,026) and 3.1%(681/21,938) respectively in 1997 and 0.6%(257/41,946), 2.7%(379/13,652) and 2.4%(628/25,277) respectively in 1998. The positive rates of HBsAg in children under 19 years of age in 1985, 1990 and 1995 were 6.1, 5.2 and 3.5% respectively. Conclusion : The positive rates of HBsAg in elementary school children in 1997 and 1998 has decreased compared with those of the previous studies. The majority of elementary school children were given hepatitis B vaccination. These results suggest that hepatitis B vaccines used in Korea were effective for prevention of hepatitis B infection in school children.

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Utility of polymerase chain reaction(PCR) according to sampling time in CSF and stool specimens from patient with aseptic meningitis (무균성 뇌막염 환자에서 뇌척수액과 대변 채취 시점에 따른 PCR의 유용성)

  • Kim, Myo Jing;Lee, Hye Jin;Choi, Jung Mi;Jung, Soo Jin;Huh, Jae Won
    • Clinical and Experimental Pediatrics
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    • v.49 no.7
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    • pp.745-750
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    • 2006
  • Purpose : Enteroviruses are the most common cause of aseptic meningitis in patients of all ages. A definite diagnosis of enteroviral meningitis can be established by detection of virus directly in CSF specimens. But this is time-consuming and lacks sensitivity, so polymerase chain reaction(PCR) detecting of viral RNA in patient specimens such as CSF, stool has been demonstrated. But little is known about the influence of sampling time on the results of CSF PCR and stool PCR. We investigated diagnostic utility of PCR of CSF and stool according to sampling time after the onset of symptoms. Methods : PCR results were analyzed according to sampling time for 42 patients diagnosed aseptic meningits in our hospital from $11^{th}$ January to $30^{th}$ August, 2005. Results : The diagnostic yield of the test was higher of CSF specimens obtained ${\leq_-}2$ days after clinical onset(positive PCR results 9/18, 50 percent), compared with CSF collected >2 days after onset(positive PCR results 1/24, 4.2 percent)(P=0.001). Instead, positive PCR results of fecal specimens maintained highly(average 90.5 percent), 10 cases had also positive PCR results even 5-6 days after onset. 10 cases of CSF specimens had positive enterovirus PCR results containing coxsackievirus B5 (n=6), coxsackievirus B3(n=3). 38 cases of stool specimens had positive enterovirus PCR results containing echovirus 18(n=7), echovirus 9(n=3), coxsackievirus B5(n=8), coxsackievirus B3(n=3). 6 cases(coxackie B5) had positive CSF PCR and stool PCR, both. Conclusion : Stool PCR was clinically sensitive for detecting enterovirus during enteroviral meningits and could give a presumptive diagnosis throughout the disease course. A definite diagnosis was obtained by CSF PCR, but its utility was clearly lower for samples obtained >2 days after clinical onset. Therefore, it is recommended that, in addition to performance of CSF PCR, fecal samples obtained from patients with suspected enteroviral meningitis should be tested by PCR, especially when the duration of symptoms is >2 days.

Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions (심한 폐동맥 고혈압을 동반한 좌우 단락 질환 환자의 수술 후 경과)

  • Lee, Cha Gon;Jeong, Su-In;Huh, June;Kang, I-Seok;Lee, Heung Jae;Yang, Ji-Hyuk;Jun, Tae Gook
    • Clinical and Experimental Pediatrics
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    • v.53 no.2
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    • pp.195-202
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    • 2010
  • Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

The Findings and Significances of Brain SPECT in Acute Mealses Encephalitis (급성 홍역 뇌증 환아들의 뇌 SPECT 소견과 유용성)

  • Kim, Jung Chul;Choung, Ju Mi;Eun, So Hee;Lee, Dae-Yeol;Kim, Jung Soo
    • Clinical and Experimental Pediatrics
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    • v.45 no.11
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    • pp.1373-1380
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    • 2002
  • Purpose : Acute measles encephalitis(ME) is characterized by an abrupt onset of fever and obtundation, frequently accompanied by seizures and multifocal neurological signs. The aim of this study was to clarify the clinical manifestation, progression and the brain SPECT patterns in patients with acute ME. Methods : This study included 11 children with acute ME admitted to Chonbuk National University Hospital. Ten patients received a first dose of measles vaccine, one patient did not receive a first dose, and no patients received a second dose. ME was diagnosed based on characteristic clinical pictures, measles antibodies by ELISA and abnormal CSF findings. Brain MRI and brain SPECT were performed in 11 patients with acute ME. Results : There were four males and seven females whose ages at onset ranged between 18 months and 14 years(mean : 10.5 years). The main clinical neurologic pictures were loss of consciousness( 10) and seizure(five). The titer of IgG and IgM antimeasles antibodies in serum were positive in 10 patients. In CSF, nine patients had IgG antibodies and one patient had IgM antibodies. The concentration of protein(mean : $124{\pm}60mg/dL$) and WBC counts(mean : $158{\pm}157/{\mu}L$) in CSF were elevated in all patients. In electroencephalographic examination, nine patients showed increased slow waves. Seven of 11 patients(63.6%) revealed high signal intensity on the brain MRI. In contrast, all patients showed hypoperfusion in brain SPECT examination. According to brain SPECT, the perfusion deficits were frequently observed in the frontal lobe(nine), temporal (nine), parietal(eight) and thalamus(eight). Conclusion : Brain SPECT is more sensitive than MRI for the evaluation of brain damage in early stages of acute ME.

Allele Distribution and Frequency of Human Surfactant Protein-A1 in Korean Neonates (한국 신생아의 폐 표면 활성제 단백-A1 (Human Surfactant Protein-A1) 유전자 대립형질의 분포와 빈도)

  • Lee, Kyung Shin;Kim, Young Hee;Suk, Jung Su;Ko, Jung Ho;Yoo, Ook Joon;Lee, In Kyu;Oh, Myung Ho;Bae, Chong Woo
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1497-1502
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    • 2002
  • Purpose : We evaluated allele frequencies and distribution of surfactant protein A1(SP-A1) in Korean neonates in order to estimate prevalence of RDS to find out new SP-A alleles, and to establish new steroid therapy. Methods : Genomic DNA was extracted from 100 neonates and served as a template in PCR for genotype analysis. SP-A gene-specific amplications and gene-specific allele determinations were performed using PCR-RFLP methods. Results : The distribution for the alleles of the SP-A1 gene in the study population were 6A, $6A^2$, $6A^3$, $6A^4$, $6A^8$, $6A^9$, $6A^{10}$, $6A^{11}$, $6A^{12}$, $6A^{13}$, $6A^{14}$, $6A^{15}$, $6A^{16}$, $6A^{17}$, $6A^{18}$, $6A^{20}$. The specific frequencies for the alleles of the SP-A1 gene in the study population were : $6A^2=21%$, $6A^3=45%$, $6A^4=11%$, $6A^8=9%$, $6A^{14}=8%$. Conclusion : The frequency of $6A^3$ was higher than the other SP-A1 alleles in Korean neonates. This finding suggests that the prevalence of RDS in Korea may be low compared with other countries. However, this finding also suggests that Korean neonates have a high risk of infection.

The Usefulness of $^{18}F$-FDG PET/CT for Predicting the Response of Locally Advanced Rectal Cancer to Neoadjuvant Chemoradiation Therapy (국소 진행된 직장암의 $^{18}F$-FDG PET/CT를 이용한 항암방사선치료의 반응성 예측)

  • Kang, Jin-Kyu;Kim, Mi-Sook;Choi, Chul-Won;Jeong, Su-Young;Yoo, Seong-Yul;Cho, Chul-Koo;Yang, Kwang-Mo;Yoo, Hyung-Jun;Cheon, Gi-Jeong;Shin, Young-Joo;Seo, Young-Seok
    • Radiation Oncology Journal
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    • v.27 no.3
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    • pp.111-119
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    • 2009
  • Purpose: This study aimed at assessing the value of fluorine-18 fluorodeoxyglucose positron emission tomography ($^{18}F$-FDG PET) for predicting the response of locally advanced rectal cancer to neoadjuvant CRT. Materials and Methods: Between August 2006 and January 2008, we prospectively enrolled 20 patients with locally advanced rectal cancer and who were treated with neoadjuvant CRT at the Korea Institute of Radiological and Medical Sciences. The treatment consisted of radiation therapy and chemotherapy, and this was followed by curative resection 6 weeks later. All the patients underwent $^{18}F$-FDG PET/CT both before CRT and 6 weeks after completing CRT. The measurements of the FDG uptake ($SUV_{max}$), the absolute difference (${\Delta}SUV_{max}$) and the percent $SUV_{max}$ difference (response index, $RI_{SUV}$) between the pre- and post-CRT $^{18}F$-FDG PET/CT scans were assessed. The measurements of the metabolic volume, the absolute difference (${\Delta}$metabolic volume) and the percent metabolic volume difference (response index, $RI_{metabolic\;volume}$) were also assessed. Results: Of the 20 patients who underwent surgery, 11 patients (55%) were classified as responders according to Dworak's classification. The post-CRT $SUV_{max}$ was significantly lower than the pre-CRT $SUV_{max}$. However, there were no significant differences in the $SUV_{max}$ and the metabolic volume reduction between the responders and non-responders. We used a minimum $SUV_{max}$ reduction of 67% as the cut-off value for defining a response, with a sensitivity of 45.5%, a specificity of 88.9%, a positive predictive value of 77% and a negative predictive value of 53.8%. Conclusion: Although there were no statistically significant results in this study, other studies have revealed that $^{18}F$-FDG PET/CT has the potential to assess the tumor response to neoadjuvant CRT in patients with locally advanced rectal cancer.

Radiation Response Modulation of GW572016 (EGFR/HER2 Dual Tyrosine Kinase Inhibitor) in Human Breast Cancer Xenografts (인간 유방암 세포 이식마우스에서 EGFR/HER2 복합 Tyrosine Kinase 억제제인 GW572016에 의한 방사선증진효과)

  • Kim, Yeon-Sil;Roh, Kwang-Won;Chae, Soo-Min;Mun, Seong-Kwon;Yoon, Sei-Chul;Jang, Hong-Seok;Chung, Su-Mi
    • Radiation Oncology Journal
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    • v.25 no.4
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    • pp.233-241
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    • 2007
  • Purpose: We examined the effect of the dual EGFR/HER2 tyrosine kinase inhibitor, GW572016, on EGFR/HER2 receptor phosphorylation, inhibition of downstream signaling and radiosensitization in either an EGFR or HER2 overexpressing human breast cancer xenograft. Materials and Methods: We established SCID mice xenografts from 4 human breast cancer cell line that overexpressed EGFR or HER 2 (SUM 102, SUM 149, SUM 185, SUM 225). Two series of xenografts were established. One series was established for determining inhibition of the EGFR/HER2 receptor and downstream signaling activities by GW572016. The other series was established for determining the radiosensitization effect of GW572016. Inhibition of the receptor and downstream signaling proteins were measured by the use of immunoprecipitation and Western blotting. For determining the in vivo radiosensitization effect of GW572016, we compared tumor growth delay curves in the following four treatment arms: a) control; b) GW572016 alone; c) radiotherapy (RT) alone; d) GW572016 and RT. Results: GW572016 inhibited EGFR, HER2 receptor phosphorylation in SUM 149 and SUM 185 xenografts. In addition, the p44/42 MAPK (ERK 1/2) downstream signaling pathway was inactivated by GW572016 in the SUM 185 xenograft. In the SUM 225 xenograft, we could not observe inhibition of HER2 receptor phosphorylation by GW572016; both p44/42 MAPK (Erk1/2) and Akt downstream signal protein phosphorylation were inhibited by GW572016. GW572016 inhibited growth of the tumor xenograft of SUM 149 and SUM 185. The combination of GW572016 and RT enhanced growth inhibition greater than that with GW572016 alone or with RT alone in the SUM 149 xenograft. GW572016 appears to act as an in vivo radiosensitizer. Conclusion: GW572016 inhibited EGFR/HER2 receptor phosphorylation and downstream signaling pathway proteins. GW572016 modestly inhibited the growth of tumor in the SUM 185 xenograft and showed radiosensitization in the SUM 149 xenograft. Our results suggest that a better predictor of radiation response would be inhibition of a crucial signaling pathway than inhibition of a receptor.