No abstract available.
Angiotensins* ; Diabetes Mellitus* ; Peptidyl-Dipeptidase A* ; Renal Insufficiency*

No abstract available.

To investigate the mechanism of crescent formation, sequential pathologic changes from the New Zealand White rabbits with anti-GBM antibody induced GN by administration of guinea pig anti-GBM IgG were studied by light (LM), immunofluorescent (IF) and electron (EM) microscopy. Although no glomerular changes were observed in LM, swelling of the endothelial cells and the epithelial cells were noted in EM by day 2. By day 7, early and cellular crescents were evident. Proteinaceous materials and fibrins were noted in the glomerular capillary lumina (GCL) and Bowman's space (BS) associated with segmental hypercellularity. The GBM damage became progressively severe, followed by focal detachment of the visceral epithelial cells from the GBM. At day 14, fibrin strands, mononuclear cells and collagen fibrils were present between the proliferating extracapillary cells. At day 31, fibrocellular crescents were predominated. Elongated spindle cells, morphologically resembling myofibroblasts, were noted near the Bowman's capsule (BC). A degree of tubular atrophy, interstitial fibrosis, and inflammatory infiltrates increased as it did with fibrous organization of crescent. Intense linear IF staining for IgG and C3 were seen throughout the experiments along the GBM. In conclusion, the progression of crescent from an early "proteinaceous" stage through cellular, fibrocellular and fibrous stages was well documented in this study. Inflammatory cells and coagulation mechanism may activate the initiation of the GBM damage at the early stage. Activated periglomerular mononuclear cells may also cause disruption of BC which facilitates entry of activated periglomerular cells and fibroblasts into BS leading to progressive fibrous crescent formation.
Animals ; Atrophy ; Bowman Capsule ; Capillaries ; Collagen ; Endothelial Cells ; Epithelial Cells ; Fibrin ; Fibroblasts ; Fibrosis ; Glomerulonephritis ; Guinea Pigs ; Immunoglobulin G ; Microscopy ; Myofibroblasts ; Rabbits*

Paroxysmal nocturnal hemoglobinuria(PNH) is a rare, acquired disease involving multiple hematopoietic lines. Characteristics of PNH are intrinsic hemolytic anemia, iron deficiency anemia and venous thrombosis. report a case of PNH with characterostoc MR and CT findings. The signal intensity of renal cortex was lower than that of medulla on both T1- and T2- weighted MR imaging. On T2 weighted MR images, the liver showed very low signal intensity but the signal intensity of the spleen was normal. On precontrast CT the attenuation of renal cortex was higher than that of renal medulla and the attenuation of liver was higher than that of the spleen. These findings of MR imaging and CT were the result from the deposition of hemosiderin in the cells of proximal convoluted tubules and transfusional hemosiderosis of liver.
Anemia, Hemolytic ; Anemia, Iron-Deficiency ; Hemoglobinuria, Paroxysmal* ; Hemosiderin ; Hemosiderosis ; Liver ; Magnetic Resonance Imaging ; Spleen ; Venous Thrombosis

No abstract available.
Interleukin-6* ; Kidney Transplantation* ; Kidney*

Progressive renal fibrosis is considered to be the final common pathway leading to chronic renal insufficiency, however, the mechanism regarding renal fibrosis in renal injury is not well understood. Recently, several kinds of cytokines have been known to be related to fibrosis after renal injury. The interaction between elements regulating fibrogenesis would be better understood by looking at the effect of TGF-beta1 on the synthesis and accumulation of extracellular matrix, especially collagenous proteins. Crescentic glomerulonephritis (CGN) was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG; and their kidneys were analyzed for the development of crescents and fibrosis through sequential renal biopsies. Serum creatinine levels in a time course progressively increased until day 15. We semi-quantitatively assayed the levels of the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA factored for GAPDH mRNA using RT-PCR. We observed a progressive interstitial fibrosis and the expression of collagen I both in the cortex and medulla. The effect of repeated renal biopsy itself on pathology and on the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA in a time course were not significant, but a very mild increase of the expression of alpha1(I) collagen mRNA was noted at day 15. Histology showed a progressive crescent formation and interstitial fibrosis in a time course that roughly paralleled the expression of alpha1(I) collagen mRNA in both cortex and medulla. TGF-beta1 mRNA was hardly expressed at day 0 in cortex as well as in medulla. It was elevated from day 1, peaked at day 7, and then decreased. In medulla, TGF-beta1 mRNA was noticeably expressed at day 1, peaked at day 4, and then decreased. The expression of alpha1(I) collagen mRNA was seen even before inducing CGN. It was gradually and continuously increased until day 15 both in cortex and medulla. These results suggest that the expression of TGF-beta1 mRNA precedes that of alpha1(I) collagen mRNA in the early stage of CGN and has a central role for provoking the accumulation the collagen I, the most representative interstitial extracellular matrix, in the rabbit model CGN induced by anti-GBM antibody. We conclude that the measurement of the expression of TGF-beta1 mRNA and/or alpha1(I) collagen mRNA in a biopsy sample can be a useful predictor for renal outcome.
Animals ; Anti-Glomerular Basement Membrane Disease ; Biopsy ; Collagen ; Creatinine ; Cytokines ; Extracellular Matrix* ; Fibrosis ; Glomerulonephritis* ; Guinea Pigs ; Immunoglobulin G ; Kidney ; Pathology ; Rabbits ; Renal Insufficiency, Chronic ; RNA, Messenger* ; Transforming Growth Factor beta ; Transforming Growth Factor beta1

BACKGROUND: For the evaluation of pulmonary nrtery branches after operation of right ventricular outflow obstructive disease, we reviewed the usefulness of both simple X-ray and lung perfusion scan with 99mTc-macroaggregated albumin compared with cardiac angiography. METHODS: From March, 1986 to July, 1993, at Seoul National University Children;s Hospital, we chose 23 cases who underwent both simple X-ray and cardiac angiography among the patients who had distinct difference between right and left pulmonary blood flow in lung perfusion scan after operation of right ventricular outflow obstructive disease. So we calculated the ratio of right and left pulmonary blood flow and Spearman correlation coefficient. RESULTS: Of the 23 cases, 17 cases were tetralogy of Fallot, 4 cases pulmonary atresia, 1 case double outlet right ventricle, and 1 case truncus arteriosus. The mean age at poeration was 27 months, and the mean postoperative follow-up period was for 68 months. The correlation coefficient between cardiac angiography and simple X-ray was 0.86(p<0.01), and that beteen cardiac angiography and lung perfusion scan was 0.80(p<0.01). CONCLUSION: The cardiac angiography has been known as the best diagnostic tool for the evaluation of pulmonary artery branches after operation of right ventricular outflow obstructive ddisease, but we proposed that the lung perfusion scan should be widely used because its results were similar with those of cardiac angiography.
Angiography ; Double Outlet Right Ventricle ; Follow-Up Studies ; Humans ; Lung* ; Perfusion* ; Pulmonary Artery* ; Pulmonary Atresia ; Seoul ; Tetralogy of Fallot ; Truncus Arteriosus

It has been postulated that programmed cell death via apoptosis may be critical for remodelling of glomeruli after inflammatory injury. To understand the regulatory mechanism of apoptosis in experimental crescentic glomerulonephritis (CGN), we examined the MIB-1 score (proliferation index, PI) and apoptotic index during the progression of experimental CGN to end-stage renal failure. CGN was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG and their kidneys were analyzed for the development of crescents through sequential renal biopsies. Serum creatinine levels progressively increased in a time course until day 45. The PI in glomeruli, tubular epithelial cells, and interstitium progressively increased during the progression of experimental CGN. The mean numbers of MIB-1 positive intraglomerular nuclei (PI) were significantly correlated with degrees of crescent formation and the numbers of apoptotic cells in the glomeruli, tubules, and interstitium. Significant apoptosis was present from day 1 (15.8 10.16 cells/glomerular cross section) and increased in number with the proliferative lesions as glomerular inflammation continued. Moreover, apoptosis increased during the resolution of the glomerular inflammation, and many apoptotic cells were present in the sclerotic lesions in day 17 (18.6 12.99 cells/glomerular cross section). As glomerular inflammation subsided, cellular crescents progressed to fibrous crescents with a reduction of cellularity by day 45. On day 45, the glomerular PI and the numbers of apoptotic cells were markedly decreased. The correlations found in CGN between the creatinine level and the percentage of crescents, between the percentage of crescent and PI, and between the PI and number of apoptotic cells support the hypothesis that there is a change in the glomerular and tubulo-interstitial apoptosis under pathologic conditions. These findings indicate that apoptosis plays an essential role in the resolution of intra- and extraglomerular inflammation and in the elimination of glomerular cells within the sclerotic regions for progressive CGN. The regulation of the apoptotic phenomenon and increased PI during CGN may be important in the progression of glomerular inflammation and the development of pathologic glomerular sclerosis.
Animals ; Anti-Glomerular Basement Membrane Disease ; Apoptosis* ; Biopsy ; Cell Death ; Creatinine ; Epithelial Cells ; Glomerulonephritis* ; Guinea Pigs ; Immunoglobulin G ; In Situ Nick-End Labeling ; Inflammation ; Kidney ; Kidney Failure, Chronic ; Rabbits ; Sclerosis

We reviewed 26 cases of cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was "daehakgyo".23. Their cardiac rhabdomyomas were diagnosed from fetal period through 12 years after birth. Median age was 9."uiwon" months. Fifteen patients were males and rest 11 patients were females. Cardiac symtoms and signs at initial diagnosis were present in 12 cases. They consisted of cardiac murmur in "hakgyo", arrhythmia in 8 and congestive heart failure in "daehakgyo" cases (one with murmur and the other with arrhythmia). Cardiomegaly was seen in 9 cases on chest X-ray and other "byeongwon" case showed abnormal left cardiac border. Electrocardiography and 24 hour Holter monitorings showed arrhythmia in 15 cases. Clinically significant arrhythmias were found in 7 cases on 24 hour Holter monitorings. They consisted of 1with frequent premature ventricular contractions, "daehakgyo uigwadaehak" with frequent premature atrial contractions, "daehakgyo" with atrial tachycardia and "daehakgyo uigwadaehak" with atrio-ventricular block ("daehakgyo" cases of atrio-ventricular block had other arrhythmias also). The locations of tumors were left ventricle in 21, interventricular septum in 21, right ventricle in 13 and atrium in "daehakgyo uigwadaehak" tumors. Mean tumor count per patient in fetal period and infancy was "daehakgyo".86 and it was statistically much more than that after infancy which was "byeongwon".5. Mean tumor area which was calculated on two dimensional echocardiography was larger in fetal period and infancy(128.9 +/- 38.2mm2)than that after infancy(47.8 +/- 11.2mm2). Mean relative tumor area to aortic root dimension was also significantly larger in fetal period and infancy than that after infancy ("daehakgyo".07vs 0.30). In tuberous sclerosis, mean mass area and mean relartive tumor area to aortic root dimension in fetal period and infancy was significantly larger than those in patients after infancy. Of 26 cases, tumorectomy was done in "daehakgyo uigwadaehak" cases due to hemodynamic obstruction and arrhythmia. One case died immediately after operation and the other "daehakgyo" cases are still alive with the follow up period of 30 and 44 months each. Of the 7 patients with significant arrhythmia on 24 hour Holter monitorings, pacemaker insertion was done in "byeongwon" case who received tumorectomy and other 1 case was treated with anti-arrhythmic drugs. All the other patients did not need anti-arrhythmic drugs or other management.
Arrhythmias, Cardiac ; Atrial Premature Complexes ; Cardiomegaly ; Child* ; Diagnosis ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Failure ; Heart Murmurs ; Heart Ventricles ; Hemodynamics ; Humans ; Male ; Parturition ; Rhabdomyoma* ; Seoul ; Tachycardia ; Thorax ; Tuberous Sclerosis ; Ventricular Premature Complexes

BACKGROUND: Valve replacement in children has many problems such as the durability of prosthetic valve, thomboembolism and hemorrhage. But recently, the necessity of valve replacement in children increased and the above problems were solved party. So the number of valve replacement in children increased progressively. RESULTS: Valve replacement in 47 children were done at Seooul National University Chidren's Hospital from March 1986 to July 1991. The patients were composed of 25 males and 33 females. 25 patients had congenital heart disease and 22 patients rheumatic heart disease. 45 patients received single valve repalcement, 2 patients double valve repalcement, and among all of them, 2 patients redo-replacement. The major valve lesion was mitral insufficiency and post-operative status in view of NYHA functinonal class was improved in most patients. The mechanical valves were applied to 45 patients and tissue valves to 2 patients. The indication of valve replacement were progressive increase in ventricular volume, major regurgitant fraction over grade III decrease in exercise tolerance and vegetation. The overall early mortality was 8.5% and late mortality 0%. There were post-operative complication rate of 30% and late complication rate 14%, and among the later, valve faliure was reported in 2 pantients and thromboembolism in 1 patient. The complication-free rate was 97.7% at post-operative 1 month, 91.3% at 12 months, 90% at 36 months and 60% at 48 months. 43 patients received anticoagulation and/or antiplatelet therapy, but there was no critical indication for this. CONCLUSION: These results suggest that cardiac valve replacement in children have been effective therapeutic modality even though various problems still remain, but we propose that sufficient long-term follow-up and clinical research be needed.
Child* ; Exercise Tolerance ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Valves* ; Hemorrhage ; Humans ; Male ; Mitral Valve Insufficiency ; Mortality ; Rheumatic Heart Disease ; Thromboembolism