To investigate the significance of liver biopsy in children with nephrotic syndrome,liver and renal biopsy samples were simultaneously examined with light microscopy,elctronic microscopy,and immunofluorescence in 10 patients. The results showed that all patients had abnormal liver histologic changes.Focal fatty degeneration in liver tissue was found under microscopic examination,and cytolysis and mild fatty degeneration were found in hepatocytes under electronic microscopic examination.The results suggested that disorder of lipid metabolism not only was toxic to glomeruli, but also deleterious to hepatocytes,then in turn enhanced abnormality in lipid metabolism. It is believed that hyperlipidemia is related to an increased anabolism of lipid and apolipoprotein, and reduction in catabolism of chylomicron and very low density lipoprotein .Therefore, with treatment of hyperlipidemia in these patients, it is possible to arrest progressive renal injury.

We have established adriamycin(ADR) nephropathy model, the expression of cyclophilin(CyP)mRNA was measured with RT PCR, and its relationship with the levels of total protein, albumin, cholesterol, 24 hour urine protein, BUN, and creatinine were determined.After four weeks,the levels of urine protein in ADR nephropathy animals were higher than 100mg/24h, whereas the levels in the control animals were lower than 6mg/24h. It was showed that the expression of CyP gene of kidney tissue in ADR nephropathy rats was significantly higher than that of the control animals. Meanwhile, it was found that there was negative correlation was found between the expression of Cyp gene and the levels of total protein and albumin,but positive correlation between the expression of CyP gene and the levels of 24 hour urine protein and cholesterol,and the expression of CyP gene had no correlation with the levels of BUN and creatinine. Our results suggested that the high levels of Cyp mRNA expression might contribute to renel tissue damage in ADR nephropathy,and it might reflect the degree of severity and prognosis in ADR nephropathy.

Objective Alport syndrome is one of the diseases that may lead to the end-stage renal disease ( ESRD) in chil-dren, and the methods for its diagnosis and treatment remain quite limited.This study aimed to investigate the clinical and genetic di-agnosis of a Chinese family with hematuria companied by genetic nephritis. Methods We analyzed the renal pathology of 7 patients in a family, performed immunofluorescence staining of type-Ⅳcollagen in the nephridial and skin tissue, conducted gene sequencing i-dentification using the exon sequence method, and examined the blood and urine samples from the patients. Results Renal patholo-gy manifested mesenterium hyperplasia in the index patient, with IgM+under the light microscope, no thickening or thinning under the electromicroscope, and no absence of type-Ⅳcollagen on immunofluorescence analysis.Mutation of c.1365_1373del TCCAGGCCC (p.Pro456_Pro458del3) was observed in exon 21 of the COL4A5 gene.Only 1682 amino acids were found in the mutated protein as compared with 1685 in the wild type. Conclusion This is the first case of Alport syndrome induced by gene deletion mutation ever reported in China and abroad.There are many female patients in this family, all with a high risk of reproduction failure.Antepartal gene diagnosis or genetic diagnosis before embryo transfer may contribute to the prevention of the disease.

Objectives To explore the clinical manifestations, treatment and prognosis of a case of blindness caused by nephrotic syndrome with cerebral venous sinus thrombosis (CVST). Methods The clinical manifestations, diagnosis and treatment of a case of NS with CVST were analyzed. The latest domestic and foreign reseach progresses in treatment for CVST in children were reviewed. Results Epilepsy suddenly appeared with diplopia, binocular vision loss and blindness in anticoagulant therapy for the child with NS. Brain magnetic resonance venography (MRV) suggested CVST. MRV reexam-ined showed that the intracranial thrombosis was completely dissolved after urokinase thrombolysis for one month followed by ineffective heparin anticoagulation. At present, international standards of anticoagulant therapy have been adopted in the treatment for CVST patients. Coagulation function (e.g.APTT) and international standardization ratio were monitored in order to prevent bleeding. Conclusions It is better to perform neural imaging examination early in suspected CVST patients. Anti-coagulation and thrombolytic therapy should be given immediately once the risk of bleeding was excluded and used for 3-6 months.

Objective:To invesgate the correlation between Angiotensin converting enzyme(ACE) gene polymorphism and Henoch-Schonlein purpura nephritis(HSPN) in children. Methods:According to the clinical features,pathological changes,48 HSPN children have been devided into 4 clinical types,Uab,I-GH,R-GH and NS type,and Ⅱ-Ⅳ 3 histological degrees respectively.The correlations between serum ACE level,ACE gene polymorphism and clinical presentation,pathlogical changs,effect to therapy were analyzed.Results:In the 48 patients,35(72.9%),4(8.3%),4(8.3%) and 5(10.5%) patients belonged to Uab type,I-GH type,R-GH type and NS type respectively.20(41.7%),19((39.6%)) and 9(18.7%) patients belonged to Ⅱ,Ⅲ and Ⅳ histological degrees respectively.5 patients had DD genotype,25 patients had ID genotype and 18 patients had II genotype.The mean serum ACE level of DD genotype patients was(39.60?11.40)U/L,which was significant higher than that of ID genotype patients[(24.29?11.62)(U/L) and II genotype patients(4.49?11.41)U/L](P

Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) is an autoimmune disease with multi organ involvement characterized by vascular wall inflammation and fibrinoid necrosis, including microscopic polyangitis (MPA), granuloma polyangitis (GPA), and eosinophilic granuloma polyangitis (EGPA). Because its clinical manifestations are complicated and non-speciifc, it is dififcult to make early diagnose. In recent years, some new progress has been made in diagnosis and treatment of this disease. The article will review the related information.

ObjectiveTo estimate the application of mycophenolate mofetil (MMF) and cyclophosphamide(CTX) intravenous pulse therapy on diffuse proliferative lupus nephritis (DPLN).MethodPubMed,Medline,EMBASE and CNKI were searched from the establishment of the database.Meta-analysis of 14 comparative studies on MMF and CTX in treatment of DPLN was performed,taking the remission,the relapse,the death of MMF and CTX for DPLN as primary efficacy variable,mean while taking the herpes zoster as safety evaluating indicator.ResultsMMF was better than CTX in remission rate ( P ＜ 0.05 ).There was no difference between in incidence rate of the relapse,the death and the herpes zoste MMF and CTX for DPLN ( P ＞ 0.05 ).ConclusionMMF was better than CTX on the efficacy and safety in DPLN.

Objective Mizoribine ( MZR) is a new immunosuppressant , however , little domestic research has been done on MZR for treatment of nephrotic syndrome in children .This study was to investigate curative effect and adverse reaction of MZR in the treatment of children with frequently relapsing nephrotic syndrome , using prospective controlled trials . Methods A total of 59 pa-tients with frequency relapsing nephrotic syndrome were randomly divided into two groups .29 patients of treatment group were treated with MZR +glucocorticoid , while 30 patients of control group were given Tripterygium wilfordii ( TW)+glucocorticoid treatment , and the course of treatment lasted for 12 months.24-hour urine protein, urinary N-acetyl β-glucosidase (NAG), serum albumin, serum cholesterol, serum creatinine, recurrence frequency, and average prednisone dosage were observed . Results At the end of treat-ment, Serum albumin in treatment group was higher than that in control group [(40.95 ±6.12)g/L vs (30.25 ±9.02)g/L], and Se-rum cholesterol ([5.45 ±0.82]mmol/L vs [7.53 ±2.74]mmol/L), urinary protein ([0.89 ±0.52]g/24 h vs [1.63 ±2.02]g/24 h), urinary NAG enzyme ([21.43 ±14.16]U/g· Cr vs [41.67 ±12.35]U/g· Cr) levels were lower compared with control group . There was significant difference between the two groups .In terms of mean recurrence times , no significant difference was found at 6th months of follow-up between the two groups, however, treatment group had lower recurrence rate than control group at 3rd month, 9th month, 12th month of follow-up, which was of significant difference .The average amounts of hormone of treatment group were lower than those of control group ([0.56 ±0.16] mg/kg· d vs [0.72 ± 0.34]mg/kg· d)、([0.64 ±0.35]mg/kg· d vs [0.67 ±0.52]mg/kg· d)、([0.53 ±0.41] mg/kg· d vs [0.83 ±0.37] mg/kg· d)、([0.34 ±0.15] mg/kg· d vs [0.54 ±0.26] mg/kg· d) at 3rd month, 6th month, 9th month, 12th month of follow-up, which was of significant difference . Conclusion Compared to Tripterygium wil-fordii combined with hormone therapy , MZR combined with prednisone therapy in children with recurrent NS frequency can reduce the relapse rate and dosage of corticosteroid to improve the clinical remission rate .

ObjectiveTo investigate the clinical application of tacrulimus (TAC, FK506) in children with primary nephrotic syndrome (NS). MethodsSixty-five primary NS children received routine or decreased-dosage glucocorticosteroid according to clinical NS types after hospitalization. At the same time, TAC was given orally with the dosage of 0.1 to 0.15 mg/kg, once every 12 hours, for 6 to 24 months. And the serum concentration of TAC was monitored during the course. ResultsAfter the treatment of TAC for 1 to 2 months, 65 patients were recovered with gradually reduced urinary protein, rapidly increased serum albumin, and improvement of cholesterol and triglycerides. Total remission rate was 83.1% and onset time was 7 to 54 days. Twelve cases experienced recurrence. Increased CD4, as well as 3/3 or 3/1 TAC genotype, indicated higher remission rate. Various pathological types had different remission rates or ratio, which were as follows: minimal change nephropathy (96.4%), mesangial proliferative glomendonephritis (90.0%), membranous nephropathy (2/3), membranous proliferative glomerulonephritis (3/5), focal segmental glomerulosclerosis (4/9). The patients would recover in the course of treatment under the conditions of TAC initial dose as 0.1 to 0.15 mg /kg per 12 hours and controlled serum concentration as 5 to 10 g/L. During the treatment, 12 cases appeared gastrointestinal symptoms, mainly as anorexia, nausea and vomiting, 1 abdominal pain, 2 headache, 1 tremor, 1 paresthesia, 3 insomnia, 4 transient increased Scr, 8 slightly increased NAG, 6 increased C3 and α-2 macroglobulin. The symptoms disappeared within one week or after stopping TAC. ConclusionsTAC is effective in primary NS children, even with abnormal liver function or tuberculosis infection. TAC can also be a substitute to cyclosporine A.

Objectives: The aim of the study was to evaluate the therapeutic effects of 6-mercaptopurine in the treatment of refractory childhood nephrotic syndrome (NS). Methods: According to the varieties of NS, 6-mercaptopurine (2 mg/kg body weight daily) combined with corticosteroid or 6-mercaptopurine (2 mg/kg body weight daily) alone after tapering of steroids were given to 28 consecutive children with primary NS in our hospital. Results: One month after the use of 6-mercaptopurine, proteinuria was decreased. The duration of improvement was 9～28 days, with mean duration of 17 days. Over-all effective rate was 85.7%. Among different varieties of NS, the best therapeutic effect was noted in steroid-dependent children; the better therapeutic effect in steroid-resistant children; and good therapeutic effect in frequently relapsing children. The effective rates were 100%, 84.6%, 81.8% respectively. All the pathological varieties of 28 children were confirmed by renal biopsy. The better therapeutic effects were noted in slight mesangial proliferative glomerulonephritis (MsPGN) and minimal change nephrotic syndrome (MCNS). The less therapeutic effect was noted in membranoproliferative glomerulonephritis (MPGN). Their therapeutic effective rates were 92.9%, 80%, 66.7% respectively. Unfortunately, drug-induced aplastic anemia was seen in 2 cases. Slight gastrointestinal reactions were present in 6 cases. There were no side reaction on the gonad. Conclusions: The great difference in the therapeutic effects is related to the different pathologic varieties of NS. With regard to the treatment of refractory NS in children, the pathological varieties should be confirmed by renal biopsy as soon as possible. Based on the renal biopsy, 6-mercaptopurine can be considered in the treatment of MsPGN and MCNS. As a result, relapses could be reduced; the duration of remission could be prolonged, and the side reactions from steroid treatment could be avoided. The use of 6-mercaptopurine for the treatment of refractory NS is one of the effective therapy.