• Tuberculosis and Respiratory Diseases
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• v.43 no.3
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• pp.461-466
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• 1996

Diffuse alveolar hemorrhage is a very rare manifestation in Henoch-Schoenlein purpura. Recently we experience a case of diffuse alveolar hemorrhage associated with Henoch-Schoenlein purpura which was diagnosed by typical clinical manifestation and renal biopsy. A 25 year old male was admitted due to hemoptysis and dyspnea. Chest X-ray, HRCT and BAL revealed diffuse alveolar hemorrhage. He also had a history of skin rash, polyarthralgia, and hematochezia with abdominal pain. Renal biopsy which was taken for the evaluation of microscopic hematuria showed IgA nephropathy. Under the diagnosis of Henoch-Schoenlein purpura, we treated him with solumedrol pulse therapy, plasma-pheresis and prednisolone with cytoxan. After then he showed marked improvement in clinical manifestation and was discharged with prednisolone and cytoxan.

• Tuberculosis and Respiratory Diseases
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• v.44 no.6
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• pp.1382-1389
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• 1997

Hantavirus pulmonary syndrome(HPS) is a systemic disease that is caused by a newly discorved and characterized virus of the Hantavirus genus, which is most frequently referred to as the sin nombre virus. The clinical syndrome resembles other hantavirus syndromes worldwide, except that it is characterized by a brief prodromal illness followed by rapidly progressive, noncardiogenic edema, and that it is more deadly than any previously recognized hantavirus infection. The clinical manifestations of HPS are characterized by four clinical phases : prodrome, pulmonary edema and shock, diuresis, and convalescence. Mortality is greatest in the first 24 hours of the pulmonary edema and shock phase of the illness. These phases are strikingly similar to the clinical phases of Hemorrhagic fever with renal syndrome(HFRS) induced by Hantaan virus, except that HPS has not been associated with renal failure and Disseminated intravascular coagulation(DIC). We here report a case of hantavirus pulmonary syndrome developed in a 58 year-old man. He had a flu-like illness followed by the rapid onset of respiratory failure due to noncardiogenic pulmonary edema. HPS was diagnosed by clinical manifestations, identification of high titer antibody to Hantaan virus antigen and histologic finding of transbronchial lung biopsy (TBLB) specimen. The patient was treated with mechanical ventilation and initial corticosteroid pulse therapy resulting in successful outcome.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.1-9
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• 2010

Slowing the progression of chronic kidney disease is much more important in children and adolescents with a relatively longer remaining life span. A practical way to assess the rate of progression of chronic kidney disease is to measure the change of GFR estimated by formulae. To slow the progression, hypertension and proteinuria have to be controlled strictly, and hypoplastic anemia must be treated with erythropoietin. If not contraindicated, ACE inhibitor or angiotensin receptor blocker is recommended with monitoring of the side effects. Trials to slow the progression should be commenced as soon as the chronic kidney disease is confirmed and needs to be continued until renal transplantation as long as residual renal function remains. An online system, the Korean Pediatric Chronic Kidney Disease Registry (http://pedcrf.or.kr/), provides tools that are useful in evaluation and management of the children and adolescents with chronic kidney diseases.

• Journal of Preventive Medicine and Public Health
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• v.52 no.1
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• pp.1-13
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• 2019

Objectives: In recent years, serious concerns have been raised regarding the impacts of rising temperatures on health. The present study was conducted to investigate the relationship between elevated temperatures and kidney disease through a systematic review and meta-analysis. Methods: In October 2017, 2 researchers independently searched related studies in PubMed and Embase. A meta-analysis was conducted using a random-effects model, including only studies that presented odds ratios, relative risks, or percentage changes, along with 95% confidence intervals (CIs). The characteristics of each study were summarized, and the Egger test and funnel plots were used to evaluate publication bias. Results: Eleven studies that met the criteria were included in the final analysis. The pooled results suggest an increase of 30% (95% CI, 20 to 40) in kidney disease morbidity with high temperatures. In a disease-specific subgroup analysis, statistically significant results were observed for both renal colic or kidney stones and other renal diseases. In a study design-specific subgroup analysis, statistically significant results were observed in both time-series analyses and studies with other designs. In a temperature measure-specific subgroup analysis, significant results were likewise found for both studies using mean temperature measurements and studies measuring heat waves or heat stress. Conclusions: Our results indicate that morbidity due to kidney disease increases at high temperatures. We also found significant results in subgroup analyses. However, further time-series analyses are needed to obtain more generalizable evidence.

• Childhood Kidney Diseases
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• v.26 no.1
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• pp.40-45
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• 2022

Purpose: Chronic kidney disease (CKD) has various underlying causes in children. Identification of the underlying causes of CKD is important. Genetic causes comprise a significant proportion of pediatric CKD cases. Methods: In this study, we performed whole-exome sequencing (WES) to identify genetic causes of pediatric CKD. From January to June 2021, WES was performed using samples from pediatric patients with CKD of unclear etiology. Results: Genetic causes were investigated using WES in 37 patients (17 males) with pediatric CKD stages 1 (n=5), 2 (n=7), 3 (n=2), 4 (n=2), and 5 (n=21). The underlying diseases were focal segmental glomerulosclerosis (n=9), congenital anomalies of the kidney and urinary tract including reflux nephropathy (n=8), other glomerulopathies (n=7), unknown etiology (n=6), and others (n=7). WES identified genetic causes of CKD in 12 of the 37 patients (32.4%). Genetic defects were discovered in the COL4A4 (n=2), WT1 (n=2), ACTN4, CEP290, COL4A3, CUBN, GATA3, LAMA5, NUP107, and PAX2 genes. WT1 defects were found in patients whose pathologic diagnosis was membranoproliferative glomerulonephritis, and identification of CUBN defects led to discontinuation of immunosuppressive agents. Genetic diagnosis confirmed the clinical diagnosis of hypoparathyroidism, sensorineural deafness, and renal disease; Alport syndrome; and Joubert syndrome in three of the patients with CKD of unknown etiology (COL4A4 [n=2], CUBN [n=1]). Extrarenal symptoms were considered phenotypic presentations of WT1, PAX2, and CEP290 defects. Conclusions: WES provided a genetic diagnosis that confirmed the clinical diagnosis in a significant proportion (32.4%) of patients with pediatric CKD.

• Development and Reproduction
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• v.23 no.2
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• pp.79-92
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• 2019

Humanized mice, containing engrafted human cells and tissues, are emerging as an important in vivo platform for studying human diseases. Since the development of Nod scid gamma (NSG) mice bearing mutations in the IL-2 receptor gamma chain, many investigators have used NSG mice engrafted with human hematopoietic stem cells (HSCs) to generate functional human immune systems in vivo, results in high efficacy of human cell engraftment. The development of NSG mice has allowed significant advances to be made in studies on several human diseases, including cancer and graft-versus-host-disease (GVHD), and in regenerative medicine. Based on the human HSC transplantation, organ transplantation including thymus and liver in the renal capsule has been performed. Also, immune reconstruction of cells, of the lymphoid as well as myeloid lineages, has been partly accomplished. However, crosstalk between pluripotent stem cell derived therapeutic cells with human leukocyte antigen (HLA) mis/matched types and immune CD3 T cells have not been fully addressed. To overcome this hurdle, human major histocompatibility complex (MHC) molecules, not mouse MHC molecules, are required to generate functional T cells in a humanized mouse model. Here, we briefly summarize characteristics of the humanized mouse model, focusing on development of CD3 T cells with MHC molecules. We also highlight the necessity of the humanized mouse model for the treatment of various human diseases.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.156-163
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• 2001

Purpose We performed urinary mass screening(UMS) program for 2,804 children of second grade elemantary school 8 years of age in Paju city with cooperation of Paju City Health Center to determine the prevalence of asymptomatic proteinuria and hematuria, and to estimate the risk of incipient renal diseases. Also we attempted to evaluate the significance of hematuria in UMS in addidtion to proteinuria. Methods : 2,804 children of the 2nd grade of elementary school who lived in Paju city were included to our UMS program in 2000. They were constituted with 1,428 boys and 1,376 girls. The screening program was carried out in 3 steps The 1st screenig test was performed at schools and then students with abnormal results were examined repeatedly at Paju City Health Center and our hospital. Those students who showed proteinuria and/or hematuria in the 1st and 2nd test were referred to our hospital to undertake the 3rd close examination including physical examination, laboratory tests and radiologic tests. Results : (I) The prevalence of urinary abnormality in the 1st screening test was $8.3\%$(233 students), comprised of $5.9\%$ of boys, $10.8\%$ of girls. (2) Among 2,804 children tested in the first screening, prevalences of asymptomatic proteinuria and isolated hematuria were 64($2.3\%$), 163($5.8\%$) respectively, and the prevalence of proteinuria with hematuria was 6($0.2\%$). (3) Among 233 students with urinary abnormalities at the 1st screening test, 102 students applied to the 2nd test. 32 children, about one third of them, were also found to have abnormal urinary findings ; isolated hematuria 30, proteinuria with hematuria 2. (4) Those findings of clinical evaluation for children with isolated hematuria at the hospital showed as follows: idiopathic isolated microscopic hematuria 21, normal 6, urinary tract infection 1, idiopathic hypercalciuria 1 and simple renal cyst 1. Those 2 students with proteinuria and hematuria seemed to have chronic glomerulonephritis. Conclusion : (1) The clinical evaluation for children who showed positive results at the 1st screening test should be done judiciously. Because of high false positive rate, almost who showed positive results was normal, only a few of them had pathologic conditions. In this study, actual incidence of incipient renal diseases in children of 8 year old was calculated to be $0.4\%$. (2) The definite conclusion whether a urinary mass screening test can alter the prognosis of incipient renal diseases could not be drawn with this study. Further study must be necessary (3) We could acknowledge the significance of hematuria in UMS, but it is necessary that one should be judicious in managing and follow-up those that show abnormal results. (J Korean Soc Pediatr Nephrol 2001;5 : 156-63)

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.203-208
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• 1999

Purpose: The information on the change of the relative renal function after operation is essential to set the initial treatment plan in unilateral hydronephrosis. So we analyzed the preoperative and postoperative relative renal function, and observed the functional changes by operation and reliability of the various factors in those changes. Materials and Methods: A retrospective trial was done of 81 patients, 35 ureteropelvic junction(UPJ) obstruction and 46 vesicoureteral reflux(VUR), among 393 patients undergoing operations at our institution from March. 1992 to February. 1997. The patients who had infravesical abnormalities, abnormal contralateral kidney and insufficient research data were excluded. We determined the relative renal functions using DTPA and MAG3 scan in UPJ obstruction and DMSA scan in VUR. The mean observation period after operation was 2.01(0.25-4) years. Results: A. UPJ Obstruction 1. The relative renal function was improved significantly after operation(P=0.0007). 2. The kidneys which have preoperative functions between 20% and 40% improved significantly in relative renal function comparing to the kidneys of the other functions(P=0.0046). B. VUR 1. The renal functions didn't improve significantly after operation(P>0.05). 2. There was no significant factor affecting the prognosis in renal functions. Conclusions: 1. The kidneys with UPJ Obstruction show the difference in functional improvement after operation according to the degree of the preoperative renal function, but more investigations about the exact value are needed. 2. In VUR there was no difference between preoperative and postoperative functions, the theoretical basis on objectives of the operation could be the symptoms or factors except the operation.

• Pediatric Infection and Vaccine
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• v.5 no.2
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• pp.221-229
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• 1998

Purpose : The symptom of urinary tract infection in children is non-specific and systemic compared to that of adults. So the diagnosis of UTI in children can be delayed. If UTI in children is not appropriately managed, it may he resulted in renal failure, hypertension, growth failure in adolescence because there are already documented renal scar or urinary tract abnormality in infection period. Especially upper UTI that involve renal parenchyme may be result in fatal complication. The purpose of this study is analyzing the relationship between acute reactive marker and $^{99m}TC$-DMSA renal scan in upper urinary tract infection. Methods : This study included 56 children admitted at Dankook University Hospital Pediatric Department in Jan. 1995~May. 1998. We analyzed quantatively the results of acute reactive marker(CRP, ESR, WBC), pyuria, fever and compared to those of sonographically find ing and $^{99m}TC$-DMSA renal scan. Comparison between groups were performed by the chi-square (x2) test and a p value of less than 0.05 was considered statistically significant. Results : 1) The number of boys less than 1 year of age was larger than that of girls. But the number of boys more than 1 year of age was reversed. 2) The higher me level of reactive marker (CRP, WBC), the more the probability of upper UTI. 3) The higher fever, the more the probability of upper UTI. 4) The more pyuria, the more probability of upper UTI. 5) The more higher the grade of vesicoureteral reflux, the more probability of upper UTI. 6) $^{99m}TC$-DMSA renal scan is more sensitive and more specific diagnostic tool than renal sonogram. Conclusion : The appearance of an abnormal $^{99m}TC$-DMSA renal scan is correlated with acute reactive marker (CRP, ESR, WBC), fever, pyuria. $^{99m}TC$-DMSA renal scan can be a good valuable predictor tool in upper UTI. So we can start early treatment and decrease the incidence of complication of upper urinary tract by above indicators before knowing the result of urine culture. And we can follow up the patients in more good relationships with their parents by telling them the duration of treatment and follow-up plan.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.92-101
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• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.