• The Korean Journal of Nuclear Medicine
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• v.34 no.6
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• pp.497-507
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• 2000

Purpose: We have examined the utility of $^{99m}Tc$-MAG3 perfusion indices for assessing renal graft function in early post-transplantation period. Materials and Methods: Our study included 80 renal transplant recipients (48 men and 32 women, mean age: 40.3 years). Diagnosis was based on biopsy, laboratory data and clinical course. Renal scintigraphy (RS) was obtained using 100 MBq of $^{99m}Tc$-MAG3 from 11 days to 23 days of kidney transplantation. We measured 5 indices in whole-kidney (WK) and cortical (C) renograms; Hilson's perfusion index (PI), transplant perfusion index (TP) and transplant function index (TF) as perfusion parameter, and the time to peak activity (Tmax) and the ratio of renal counts at 20 min to that at 3 min (K20/3) as functional parameter. Results: The diagnoses at the day of RS were normal graft (NG) in 44, acute rejection (AR) in 14, acute tubular necrosis (ATN) in 10, and Cyclosporine A nephrotoxicity (CsA) in 12. TP and TF were significantly decreased in AR, ATN and CsA, compared to those in NG. K20/3 of AR and ATN wore significantly greater than that of NG. WK-Tmax of AR was significantly longer than that of NG. K20/3 of AR and C-K20/3 of ATN were significantly prolonged relative to those of CsA. There were no statistically significant perfusion indices among complication groups. Conclusion: TP and TF reflecting microperfusion and initial tubular extraction are reliable in assessing graft function. However, it is required to correlate perfusion indices with functional indices and clinical course in differentiating from one another among complication groups.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.1-8
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• 2001

Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.48-55
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• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.154-165
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• 2010

Purpose : We know little about the natural course of IgA nephropathy (IgAN) in association with histologic changes especially in children. We investigated clinicopathologic features with long-term follow-up biopsy to clarify the outcomes and prognostic indicators for childhood IgAN. Methods : From our patients' medical records, we retrieved 20 patients with IgAN, to whom renal biopsies had been performed for the initial diagnosis and follow-up to find out any histologic changes. Initial and follow-up biopsies were classified by Haas classification. The changes of these parameters were compared with the evolution of clinical features. Results : Patients were treated with angiotensin-converting enzyme inhibitors in combination with angiotensin receptor blockers (in subclass II or above) and short-term cyclosporine A(in patients showing nephrotic syndrome). Histologic improvement in 7 cases and deterioration in 3 cases were observed. At the time of last biopsy, 10 cases (50%) showed clinical remission and the others showed improved clinical features. These clinical outcomes did not correlate with initial Haas classifications. Hypertension at onset observed in 5 cases (25%) revealed significant correlation with clinical outcome (P =0.01) and last Haas classification (P =0.007). None of the cases showed progression to CRF or ESRD. Conclusion : During a mean follow-up of $10.8{\pm}3.4$ years, childhood IgAN showed good clinicopathologic outcome. Hypertension at onset was only a strong predictor of clinicopathologic outcomes, but initial Haas classification cannot predict outcomes in children. Histologic change of IgAN in long term follow-up period cannot be completely predicted by clinical data and vice versa. Therefore, a renal biopsy should be considered as a part of follow-up plan.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.61-67
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• 2002

Purpose: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. Results: The classification of isolated proteinuria was as follows. Transient proteinuria $32\%$, orthostatic proteinuria $65\%$, persistent proteinuria $3\%$, In orthostatic proteinuria group, daytime and nighttime proteinuria were $319.2{\pm}189.1\;mg/dL$ and $56.5{\pm}56.1\;mg/dL$. In persistent proteinuria group, daytime and nighttime proteinuria were $1140{\pm}540.5\;mg/dL$ and $289{\pm}58\;mg/dL$. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. Conclusion: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow- up with quantitation of proteinuria is warranted.(J Korean Soc Pediatr Nephrol 2002 ; 6 61-7)

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• 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.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.17-24
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• 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.

• Archives of Plastic Surgery
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• v.34 no.5
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• pp.659-662
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• 2007

Purpose: Multiple skin leiomyoma and uterine myoma bearing autosomal dominant traits are benign smooth muscle tumors which originate in skin or female uterus. Skin leiomyoma occurs after gene mutation originating from arrector pili muscle of hair follicle where its clinical manifestations vary significantly from person to person. Our department hereby reports the histological findings and genetic evaluations of this very rare disease. Methods: A 57-year-old woman presented in our institute with multiple tumors in the left and central parts of her back that started to appear since 19 years ago. The patient was diagnosed as having uterine myoma 15 years ago and underwent hysterectomy. Biopsy has been done on the specimen, and genomic DNA was separated from Fumarate hydratase gene for it to go through PCR amplification. The results of PCR amplification were aligned by sequencer. Results: According to the results of biopsy, tumor cells were spindle-shaped and were aligned in a bundle where there was no dysplasia or mitosis. Moreover, these cells had abundant eosinophilic cytoplasm with elongated nucleus, and benign leiomyoma that showed positive reactions to SMA stain were found. In genetic examination, mutations such as heterozygous single nucleotide substitutions were found in alignments of amplified DNA. Conclusion: Multiple skin leiomyoma and uterine myoma are relatively uncommon diseases that are transmitted through autosomally dominant traits from genetic mutations. When a patient's chief complaint lies upon skin-colored or brown masses that occur in multiples appearing in the trunk or extremities with characteristic clinical symptoms and histological findings, and when the patient's family history is acknowledged such as skin or uterine leiomyoma or renal tumor, necessary genetic examination on multiple skin leiomyoma and uterine myoma could be done, and thereby precise diagnosis could also be made.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.128-131
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• 2014

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.