Primary membranous nephropathy in two siblings with one combined with anti-glomerular basement membrane disease: a case report.

BACKGROUND: The phospholipase A2 receptor (PLA2R) associated with membranous nephropathy (MN) is an organ-specific autoimmune disease associated with PLA2R and human leukocyte antigen (HLA) genes. Familial PLA2R-related MN is rarely reported. The combination of anti-GBM disease and MN has been well documented, though the mechanism behind it remains unclear. CASE PRESENTATION: We describe two siblings diagnosed with pathology-confirmed PLA2R-related MN 1 year apart. And one of the two siblings developed an anti-GBM disease. The high-resolution HLA typing showed identical alleles in both siblings, specifically heterozygotes of DRB1*15:01/*03:01. CONCLUSION: We describe a familial case of PLA2R-related MN supporting the role of genetic factors that HLA-DRB1*15:01 and DRB1*03:01 predispose patients in the development of PLA2R-related MN in the Han Chinese population. The combination of MN and anti-GBM disease may also partially be associated with the same susceptible HLA allele DRB1*15:01.

Identifying the pulsed neuron networks' structures by a nonlinear Granger causality method.

BACKGROUND: It is a crucial task of brain science researches to explore functional connective maps of Biological Neural Networks (BNN). The maps help to deeply study the dominant relationship between the structures of the BNNs and their network functions. RESULTS: In this study, the ideas of linear Granger causality modeling and causality identification are extended to those of nonlinear Granger causality modeling and network structure identification. We employed Radial Basis Functions to fit the nonlinear multivariate dynamical responses of BNNs with neuronal pulse firing. By introducing the contributions from presynaptic neurons and detecting whether the predictions for postsynaptic neurons' pulse firing signals are improved or not, we can reveal the information flows distribution of BNNs. Thus, the functional connections from presynaptic neurons can be identified from the obtained network information flows. To verify the effectiveness of the proposed method, the Nonlinear Granger Causality Identification Method (NGCIM) is applied to the network structure discovery processes of Spiking Neural Networks (SNN). SNN is a simulation model based on an Integrate-and-Fire mechanism. By network simulations, the multi-channel neuronal pulse sequence data of the SNNs can be used to reversely identify the synaptic connections and strengths of the SNNs. CONCLUSIONS: The identification results show: for 2-6 nodes small-scale neural networks, 20 nodes medium-scale neural networks, and 100 nodes large-scale neural networks, the identification accuracy of NGCIM with the Gaussian kernel function was 100%, 99.64%, 98.64%, 98.37%, 98.31%, 84.87% and 80.56%, respectively. The identification accuracies were significantly higher than those of a traditional Linear Granger Causality Identification Method with the same network sizes. Thus, with an accumulation of the data obtained by the existing measurement methods, such as Electroencephalography, functional Magnetic Resonance Imaging, and Multi-Electrode Array, the NGCIM can be a promising network modeling method to infer the functional connective maps of BNNs.

[Evaluation of the color stability of infiltrant resin in comparison to aesthetic composite resins].

OBJECTIVE: To investigate the color stability of infiltrant resin together with three other composites exposed to staining solution. METHODS: Three types of anterior aesthetic composite resins (Filtek Z250, Filtek Z350 XT, and Beautiful Flow Plus F00) and artificial caries with Icon infiltrant resin were used. The samples were randomly divided into two groups (each contained 10 specimens): coffee solution and distilled water immersion for three weeks. Color parameters were deter-mined with the Crystaleye spectrophotometer. RESULTS: Artificial caries lesions infiltrated by Icon showed much higher ΔE than those infiltrated by other aesthetic resins (P<0.05). The discoloration of all the experimental resins and the artificial caries infil-trated with Icon increased with time, but the discoloration on the 1st week was significantly higher than those on the 2nd and 3rd weeks. CONCLUSIONS: Artificial caries infiltrated with Icon was more vulnerable and susceptive to food color than those infiltrated with other aesthetic composite resins.

Radiographic Measurement of Femoral Lateral Bowing and Distal Femoral Condyle Resection Thickness: Variances and Effects on Total Knee Arthroplasty Planning.

BACKGROUND: Accurate evaluation of the plain radiography of lower limb is critical for preoperative planning of total knee arthroplasty (TKA). We aimed to investigate the effect of femoral lateral bowing and rotation on the radiographic measurements of distal femoral condyle resection thickness (DRT) and the distal femoral resection valgus angle (FVA). METHODS: We analyzed 246 three-dimensional femoral models generated from computed tomography images of 123 patients, acquiring projected contours in seven positions - 20° and 10° internal rotation; 0° rotation; 10°, 20°, 30°, and 40° external rotation - for each model. Medial and lateral condyle DRTs, femoral shaft lateral bowing angle (FBA), and distal FVA were determined for each position. Linear mixed effect model was used to determine the effect of degree of femur rotation on repeated measurements of DRT or FVA. RESULTS: FBA significantly affected the FVA and DRT (Pearson's R = 0.767 and -0.408, respectively; P < 0.000). Samples were divided into three groups according to the FBA measured in neutral position: FBA <0°: DRT 3.75 ± 1.30 mm, FVA 4.53° ± 1.27°; FBA >0° but <3°: DRT 3.39 ± 1.31 mm, FVA 5.92° ± 1.31°; FBA >3°: DRT 2.22 ± 1.31 mm, FVA 7.37° ± 1.31°. From simulated 20° internal rotation to 40° external rotation in each femoral model, the average variation ranges of radiographically measured DRT, FVA, and FBA were 0.50 ± 0.28 mm, 2.93° ± 0.96°, and 10.33° ± 1.90°, respectively, with no significant differences among the FBA groups. The degree of femoral rotation significantly affected the FVA (F = 62.148, P < 0.000), whereas there was no effect on condyle resection thickness (F = 0.4705, P = 0.494). CONCLUSIONS: Axial femoral rotation has less effect on radiographic measurements of differences in the DRT than on those of the distal FVA.

Restrictive Cardiomyopathy Resulting from a Troponin I Type 3 Mutation in a Chinese Family.

Objective To identify the pathogenic variant responsible for restrictive cardiomyopathy (RCM) in a Chinese family.Methods Next generation sequencing was used for detecting the mutation and Results verified by sequencing. We used restriction enzyme digestion to test the mutation in the family members and 200 unrelated normal subjects without any cardiac inherited diseases when the mutation was identified.Results Five individuals died from cardiac diseases, two of whom suffered from sudden cardiac death. Two individuals have suffered from chronic cardiac disorders. Mutation analysis revealed a novel missense mutation in exon 7 of troponin I type 3 (TNNI3), resulting in substitution of serine (S) with proline (P) at amino acid position 150, which cosegregated with the disease in the family, which is predicted to be probably damaging using PolyPhen-2. The mutation was not detected in the 200 unrelated subjects we tested.Conclusion Using next generation sequencing, which has very recently been shown to be successful in identifying novel causative mutations of rare Mendelian disorders, we found a novel mutation of TNNI3 in a Chinese family with RCM.

Expression patterns of transcription factor PPARγ and C/EBP family members during in vitro adipogenesis of human bone marrow mesenchymal stem cells.

In the past decades increasing lines of evidence have demonstrated that adipose tissue, as an endocrine organ plays a central role in metabolic homeostasis and its related maladies. CCAAT/enhancer-binding protein (C/EBP) family members and the nuclear receptor peroxisome proliferator-activated receptor gamma (PPARγ) were known to be the vital transcription factors in the regulation of adipogenesis. However, the exact mechanism for increased marrow fat in patients with bone metabolic diseases, such as osteoporosis, is still poorly understood. Herein, we studied the expression pattern of PPARγ and C/EBPs in human bone marrow mesenchymal stem cell (hBMSC) adipogenesis and evaluated the effects of individual components of an adipogenic cocktail on the differentiation and transcription factor expression. We furthermore examined whether the ERK signaling pathway was involved in mediating these effects. These findings showed that C/EBPß and C/EBPδ were detected in undifferentiated hBMSC and maintained during the whole process of adipogenesis, and could initiate the expression of PPARγ1 under the treatment of dexamethasone and IBMX. Subsequently, the activation of PPARγ1 by indomethacin, its exogenous ligand, activated C/EBPα, which, together with IBMX, up-regulated PPARγ2 expression and therefore the fullest adipogenesis. Insulin and its downstream signal pathway extracellular signal-regulated kinases (ERK), however, were found not necessary for hBMSC adipogenesis. Our results revealed some unique characteristics of human adipocyte formation, which may help to understand the molecular mechanisms of bone marrow adipogenesis and give insights into the treatment of osteoporosis.

[Surface roughness and staining stability of infiltrant resin for enamel white spot lesion].

OBJECTIVE: To investigate the influence factors on the surface roughness and staining susceptibility of infiltrant resin. METHODS: In the study, 30 human third molars were used, and each sample had three open enamel windows. The samples were randomly divided into three groups according to their different demineralized time. Each sample had at least one intact spot (A), one infiltrant resin spot (B) and one artificial white spot lesion (C). The surface roughness was tested before color staining for all the three spots of each specimen. The specimens were stored in a staining solution (coffee) for a period of 21 days, before and after which the color Commission Internationaled' Eclairage (CIE)L*a*b* was recorded for A, B and C spots. RESULTS: The B spot's surface roughness of each group was(0.15 ± 0.02)µm,(0.31 ± 0.03)µm and(0.40 ± 0.02)µm, respectively. And the C spot's surface roughness each was (1.08 ± 0.10)µm,(2.89 ± 0.13)µm and(3.41 ± 0.14)µm. The surface roughness of B and C of the three groups increased with demineralization time longer, and had significant difference for both B and C (P < 0.01). The ΔE of the three groups between A and B before staining had significant difference (P < 0.01). And the ΔE of group1 was less than 3.7, but the other two groups' more than 3.7. After staining, the ΔE of groups 1 and 2 was less than 3.7 but that of group 3 was more than 3.7. There were significant differences between groups 1 and 3, and also between groups 2 and 3(P < 0.01). CONCLUSION: The degree of the lesion's demineralization has effect on the surface roughness and color susceptibility of infiltrant resin. The increased surface roughness of infiltrant resin has positive effect on masking enamel white spots.

[Prevalence and risk factors of atherosclerotic renal artery stenosis].

OBJECTIVE: To explore the prevalence and risk factors of atherosclerotic renal artery stenosis (ARAS) in patients undergoing coronary angiography. METHODS: A total of 2506 patients with suspected and known coronary heart disease (CAD) at our hospital underwent simultaneous coronary and renal angiography. Renal artery stenosis was defined as at least one of renal artery stenosis ≥ 50% narrowing of luminal diameter. The prevalence of ARAS was summarized from the results of angiography. And single- and multi-variable logistic regression analysis was used to assess the relationship between clinical characteristics and ARAS. RESULTS: Among them, there were 1479 males and 1027 females with a mean age of 62.7 ± 11.4 years. ARAS was detected in 409 patients (16.3%), including 214 (8.5%) with significant stenosis (≥ 75%); Bilateral ARAS was detected in 98 patients (3.9%), including 27 (1.1%) with significant stenosis (≥ 75%). Left and right ARAS were detected in 162 patients (6.5%) and 148 patients (5.9%) respectively, including 84 patients (3.4%) with significant stenosis (≥ 75%) in left ARAS and 72 patients (2.9%) in right ARAS. Univariate analysis indicated that age, females, diabetes, hypertension, stroke, peripheral artery disease, coronary heart disease, renal insufficiency, dyslipidemia and hyperuricemia were the predictor for ARAS (P < 0.05 or 0.01). Multivariate regression analysis demonstrated that age ≥ 65 years (P = 0.025, OR = 1.358), females (P < 0.01, OR = 1.678), hypertension (P < 0.01, OR = 1.650), peripheral artery disease (P < 0.01, OR = 14.678), renal insufficiency (P < 0.01, OR = 1.835), coronary heart disease including 3-vessel (P < 0.01, OR = 1.746) and left main coronary (P < 0.01, OR = 3.416)disease were independent risk factors for ARAS. CONCLUSION: Renal angiography should be routinely performed in female patients aged ≥ 65 years with hypertension, peripheral artery disease, elevated creatinine and coronary heart disease, especially for 3-vessel and left main coronary disease to identify ARAS in time.

Total hip arthroplasty for vascular necrosis of the femoral head in patients with systemic lupus erythematosus: a midterm follow-up study of 28 hips in 24 patients.

OBJECTIVE: Avascular necrosis of the femoral head is one of the most frequently reported complications in patients with systemic lupus erythematosus (SLE) and often requires total hip arthroplasty (THA). Our objective was to analyze the perioperative management, technical problems, clinical outcomes, and complications associated with THA in patients with SLE. METHODS: A total of 28 total hip arthroplasties performed for 24 patients with SLE, including 19 women and 5 men with a mean age of 38.8 years performed from 1998 to 2011 were retrospectively reviewed. SLE disease activity index and ASA class were evaluated preoperatively. WOMAC, HHS, and SF-36 scores were also evaluated in all cases pre- and post-operatively for functional recovery of the hip and health-related quality of life (HRQOL). RESULTS: The average SLE disease activity index was 3.5 points. Three patients were in ASA class I, 12 class II, and 9 were class III (37.5%). The average duration of follow-up was 67.5 months. None of the patients required a revision, and 3 patients died during the follow-up period. A statistically significant improvement in all scores was found comparing pre- and post-operative conditions (P < 0.001). The complication rate was 11.1% with 2 wound infections and 1 urinary tract infection. CONCLUSION: THA is an acceptable method for achieving functional recovery and increasing HRQOL in patients with SLE and ANFH who receive proper perioperative management.

The influence of body mass index on life quality and clinical improvement after total hip arthroplasty.

BACKGROUND: The effect of body mass index (BMI) on the outcome of total hip arthroplasty (THA) remains controversial. The purpose of this study was to examine whether revision rate and postoperative outcomes following THA were influenced by BMI. MATERIALS AND METHODS: We retrospectively evaluated 714 patients (751 hips) who underwent primary THA in our department between March 1991 and April 2006. They were followed prospectively for 5-20 years with 24 deaths (24 hips) and 33 losses (34 hips). Patients were separated into three groups based on BMI: underweight, normal and obese groups. A survival analysis was performed using revision as the endpoint, and a case-matched study that was matched for age, gender, and laterality was designed; outcomes were assessed with the Harris Hip score, 36-item short-form health survey, complication rate and radiological examination. RESULTS: The preoperative scores were lower for the obese group, and the postoperative scores were higher for the normal group. Patients in the obese group obtained the greatest overall improvement in clinical scores from admission to the last follow-up. We found a significantly higher complication rate in the obese group and underweight group. It appears that being underweight was associated with an increased dislocation rate, and obese patients were more likely to have osteolysis, deep vein embolism, and pulmonary thrombosis. The log rank test for survival showed no significant differences among the three groups. CONCLUSIONS: Abnormal BMI does not prevent functional rehabilitation after THA; however, patients with abnormal BMI have to face higher complication rates and poorer clinical outcomes following this operation.

[Risk factors and coronary angiography characteristics of female patients with acute coronary syndrome].

OBJECTIVE: To assess the risk factors and coronary angiography characteristics of female patients with acute coronary syndrome (ACS). METHODS: A total of consecutive 986 inpatients with ACS who had undergone coronary angiography from March 2009 to August 2010 in our hospital were enrolled in this study. There were 303 female patients and 683 male patients. Clinical data were collected by physicians and the severity of coronary artery stenosis was analyzed via the international Gensini Score system. RESULTS: Incidence of ACS under the age of 60 years [8.6% (26/303) vs. 16.5% (113/683), P < 0.05], family history of coronary artery disease [15.8% (48/303) vs. 23.0% (157/683), P < 0.05], and smokers [19.1% (58/303) vs. 71.7% (490/683), P < 0.001] were significantly less while hypertension [81.5% (247/303) vs. 64.0% (437/536), P < 0.001] and diabetes rate [51.8% (157/303) vs. 44.0% (298/683), P < 0.05] were significantly higher in female patients than in male patients. The comorbidities of dyslipidemia, adiposity, hyper-C-reaction protein and hyperfibrinogenemia were similar between male and female patients (P > 0.05). Unstable angina and non-ST-segment elevation myocardial infarction were more often [86.1% (261/303) vs. 78.5% (536/683)], while ST-segment elevation myocardial infarction was less [13.9% (42/303) vs. 21.5% (147/683), P = 0.005] in female patients than in male patients. There were significantly more incidence of mild coronary artery stenosis [15.0% (47/303) vs. 10.0% (68/683), P = 0.012] and less severely stenotic lesions [84.2% (255/303) vs. 89.8% (613/683), P = 0.013] in female patients than in male patients. Gensini score, percutaneous intervention rate and in-hospital mortality rate were similar between male and female patients with ACS (P > 0.05). CONCLUSIONS: Prevalence rates of diabetes mellitus and hypertension are higher while positive family history on coronary artery disease and smoking rate are lower in female patients with ACS than in male ACS patients. Female ACS patients are often presented with unstable angina or non-ST-segment elevation myocardial infarction and with mild coronary artery stenosis compared to male ACS patients.

[Root resection by Er:YAG laser: a scanning electron microscope study].

OBJECTIVE: To compare the difference of the surface of root resection by Er:YAG laser, ultrasonic or high-speed handpiece with scanning electron microscope (SEM), and to evaluate the possibility of using Er:YAG laser on the root resection in clinical application. METHODS: Thirty maxillary central incisors were divided into three groups (Er:YAG laser group, ultrasonic group, high-speed handpiece group), and the root resection were made at root tip 3 mm with Er:YAG laser, ultrasonic instrument or long needle diamond bur according to grouping. The surface of the root resection by SEM in the aspects of debris, smear layer, opened dentinal tubules, cracks and ablation characteristics were compared. RESULTS: The examination revealed that Er:YAG laser group and ultrasonic group had no or little debris and smear layer and with opened dentinal tubules. High-speed handpiece group had great amount of debris and smear layer and without opened dentinal tubules. Cracks were observed in ultrasonic group and high-speed handpiece group, no in Er:YAG laser group. There were ablation characteristics in ultrasonic group and high-speed handpiece group, but no in Er:YAG laser group. CONCLUSION: From the morphological aspect, Er:YAG laser has much more advantage than ultrasonic instrument and diamond bur for the root resection.

[Comparison of clinical effect of different tapered gutta-percha root filling with warm vertical condensation].

OBJECTIVE: To compare the clinical effect of different tapered gutta-percha root filling by warm vertical condensation with immediate postoperative radiographs and one year follow-up. METHODS: 40 maxillary anterior teeth with single, straight canals were divided into two equal groups. The teeth were instrumented with Hero 642 rotary nickel-titanium files to a master file 0.06 taper No. 30. Group 1 was obturated with 0.02 tapered gutta-percha using System B for downpack and Obtura II for backfilling. Group 2 was the same but 0.06 tapered gutta-percha. Every tooth's X-ray radiographs of immediate postoperative and one year follow-up were taken. In order to compare the quality of root canal filling, the rate of filling material extrusion, and the rate of obturation of lateral canals in each group were evaluated by X-ray radiographs. The clinical effect of one year follow-up's radiographs of the two groups was compared too. RESULTS: There was no significant difference in two groups on the quality of the root canal filling, obturation of lateral canals, and filling material extrusion. The success of the therapy was similar. But the 0.06 tapered gutta-percha group showed more quickly healing trend on apical periodontitis. CONCLUSION: When single, straight root canals were obturated using warm vertical condensation, adaptively tapered gutta-percha showed better clinical effect.

[Quality of apical seal of differently tapered gutta-percha cone using warm vertical condensation technique].

OBJECTIVE: The purpose of this study was to compare the quality of apical seal of the canals that obturated with differently tapered gutta-percha cone using continuous wave technique. METHODS: 62 extracted human mandible incisors were prepared with Gates-Glidden drill and Hero642 to a final file of No. 30 and 0.06 taper. The teeth were randomly separated into 0.02 taper group (30 teeth), 0.06 taper group (30 teeth) and positive control group (2 teeth). The teeth of 0.02 taper group and 0.06 taper group were respectively obturated with a 0.02, or 0.06 tapered gutta-percha cone and Cortisomol sealer using warm vertical condensation technique separately. The teeth of positive control group were not obturated. In 0.02 taper group and 0.06 taper group, 10 teeth were placed in India ink for 24 hours, 10 teeth were placed in India ink for 10 days, 10 teeth were placed in India ink for 90 hours after 67 days storage in Hank's balanced salt solution. The teeth of positive control group were placed in India ink for 24 hours. The apical leakage was evaluated by the linear measurement under the stereomicroscope. RESULTS: The dye penetration of positive control group was along the whole canals. The apical leakage of 0.02 taper group increased along with time, while no difference was found among 0.06 taper group. There was a significant difference in the degree of leakage between 0.02 taper group and 0.06 taper group in 67 days (P = 0.041), but not in 24 hours and 10 days groups (P = 0.601, P = 0.471). CONCLUSION: Better apical seal was obtained when using the same tapered gutta-percha cone with root canal.

[Expressions of PDCD5 and p53 in oral leukoplakia and oral squamous cell carcinoma].

OBJECTIVE: To study the relationship of the expressions of new apoptosis-related gene PDCD5 and p53 in oral normal mucosa, oral leukoplakia and oral squamous cell carcinoma. METHODS: The expressions of PDCD5 and p53 were observed separately in 17 samples of oral normal mucosa, 60 of oral leukoplakia, and 30 of oral squamous cell carcinoma by Immunohistochemical means. RESULTS: PDCD5 positive rate in oral normal mucosa was 88.2%, in oral leukoplakia was 63.3%, and in oral squamous cell carcinoma was 30%. P53 positive rate in oral normal mucosa was 0, in oral leukoplakia was 31.7%, and in oral squamous cell carcinoma was 60%. There was a negative relationship between PDCD5 SII and P53 SII in every lesion. CONCLUSION: It suggests that both PDCD5 and p53 could be used as molecular markers of carcinogenesis for oral epithelium.