Kim Hyung-Tae;Seo Jung-Wook;Kim Han-Seong;Lee Chong-Guk
Childhood Kidney Diseases
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v.7
no.2
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pp.217-222
/
2003
Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.
The purpose of this study is to evaluate urine protein-to-creatinine ratio as a parameter for early detection of nephrotic syndrome and as a parameter for monitoring effectiveness in early course of treatment. Nine healthy dogs were sensitized by intravenous injection with 1 $\mu$g of endotoxin and 5 mg of native bovine serum albumin. After 1 week, 120 mg of cationized bovine serum albumin was injected intravenously 5 times a week. Among nine dogs, five dogs were confirmed as having developed glomerulonephritis and nephrotic syndrome by increase of urine protein-to-creatinine ratio(>1.0), hypoalbuminemia (<1.5 g/dl), hypercholesterolemia (> 240 mg/dl) and azotemia (BUN>40 mg/dl). During the induction of glomerulonephritis and the progression to nephrotic syndrome, the increase of urine protein-to-creatinine ratio was firstly detected. 1 to 4 weeks later, hypoalbuminemia, hypercholesterolemia, and azotemia were detected. Prednisolone (2.2 mg/kg, bid) was administered orally to the dogs with induced nephrotic syndrome. In early stage of treatment, the increase of serum albumin and decrease of serum cholesterol were detected. 1 to 4 weeks later, decrease of urine protein-to-creatinine ratio was detected. It was concluded that urine protein-to-creatinine ratio is a useful parameter for early detection of nephrotic syndrome, and serum albumin and cholesterol are useful parameters for the monitoring in early course of treatment in nephrotic syndrome.
Kim Se Heui;Roh Hyun Kyung;Lee Young-Mock;Kim Ji Hong;Kim Pyung-Kil;Hong Soon Won;Jeong Hyun Joo
Childhood Kidney Diseases
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v.5
no.2
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pp.196-205
/
2001
Purpose : Membranous glomerulopathy is a glomerular disease characterized by the presence of subepithelial immune deposits with thickening of the capillary wall of the glomerulus without inflammatory change. The pathogenesis of membranous glomerulopathy is still unknown. Its incidence is higher in males, and it is rarely found in infants and adolescents. Among the clinical manifestations proteinuria is most common, while edema and hematuria are present. According to reports from other countries, among few patients diagnosed with membranous glomerulopathy by renal biopsy, show isolated microscopic hematuria without the clinical manifestations. Little research in this area has been performed in Korea, and so we conducted retrograde studies on membranous glomerulopathy associated with isolated microscopic hematuria. Materials and Methods : We analyzed retrogradely 109 cases of asymptomatic isolated microscopic hematuria that were diagnosed as membranous glomerulopathy by renal biopsy at Yonsei University Severance hospital from January, 1992 to July, 2001. Results : In 87 of the 109 cases patients were over 15 years old while in 22 cases patients were under 15 at the time of dignosis. Only three patients showed isolated microscopic hematuria without the clinical manifestations and abnormal laboratory findings and they were all male patients under 15 years old. Conclusion : Few cases of the membranous glomerulopathy show only asymptomatic isolated microscopic hematuria However, since membranous glomerulopathy can be found in patients who present with asymptomatic isolated microscopic hematuria only, if adequate indication for renal biopsy is present, we conclude that renal biopsy must be aggresively pursued in order to find the underlying disease. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 196-205)
Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.
The purpose of this study is to investigate the vasodilatation effect of kirenol isolated from Sigesbeckia pubescens on the rabbit basilar artery. In this study, to determine the vasodilatation effect of kirenol on the rabbit basilar artery, arterial rings with intact or damaged endothelium were used for the experiment. And used an organ bath and force transducer were contracted by endothelin. Kirenol, major active constituents of S. pubescens, showed a moderate vasodilatation effect on the basilar arteries of rabbits. Therefore, treatment with kirenol may selectively accelerate cerebral blood flow through dilatation of the basilar artery. This result suggests a potential role of kirenol isolated from S. pubescens as a source of vasodilatation agent.
Purpose : The urinary mass screening program for the detection of proteinuria in school aged population has been performed in Seoul since 1981. Systematic evaluation in corporation with the Seoul School Health Center for students with proteinuria identified in the mass screening has been performed from 1987. The results of urinary mass screening up to 1994 was reported. I report here the results of urinary mass screening from 1995 to 1997 and compare them with previous results and attempt to reveal the significance of urinary mass screening. Objects and Methods : In the 3-year period between 1995 and 1997, annually about 460,000 students comprising 3 different age groups; 5th grade of elementary school, 2nd grade of middle school and 2nd grade of high school were chosen, corresponding to the approximate ages of 11, 14, and 17 years, respectively. These subjects accounted for 26% of total school aged children in Seoul. The screening program was carried out in 3 steps. The 1st test was performed with dipstick at school and the 2nd at the Seoul School Health Center. Those students who showed proteinuria in the 1st and 2nd tests were referred to the hospital. Laboratory examinations including renal biopsies were performed to those students with pathologic proteinuria to clarify the incipient renal diseases. Results : 1) The prevalence of asymptomatic proteinuria was 0.28% in the 1st test. It peaked at the group of 14 years old as 0.34%, compared with 0.26% at the group of 11 years old and 0.24% at the group of 17 years old. It reached to 0.26% in male and 0.30% in female. 2) 25 percent of those having proteinuria at the first test were positive at the second test. 3) The proportion of patients with proteinuria by 3rd test were as follows; 25% of transient proteinuria, 55% of orthostatic proteinuria, 6% of constant proteinuria, 12% of proteinuria with hematuria, and 2% of transient proteinuria with isolated hematuria. Pathologic proteinuria were totaled as 20%. The prevalence of renal diseases among the age group of 7-18 years old was estimated to be 1.4 per 10,000. 4) Renal biopsy performed on 38 children with proteinuria at the third test revealed IgA nephropathy in 17(44%), focal segmental glomerusclerosis in 5(13%), minimal change disease in 4(11%), membranoproliferative glomeronephritis in 3(8%), $Henoch-Sch\"{o}nlein$ purpura nephritis in 3(8%), and others in 6(16%). Therefore, the prevalence of IgA nephropathy among the age group of 7-18 years old was estimated to be 0.64 per 10,000. 5) The prevalence of chronic renal failure was estimated to be 5.7 per 1 million of 7 to 18 years age group. Conclusions : 1) The prevalence of proteinuria in the first screening test was 0.28% and finally only 5% of them showed the pathologic proteinuria at the third test. 2) The prevalence of IgA nephropathy and chronic renal failure were 0.63 per 10,000 and 5.7 per 1 million, respectively among school-aged children in Seoul.
Purpose : Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A$\beta$-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. Methods : Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A $\beta$-hemolytic streptococcal infection were evaluated. Results : Six(12.5$\%$) patients showed elevation of serum creatinine level but there was no patient with Persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9$\%$). Hematuria disappeared within 6 months(79$\%$) and proteinuria within 6 months(100$\%$) from the disease onset. Conclusion : Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4$\%$) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequoia. (J Korean Soc Pediatr Nephrol 2005;9:137-142)
The purpose of this study was to analysis the correlation between kidneys function indicators and these size in ultrasonography. A total of 170 (male:86, female:84) patients of sex and age groups were examined by abdominal ultrasonography. The patients classified as those in their 20's, 30's, 40's, and over 50's. We measured the length, width, and cross-sectional height of the kidneys twice. At this time, the length of these were measured from the maximum upper to the maximum lower pole and the widest width in the same ultrasonography was measured to obtain the cross-sectional area. Other relevant indicators included body surface area, serum creatinine, glomerular filtration rate (GFR), MDRD (Modification of diet in renal disease) and C-G (Cockcroft-Gault). Significant comparisons of differences between relevant factors by age groups and sex were conducted with a one-way distribution analysis. Correlation analysis was also performed between relevant factors by using Pearson and Spearman correlation coefficient. It was defined as meaningful when the p-value was less than 0.05. As a result, the length, the width, and the cross-sectional area of kidneys were correlated with GFR, C-G, MDRD. Therefore, it is expected that the accuracy of diagnosis of kidneys disease will be increased if the relevant indicators are evaluated together rather than measuring only length of these in ultrasonography.
Lee Ji Eun;Yook Jinwon;Lee Eui Seong;Kim Ji Hong;Kim Pyung-Kil;Chung Hyun Joo
Childhood Kidney Diseases
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v.4
no.1
/
pp.17-24
/
2000
Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.
In general, specific gravity (SG) and urinary creatinine (CR) have been used to adjust urinary cadmium (Cd) concentrations. However, the validity of correction methods has been controversial. We compared the two adjustments to evaluate associations between urinary Cd and various renal damage markers and to evaluate the relationship between urinary Cd concentration and renal disease markers, such as estimated glomerular filtration rate (eGFR), in a relatively large general population sample. Among the 1,086 volunteers who were enrolled in this study, 862 healthy volunteers who did not have kidney disease were included in the final analysis. Urinary Cd, malondialdehyde (MDA), and N-acetyl-${\beta}$-D-glucosaminidase (NAG) concentrations were measured, the creatinine-based eGFR was calculated, and the relationships between these markers were subsequently analyzed. This study showed the use of urinary Cd concentration adjusted with SG rather than with urinary creatinine may be appropriate in studies evaluating renal function based on Cd exposure. Urinary Cd concentration adjusted with SG had a positive correlation with urinary MDA levels and a negative correlation with eGFR. This relationship was relatively stronger in women than in men. This study showed that urinary Cd level was associated with decreased eGFR in the general population, and oxidative stress was likely to act as an intermediator in this process. These results suggest that eGFR can be a very good indicator of kidney damage caused by Cd exposure in the general population.
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