Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Humans ; Kidney Diseases ; therapy ; Nephrotic Syndrome ; therapy ; Rituximab

OBJECTIVETo analyze the clinical features and gene mutations of 6 Chinese children with Dent's disease.

METHODThe clinical and laboratory data of 6 children with Dent's disease were summarized. CLCN5 gene was analyzed using PCR amplification and DNA sequencing.

RESULTAll the six patients presented with low molecular weight proteinuria and hypercalciuria, including 3/6 hematuria, 4/6 nephrocalcinosis, 3/6 hypophosphatemia, 1/6 rickets. Six mutations of the CLCN5 gene were revealed, including L594fsX595, R637X, R467X, IVS4-2A > G, S244L and V505G. The mutation L594fsX595, IVS4-2A > G and V505G was never reported before.

CONCLUSIONLow molecular weight proteinuria and hypercalciuria were the main clinical features of the six Chinese boys with Dent's disease. Dent's disease could be associated with a Bartter-like syndrome, which make the gene diagnosis more important.

Adolescent ; Child ; Child, Preschool ; Chloride Channels ; genetics ; Dent Disease ; complications ; diagnosis ; genetics ; Humans ; Hypercalciuria ; diagnosis ; genetics ; Male ; Mutation ; Pedigree ; Proteinuria ; diagnosis ; etiology ; genetics

Objective To observe the therapeutic effects of low frequency ultrasound enhanced thrombolysis (LFUET) on acute cerebral infarction (ACI) in rats.Methods The ACI animal models were established by injec- ting auto-thrombus into the rats' left middle cerebral arteries.They were then treated with urokinase,and received transcranial LFUET treatment at the same time.Nervous system functioning was assessed using NSS,and infarct vol- umes (IVs) were measured through tetrazolium chloride (TTC) staining.Results The NSS scores in the large- dose urokinase group (LDU group),the ultrasound plus small-dose urokinase group (USMU group) and in the in- farct group (Ⅰgroup) at 24 h after treatment were significantly lower than those before treatment.IVs in the two treat- ment groups are lower than those in theⅠgroup,but there was no significant difference between the LDU group and USMU group volumes.Conclusion LFUET can accelerate the recovery of nervous system function in rats after ACI,minimize IVs,and reduce the required dosage of urokinase.

The distribution information of Lomatogonium rotatum. was collected by interview investigation and field survey, and 55 related environmental factors were collected, the habitat suitability study was conducted based on geographic information system (GIS) and maximum entropy model. The AUCs of ROC curve were both above 0.99, indicating that the predictive results with the maximum model were highly precise. The results showed that 13 major environmental factors have obvious influence on ecology suitability distributions of L. rotatum, including month average temperature of February et al., the suitable distribution areas are mainly concentrated in the east-central of Inner Mongolia, including Hexigten banner, Duolun county, Zhenglan banner et al., The zoning results basically coincide with the genuine producing areas, and further afford new suitable distribution areas, which can provide reference for L. rotatum's wild nursery and the siting of introduction and cultivation.
China ; Ecosystem ; Environment ; Gentianaceae ; growth & development ; Geographic Information Systems ; Rain ; Temperature

OBJECTIVETo investigate the deletion of p53 gene and amplification of HER-2 oncogene at chromosome 17 in primary hepatocellular carcinoma (HCC) and the clinical significance.

METHODSInterphase dual fluorescence in situ hybridization (FISH) was applied to detect the ratio of the number of p53 gene copy or HER-2 oncogene copy to that of chromosome 17 copy, to determine the p53 gene deletion and HER-2 oncogene amplification in nuclei prepared from 42 surgical specimens of HCC. Statistical analysis for their clinical significance was performed.

RESULTSLoss of p53 gene and amplification of HER-2 oncogene were detected in 27 (64.3%) and 9 (21.4%) of the 42 HCC respectively including 4 cases with low and 5 with high copy amplification. Six (14.3%) of 42 HCC showed simultaneously p53 gene deletion and HER-2 oncogene amplification. 61.9% (26/42) of HCC were polysomy 17, which correlated positively with p53 gene deletion (chi(2) = 12.286, P < 0.001). No close correlation between p53 gene loss and HER-2 oncogene amplification was found (chi(2) = 0.00, P = 1.00). Loss of p53 gene was related to the serum alpha-fetoprotein (AFP) level and the tumor size (P < 0.05). The postoperative 2-year survival rate (18.5%) of HCC patients with p53 gene deletion was significantly lower than postoperative 2-year survival rate (60.0%) of those without p53 gene loss (chi(2) = 7.467, P = 0.006). Meanwhile, HER-2 oncogene amplification showed a tendency of correlation with the tumor size (chi(2) = 2.973, P = 0.085), and the postoperative 2-year survival rate (0/9) of HCC patients with HER-2 oncogene amplification was significantly lower than those (42.4%) without HER-2 oncogene amplification (chi(2) = 3.977, P = 0.046).

CONCLUSIONThere were a high frequency of p53 gene deletion and a low frequency of HER-2 oncogene amplification in primary HCC, which might be involved in initiation and development of a subset of primary HCC.

Adult ; Carcinoma, Hepatocellular ; genetics ; pathology ; Chromosomes, Human, Pair 17 ; Female ; Gene Amplification ; Gene Deletion ; Genes, erbB-2 ; Genes, p53 ; Humans ; In Situ Hybridization, Fluorescence ; Liver Neoplasms ; genetics ; pathology ; Male ; Middle Aged ; Polyploidy ; Survival Rate ; alpha-Fetoproteins ; metabolism

OBJECTIVETo investigate the clinicopathological feature and treatment of idiopathic membranous nephropathy (IMN) in children.

METHODA retrospective analysis of 25 cases of biopsy-proven IMN seen between January 2004 and December 2009.

RESULTThe incidence of IMN was 3.81% in all the children patients who underwent renal biopsy. Of 25 patients with IMN, nine were boys and sixteen were girls. The mean age at onset was (9.4 ± 3.4) years with a range of 2 - 14 years. Renal biopsies were performed at a median 2.5 months (range 0.4 - 11 months) after onset. The clinical manifestations included nephrotic syndrome (NS) nephritic type in 21 cases (84%) and glomerulonephritis in 4 cases. All patients presented with hematuria, and 7 had macroscopic hematuria. Hypertension was noted in 4 patients. Two patients were complicated with thrombosis. One patient was in a chronic renal insufficiency(CRI)state. According to the MN staging criteria, 21 cases were in stage II IMN (84%). Six patients showed moderate or severe tubulointerstitial lesion. Focal segmental glomerulosclerosis (FSGS) was found in two patients. Of the 22 patients with NS and nephrotic proteinuria, 21 cases were treated with prednisone initially and in 20 of them the efficacy of corticosteroid therapy was evaluated:one of them was steroid sensitive (became steroid-resistant after relapse) and all the others were steroid-resistant (95%). The subsequent treatment: eight of them were treated with prednisone followed by a taper to alternate-day therapy. Five of them had complete remission and three partial remission. Twelve cases were treated with combined therapy of prednisone and immunosuppressive agents. Of these 12 cases together with one case who received initially combined treatment with prednisone and immunosuppressive agent and one case treated with prednisone initially for five weeks then with combined therapy contained another immunosuppressive agent, totally 14 cases, 5 had complete remission, 2 partial remission, 3 did not achieve remission, and 3 had unknown response.

CONCLUSIONOf the patient cohort, the predominant presenting feature was nephrotic syndrome, and with different degree hematuria. Almost all of them were steroid resistant, but followed by a taper to alternate-day therapy, some could achieve remission. The effect of a combination of prednisone and immunosuppressive agent is needed to be further proven in children.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerulonephritis, Membranous ; pathology ; therapy ; Humans ; Male ; Nephrotic Syndrome ; pathology ; therapy ; Retrospective Studies

Objective To Analyze the change trend of the incidence and mortality of breast cancer in Chinese women from 2005 to 2013 in the Chinese Cancer Registry Annual Report in order to provide references for the implement of the prophylaxis and treatment of breast cancer. Methods Extracted all the records of the incidence and mortality of breast cancer in Chinese women from 2005 to 2013, applied the Joinpoint regression model to analyze the change trend of the incidence and mortality of breast cancer in Chinese women. Results The incidence levels of breast cancer among urban women in China was higher than that in rural from 2005 to 2013, the change trend of urban incidence was stable(t=-0.2, P=0.828), the rural incidence showed an increasing trend(t=7.8, P<0.001). The peaks of urban and rural incidence were in the age group of 50- and the age group of 45- respectively.The mortality of breast cancer among urban women in China was higher than that in rural from 2005 to 2013, the change trend of urban mortality was stable(t=0.8, P=0.458), and the rising trend of rural mortality was obvious(t=3.3, P=0.014). The mortality of urban women began to rise after the age of 30, accelerating to rise after the age of 75, the mortality of rural women began to rise after the age of 30, the change tended to be stable at the age of 55-69, and began to rise after the age of 70 again. Conclusions The incidence and mortality of breast cancer among urban women in China were all higher than that in rural from 2005 to 2013, the rising trend of the incidence and mortality of breast cancer among rural women was obvious, so the prophylaxis and treatment measures of breast cancer should be actively formulated and perfected.

Background:The role of local treatment in oligometastatic prostate cancer (PCa) is gaining interest with the oligometastases hypothesis proposed and the improvement of various surgical methods and techniques.This study aimed to compare the short-term therapeutic outcomes of robotic-assisted laparoscopic radical prostatectomy (RALP) for oligometastatic prostate cancer (OPC) vs.localized PCa using propensity score matching.Methods:Totally 508 consecutive patients underwent RALP as a first-line treatment.The patients were divided into two groups according to oligometastatic state:the OPC group (n =41) or the localized PCa group (n =467).Oligometastatic disease was defined as the presence of two or fewer suspicious lesions.The association between the oligometastatic state and therapeutic outcomes of RALP was evaluated,including biochemical recurrence (BCR) and overall survival (OS).A Cox proportional hazards model was used to assess the possible risk factors for BCR.Results:Totally 41 pairs of patients were matched.The median operative time,the median blood loss,the overall positive surgical margin rate,the median post-operative hospital stays,and the post-operative urinary continence recovery rate between the two groups showed no statistical significance.The 4-year BCR survival rates of the OPC group and localized PCa group were 56.7％ and 60.8％,respectively,without a significant difference (P=0.804).The 5-year OS rates were 96.3％ and 100％,respectively (P =0.326).Additionally,the results of Cox regression showed that oligometastatic state was not an independent risk factor for BCR (P =0.682).Conclusions:Our findings supported the safety and effectiveness of RALP in OPC.Additionally,oligometastatic state and sites did not have an adverse effect on BCR independently.

Objective To explore the application effect of systematic health education in patients with nasopharyngeal carcinoma undergone radiotherapy.Methods Eighty-three patients with nasopharyngeal carcinoma from the Department of Oncology,Affiliated Hospital of Guangdong Medical College from January 2009 to January 2011 were randomly divided into two groups:the control group (41 patients) and the observational group (42 patients).The control group received routine health education,while the observational group received systematic health education with a health education guidebook throughout the treatment period.Questionnaire on the radiotherapy knowledge was used on the 10th day during radiation treatment to evaluate patients' mastering of the knowledge and their satisfaction with medical personnel.Results 98％ patients in the observational group knew the aetiological agent of nasopharyngeal carcinoma,95％ of them knew the importance of radiotherapy,95％ patients knew the secondary reaction of radiotherapy and matters need attention,98％knew the dietary instruction and 98％ knew the discharge guidance,all of which were higher than those in control group (76％,78％,80％,78％,78％,respectively).The differences were statistically significant (x2 =8.742,5.332,4.260,7.499,7.499,respectively; P ＜ 0.05).In observational group,the incidence rate of mucous membrane of mouth reaction was 35.7％,radiodermatitis accounted for 35％,mucous membrane of nasopharynx accounted for 30.9％,jaw joint functional disorder accounted for 38％,all of which were lower than those in the control group (73.1％,70.7％,65.8％,75.6％,respectively).The differences were statistically significant (Hc =10.266,10.173,8.772,10.414,respectively; P ＜ 0.05).The satisfaction with medical personnel in observational group was 95％,was higher than that in the control group (80％),and the difference were statistically significant (x2 =4.26,P ＜ 0.05).Conclusions Systematic health education can increase the nasopharyngeal carcinoma patients' knowledge about radiotherapy,reduce the incidence rates of adverse reactions,improve their satisfaction with the nursing,and promote good communication between patients and nurses.

Objective To study the tolerability and safety of single and multiple doses of Picika oral solution in healthy volunteers.Methods A single center, randomized, single -blind, placebo -controlled, dose -escalation study was designed.50 patients were given single dose, and 10 cases were given multiple doses.All of them had half male and fe-male.Single -dose group: 20 mL(4 subjects), 40 mL(6 subjects), 60 mL(10 subjects; 2 using placebo), 90 mL(10 subjects; 2 using place-bo), 120 mL (10 subjects ; 2 using placebo), 160 mL (10 subjects; 2 using placebo); multiple doses group: 10 subjects(2 using placebo), 40 mL? times-1 , tid, continuous medication for 10 days.Results Of the sixty healthy subjects enrolled , 58 finally completed the trial, and two shed.One case (female) of adverse event in single -dose 160 mL group was reported: her ctivated partial thromboplastin time (APTT) was ab-normal with clinical significance.It may not be associated with the medi-cation.In multiple doses group, one case of abdominal pain (female) was reported, may not be associated with the medication.Conclusion Single and multiple doses of Picika oral solution are safe and well tolera -ted in healthy subjects.