Atypical hemolytic uremic syndrome (aHUS) is characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. IgA nephropathy (IgAN) is a common primary glomerular disease, while aHUS combined with IgAN is rare, and has been reported rarely in the past. This paper reported a case of children with aHUS combined with IgAN. In the acute stage, she was treated with plasma therapy and glucocorticoid. After remission, she was treated with glucocorticoid combined with immunosuppressants. During the follow-up period, aHUS did not recur, the renal function was normal, and urinary protein decreased to weakly positive.

Objective: To study the role of alternative complement pathway overactivation in malignant nephrosclerosis. Methods: (1) Fifty patients with confirmed malignant nephrosclerosis by renal needle biopsy were enrolled. Meanwhile, twenty-five cases of time-zero renal needle biopsy were enrolled as control subjects. Enzyme linked immunosorbent assay (ELISA) was used to detect alternative complement pathway of the complement initiation factor B, positive regulation factor P, negative regulation factor H, and the complement end products C3a and C5a in the plasma and urine. (2) Immunohistochemistry was used to detect the deposition of the complement end product C5b-9, C4d and mannan binding lectin (MBL) of lectin pathway in the renal biopsies. Double immunofluorescence labeling method was used to assay the deposition of C5b-9 and CD34 (endothelial cell marker) in the arteriolar endothelium and glomerular capillary endothelium. Results: (1) The plasma and urine levels of complement factor B, factor P, C3a and C5a in malignant nephrosclerosis patients were significantly higher than those in control subjects (all P<0.05), while the plasma and urine levels of complement factor H in malignant nephrosclerosis patients were lower than those in control subjects (all P<0.05). (2) The plasma level of factor P was positively correlated with 24 h urine protein (r_s=0.465, P=0.001). Urinary factor B/urinary creatinine, urinary factor P/urinary creatinine and urinary C3a/urinary creatinine were positively correlated with serum creatinine in malignant nephrosclerosis patients (r_s=0.483, P<0.001; r_s=0.352, P=0.012; r_s=0.319, P=0.024), while urinary factor H/urinary creatinine was negatively correlated with serum creatinine and 24 h urine protein (r_s=-0.299, P=0.035; r_s=-0.342, P=0.015). Urinary C5a/urinary creatinine was positively correlated with serum creatinine and 24 h urine protein (r_s=0.525, P<0.001; r_s=0.496, P<0.001). (3) Immunohistochemical results showed that there were C5b-9 deposited in the arterioles and glomerular capillary wall in malignant nephrosclerosis patients, and no deposition in control renal tissues. Meanwhile, the semi-quantitative scores showed that C5b-9 deposition intensity was positively correlated with serum creatinine and 24 h urine protein (r_s=0.791, P<0.001; r_s=0.345, P=0.014). The double immunofluorescence labeling analysis showed that the C5b-9 and CD34 deposited in the arteriolar endothelium and glomerular capillary endothelium. (4) Plasma level of factor B in malignant nephrosclerosis patients was positively correlated with plasma C3a level (r=0.331, P=0.022). Plasma level of factor P was positively correlated with C5b-9 score (r_s=0.300, P=0.034). Urinary B was positively correlated with urinary C3a, C5a and C5b-9 score (r_s=0.311, P=0.028; r_s=0.465, P=0.001; r_s=0.428, P=0.002). Urinary factor P was also positively correlated with urinary C3a and C5a (r_s=0.307, P=0.030; r_s=0.442, P=0.001). Immunohistochemical result showed that there were C4d deposited in the arterioles and glomerular, and no deposition of MBL. Conclusion Complement activation via the alternative pathway may be involved in malignant nephrosclerosis and related to the severity of the disease.

Objective To study the role of C3a and C5a in focal segmental glomerulosclerosis (FSGS) patients. Methods (1) A total of 66 patients with FSGS confirmed by renal biopsy were selected, including 18 cases of tip lesion, 11 cases of perihilar, 22 cases of not otherwise specified (NOS), 10 cases of cellular, and 5 cases of collapsing FSGS. The normal renal tissue resected from patients with kidney tumor was taken as a negative control. The expression of C3a and C5a in renal tissues was detected by immunohistochemistry. (2) Serum and urine samples from these 66 FSGS patients were collected, and serum and urine samples from 10 healthy adult selected from the same physical examination center in the same term were used as normal controls. The levels of C3a and C5a in serum and urine were detected by enzyme - linked immunosorbent assay (ELISA). Results (1) Immunohistochemical results showed that C3a and C5a were deposited in glomerulus of FSGS patients, and no deposition in normal renal tissues. The semi - quantitative score showed that kidney C3a score was significantly correlated with serum creatinine (r=0.547, P＜0.001) and 24 h urine protein (r=0.329, P=0.007) in FSGS patients, and kidney C5a score was also significantly correlated with serum creatinine (r=0.415, P＜0.001) and 24 h urine protein (r=0.414, P＜0.001) in FSGS patients. (2) The levels of serum C3a and C5a in FSGS patients were higher than those in healthy adults (both P＜0.05), but there was no significant difference among the five pathological types (P＞0.05). The levels of urinary C3a/urinary creatinine, urinary C5a/urinary creatinine were higher in FSGS patients than those in healthy adults (all P＜0.05). The levels of urine C3a/urinary creatinine and urinary C5a/urinary creatinine in collapsing FSGS were higher than other FSGS types (all P＜0.01), but there was no significant difference among the tip lesion, the perihilar, the not otherwise specified and the cellular (P＞0.05). (3) Urinary C3a/urinary creatinine levels were significantly correlated with serum creatinine (r=0.774, P＜0.001) and 24 h urine protein (r=0.430, P＜0.001) in FSGS patients, and urinary C5a/urinary creatinine levels were also significantly correlated with serum creatinine (r=0.677, P＜0.001) and 24 h urine protein (r=0.333, P=0.007) in FSGS patients. Conclusion Complement C3a and C5a may be involved in the pathogenesis of FSGS and may be related to the severity of FSGS.

Objective To investigate the survival status of people with disabilities in urban areas of Lanzhou, China and discuss the correlation of their quality of life to disability attitude and quality of care and support of disabled people. Methods From August to November, 2016, 606 persons with disability registered in Chengguan District of Lanzhou were selected by multistage stratified cluster sampling. They were investigated in home-based visit with World Health Organization Quality of Life-Disability Scale for physical disability, World Health Organization-Disability Attitude Scale and World Health Organization Quality of Care and Support Scale-Disability Scale. Results The quality of life scored in average of (40.76±14.79), and different with the demographic characteristics (P<0.05). Pearson's correlation analysis showed that the score in all fields of quality of life and the total score positively correlated with most dimensions of disability attitude score (r>0.080, P<0.05) and most dimensions score of quality of care and support (r>0.083, P<0.05).Conclusion It is important to strengthen the precise security system for people with disabilities, and strive to establish a full-cycle, all-round social support system for them.

Obejective To explore the diagnostic and curative evaluation value of gastrin-releasing pep-tide precursor (ProGRP) and neuron specific enolization enzyme (NSE) in small cell lung cancer (SCLC). Methods Sixty SCLC patients, sixty non-small cell lung cancer (NSCLC) patients and forty patients with be-nign pulmonary disease were collected fromJanuary 2014 to October 2015. The levels of serum ProGRP and NSE in all patients were determined by ELISA method and radioimmunoassay respectively then the clinical value of ProGRP and NSE on SCLC was evaluated. Results The levels of ProGRP and NSE in SCLC group were signif-icantly higher than those in NSCLC group and those in lung benign disease group (P < 0.05). The levels of Pro-GRP and NSE in extensive stage were higher than those in limited stage in SCLC group (P < 0.05). The bound-ary value of SCLC through ProGRP identified through ROC curve was 64.68 pg/mL. The diagnostic sensitivity , specific degree and Youdenindex of ProGRP in SCLC were 86.7%, 97.5% and 0.842 respectively, which were significantly higher than NSE (P < 0.05). After 2 cycles of chemotherapy, serum ProGRP in SCLC disease con-trol groupwere significantly decreased(P < 0.05) but on difference of serum ProGRP was found in SCLC progres-siongroup (P > 0.05). Conclusion ProGRP and NSE can be used as markers for the diagnostic and curative evaluation of SCLC. And ProGRP has higher sensitivity and specificity than NSE and can be promoted in clinic.

Objective To analyze the distribution of various genotypes of human cytomegalovirus glycoprotein N ( HCMV gN) in patients with HIV infection; to investigate the effects of HCMV-HIV co-in-fection on disease progression and the relationships between HCMV gN genotypes and disease progression. Methods Patients with active HCMV infection were screened out from 359 patients with HIV infection by using the pp65 antigenemia assay.The genes encoding HCMV gN ( UL73 ) were amplified by nested PCR ( nPCR) .The amplicons were digested by restriction enzymes including MboⅠ, ScaⅠ and SalⅠ.Then, the restricted fragment length polymorphisms were further analyzed on 4%agarose gel.The relationships be-tween HCMV genotypes and the morbidity and mortality of acquired immune deficiency syndrome ( AIDS ) were investigated via a prospective study.Results Among the 359 patients with HIV infection, 28 subjects were positive for the HCMV pp65 antigenemia assay.The HCMV gN genotypes in 20 patients with active HCMV infection were distributed as: gN-3a (4/20, 20%), gN-1 (4/20, 20%), gN-4d (1/20, 5%), gN-4b (1/20, 5%) and mixed infection (10/20, 50%).Patients with HCMV-HIV co-infection were more likely to develop AIDS during the follow-up period (RR=9.78).Patients harboring HCMV gN-1 and gN-4 genotypes would seem likely to have 4.6 times of chance leading to AIDS-associated death than those harbo-ring other HCMV gN genotypes.Conclusion HCMV infection ( especially gN-1 and gN-4 genotypes) might accelerate the progression of HIV infection.

Objective:To investigate the role of C3a,C5a and their receptors in the pathogenesis of IgA nephropathy (IgAN). Methods:A total of twenty-eight 6-8 weeks old female BALB/c mice were investigated.And they were negative control group , WT group,C3aR-/-group,C5aR-/-group(the latter three groups were named as experimental groups ),seven mice in each group.All the mice were infected through respiratory tract with infectious SV (experimental groups) or PBS(negative control group),combined with tail vein challenge to make IgAN animal model.Testing 24 h total urinary protein , serum urea nitrogen ( BUN ) and creatinine ( Cr ) , using direct immunofluorescence to test the renal deposition of IgA and C 3,observing renal pathologic lesion under PAS staining with light microscopy.RT-qPCR was used to test the relative mRNA expression of TNF-α,TGF-β,IL-1β,IL-6,MCP-1.Results: After 15 weeks,the level of UTP in experimental group was significantly higher than negative control group ,and the same results as WT group than C3aR-/-group and C5aR-/-group.There was no significant difference among groups for BUN and Cr.Combined with negative control group , experimental groups had significant renal pathological lesions , and the changes of WT group was more severe than C3aR-/-group and C5aR-/-group.The results of relative mRNA expression of TNF-α,TGF-β,IL-1β,IL-6,MCP-1 was the same as the level of 24 h UTP,at the same time,the relative mRNA expression of IL-1β,IL-6,MCP-1 in C3aR-/-group was significantly less than C5aR-/-group.Conclusion:The deficiency of C3a/C5a receptors can protect kidney from injury in IgAN ,and C3a receptor has more significant role in protect kidney from injury in IgAN.

BACKGROUNDMycophenolate mofetil (MMF) and cyclophosphamide (CTX) are widely used in treating various kidney diseases. However, whether they are effective and which one is better for treating IgA nephropathy patients with proliferative pathological phenotype in renal diseases, such as endocapillary proliferation, cellular crescents, and/or capillary loops fibrinoid necrosis is still unknown. We, therefore, initiated a study to compare the effects of MMF and CTX in treating IgA nephropathy with the above pathological lesions.

METHODSOne hundred and nineteen patients with IgA nephropathy who had at least one of the three aforementioned lesions were enrolled. All patients were treated with prednisone; 48 patients received prednisone only (Pred group), 40 received MMF and prednisone (MMF + Pred group), and 31 were treated with CTX and prednisone (CTX + Pred group). The median time of follow-up was 30 months (maximum: 96 months). The primary endpoint was defined as renal survival. The incidence of remission of proteinuria was the secondary endpoint.

RESULTSSerum creatinine in all groups declined significantly at different follow-up times (P = 0.002), and the differences among the three groups were significant (P < 0.001). At 24 months of follow-up, the decline rates were 12.35%, 32.95%, and 24.14% in the Pred, MMF + Pred, and CTX + Pred groups respectively. For urine protein excretion, the decline rates were 49.12% (Pred), 73.67% (MMF + Pred), and 63.53% (CTX + Pred) respectively at 24 months of follow-up. The differences among the three groups were not significant (P = 0.714). Renal survival (the primary endpoint) was significantly different (P = 0.027); however, the sencondary endpoint was similar for all the three groups (P = 0.100).

CONCLUSIONSFor IgA nephropathy patients with endocapillary proliferation, cellular crescents, and/or fibrinoid necrosis of capillary loops, prednisone combined with MMF was more effective in lowering the serum creatinine than with CTX. Combined MMF and prednisone treatment led to a better renal survival compared to that of prednisone with CTX.

Adult ; Female ; Glomerulonephritis, IGA ; drug therapy ; pathology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Male ; Mycophenolic Acid ; analogs & derivatives ; therapeutic use ; Retrospective Studies ; Young Adult

Objective To develop a RP-HPLC method for the determination of Brucine and Strychnine in Semen Strychni and its extractive of total alkaloids. Methods A chromatographic column of Licrospher C18 (4.6 mm×250 mm, 5 μm) was used with the mobile phase of acetonitrile∶0.01 mol/L sodium heptane sulfonate and 0.02 mol/L potassium dihydrogen phosphate mixed with equal amount (adjusted pH to 2.8 with 10% phosphonic acid)=27∶73, detection wavelength at 260 nm, column temperature of 30 ℃ and flow rate of 1 mL/min. Results The calibration curves of Brucine and Strychnine were both in good linearity in the ranges of 0.1-1.0 μg and 0.12-1.2 μg (r=1.000) respectively. The average recovery rates of Brucine and Strychnine were 99.88% (RSD=1.06%) and 100.06% (RSD=0.78%) respectively. Conclusion The method is realiable and accurate, which can be applied to determination of Brucine and Strychnine in Semen Strychi and its extractive.

Objective To explore the clinicopathological characteristics of lupus nephritis (LN) with antinucleosome antibody (AnuA).Methods Data of 481 patients with biopsy-proven LN in the First Affiliated Hospital of Zhengzhou University from 2004 to 2011 were analyzed retrospectively.The patients were divided into two groups:AnuA-positive group (76 patients) and AnuA-negative group (405 patients).The clinical manifestations,laboratory examinations,histopathologic classes of LN,disease activity measured by SLE disease activity index (SLEDAI) of two groups were investigated and compared.Results There were 15 male patients in positive group (15/76,19.74％) with mean age of (27.99±10.88) years and 45 patients in negative group (45/405,11.11％) with mean age of (31.15±12.15) years respectively,which showed that male patients were more common in positive group (P＜0.05).Incidences of oral ulcer,fever,anemia,low complement and positive anti-dsDNA antibody were higher in positive group (P＜0.05).Percentage of diffuse proliferative lupus nephritis (class Ⅳ ) and pathological activity index (AI) in positive group were higher compared to negative group (all P＜0.05),while no significant differences of other pathological types,chronic index (CI) and SLEDAI were found between two groups.Conclusion LN patients with positive AnuA have special clinicopathological characteristics and AnuA may be used as a promising biomarker for the proliferative LN.