• Childhood Kidney Diseases
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• v.4 no.2
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• pp.136-143
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• 2000

Purpose : The hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia thrombocytopenia, and acute renal failure. It is ole of tile most common cause of acute renal failure in children but few reports are available in Korea. Thus we investigated the 23 patients diagnosed as HUS during last 14 years. Method : We retrospectively investigated the etiologic factor, clinical manifestations laboratory findings, treatment modalities, and final outcomes of the patients. Then patients were divided into two groups according to outcome, md comparison was performed. Group A(8) comprised patients who progressed to end-stage renal disease or expired. Group B(15) comprised patients who completely recovered after dialysis treatment. Result The number of patients aged less than 4 years were 17; between 5 and 10 were 4 and more than 10 were 2. The gende ratio was M:F=2 : 1. The etiologic factors were as follows: acute gastroenteritis in 14 patients including 4 bloody diarrhea, upper respiratory tract infection in 7 patients, and 1 patient with herbal mediation. The overall mortality rate was 22$\%$: 2 patients died of US complications, 2 patients died of sepsis, and 1 patient died of pulmonary hemorrhage. Group A (Hb 4.8${\pm}$1.2 g/dL) showed lower value in hemoglobin than group B (Hb 6.3${\pm}$1.7 g/dL) during hospital stay (P< 0.05), And the time interval between tile disease onset and dialysis treatment was significantly longer in group A ($11.9{\pm}9.1\;days\;vs\;2.8{\pm}2.1\;days$) (P< 0.05). Conclusion : Overall mortality rate was 22$\%$. Low hemoglobin value and the prolonged time interval between the disease onset and dialysis treatment were related with poor prognosis. So early diagnosis and appropriate intensive care including dialysis treatment is essential to achieve better outcome in children.

• 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.

• Journal of Chest Surgery
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• v.37 no.4
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• pp.297-306
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• 2004

Current artificial heart valves have several disadvantages, such as thromboembolism, limited durability, infection, and inability to grow. The solution to these problems would be to develop a tissue-engineered heart valves containing autologous cells. The aim of this study was to optimize the protocol to obtain a porcine acellular matrix and seed goat autologous endothelial cells on it, and to evaluate the biological responses of xenograft and xeno-autograft heart valves in goats. Material and Method: Fresh porcine pulmonic valves were treated with one method among 3 representative decellularization protocols (Triton-X, freeze-thawing, and NaCl-SDS). Goat venous endothelial cells were isolated and seeded onto the acellularized xenograft leaflets. Microscopic examinations were done to select the most effective method of decellularizing xenogeneic cells and seeding autologous endothelial cells. Two pulmonic valve leaflets of. 6 goats were replaced by acellularized porcine leaflets with or without seeding autologous endothelial cells while on cardiopulmonary bypass. Goats were sacrificed electively at 6 hours, 1 day, 1 week, 1 month, 3 months, and 6. months after operation. Morphologic examinations were done to see the biological responses of replaced valve leaflets. Result: The microscopic examinations showed that porcine cells were almost completely removed in the leaflets treated with NaCl-SDS. The seeded endothelial cells were more evenly preserved in NaCl-SDS treatment. All 6 goats survived the operation without complications. The xeno- autografts and xenografts showed the appearance, the remodeling process, and the cellular functions of myofibroblasts, 1 day, 1 month, and 3 months after operation, respectively. They were compatible with the native pulmonary leaflet (control group) except for the increased cellularity at 6 months. The xenografts revealed the new endothelial cell lining at that time. Conclusion: Treatment with NaCl-SDS was most effective in obtaining decellularized xenografts and facilitate seeding autologous endothelial cells. The xenografts and xeno-autografts were repopulated with myofibroblasts and endothelial cells in situ serially. Both of grafts served as a matrix for a tissue engineered heart valve and developed into autologous tissue for 6 months.

• Journal of Chest Surgery
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• v.36 no.5
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• pp.321-328
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• 2003

Background: The St. Jude Medical prosthesis is one of the popularly used artificial prosthesis, therefore the National Medical Center reports the long-term clinical results of patients who underwent prosthetic valve replacement with St. Jude medical valve for 18 years. Material and Method: Between January, 1984 and June, 2002, a series of 163 consecutive patients who had Implanted St. Jude prosthesis at the National Medical Center were reviewed. Mean age was 42.9$\pm$15.1 years and male to female ratio was 69:94. The operative procedure comprised of 87 MVR, 30 AVR, 45 DVR, and 1 TVR. The reoperative procedure comprised of 21 MVR, 2 AVR, and 14 DVR. Follow-up rate was 96.9%, and cumulative follow-up was 823.8 patient-years. Result: Early mortality rate was 7.9% (13 patients), late mortality rate was 8.7% (13 patients) and late mortality due to valve related complication was 47% (7 patients). Actual survival rate at 10 and 18 years were 91.7$\pm$2.1% and 91.0$\pm$1.9%. Linearized Incidence was as follows: thromboembolism, 1.09%/ patient-year; anticoagulant related hemorrhage, 0.36%/patient-year; valve thrombosis, 0.24%/patient-year; paravalvular leakage, 0.12%/patient-year; and prosthetic bacterial endocarditis, 0.12%/patient-year linearized incidence of over all valve related complication was 1.94%/patient-year. Freedom from valve related complication at 10 and 18 years were 89.1$\pm$3.3% and 88.4$\pm$3.9%. Freedom from valve related death at 10 and 18 years were 95.1$\pm$1.2% and 95.1$\pm$1.0%. Valve related complication was related the age of patient, especially anticoagulant related hemorrhage was more common in patients over 60 years of age. Valve related complication, death were higher in DVR than AVR or MVR, and valve related death was higher in reoperation. There was no relationship between valve related complication or death and implant valve of size. Conclusion: The long-term clinical results of patients implanted with St. Jude Mechanical prosthesis was quite satisfactory with a low incidence of valve related complication and mortality.