• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

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.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.769-776
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• 2006

Purpose : Pulse wave velocity(PWV) and ankle brachial index(ABI) are not only noninvasive methods used to assess arterial stiffness in adults, but also useful, simple ways to estimate the severity of hypertension, end stage renal disease and atherosclerosis in adults. But there are few studies on PWV and ABI in adolescents and children. In this study, hypertensive adolescents were compared to normal individuals to find out the usefulness of PWV as the index of progress in the disease. Methods : 413 teenagers were divided into three groups. The first group consisted of adolescents that only had hypertension(Group 1 : 23 teenagers). The second group consisted of the adolescents who had hypertension with obesity(Group 2 : 17 teenagers), and the last group was the normal subjects(Group 3 : 328 teenagers). Weight, height and body mass index were measured. Simultaneous measurements of systolic, diastolic and average blood pressure were obtained from the four extremities. PWV, ABI, ejection time and preejection period were also measured. Results : Right brachial-ankle PWV was significantly higher in both group 1 and group 2 compared with group 3, and significantly higher in group 2 compared with group 1. Likewise, left brachial-ankle PWV also showed significantly higher values in both group 1 and group 2 compared with group 3, and also higher values in group 2 compared with group 1. Positive correlations were found between the systolic, diastolic, average blood pressure and PWV. There were also positive correlations between the blood pressure and weight, body mass index. Conclusion : Higher PWV was demonstrated in adolescents with essential hypertension compared to normal subjects. Follow-up study is needed to evaluate the progress.

• Korean Journal of Head & Neck Oncology
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• v.23 no.2
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• pp.153-156
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• 2007

Objectives : We study the feasibility and pharmacokinetics of cisplatin concurrent chemoradiation for advanced head and neck cancer patient undergoing hemodialysis. Materials and Methods : A 57-year old male with end stage renal disease developed stage III external auditory canal cancer. Complete resection surgery was done. Postoperative 6 months, local recurrence was occurred. Despite excision and adjuvant radiotherapy, local tumor was recurred. We decided to treat a cisplatin concurrent chemoradiotherapy. Cisplatin was administered at a dose of $20mg/m^2$ for 30 min. Hemodialysis was started 30 min after completion of the cisplatin infusion and performed for 4 hours. Hemodialysis was performed on day 3 and 5 of chemotherapy. Plasma samples were collected at specified times after administration of cisplatin. Result : At the end of the third cycle of cisplatin concurrent chemoradiotherapy, the tumor size was markedly decreased. The maximum plasma concentrations of plasma platinum and free platinum were 0.74 and $0.37{\mu}g/ml$ respectively. The area under the curve of plasma platinum and free platinum were 94.7 and $11.3{\mu}g{\cdot}h/ml$ respectively. Conclusion : We report a case of Cisplatin concurrent chemoradiation for hemodialysis patient with advanced head and neck cancer and suggest full dose cisplatin concurrent chemoradiotherpay is tolerable for these patients.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.150-156
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• 2003

Purpose : Efforts to predict the clinicopathological outcome of IgA nephropathy have been made but have yielded conflicting results and have not helped in deciding the appropriate timing of the renal biopsy. In this study, we reviewed the predictive factors of clinicopathological outcome for finding out the criteria of renal biopsy timing of IgA nephropathy. Methods : Forty children diagnosed with biopsy proven IgA nephropathy at Wonju Christian Hospital were studied retrospectively, based on medical records. Results : Among 39 patients, 2 children progressed to higher serum creatinine level. One of them reached to the end stage renal disease within 2 year 7 months. According to WHO histopathological classification, there were 15 cases of class I, 14 cases of class II, 7 cases of class III, and 3 cases of class IV. In the mild histological classes(class I, II), gross hematuria was shown in 23 out of 29 children(P=0.02). In the severe histological classes(class III, IV), gross hematuria was noted in 4 out of 10(P>0.05). The tubulointerstitial changes were grade 1 in 24 cases, grade 2 in 4 cases, grade 3 in 8 cases, and grade 4 in 3 cases. With an increase in the tubulointerstitial grade, the 24 hour urine protein/albumin ratio increased. Serum creatinine less than 0.79 mg/dL could predict the lower grade(grade 1 and 2) of tubulointerstitial changes. But serum creatinine greater than 1.13 mg/dL could predict the higher grade(grade 3 and 4) of tubulointerstitial changes. In children with gross hematuria(n=27), serum creatinine was lower(0.78 vs 1.09 mg/dL, P=0.027), serum IgA was higher(316.3 vs 198.8 mg/dL), and the cases of lower WHO classification(I and II) were more common(23 vs 4, P=0.029) than the children with microscopic hematuria. Conclusion : Serum creatinine less than 0.79 mg/dL, macroscopic hematuria, and higher 24 hour urine protein/albumin ratio would predict the lower grade glomerulo tubulointerstitial lesion in IgA nephropathy and could be used as the criteria delaying the renal biopsy.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.120-126
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• 2000

Purpose : This retrospective study has been undertaken to find out the clinical outcome of children with HS nephntis and its relationship with initial clinical presentation and/or renal pathologic finding. Patients and methods : Study population consisted of 59 children with HS nephritis who have been admitted to the Pediatric department of Kyungpook University Hospital from 1987 to 1999, and biopsy was done with indications of heavy proteinuria (> 1 g/m2/day) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 year. Patients were divided clinically into 3 groups ; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings ore graded from I-V according to International Study of Kidney Disease in Children (ISKDC). Results : Mean age of presentation was $8.1{\pm}3.0$ years and slight male preponderance m noted (33 boys md 26 girls). Histopathologic grading showed Grade I ; 2, Grade II ; 44, and Grade III ; 13 cases. Clinical outcome at the follow-up period of 1-2 year (49 cases) and 3-4 years (30 cases) shooed normal urinalysis in 75 (30.6$\%$) and 18 cases (60.0$\%$), persistent isolated hematuria in 20 (40.8$\%$) and 2 cases (6.7$\%$), hematuria with proteinuria in 11 (22.5$\%$) and 8 cases (26.6$\%$), and persistent heavy proteinuria in 3 (6.1$\%$) and 2 cases (6.7$\%$) respectively. Clinical outcome according to histopathologic grading showed the frequency of normalization of urinalysis being lower in Grade III compared to grade I or II. Clinical outcome according to initial clinical presentation showed no relationship to the normalization or urinalysis at follow-up periods. However, 15-20$\%$ of children with initial heavy proteinuria showed persistent heavy proteinuria (3 out of 20 cases at 1-2 years, and 2 out of 10 case at 3-4 years of follow-up periods). Conclusion : The majority of children with HS nephritis (histopathologic grade I, II, III) improved within 3-4 years and persistent heavy proteinuria was seen only in a kw of children with initial clinical presentation of heavy proteinuria.