• 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.4 no.2
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• pp.144-153
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• 2000

Purpose : Effects to predict tile progression of chronic renal failure (CRF) in children, using mathematical models based on transformations of serum creatinine (Scr) concentration, have failed. Error may be introduced by age-related variations in creatinine production rate. Height (Ht) is a reliable reference for creatinine production in children. Thus, Scr, factored for Ht, could provide a more accurate predictive model. We examined this hypothesis. Methods : The progression of of was detected in 63 children who proceeded to end-stage renal disease. Derivatives of Scr, including 1/Scr, log Scr & Ht/Scr, were defined fir the period Scr was between 2 and 5 mg/dl. Regression equation were used to predict the time, in months, to Scr > 10 mg/dl. The prediction error (PE) was defined as the predicted time minus actual time for each Scr transformation. Result : The PE for Ht/Scr was lower than the PE for either 1/Scr or log Scr (median: -0.01, -2.0 & +10.6 mos respectively; P<0.0001). For children with congenital renal diseases, the PE for Ht/Scr was also lower than for the other two transformations (median: -1.2, -3.2 & +8.2 mos respectively; P<0.0001). However, the PEs for children with glomerular diseases was not as clearly different (median: +0.9, +0.5 & +9.9 respectively). In children < 13 yrs, PE for Ht/Scr was tile lowest, while in older children, 1/Scr provided the lowest PE but not significantly different from that for Ht/Scr. The logarithmic transformation tended to predict a slower progression of CRF than actually occurred. Conclusion : Scr, floored for Ht, appears to be a useful model to predict the rate of progression of CRF, particularly in the prepubertal child with congenital renal disease.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.73-79
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• 2012

Purpose: The association of mitochondrial DNA (mtDNA) mutations, deletions and copy number with progressive changes in patients with some glomerular disease and end-stage renal disease have been reported. In this study, we performed mtDNA mutation analysis in children with IgA nephropathy to investigate its role in progressive clinical course. Methods: Seven children with IgA nephropathy were involved in this study. MtDNA isolated from platelet was amplified by PCR and sequenced entirely. Results: The mean age at renal biopsy was $11.5{\pm}2.2$ year and the mean age at latest evaluation was $17.9{\pm}3.2$ year. The mean follow-up period were $7.8{\pm}3.1$ years. Patients was divided into 2 groups according to the amount of proteinuria at presenting manifestation. Group 2 patients were nephrotic syndrome. Renal function reveals within normal range in all patients. In group 2 patients, the mean serum albumin level was significantly lower than those of group 1 ($3.7{\pm}0.6g/dL$ vs. $4.7{\pm}0.2g/dL$, P=0.0241) and the mean total cholesterol level was significantly higher than those of group 1 ($222.7{\pm}35.7mg/dL$ vs. $148.3{\pm}29.1mg/dL$, P=0.0283). In Group 2 patients, total amount of protein of 24 hour collected urine also significantly higher than those of group 1 ($1,466.0{\pm}742.5mg$ vs. $122.5{\pm}48.1mg$, P=0.0135). Pr/Cr ratio in random urine sample was also higher in group 2 than those of group 1 but the statistical significance was not noted ($1.8{\pm}1.6$ vs. $0.2{\pm}0.2$, P=0.0961). Deletion of mtDNA nt 8272-8281 were observed in two patients, one patient in each groups, respectively. This is noncoding lesion. No patients demonstrated the mtDNA mutations. Conclusions: We have identified a deletion of mtDNA nt 8272-8281 in two children with IgA nephropathy. Further studies are needed to clarify the role of mitochondrial function in the progressive change of IgA nephropathy.

• Nutrition Research and Practice
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• v.3 no.3
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• pp.247-252
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• 2009

The present study was to investigate the nutritional status and factors related to malnutrition in end-stage renal disease (ESRD) patients requiring hemodialysis (HD) in South Korea. Subjects were ESRD outpatients from general hospitals or HD centers in Seoul referred to the dialysis clinic for maintenance HD care. A total of 110 patients (46 men and 64 women; mean ages $58.6{\pm}1.0y$) were eligible for this study. The family history of chronic renal failure (CRF) was considered positive if a patient reported having either a first-degree or second-degree relative with CRF. Malnutrition was defined as a triceps skinfold thickness or mid-ann muscle circumference below the fifth percentile for age and sex and forty-seven of the 110 patients were malnourished. Almost all (94%) patients had anemia (hemoglobin: <13 g/dL for men and <12 g/dL for women). Energy intake was below the recommended intake levels of energy [30-35 kcal/kg ideal body weight (IBW)] and protein (1.2 g/kg IBW) in 60% of patients. The duration of HD was longer in malnourished HD patients (P=0.0095). Malnutrition was more prevalent in women (P=0.0014), those who never smoked (P=0.0007), nondiabetic patients (P=0.0113), and patients with bone diseases (P=0.0427), adequate HD (spKt/$V{\geq}1.2$) (P=0.0178), and those with a family history of CRF (P=0.0255). Multiple logistic regression was used to examine the relationship between malnutrition and potential risk factors. After adjusting for age, sex, and other putative risk factors for malnutrition, the OR for malnutrition was greater in HD patients with a family history of CRF (OR, 3.290; 95% CI, $1.003{sim}10.793$). Active nutrition monitoring is needed to improve the nutritional status of HD patients. A family history of CRF may be an independent risk factor for malnutrition in Korean HD patients. A follow-up study is needed to investigate whether there is a causal relationship between a family history of CRF and malnutrition in Korean ESRD patients.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Health Policy and Management
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• v.19 no.3
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• pp.92-108
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• 2009

This study suggests a model for continuing and comprehensive management of hypertension or Type 2 diabetes mellitus (T2DM) in Korea. Moreover, this paper computed the contribution cost of hypertension or T2DM management using the healthcare medical cost, which could have occurred from stroke, myocardial infarction (MI), and end-stage renal disease (ESRD) that were successfully prevented from the effective hypertension or T2DM management. Additionally, these costs were compared with the cost of implementing the hypertension or T2DM management model suggested in this study. This study used the medical fee summary of the health insurance claims submitted to National Health Insurance Corporation by medical facilities for services provided during the period from January 1st 1999 to December 31st 2006. The prevalence rate with treatment referred to cases in which patients submitted their medical claims at least once during the period, along with an accordant diagnosis. The incidence rate with treatment referred to cases in which patients who never submitted claims for the accordant disease during the five years from 1999 to 2003 submitted claims for the accordant disease in 2004 and 2005. The relative risk of the occurrence of stroke, MI and ESRD was 11.0, 13.6, and 30.3, respectively. The attributable risk of hypertension or T2DM for stroke was 0.730, and that for MI and ESRD were 0.773 and 0.888, respectively. Based on these, the contribution cost of hypertension or T2DM is estimated to be 986.3 billion Korean Won(KRW) for stroke patients, 330.5 billion KRW for MI patients, and 561.7 billion KRW for ESRD patients as in 2005. Hence, the total contribution cost of hypertension or T2DM to stroke, MI, and ESRD is 1.878 trillion KRW. The estimate for operational costs included an annual expenditure of 50,000 KRW per each recipient and an annual subsidy of 0.22 million KRW per person for the 1.6 million low.income individuals with hypertension or T2DM to cover their out.of.pocket medical expenses. Under this assumption, it took approximately 0.6 trillion KRW to manage 5 million high.risk patients in the low. and mid.income range, coverings up to 50% of costs. In conclusion, considering the potential benefits of preventing stroke, MI, and ESRD, the costs seems to be reasonable.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.62-69
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• 2008

Purpose: The most concerning issue in children with urinary tract infection(UTI) is the probability of underlying genitourinary anomalies and vesicoureteral reflux (VUR), which is frequently associated with renal scarring and eventually end-stage renal disease. Therefore, voiding cystourethrography(VCUG) is usually recommended at the earliest convenient time for children with UTI. However, VCUG is an invasive procedure that requires catheterization and exposure to X-ray. In this study, we aimed to determine the predictability of clinical, laboratory and imaging parameters for VUR in children with UTI. Methods: Data of children with bacteriologically proven UTI who underwent VCUG were evaluated retrospectively for clinical(age, gender, fever), laboratory(leukocytosis, ESR, CRP, pyuria, blood urea nitrogen, serum creatinine) and imaging(renal ultrasound and DMSA renal scan) findings. First, children with UTI were divided into two groups according to the presence of VUR as non-VUR group and with VUR group, and clinical, laboratory variables were compared between these groups. Second, patients who had VUR were reclassified as low-grade VUR(grade I-II) group and high-grade(grade III-V) VUR group according to grading of VUR, and clinical, laboratory and imaging variables were compared between these groups. Results: Among 410 children with UTI, 137 had VUR and 78 high-grade VUR. Fever, leukocytosis, ESR, CRP, pyuria were associated with VUR. In addition, abnormal findings of ultrasonography and DMSA renal scan were closely related to VUR. However, these clinical and laboratory variable in patients with high grade VUR were not different significantly, compared to those with low-grade VUR group. Conclusion: Fever, leukocytosis, ESR, CRP seems to be potentially useful predictors of VUR in pediatric patients with UTI. In addition, renal ultrasonography and DMSA renal scan findings supported the presence of VUR. Further study of these findings could limit unnecessary VCUG in patients with UTI.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.120-122
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• 2002

A 9-year-old boy of B blood group with end-stage renal disease due to IgA nephropathy received group O kidney transplantation from his father On day 9, he developed intravascular hemolysis, and anti-B autoantibody formation was confirmed. We diagnosed as immune hemolytic anemia due to passenger lymphocyte from donor, and cyclosporine withdrawl was done. Anemia resolved spontaneously, but on day 18, graft dysfunction developed, and graft biopsy revealed acute allograft rejection. Although hemolysis due to autoantibody is very rare and often mild, and the role of hemoglobinuria on acute rejection in this case is not certain, we recommend consideration of aggressive management on severe hemolysis after minor mismatched kidney transplantation. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 120-2)

• Tuberculosis and Respiratory Diseases
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• v.66 no.6
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• pp.463-466
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• 2009

Streptococcus constellatus (S. constellatus) is a commensal microorganism belonging to the "Streptococcus milleri" group, but may cause infections in different locations in immunocompromised patients. The infection of S. constellatus has high mortality and morbidity due to its tendency to cause abscesses in infected patients, which require immediate surgical drainage for effective treatment. We report on a 72-year-old woman with end stage renal disease, who suffered from dyspnea and general weakness that had developed over 7 days. Chest CT showed loculated pleural effusion. S. constellatus was cultured from exudative pleural effusions and confirmed by an analysis of 16S rRNA sequence. The patient was treated with drainage of pleural effusion and piperacillin/tazobactam for 5 weeks.

• Journal of the Korea Convergence Society
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• v.11 no.9
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• pp.395-404
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• 2020

This was a descriptive study to investigate the effect of social support and health literacy on sick role behaviors in hemodialysis patients. A total of 149 outpatients receiving regular hemodialysis at four dialysis centers located in J city were surveyed. Data were analyzed using descriptive statistics, t-test, one-way ANOVA, Pearson correlation coefficient, and multiple linear regression. Factors including social support (β=.37, p<.001), health literacy (β=.16, p=.035), and religion (others) (β=-.25, p=.001) had an effect on sick role behavior. The explanatory power of model in this study was 22.0%. Therefore, the improvement of sick role behavior in patients undergoing hemodialysis requires specific strategies and interventions that are effective in increasing the social support and health literacy.