Drugs, Chinese Herbal ; therapeutic use ; Glomerulosclerosis, Focal Segmental ; drug therapy ; Humans ; Sclerosis

Adult ; Cyclosporine ; Glomerulosclerosis, Focal Segmental/drug therapy* ; Humans ; Leflunomide/therapeutic use* ; Nephrosis, Lipoid/drug therapy* ; Nephrotic Syndrome ; Steroids

The aim of this article was to investigate the mechanisms of emodin in antagonizing against organ fibrosis, and to illustrate that emodin can be an effective Chinese herbal preparation for treatment of organ fibrosis.
Animals ; Emodin ; therapeutic use ; Fibrosis ; drug therapy ; Glomerulosclerosis, Focal Segmental ; drug therapy ; etiology ; Humans ; Kidney ; metabolism ; pathology ; Liver Cirrhosis ; drug therapy ; Phytotherapy ; Pulmonary Fibrosis ; drug therapy

PURPOSE: Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. METHODS: Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87 patients were selected for this study. They showed poor response to 4-week daily steroid therapy either initially (initial nonresponder) or subsequently in the disease course (subsequent nonresponder). The medical records including renal pathologic findings were analyzed retrospectively. RESULTS: The mean age at the onset of nephrotic syndrome was 7.3+/-4.1 years and male to female ratio was 62:25. Pathologically, 28 (32%) had minimal change lesion (MCL), 47 (54%) had focal segmental glomerulosclerosis (FSGS) and 12 (14%) had others. There were 15 (54%) initial nonresponders and 13 (46%) subsequent nonresponders in the MCL group, and there were 26 (55%) and 21 (45%), respectively, in the FSGS group. The incidence of hematuria was less frequent in the MCL group. The frequencies of hypertension and azotemia were not significantly different between in the MCL and the FSGS group. Among 10 patients with MCL in whom the steroid therapy were extended to 6 weeks, 3 patients responded subsequently. And 1 of 3 patients among the FSGS group responded to 8-week daily steroid therapy. The 2nd line drug therapy such as oral cyclophosphamide, intravenous pulsed methylprednisolone, enalapril, dipyridamole, etc. was tried in 26 patients with MCL and all 47 patients with FSGS. In the MCL group, 7 of 13 initial nonresponders and 9 of 13 subsequent nonresponders responded to these 2nd line drug therapies. In the FSGS group, 10 of 26 initial nonresponders and 11 of 21 subsequent nonresponders responded to these therapies. While only 1 subsequent nonresponder in the MCL group progressed to chronic renal failure, 9 initial and 4 subsequent nonresponders progressed in the FSGS group. CONCLUSIONS: The FSGS group formed about a half and the MCL group formed about a third of steroid-resistant nephrotic syndrome in children. Although the response to 2nd line drug therapies was not different between 2 groups, the incidence of progression to chronic renal failure was significantly higher in the FSGS group.
Azotemia ; Child ; Cyclophosphamide ; Dipyridamole ; Drug Therapy ; Enalapril ; Female ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Male ; Medical Records ; Methylprednisolone ; Nephrotic Syndrome* ; Retrospective Studies ; Seoul

OBJECTIVE: To determine the factors associated with the eff ect of steroid on adult primary nephrotic syndrome. METHODS: The general information, laboratory examination and renal pathological type of 425 patients with primary nephrotic syndrome were retrospectively analyzed. RESULTS: Th ere were significant differences in the response to steroid among the pathological types of minimal change disease, focal segmental glomerulosclerosis and IgA nephropathy. Th e patients in the age of 14-24 years old showed the strongest response to steroid (P<0.05). Th e IgA level in the steroid resistance group was lower than that in the non-steroid resistance group (P<0.05). There was no significant difference in urine protein in 24 hour quantitation in the steroid resistance group between pre- and post-treatment (P>0.05), while there was significant difference in urine protein in 24 hour quantitation in the non-steroid resistance group between pre- and post-treatment (P<0.05). CONCLUSION Pathological types and ages of the patients are related to the steroid curative effect. The decrease in IgA probably affects the effect of steroid on primary nephrotic syndrome.
Adolescent ; Adult ; Glomerulonephritis, IGA ; drug therapy ; Glomerulosclerosis, Focal Segmental ; drug therapy ; Humans ; Kidney ; physiopathology ; Nephrosis, Lipoid ; drug therapy ; Nephrotic Syndrome ; drug therapy ; Proteinuria ; Retrospective Studies ; Steroids ; therapeutic use ; Urinalysis ; Young Adult

OBJECTIVETo observe the effects of Shensu II Recipe on the renal function, mesangial extracellular matrix (ECM) accumulation, the expressions of transforming growth factor-beta1, (TGF-beta1), and plasminogen activator inhibitor-1 (PAI-1) in the focal segmental glomerulosclerosis (FSGS) rats.

METHODSFSGS SD rat model was induced by injecting adriamycin. They were randomly divided into the model group, the Western medicine group, and the Chinese medicine group according to body weight. Besides, another 12 rats was taken as the blank control group. Of them, benazepril (0.33 mg/100 g) was given to rats in the Western medicine group by gastrogavage, while Shensu II Recipe (3.5 g/100 g) was given to rats in the Chinese medicine group by gastrogavage. Normal saline was given to rats in the control group and the model group by gastrogavage. Six rats died during the experiment process, among which, one in the control group, two in the model group, one in the Western medicine group, and two in the Chinese medicine group. The changes of 24 h urinary protein (24 hU, pyrogallol red method), blood urea nitrogen (BUN, urease method), serum creatinine (SCr, enzymatic assay of creatinine), serum total protein (TP, biuret colorimetry), serum albumin (ALB, bromocresol green colormetry) were detected. The pathomorphological changes of the glomerulus were observed. Fibronection (FN), collagen IV (Col IV), glomerulus sclerosis index (GSI), ECM/glomerulus area (GA), expressions of TGF-beta1, and PAI-1 were determined by semi-quantitative analysis.

RESULTSAt the end of the 12th week, improvement was shown in the Chinese medicine group (24 hU: 38.55 +/- 2.49 mg; BUN:10.87 +/- 1.78 mmol/L; SCr: 51.70 +/- 1.50 micromol/L; TP: 68.28 +/- 2.31 g/L; and ALB: 42.43 +/- 1.95 g/L). The pathomorphological observation showed that the development of glomerulosclerosis (GS) was significantly slowed down. Semi-quantitative analysis showed significant difference when compared with the model group (GSI: 1.68 +/- 0.33 grade; ECM/GA: 7.11% +/- 2.46%; FN: 4.15% +/- 1.55%; Col IV:1.47% +/- 0.48%; TGF-beta1:19.70% +/- 5.05%; PAI-1: 22.57% +/- 10.65%) ( P < 0.05, P < 0.01).

CONCLUSIONShensu II Recipe could postpone the development of GS in FSGS rats possibly through inhibiting the expressions of TGF-beta1 and PAI-1, hindering the over-accumulation of mesangial matrix.

Animals ; Disease Models, Animal ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Extracellular Matrix ; drug effects ; Glomerular Mesangium ; drug effects ; Glomerulosclerosis, Focal Segmental ; drug therapy ; metabolism ; Male ; Plasminogen Activator Inhibitor 1 ; metabolism ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta1 ; metabolism

OBJECTIVETo investigate the effects of Tongluo Recipe on the expression of collagen IV (Col IV), fibronectin (FN), laminin (LN), transforming growth factor-beta1 (TGF-beta1) in rat renal tissues and explore the mechanism underlying these effects in rats with glomerular sclerosis.

METHODSThe pathological changes in the renal tissues of rats with glomerular sclerosis were observed microscopically, and the expressions of Col IV, FN, LN, and TGF-beta1 were detected using immunohistochemical staining and image analysis system.

RESULTSTongluo Recipe significantly decreased the expressions of Col IV, FN, LN and TGF-beta1 in the renal tissue of rats with glomerular sclerosis (P<0.05 or P<0.01) and obviously alleviated the renal pathologies (P<0.01).

CONCLUSIONThe therapeutic effects of Tongluo Recipe are probably mediated by lowered expressions of Col IV, FN, LN and TGF-beta1.

Animals ; Collagen Type IV ; biosynthesis ; Drugs, Chinese Herbal ; therapeutic use ; Fibronectins ; biosynthesis ; Glomerulosclerosis, Focal Segmental ; drug therapy ; metabolism ; pathology ; Kidney ; drug effects ; metabolism ; pathology ; Male ; Phytotherapy ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta1 ; biosynthesis ; Treatment Outcome

No abstract available.
Adolescent ; Biopsy, Fine-Needle ; Fluorodeoxyglucose F18/diagnostic use ; Glomerulosclerosis, Focal Segmental/therapy ; Graft Rejection/drug therapy ; Humans ; Immunosuppressive Agents/therapeutic use ; *Kidney Transplantation ; Liver/radiography/ultrasonography ; Lymphoproliferative Disorders/pathology/*radiography/radionuclide imaging ; Male ; Positron-Emission Tomography ; Tomography, X-Ray Computed

OBJECTIVETo investigate the mechanism of "Jianpi Qinghua Decoction" (JQD) on renal fibrosis by observing the impact of JQD on serum total cholesterol (TC), triglyceride (TG), low-density lipoprotein (LDL), very-low-density lipoprotein (VLDL), and kidney tumor necrosis factor-Α (TNF-Α) expressions in focal segmental glomerulosclerosis rats induced by nephrectomy combined with adriamycin.

METHODSTotally 56 male SD rats were divided into normal group, sham operation group, model group, JQD group, Yiqi Jianpi group, Qingre Huashi group, and Niaoduqing group (all n=8). The model of focal segmental glomerulosclerosis was established by the unilateral nephrectomy and the injection of adriamycin in caudal vein of rat at a dose of 3 mg/kg in the latter 5 groups. JQD, the disassembled prescription of Jianpi Qinghua Decoction (Yiqi Jianpi Decoction and Qingre Huashi Decoction), and Niaoduqing Capsule were administered separately for 8 weeks. The serum TC, TG, LDL, and VLDL levels and the expression of Kidney TNF-Α were determined.

RESULTSCompared with normal group and sham operation group, the serum TC, TG, LDL, and VLDL levels and the kidney TNF-Α expression in the model group were significantly higher (all P<0.01). Compared with the model group, the JQD group, Qingre Huashi group, and Niaoduqing group had significantly lower serum TC, TG, LDL, and VLDL levels and kidney TNF-Α expression (all P<0.01). Compared with the model group, the Yiqi Jianpi group had significantly lower serum TC ,TG, and VLDL levels (all P<0.01), while the serum LDL level and kidney TNF-Α expression remained unchanged (all P>0.05).

CONCLUSIONSJQD can regulate serum lipids and lower the TNF-Α expression in kidney tissue and thus improve the renal inflammation and relieve renal fibrosis. The heat-clearing and dampness-removing herbs in the prescription play a central role in fighting against renal fibrosis.

Animals ; Disease Models, Animal ; Drugs, Chinese Herbal ; therapeutic use ; Fibrosis ; Glomerulosclerosis, Focal Segmental ; blood ; drug therapy ; Kidney ; metabolism ; pathology ; Lipids ; blood ; Male ; Rats ; Rats, Sprague-Dawley ; Tumor Necrosis Factor-alpha ; metabolism

OBJECTIVETo investigate the clinical and pathological characteristics of focal segmental glomerulosclerosis (FSGS) in children.

METHODSThe data of 38 children,aged from one and half to 15 years, 25 boys and 13 girls, with primary FSGS were studied retrospectively.

RESULTSMajority of the cases in this study were school-aged children. The average age of initial onset was 8.9 +/- 3.68 years. The ratio of boys to girls was 1.92. The clinical manifestation included isolated proteinuria in 3 cases, proteinuria and hematuria in 1 and nephrotic syndrome in 34 (simple type in 16 and nephritic type in 18). Of 38 cases, 24 (63%) presented with hematuria, 11 (29%) with hypertension and 7 (18%) with decreased creatinine clearance. The pathologic classification included perihilar variant in 17 cases, peripheral variant in 14 and tip variant in 7. The predominant clinical feature of children with tip variant was simple type of nephrotic syndrome (86%). Microscopic hematuria was not common (29%). Blood pressure and renal function were normal. The children with diffuse mesangial hypercellularity superimposed on changes of FSGS (in 21 of 38 cases) were more likely to have hematuria (76%) and less simple nephrotic syndrome (30%). The initial treatment response to prednisone in 34 cases with nephrotic syndrome showed sensitive in 12 cases, resistant in 21 and unknown in 1. Transition from sensitive to resistant occurred in six of 12 children. Three of 4 cases with non-nephrotic syndrome showed no response and the remaining one had unknown response. It was found that 44% of children who received cyclophosphamide and 83% of children who received pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A in addition to oral steroids had complete or partial remission. Correlation analysis showed that the level of proteinuria after treatment was correlated directly with renal tubulointerstitial lesion and renal function (Pr = 0.48, P < 0.05; Pr = 0.45, P < 0.05).

CONCLUSIONFSGS was common in school-aged children. The predominant presenting feature was nephrotic syndrome. Hematuria was common. Hypertension and renal insufficiency were less frequently seen. The renal biopsy showed multiple variants. Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A treatments showed relatively good response.

Adolescent ; Child ; Child, Preschool ; Creatinine ; blood ; Female ; Glomerulosclerosis, Focal Segmental ; complications ; drug therapy ; pathology ; Glucocorticoids ; therapeutic use ; Hematuria ; etiology ; Humans ; Hypertension ; etiology ; Infant ; Male ; Methylprednisolone ; therapeutic use ; Prognosis ; Proteinuria ; etiology ; Retrospective Studies ; Treatment Outcome