Objective To study application of the quality management of Blood Cell Analyzer according to the requirement of biological variation determination.Methods Collected the indoor imprecision value(CV％) from 8 items detected by blood cell analyzer during from April to Nov.of 2016,and the bias (Bias％) of 8 items of two EQA (external quality assessment) from the ministry of health in 2016.Then according to the 3 levels of the minium,appropriate and optimal quality specifications derived from the biological variability the rates of imprecision and bias were culculated.The pass rate of the imprecision and bias was calculated.By using mean bias and mean imprecision and biological variation 3 levels of total error (TEa) crite rion,and to calculate the corresponding σ and QGI value,so as to evaluate the performance of whole blood cell analyzer.Then improved the quality.Results For the imprecision value of 8 items,except the MCHC average value,all others were all 100 ％ meeting the appropriate level of quality requirements.For the bias value (Bias ％) from 8 items,except MCH,all others were over 80 ％ meeting the appropriate level of quality requirements.While for the calculated σ value,based on the best level of quality requirements,except the σ value of WBC was 4.6,the σ value of all other items were all＜3.Based on the appropriate level of quality requirements,except the σvalue of MCHC was 1.9,the value of σ of all other items were all＞ 3,and based on the minimal requirements,the σ value of all 8 items were all ＞3.After analysis,this blood cell analyzer,except that MCHC should use the minimal quality standard requirements,all other examination items could used the proper quality standard requirements,and the calculated QGI were all ＜0.8.Conclusion Based on the biological variation determination requirement and calculated σ and QGI value,this method could be used to more accurate quality evaluation of blood cell ana lyzer.Which is a higher levelof quality management,will be more conducive to quality improvement and better serve the clinical.

Objective To assay the expression chinse of ED1 positive cell in focal segmental glomemlosclerosis in rats,we investigated the relationship between the infiltration of mononuclear phagocytes and the progression of glomendar sclerosis.Methods We used 12 Wistar ratswhichwere dividedintotwo groups.1est group and coutrol group.Themodel offocal segmental glomerursclerosiswas ulade by in jecting PAN 9 mg/100g body weights.The rats ofcontrol group were injected 3ml 0.9％80diuln chloride.The proteinuria,serum creatinine,fipi&and protein of the rats were examined.The rata were killed at the 20th week.All the kidneys were kept and nlade into pathologic slEun-pie.1mmunohistochemical method was applied to detect the protein expression of FIN and the EDI positive cell in renal tissue of all the rats,and the number of Edl positive cell W88 counted.The results were analyzed by SPSS.Results The proteinuria of the mts in FSGS model group was significantly increased,the serum lipid ofthem was also increased.The pathology changes of the rat renal in model group showed that a part of giomemli appeared focal segmental sclerosis or all glomerular sclerosis,and the extracelhlar matrix accumulated.In the renal of model rats,the amount of EDI positive cells was significantly higher than that in normal rats(P

Objective To explore effect of rehabilitation training on deglutition disorders of children with cerebral palsy . Methods Twenty-seven children patients from January to June in 2013 were set as control group and thirty-one patients from July 2013 to January 2014 as experiment group. The children in the control group were treated with tube-feeding combined with spoon feeding and bottle-feeding by professional nurse. Children in the experiment group were treated with oral rehabilitation training by professional therapists and nurses apart from the same feeding as in the control group. The two groups were compared in terms of effect of deglutition within 4 weeks, time and rate of removing the stomach tube. Results The recovery of deglutition function in the experiment group was much better than that in the control group , the total effective rate and rate of removing the stomach tube within 3 months were higher and the intubation duration was significantly lower as compared with those of the control group (P < 0.01). Conclusion Rehabilitation training can improve the recovery of deglutition disorders, improve active feeding ability of children with cerebral palsy, shorten time of tube feeding and improve their life quality.

Objective To evaluate the treatment of current status and prognosis in childhood APL with APL2008 ,which was administrated since 2008 in our center .Methods A total of 43 children with newly diagnosed APL between 2008 to 2014 were studied retrospectively .Treatment options and current status were summarized from 28 patients who received APL2008 therapy . Results Studied 43 patients were at median age of 8 years and 4 months ,with 28 boys and 15 girls .The main clinical manifestations were infection ,anemia ,bleeding ,fever ,hepatomegaly ,splenomegaly and lymphadenopathy .The proportions of low ,intermediate and high risk groups were 27 .9% ,48 .8% and 23 .3% ,respectively .Eleven cases could be diagnosed as DIC .Bone marrow morphology showed abnormal elevation of promyelocyte .37 patients had distinctive immunophenotype such as frequent expression of CD33 , CD117 and MPO .PML/RARαfusion gene positive rate was 100% in 43 children and cytogenetic analysis were positive in 37 cases , of which specific genetic lesion in APL cells with t (15 ;17)(q22 ;q12) was found in 28 cases ,and karyotypes was found in 9 cases as infrequent chromosomal abnormalities .In 43 patients ,4 cases were early dead from intracranial hemorrhage at early stage ,and 11 cases were given up early .There were only 2 cases dead ,2 cases relapsed and 1 case lost among 28 APL children ,which enabled ef‐ficacy analysis possible .96 .4% of these 28 cases achieved HCR .The 2 year Kaplan Meier estimates of OS and EFS were 85 .9% ± 7 .6% and 80 .4% ± 8 .8% .But OS and EFS would be 94 .7% ± 5 .1% and 88 .9% ± 7 .4% if 3 patients who had non standard treat‐ment were excluded .Conclusion Childhood APL were characterized by anemia ,bleeding ,fever and infiltration .APL′s coincidence rate between PML/RARa fusion gene and morphology ,immunology and cytogenetics were 95 .3% ,90 .2% and 86 .5% ,respective‐ly .APL2008 significantly improved the prognosis of APL .

Primary tracheobronchial amyloidosis (TBA) is a rare pulmonary disease. A systematic review was performed on 64 cases of primary TBA in China and progress in the diagnosis and treatment of this disease is discussed. The Chinese biological and medical databases from 1970 to 2010 were searched and 75 cases of complete clinical and pathological data were identified. The clinical characteristics of the disease were summarized and longitudinal comparisons were made of diagnostic and treatment methods over time. The results showed that the morbidity associated with primary TBA has increased over recent years. The clinical manifestations were non-specific. Progressive dyspnea, cough and sputum were the most common symptoms. The percentage of patients undergoing computed tomography (CT) scan has increased over the years. The bronchoscopy and transbrochial lung biopsy (TBLB) were usually sufficient to establish the diagnosis. Treatment was reported for a total of 44 cases. Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration such as steroids and colchicines were reported to be effective in some patients. It is concluded that the demographic characteristics and clinical manifestations of primary TBA patients in China are largely consistent with findings reported in other countries. Dramatically more cases were reported in recent years, mainly due to the extensive application of bronchoscopy since 1990s. Chest CT scan provides important clues for the diagnosis of the disease. The definite diagnosis was confirmed by bronchoscopic findings and Congo red staining of biopsy specimen. Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration, such as steroids and colchicines were reported to be effective in some patients.

Objective To measure serum surfactant protein (SP) A and D levels in patients with interstitial lung disease associated with rheumatoid arthritis (RA).Methods Serum SP-A and SP-D levels of RA,RA-ILD patients and healthy controls were assessed using a sensitive enzyme-linked immunosorbent assay (ELISA).The relationship between SP-A and SP-D and RA-ILD was analyzed.The serum SP-A and SP-Dpositive rate was calculated for the three groups.The correlation between SP-A and SP-D with RF,anti-CCP,antinuclear antibody,antikeratin antibody,anti-perinuclear factor,C-reactive protein,erythrocyte sedimentation rate,were analyzed.Mean value of groups were compared with variance analysis,Spearmam rank correlation test was used for correlation analysis.Results The levels of serum SP-A in RA-ILD patients and RA patients as well as in healthy controls were [ (51.2±9.2),(25.9±2.6),( 15A±0.3 ) μg/L] respectively.The level of serum SP-D of the three groups was [ ( 42.5 ±8.1 ),(20.8 ± 1.5 ),( 16.6±0.8 ) μg/L ] respectively.The levels of serum SP-A and SP-D in patients with RA complicated with ILD were higher than those simple RA patients and healthy controls (P＜0.05).The levels of serum SP-A and SP-D in patients with RA were not significantly higher than those in healthy controls (P＞0.05).The positive rate of serum SP-A and SP-D in RA-ILD patients were significantly higher than those in simple RA patients and healthy controls.The positive rate of serum SP-D of RA-ILD patients was higher than that of SP-A.The levels of serum SP-A and SP-D in patients with RA complicated with ILD were correlated positively with age,C-reactive protein.The level of serum SP-D was correlated positively with RF,anti-CCP,antikeratin antibody.There was no correlation between the level of serum SP-A and SP-D with RA-ILD and antinuclear antibody,antiperinuclear factor,erythrocyte sedimentation rate.Conclusion The levels of serum SP-A and SP-D are correlated with RA-ILD and may be useful markers for ILD in patients with RA.These two paramenters may be helpful to early diagnosis of RA-ILD.The Serum SP-D levels are more sensitive in predicting the development of RA-ILD than other parameters and can help in assessing the severity of lung damage.

Objective To report a case of progressive pseudorheumatoid dysplasia (PPD) with two kinds of WISP3 gene mutation. Methods A case of PPD was reported. Its clinical profile and the process of diagnosis were analyzed, and the related literature were reviewed. Results A 15-years old boy, who developed progressive joint pain and enlargement with spine involvement, was diagnosed as PPD. The erythrocyte sedimentation rate and C-reactive protein were in normal range, rheumatoid factor and anti-CCP antibody were all negative. HLA-B27 was also negative. Gene study discovered two kinds of mutations in Wnt1-inducible signaling pathway protein 3 (WISP3) gene: c.589+2T＞C and c.624dupA. Radiographic studies revealed severe osteoporosis without erosion, platyspondylia, enlargement of metaphysis and scoliosis deformity. The joint space of sacroiliac joint and articulation of pubis were significantly widened. Conclusion PPD is a rare autosomal recessive disorder characterized by cartilage homeostasis. It is associated with WISP3 gene mutations. Gene detection, laboratory examination and typical radiographic features are helpful for the diagnosis. This is the first report of c.589+2T＞C and c.624dupA mutations in patients with PPD in our country.

Objective To study the clinical characteristics and prognosis of interstitial lung disease (ILD) associated with polymyositis (PM) and dermatomyositis (DM). Methods The clinical data of 107 DM related ILD,and its frequency rate was 26.2%. Arthritis as the first symptom had a higher presenting rate in patients with ILD than in patients with non- ILD. Arthritis, dry cough and short of breath occurred more dyspnea presented in patients with DM-ILD, and there was serious myalgia and muscle weakness in patients More DM-ILD patients had high HBDH and AST than those in PM-ILD. High CK and CK-MB were more The 7 of 8 severe cases had DM-ILD in which 5 died of respiratory failure (death rate was 17.9% in PM/elevated ESR and CRP tend to complicate with ILD. The DM patients who have characteristic skin rashes and high AST level are prone to develop ILD. The PM patients who have high CK and CK-MB are susceptible to

Objective:To explore the expression of peptidyl prolyl cis-trans isomerase (Pin1) activity in peripheral blood of patients with rheumatoid arthritis (RA) and the value and correlation of T helper cell 17/regulatory cells (Th17/Treg) cells, and to analyze the effect and influence of all-transretinoic acid (ATRA) on it.Methods:① Comparing the difference of Pin1 expression and absolute counts of Th17 and Treg between RA patients before and after treatment and healthy control group, Kruskal-Wallis rank sum test was used for analysis. ② To analyze the correlation between the expression of Pin1 and its general data, activity indicators [such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and disease activity score 28 joints (DAS28) scores], Th17, Treg and some cytokines in RA patients, and to use Pearson and Spearman correlation tests. ③ To analyze the difference of Pin1 expression and Th17/Treg in peripheral blood of RA patients treated with low-dose all-trans retinoic acid (10 mg twice a week) and traditional immunosuppressants such as hydroxychloroquine for 3 months respectively. Mann-Whitney U test was used for comparison between the two groups, and the difference was statistically significant with ( P<0.05). Results:① The activity of Pin1 in peripheral blood of the newly treated group of RA was [13.62(9.16, 19.42)] higher than that of the healthy control group [8.97(7.62, 11.45)]( Z=42.82 , P<0.05), and Th17 was [18.28(12.76, 24.08)] higher than that of the healthy control group [6.04(4.96, 4.96)]( Z=48.83 , P<0.05). Treg [11.06(5.31, 21.87). It was lower than that of healthy control group [40.41(24.33, 48.52)]( Z=42.21 , P<0.05). ② the activity of Pin1 in peripheral blood of RA patients was positively correlated with CRP, the number of involved joints, DAS28 score, interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) ( r=0.396, P<0.05; r=0.683, P<0.05; r=0.466, P<0.05; r=0.315, P<0.05; r=0.416, P<0.05). ③ Compared with the newly treated RA group, the activity of Pin1 [6.94(5.96, 8.77), Z=42.82 , P<0.05] and Th17 7.38 decreased [7.38(3.85, 11.21), Z=48.83 , P<0.05], while Treg [40.41 (17.77, 33.47)] increased ( Z=42.21 , P<0.05). ④ Compared with the traditional medicine group, Treg [28.9(21.73, 37.36)] was higher in the retinoic acid group, and the difference was statistically significant ( Z=-2.683 , P<0.05). The activity of Pin1 was [6.23(5.58, 8.75)], but there was no statistical significance ( Z=-1.622 , P=0.104). Conclusion:Pin1 in peripheral blood of RA patients is over-expressed. Th17 is increased and Treg is decreased. ATRA combined with other traditional drugs can reduce Pin1 activity, promote Treg growth and improve disease activity of RApatients to a certain extent.

Objective To investigate the correlated pathogenetic factors and vaginal local immunity in vulvovaginal candidiasis (VVC). Methods A case control study was conducted to compare VVC group (60 cases) with normal group (60 cases). All of the women filled up the specific questionnaires. Routine examination, pH test and bacterial culture were done on the vaginal discharge. Cytokines of the vaginal lavage were measured by enzyme linked immanosorbent assay. Results (1) Outcomes of the questionnaires: there was no significant difference between the two groups in educational background,knowledge of gynecologic infection, history of gynecologic infection, hygienic habit, sex life, or use of medicine (P >0. 05). The incidence of chronic cervicitis in normal group (43%, 26/60) was higher than in VVC group (22%, 13/60; P <0.05). (2) There was no difference in vaginal pH between the two groups ( P > 0. 05 ). (3) Detection rate of candida albicans by vaginal discharge routine examination was 72% (43/60). (4) The concentrations of interleukin (IL) 2, and IL-4 in vaginal lavage did not show significant difference between the two groups ( P > 0. 05 ), but the concentrations of human defensin 5,human beta-defensin (HBD) 1, and HBD2 in WC group [(0.94±0.44) mg/L, (3.1±0.4) μg/L,(10±6) μg/L] were higher than normal group (P < 0.05). Conclusions VVC is a common vulvovaginitis. There is no significant correlation between the incidence of VVC and educational background,knowledge of gynecologic infection, history of gynecologic infection, hygienic habit, sex life, or use of medicine in the child-bearing period. Human defensin may be closely correlated with the pathogenesis of VVC.