Primary nephrotic syndrome (PNS) is one of the most common forms of glomerular disorder in chil dren.It is reported that over 90％ of patients are due to minimal change disease and most respond to glucocorticoid (GC) therapy.However,76％-93％ cases have relapses and 40％-50％ frequently relapses nephritic syndrome or steroid dependent nephritic syndrome when steroid is tapered.Long-term courses of GC and immunosuppressant will place them at increased risk for severe side effects.Not all patients do respond to therapy.Although most reports on rituximab use in pediatric patients have not recognized significant side effects,the long-term adverse events of rituximab are not known.Thus,controlled long-term studies are required to be done to assess the risk-benefit profile of rituximab in childhood nephrotic syndrome.

Application of Radio Frequency Identification (RFID) into transfusion management in hospital wards,for elimination of errors from transfusion.Its clinical practice in three years has significantly improved transfusion efficiency,optimized workflow and reduced nursing errors.In the meantime,it has improved informationization of supplies satisfactorily.The paper also discussed the merits and defects of RFID for reference and review of the readers.

Objective To investigate the drug effect of children with Dent disease who received therapy of po-tassium citrate and thiazide diuretics and angiotensin -converting enzyme inhibitors(ACEI),and to provide the refe-rence for the clinical treatment.Methods Dent disease patient who were followed -up in Bayi Children′s Hospital Af-filiated to Beijing Military Region General Hospital during the period of July 2006 and March 201 4 were selected.The patients were administered a therapy of potassium citrate associated with thiazide diuretics and ACEI according to the level of proteinuria and calciuria and serum potassium.The underlying changes before and after the treatment were com-pared and analyzed.Results In 1 5 children with Dent disease,they were all male cases,onset age ranged from 3 months to 1 1 years old[(2.62 ±3.1 1 )years old],and the disease duration ranged from 0.50 to 9.50 years old [(2.81 ±2.34)years].The patients were followed up for 0.50 to 7.50 years[(3.61 ±2.62)years].There was a sig-nificantly statistical difference in calcium/creatinine and daily Ca -creat ratio in contrast to before treatment[(0.41 ± 0.1 9)mg/mg vs(0.26 ±0.1 2)mg/mg,t =2.603,P =0.021 ;(6.76 ±2.0)mg/kg vs (4.34 ±1 .97)mg/kg,t =5.265,P =0.000],there was no significantly statistical difference in 24 -hour urinary protein quantity in contrast to before treatment[(0.96 ±0.62)g/24 h vs (0.87 ±0.44)g/24 h,t =1 .01 6,P =0.327].One case with kidney stone and 5 cases with nephrocalcinosis had a negative result of renal ultrasound after treatment.Conclusions Treatment of potassium citrate combination with thiazide diuretics and ACEI can significantly decrease urinary calcium excretion, make a disappearance of kidney stone and nephrocalcinosi,and it may have a role in protecting renal function.Treat-ment of benazepril can not significantly decrease the proteinuria and has no substantial improvement in low molecular weight protein urine.

OBJECTIVE:To establish a bacterial endotoxin test for monitoring pyrogen in wilsonii injection METHODS:Bacterial endotoxin test procedure and guiding principle in Chinese Phamacopoeia were used RESULTS:The test showed that the reaction with limulus agent was not interfered by wilsonii injection with a sensitivity of 0 25Eu/ml CONCLUSION:Limulus lysate method can be used to take the place of rabbit method for monitoring pyrogen in wilsonii injection

Objective To investigate the clinicopathological features and treatment of children with C3 glomerulopathy (C3 G).Methods Seven children diagnosed as C3 G by clinical and pathological characteristics were enrolled in this study.The clinicopathological data and the prognosis were analyzed.Results Of the 7 cases,4 cases were female and 3 cases were male,with the mean age of (7.7-± 3.1) years old (1.5-10.4 years old) at onset,the duration from onset to renal biopsy was (3.4 ± 2.4) months (1-6 months) and 1 of them had a second renal biopsy 4.2 years later,and mean age was (8.4 ± 3.6) years old (1.8-13.3 years old) on diagnosis.Clinical features:among the 7 patients,6 cases had hematuria,among them 1 case had gross hematuria and 5 cases had microscopic hematuria;6 cases had low level of serum complement C3,5 cases had heavy proteinuria and low serum albumin,and anemia was observed in 2 cases respectively.Five cases had complement factor H and H factor antibody by examination,and 1 of them had low serum factor H,but none of them had serum antibody to factor H.Four cases had genetic evaluation,and only 1 case revealed risk variants in the C3 gene(R304R,T612T,V807V,A915A,P1632P)and CFH gene(p.H402Y,p.E936D).Clinically,4 cases were diagnosed as nephrotic syndrome of nephritis type,2 cases were diagnosed as nephritic syndrome,and 1 case was diagnosed as nephrotic syndrome of simple type.Immunofluorescence study showed that all the cases had intense deposition of C3,and 6 cases were accompanied by the deposition of immunoglobulin.Under light microscopy,3 cases showed the feature of membrane proliferative glomemlonephritis,2 cases with endocapillary prolifera-tive glomerulonephritis,1 case with mesangial proliferative glomerulonephritis,and 1 case with endoeapillary proliferative IgA nephropathy.Under electron microscopy,3 cases who had typical ribbon-like dense deposits in glomerular basement membrane were of dense deposit disease,and the rest were C3 glomerulonephritis.All patients had steroid and immune inhibitor treatment,and during the follow-up stage of (2.6 ± 1.8) years(1.1-5.6 years),4 cases showed normal urinalysis,2 cases had microproteinurine and microscopic hematuria,and 1 case had urinary protein ± to + + and microscopic hematuria.Conclusions C3G has variety of pathological-clinical manifestation.Interpretation of individual cases depends on integration of information from the biopsy together with clinical,serological,and genetic features.Patients with steroid and immune inhibitor treatment had some clinical improvement of their urinalysis.

ObjectiveTo investigate the mechanism, diagnosis and treatment of membrane proliferative glome-rulo-nephritis (MPGN) transitioned from endocapillary proliferative glomerulonephritis (EnPGN).Methods The clinical data and the results of pathological examination of one case of MPGN transitioned from EnPGN were retrospectively analyzed.Results The child was presented with proteinuria, microscopic hematuria, and persistent low level of complement C3. The type of renal pathology was transitioned from EnPGN to MPGN. Complete remission was achieved in this child with the treatment of oral prednisolone and tacrolimus, but the level of plasma complement C3 remained low.Conclusions The type of renal pathology in children with persistent low level of complement C3 could make a transition, and the early diagnosis, timely and effective treat-ment are important.

Desmoplastic small round cell tumor( DSRCT) is a kind of highly uncommon malignant tumor which is reported in very recent years.Since it has been firstly reported by Gerald in 1991,DSRCT has only a-round one hundred reported cases globally, and very limited cases domestically.This report illustrates the first case of DSRCT in our hospital so as to share and to discuss with clinicians.

Objective To investigate the expression of myeloid antigen(MyAg) in acute lymphoblastic leukemia (ALL) and its correlation with clinical characteristics and prognosis.Methods The clinical data of 103 newly diagnosed ALL 1patients were retrospectively analyzed.These patients were divided into myeloid antigen-positive group (MyAg+ ALL) and myeloid antigen-negative group(MyAgALL) based on the flow cytometry(FCM) analysis in bone marrow.The correlation of MyAg expression with the clinical features,short-term efficacy and 5-year overall survival were assessed in the two groups.Results MyAg expression was detected in 46.5％ out of 103 cases and CD13,CD33 were the most commonly expressed MyAg(34.2％ and 21.4％ respectively).The expression of CD34 in MyAg+ ALL group was higher than MyAgALL group(75.3％ vs 60.5％,P ＜0.05).The expression of MyAg in T-ALL group (66.7％) was higher than B-ALL group (42.8％) (P ＜ 0.05).The clinical and biological characteristics of ALL patients between MyAg+ and MyAggroups showed that higher percentage of incoincidence of classification between morphology and immunology.Conclusion MyAg expression was not correlated with clinical characteristics and prognosis of ALL patients.

Objective To investigate the relationship between oxidative stress and cell apoptosis in the kidney of rabbit induced by high energy shock wave(HESW).Methods Forty healthy male rabbits were surgically managed to the mono-nephron models and randomized into 4 groups consisting of ten each: control group,1 000 HESW group,2 000 HESW group and allopurinol pretreated plus 2 000 HESW group.The rabbit kidney tissues were obtained.The activity of superoxide dismutase(SOD) and malondialdehyde(MDA) levels in the renal tissue were measured 24 hours after HESW application.The cell apoptosis was detected by TdT-mediated dUTP nick end labelling(TUNEL) and flow cytometry.Results Compared with the control group,the MDA level and the rate of apoptosis in the HESW groups increased,and the activity of SOD reduced significantly(P

Objective To study the changes of blood gastrointestinal hormones concentration and effect of early feeding in premature infants on it. Methods Seventy-two premature infants were divided into 4 groups: self-sucking (group Ⅰ),tube feeding (group Ⅱ),minimal enteral nutrition (MEN, group Ⅲ), non-nutritive sucking (NNS, group Ⅳ). The plasma motilin and serum gastrin were measured within 12 hour before enteral feeding, on day 3 and day 7 of life by radioimmunoassay. 16 full-term neonates were studied as control. Results (1)The serum concentration of gastrin in premature infants before enteral feeding, on day 3 and day 7 were (61?24)ng/L,(93?37)ng/L,(126?45) ng/L respectively, which were significantly lower than that of control(126?30) ng/L,(135?34)ng/L,and (178?46)ng/L,(P