• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Childhood Kidney Diseases
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• v.15 no.1
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• pp.66-75
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• 2011

Purpose: Early diagnosis and treatment of febrile urinary tract infection (UTI) in children is important to prevent kidney damage. This study aims to evaluate the relationship between the presence of pyuria, the severity, and underlying genitourinary anomalies in patients with UTI. Methods: We retrospectively reviewed 293 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from June, 2007 until January, 2010. We divided the patients into two groups, one with the finding of pyuria at admission, and the other without, and compared the fever duration, white blood cell counts (WBC) and C-reactive protein (CRP) in peripheral bloods, hydronephrosis, cortical defects, vesicoureteral reflux and admission period. Results: Among the 293 patients with febrile UTI, 189 patients showed findings of pyuria whereas 104 patients did not. Patients with pyuria showed an increment of WBC ($14,694{\pm}485.2$ vs. $11,374{\pm}451.2/uL$, P <0.05) and CRP ($46.9{\pm}3.9$ vs $17.1{\pm}3.6$ mg/L, P <0.05) in peripheral blood sample. The presence of cortical defects (21.7 Vs 5.8%, P <0.05) and vesicoureteral reflux (15.9 Vs 6.7%, P <0.05) was also increased in patients with pyuria compared to patients without pyuria. There were no specific differences in fever duration, admission period, and hydronephrosis. Within the group with pyuria, CRP in peripheral blood sample increased proportionally with the increment of pyuria (P <0.05). Conclusion: In patients with febrile UTI, the increment of WBC in the urine sample can be a helpful predictor for increased CRP in peripheral blood and acute pyelonephritis.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.875-881
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• 2007

Purpose : Chromosome analysis is important in genetic study and genetic counseling. This study was performed to evaluate the type and incidence of chromosome abnormalities in a single hospital for 25 years. Methods : Chromosome analyses were performed on peripheral blood lymphocytes, obtained from 4,856 patients with suspected chromosomal aberrations, referred to cytogenetic laboratory in Department of Pediatrics, Kyungpook National University Hospital from May 1981 to October 2005. Results : We analyzed 4,567 cases. Children were 3,014 cases (66.0%) and adult were 1,553 cases (34.0 %). The most common purpose of the chromosomal analysis was growth and developmental abnormality in children and infertility in adults. Total chromosomal aberration rate was 16.9% (770/4,567). Among those cases, the numerical abnormalities were 12.2% (558 cases), the structural abnormalities were 4.1% (187 cases), and others were 0.5% (25 cases). The relative frequencies of autosomal abnormalities were 6.4% (294 cases) in Down syndrome; 0.2% (7 cases) in Edwards syndrome; 0.1% (4 cases) in Patau syndrome; 0.2% (10 cases) in other abnormalities, of sex chromosome, 2.9% (131 cases) in Klinefelter syndrome; 2.2% (99 cases) in Turner syndrome; 0.2% (8 cases) in 47, XXX; 0.1% (3 cases) in 47, XYY. Among the structural abnormalities, translocation was 1.8% (84 cases), inversion was 0.8% (37 cases), deletion was 0.4% (17 cases), and insertion was 0.3% (13 cases), in order of frequency. Conclusion : In this study, the type, incidence and distribution of cytogenetic abnormalities by karyotype were reviewed. We hope that our study could be used as a basic information on the diagnosis, treatment and genetic counseling for chromosome abnormalities in Korea.

• Journal of agricultural medicine and community health
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• v.34 no.3
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• pp.334-345
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• 2009

Objectives: This study was performed to investigate the management status of chronic disease at community health centers. Methods: The study subjects were 450 employed persons at community health centers in Jeonlanamdo. General characteristics, status of chronic disease management and health education were collected for statistical analysis. The differences on management status among working areas were assessed with chi-square test and ANOVA. Results: The mean values of work duration were 18.7 years in rural, 14.4 years in fishing village, and 17.4 years in urban-rural, respectively. The number of management registration was highest in hypertension. The ways of disease management were medication check-up, diet stopping smoking. The contents of group health education were exercise, diet and prohibition of smoking. The place of health education was town assembling hall. The fields which officials wanted to be educated were symptom, diagnosis, treatment and complication. The most important field was early detection of chronic disease patients and health education. Finally, the field requiring support was work standardization. Conclusions: Community health workers have worked positively in chronic disease management. The barriers to work were the lack of professional and preliminary data. The support system with other health organ and health education were needed for the improvement of working ability.

• Tuberculosis and Respiratory Diseases
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• v.65 no.3
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• pp.191-197
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• 2008

Background: Due to the irreversible nature of chronic obstructive pulmonary disease (COPD), the treatment aim in patients with COPD is not to cure but to reduce the symptoms, increase lung function, and improve the quality of life. It has been suggested that depression is a common emotional disturbance in patients with COPD who are faced with a major physical impairment and embarrassing symptoms. This study evaluated the prevalence and risk factors of depression in patients with chronic obstructive pulmonary disease. Methods: A total of 59 patients with a registered diagnosis of chronic obstructive pulmonary disease were selected. Depression was assessed using the Centers for Epidemiologic Studies Depression (CES-D) scale. The quality of life was assessed using the Korean version of the St. George's Respiratory Questionnaire. Results: The prevalence of depression was 17.0%. In the correlation model, the interaction of the $FEV_1%$ over predicted value and SGRQ score(symptom, activity, impact, overall score) was statistically significant. The interaction of the $FEV_1%$ over predicted value and depression scale(CES-D) was also statistically significant. There was a positive correlation between the SGRQ scores(symptom, activity, impact, overall score) and the depression scale. Conclusion: The prevalence of depression in patients with chronic obstructive pulmonary disease is relatively high. The pulmonary function and the living standards were found to be significant risk factors for depression.

• The Korean Journal of Nuclear Medicine
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• v.31 no.4
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• pp.452-458
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• 1997

To evaluate the pinhole scintigraphic findings and its significance, authors retrospectively compared the pinhole bone scintigrams and corresponding radiograms of 16 lesions in 14 patients with fibrous dysplasia. They were diagnosed pathologically in 10 lesions and radiologically in 6 lesions. The mean age of patients was 41.1 years. The mean interval between two studies was 1.1 days. Locations were ribs 7, pelvic bone 4, clavicle 1, long bones 4(femur 2, tibia 1, humerus 1). The radiographic findings were as follows : the central portions were radiolucent(n=9), ground-glass opacities(n=5) or sclerotic(n=2) and the peripheral appearance were sclerotic rim(n=5), septation(n=7), cortical perforation (n=10) and invisible cortical thinning(n=9). Pinhole scintigraphic findings were as follows; Central portions showed normal 1+ uptake in 6 cases(radiolucent 5, ground-glass opacity 1), slightly increased 2+ upta- ke in 7 cases(radiolucent 4, ground-glass opacity 3), and marked 3+ uptake in 3 cases(ground-glass opacity 1, sclerotic 2). The 15 of 16 lesions showed more intense uptake in the peripheral portion: slightly increased 2+ uptake corresponding to the sclerotic rim(5/5) and unvisible cortical thinning(1/9), and irregular foci of marked 3 + uptake corresponding to septation(7/7), cortical perforation(10/10) and invisible cortical thinning (8/9). One of 16 lesions showed homogeneous 2+ uptake. In conclusion, pinhole scintigram provides information on regional activity of the fibrous dysplasia, which would be helpful in diagnosis, prediction of prognosis and determination of treatment plan.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.95-103
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• 1998

Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.

• Pediatric Infection and Vaccine
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• v.10 no.2
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• pp.186-192
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• 2003

Purposes : Recently, acute gastroenteritis broke out among the neonates transferred from postpartum care centers located in Seoul, and Gyeounggi area. Thus, we researched the cause, progress and characters of the disease by analyzing the cases from 9 hospitals in Seoul and Gyeounggi area. Methods : We conducted retrospective study of 33 neonates transferred from postpartum care centers from October 2001 to July 2002 : we divided the neonates into 2 groups. The severe group had any of following 5 conditions : blood pH <7.20, respiratory difficulty, mechanical ventilation, shock, and disseminated intravascular coagulation. And the rest were classified into the moderate group. Results : The severe group was significantly more aged than the moderate group(P= 0.005). Weight loss was significantly severe in the severe group(P=0.0512). In blood gas analysis, bicarbonate was less in the severe group than the moderate group(P=0.032). In the virus examination, rotavirus was detected in 7 cases, and astrovirus was detected in 1 case. In the severe group, 4 neonates were dead. Conclusion : In acute gastroenteritis of neonates, early diagnosis and treatment are important. Thus, the legislation of postpartum care center is needed, and the medical specialists should be stationed in postpartum care center. We assume that the severe group had severe conditions because they were treated comparatively late. However, to find out the cause of the disease and to cope with it, the nationwide epidemiologic study on acute gastrenteritis of neonates is needed.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.186-190
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• 2005

Purpose : The purpose of this study was to evaluate the responses and toxicities of risk-adapted chemotherapy in pediatric intracranial germ cell tumors(IC-GCT). Methods : Fourteen patients who were diagnosed as IC-GCT from October 2002 to December 2003 received chemotherapy as an initial treatment modality. The low risk(LR) group was defined as follows : Pure germinoma and normal AFP level. Beta-hCG level 50 mIU/mL or less. The others belonged to the high risk(HR) group. Chemotherapy was composed of cisplatin, cyclophosphamide, etoposide and vincristine. Double doses of cisplatin and cyclophosphamide was used in HR patients. Results : Pathologic confirmation was done in all but one. Median age at diagnosis was 11.6 yr (1.2-18.7 yr), and nine patients belonged to the HR group. Tumor markers were normalized after chemotherapy in all patients whose tumor markers had been elevated. Four LR patients(80 percent) and seven HR patients(77.8 percent) showed complete response(CR) at the end of chemotherapy. An additional two of the three patients with partial response(PR) achieved CR after radiation therapy (RT), and the remaining one relapsed before RT. Four LR and all HR patients experienced infectious episodes that required hospitalization. Four of the nine HR patients(44.4 percent) suffered from tinnitus, three of whom developed sensorineural hearing loss. All but one are surviving, event-free, with a median follow-up of 13.9 mo(8.1-22.3 mo). Conclusion : Risk-adapted cisplatin-based chemotherapy was effective even in HR patients, but regimen modification seems to be necessary to avoid an unacceptably high toxicity rate.