To further study the renal damage of SS with biopsy and recent determination. Methods 31 patients of SS with renal impairment from 1988 to 1995 were analyzed by routine, immunoassay,tubular function and biopsy examination. Results 25/31 cases presented dRTA, 3/25 with diabetes insipitus and 2/25 with hypokalemic paralysis. Glomerulopathy was occured in 8 of 31 (NS 5, GN 3) and mild renal failure( RF) in 4. Hypergamma-globulinemia was observed in 23 of 31 (74%). About 65% and 45% patients revealed anti ss-A and anti ss-B positive respectively. In 13 renal biopsies, 9 specimens showed CIN with large number of lymphoplasma cells infiltration and tubular atrophy and other 2 specimens revealed LN (type Ⅲ and Ⅳ ) and 2 MPGN. By IF, no positive findings were seen in 7 cases and deposition of IgA, IgM or 63 in some other patients. IgG deposits in the interstitial lymphoplasma cells in 1 patient. 19 patients were treated with prednisone and 3 combined with CTX. 14 cases remitted during the follow-up. In 4 patients with RF, Scr level returned to normal after treatment. Conclusion The renal impairment of SS may be the presenting or predominant feature. Their major clinical manifestations are dRTA and GN. The treatment with prednisone may decrease the infiltration of lymphoplasma cells in the interstitium, improve the renal function and correct RTA.

Objective To observe the therapeutic effect of interferon-? on adult patients with HBV associated glomerulonephritis.Methods Nine patients with HBV associated glomerulonephritis diagnosed in Shanghai Ruijin Hospital from September 1996 to March 2003 were treated with interferon-? at a dose of 3 million units every other day and the total period was 6 to 9 months.The proteinuria,serum HBV marks and bDNA were measured before and after therapy.Results At the end of the therapy urinary protein excretion significantly reduced(P

Objectives To investigate the incidence of deep vein thrombosis (DVT) of lower limbs and pulmonary embolism (PE) caused by hypercoagubility in patients with nephrotic syndrome and to estimate the indication and clinical effect of anticoagulation therapy. Methods 54 patients with nephrotis syndrome(thirty-one males and twenty-three females with mean age: 44 13?15 88, 24 hours proteinuria:8 43?5 64g, albuminemia: 20 48?5 41g/L) were enrolled in this study.DVT of lower limbs and PE were confirmed by radionuclide imaging (RNV) with 99mTc-MAA and the clinical effect of subcutaneous injection of low molecular weight heparin (LMWH) and oral antiplatelet coagulation therapy were evaluated as well. Results 41 out of 54 cases (76%) had DVT of lower limbs, 14 8% of which had clinical manifestations. 32 out of 54 cases (59 25%) had PE, 7 4% of which had symptoms. 70 7% of PE were caused by DVT of lower limbs. No patient died of PE after the LMWH treatment.Conclusions DVT is one of the most important complications of NS. RNV can be used to make early diagnosis of DVT of lower limbs and PE. Early anticoagulation therapy might ameliorate the prognosis of NS and lower the mortality of PE.

Objective To analyse the clinicopathological changes of Alport syndrome (AS) and to detect the deposition of type Ⅳ collagen within basement membrane of Alport patients. Methods Fourteen patients with AS (12 families) hospitalized from 1990. 1 to 1996. 6 were investigated. Eleven were male and 3 female (mean age 29. 4 years). Results Microscopic hematuria was found in 13, with recurrent gross hematuria in 7. All had proteinuria. Three patients presented nephrotic syndrome. Progressive renal failure occured in 10 of 11 male(11 - 39 years) and 1 female (40 years). Six patients were treated with hemodialysis, two of them with transplantation. Sensorineural deafness was observed in 9 patients particularly high frequency sound. Anterior lenticonus were presented in 2. It showed heterogeneitic, 50% transmitted as X-linked dominant(XD) trait. In 7 renal biopsies, the findings by light microscopy mostly revealed focal and segmental sclerosis glomerulonephritis (4/7). The results of immunofluorescenc e (IF) were ne gatt ye in 4. Ultrastructural studies showed variable thickening, thinning of glomerular basement membrane (GBM) in 7 specimens with lamellation and basket-weaving of GBM in 1. Using the iIF technique, the ?3, 4, 5 (Ⅳ) chains were absent within both GBM and EBM of 4 male XD-AS patients. Conclusions AS is not a rare hereditary disease characterized by hematuria, proteinuria and progressive renal failure with sensorineural deafness and ocular lesions. Type Ⅳ collagen within Alpori patients' basement membrane is abnormal and iIF study of type Ⅳ collagen chains distribution is useful to confirm the diagnosis of AS.

Objective\ To investigate the effect and safety of LMWH on treatment of nephrotic syndrome(NS).Methods\ We randomized 43 patients with primary NS into 2 groups.Steroid and cyclophosphamide were given in control group.LMWH with the dosage of 3200～5000IU twice per day was added in the treated group.Results\ After 4 to 6 weeks of treatment,a significant increase in AT-Ⅲ,anti-Xa activity and serum albumin and decrease in fibrinogen and proteinuria were observed in LMWH.Conclusion\ LMWH should be recommended as a useful treatment of NS.

Objective To evaluate the renal involvement and therapeutic effect on ANCA-associated vasculitis(ASV) . Methods Fifty-six cases were diagnosed as ASV from Oct 1997 to Mar 2001. Among them, 21 cases received renal biopsies. According to disease activity and severity, Use following treatment protocols were choosed: (1) double pulse therapy; (2) single pulse therapy; (3) oral prednisone and/or low dose of CTX; (4) blood purification technique and/or immunosuppressive therapy. Remission rate was also analyzed. Results 96. 4% of case suffered with renal involvement. 91. 1% with acute renal failure, 96.4% with severe renal failure, 98. 2% with multiple organs involvement. The mortality was 19. 6% and the remission rate 67. 9%. 12. 5% of cases lost follow-up. ConclusionsRenal involvement is common and serious in ASV patients. Individualized and early therapy, as well as valuable urgent renal biopsy are necessary. To those who have entered ESRD, immunosuppressive agents could be used to decrease the extra-renal complication.

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Objective To evaluate the diagnostic value of clinical laboratory indexes on judgment of hypovolemia in the patients with nephrotic syndrome (NS).Methods The blood volume of each 50 cases of healthy adult men and women was assessed with indocyanine green-pulse dye densitometry (ICG-PDD).The normal range of blood volume and the cut-off value of hypovolemia were determined.The blood volume of 81 patients with NS was also measured with ICG-PDD and then these patients were divided into the hypovolemic group (21 cases) and the non-hypovolemic group (60 cases) according to the cut-off value of hypovolemia.The test data of clinical laboratory indexes of the patients in the two groups were compared,and the indexes with statistic difference were screened out.Their diagnostic values on judgment of hypovolemia were evaluated by receiver operating characteristic (ROC) curve analysis,and finally the indexes with high judgment accuracy were selected.Results ①The cut-off values of hypovolemia are ＜ 52.9 ml/kg for the male and ＜ 52.5 ml/kg for the women,which were determined with ICG-PDD.②The five clinical laboratory indexes,including orthostatic heart rate (OHR) increase ＞ 10 bit per minute,fractional excretion of sodium (FENa) ＜ 1,transtubular potassium gradient (TTKG) ＞ 60％,blood urea nitrogen/serum creatinine ratio (BUN/Scr) ＞ 20,and urine specific gravity (SG) ＞ 1.020,were used to judge the hypovolemia in NS patients in this study.ROC curve analysis showed that the diagnostic accuracy in judgment of hypovolemia by one index or two indexes combination only belonged to medium level.However,the diagnostic accuracy in judgment of hypovolemia by the following three indexes combination,i.e.OHR increase+FeNa+BUN/Scr,FeNa+ BUN/Scr+SG,OHR increase+TFKG+BUN/Scr,or ORG increase+FeNa+TTTKG,reached high level.Conclusion This study obtained the cutoff value of Chinese adults hypovolemia are ＜ 52.9 ml/kg for the male and ＜ 52.5 ml/kg for the women,which are determined with ICG-PDD,through evaluation we recommend applying the above four specific combinations of three indexes for diagnosis of hypovolemia in NS patients.

BACKGROUND: Cell apoptosis and ventricle reconstitution following myocardial infarction are of mutual cause-effect, and they cause vicious cycle. How to reduce the apoptosis events following myocardial infarction is one of keys to saving heart function.OBJECTIVE: To observe the effect of umbilical cord blood mesenchymal stem cell (UCBSMC) transplantation on remaining myocardial tissue of dogs with acute myocardial infarction.DESIGN: A randomized controlled observation.SETTING: Department of Cardiothoracic Surgery, Xinhua Hospital.MATERIALS: This study was carried out in the Central Laboratory of Xinhua Hospital from October 2005 to May 2007.Thirty-six adult hybrid dogs, male and female in half, were provided by the Animal Experimental Center of Xinhua Hospital.METHODS: Thirty-six dogs were divided into cell transplantation group and control group, with 18 dogs in each according to table of random digit. Mesenchymal stem cells were isolated from the umbilical cord blood of full-term pregnant hybrid dogs, cultured and amplified. Then, they were labeled with Laz gene, in vitro induced with 5-azacytidine, and transplanted into the dogs with acute myocardial infarction in the cell transplantation group. Rats in the control group were injected with the same amount of normal saline. Each dog was euthanized by anesthesia for harvesting myocardial specimen 1,4 and 8 weeks after transplantation.MAIN OUTCOME MEASURES: ① Remaining and apoptosis index detected by TUNEL method. ② Myocardial cell volume and histomorphology detected by confocal microscopy. ③ Histological change of myocardial collagen network detected by haematoxylin-basic fuchsin-picric acid staining.RESULTS: Thirty-six involved experimental dogs all entered the stage of final analysis. ①The apoptosis index in the cell transplantation group was significantly lower than that in the control group 1, 4 and 8 weeks after cell transplantation (P ＜0.05). ② Myocardial cell volume in the cell transplantation group 1, 4 and 8 weeks after cell transplantation was significantly larger than that in the control group (P ＜ 0.05). ③ Collagen fiber in the myocardial tissue of dogs in the cell transplantation group was arranged in order and regularly, and in contrast that in the control group was not, and fibers in the control group fused partially.CONCLUSION: UCBSMC transplantation reduces the apoptosis of myocardial cells, promotes the hypertrophy of remaining myocardial cells, regulates myocardial collagen network and improves heart function.

Objective To investigate the underlying mutation in a late-onset Chinese patient with classic Bartter syndrome. Methods The mutation analysis of CLCNKB gene was performed by the PCR direct sequencing. The patient's parents and siblings were studied as well. Fifty normal volunteers were analyzed as control group. Results The heterozygous deletion mutation cDNA 753delG and heterozygous missense mutation G433E were detected in the patient. Her father was found to carry heterozygous G433E and her mother to carry cDNA 753delG mutation respectively. Her brother carried heterozygous G433E and her sister was normal. Conclusions Two mutations of the CLCNKB gene in this Chinese patient with late-onset classic Bartter syndrome are identified. The cDNA 753delG mutation has not been reported previously.