Objective To report a case of pulmonary alveolar microlithiasis ( PAM) in Peking Union Medical Col-lege Hospital and to summarize the clinical features and genetic characters .Methods The clinical features , ima-ging results , pathology findings and SLC34 A2 gene mutation was analyzed and reported .Results The patient was a 35 years old male, presenting with cough and sputum for 10 years and worsen with short of breath for 3 weeks. Computed tomography of lung and pathology findings support the diagnose of pulmonary alveolar microlithiasis .And a heterozygous mutation c .A910 T in exon 8 of SLE34 A2 gene was discovered through genetic testing .Conclusions Since to the treatment is non-specific in this rare disease , it's significantly important to recognize this disease through early non-specific clinical features but typical imaging findings .And the finding that c .A910 T is more common in Asia population may provide us a potential target for screening and possible genetic engineering therapy .

Objective To investigate the urinary albumin excretion of the diabetes patients and application value in the monitoring of early impairment in kidney. Methods The random urine samples from diabetes patients and controls within three days were collectod. The changes of urinary albumin excretion within day and between days were analyzed. 24-hour urine albumin was used as a standard to evaluate early kidney damage. The correlations between results of random urine albumin at the different time points and different periods were comparod. The sensitivity and specificity of random urine albumin at the different time points and different periods was evaluated and compared to deduce the best diagnostic porformance of the random urine albumin. Results There are greater variations of the levels of urinary albumin of patients with diabetes and control. After the correction with urine creatinine and urine volume the variations can be reduced (CV:49%±23% and 64%±30%). Urinary albumin excretion rate change rhythmically within the 24 h in healthy and diabetes patients. We found the best correlation between overnight ratio of urinary concentrations of albumin and creatinine (ACR) and 24-hour urinary albumin (R2 = 0.976). It was superior to urina sanguinis (R~2 = 0.900), postprandial urine (R~2 = 0.584) and random urine (R~2 =0.791). When 24 h urinary albumin was taken as the standard, receiver operating characteristic (ROC) curve analysis showed there was significant difference between male and female(male 12.8 μg/mg urine creatinine vs female 27.0 μg/mg urine creatinine),and the the cut-off value of ACR was 27.7μg/mg urine creatinine. When the smallest available negative likelihood ratio (0.011) and the greatest positive likelihood ratio (481.000) were obtained,the concentration of 13.0 μg/mg creatinine and 87.4 μg/mg creatinine were set as the cut-off value of ACR. Conclusions The correction with urinary creatinine can reduce the variation between-days compared with urine volume, but still can not completely eliminate the variability. The ACR of overnight urine has the best correlation with the 24 h urinary albumin and can replace 24 h urinary albumin. Random urine as the most convenient collecting urine samples can also replace 24-hour urinary albumin, but the gender discrepancy need to be considered. When the concentration of 13.0 μg/mg and 87.4 μg/mg was set as a random ACR exclusion value and the confirmative value, it can basically rule out and confirm the existence of microalbuminuria.

Objective To investigate the serum creatinine (SCr) level in patients with sporadic amyotrophic lateral sclerosis (sALS) and to explore the relationship between the SCr level and the clinical characteristics.Methods A total of 80 patients with sALS,80 patients with multiple system atrophy (MSA) and 80 patients with tension-type headache (TTH) were enrolled in the study.The SCr levels were compared among the three groups.The association between the SCr level and the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R),the forced vital capacity (FVC) percentage of predicted (FVC％ pred),the site of symptom onset,the duration of disease and the rate of disease progression was evaluated in the sALS group.Results The SCr level was significantly decreased in the sALS group than the other two groups [(60.86 ± 16.80) μmol/L vs (70.05 ± 12.79) μmol/L and (66.97-± 14.14) μmol/L,P ＜ 0.01].In the sALS group,the SCr level was positively correlated with the ALSFRS-R (r =0.315,P =0.005),while no correlation was found between the SCr level and the FVC％ pred,the site of symptom onset,the duration of disease and the rate of disease progression (all P ＞ 0.05).Conclusion The SCr level is an important biochemical index in the patients with sALS and might play an important role in monitoring the disease progression.

ObjectiveTo evaluate the safety and efficacy of total pelvic reconstruction surgery with Prosima in treatment of pelvic organ prolapse (POP) stage Ⅲ.Methods From July 2010 to December 2011,31 patients with POP stage Ⅲ undergoing total pelvic reconstruction surgery with Prosima were enrolled in this prospective study.Among two cases complicated with stress urinary incontinence underwent transobturator tension-freevaginaltapeconcomitantlywithtotalpelvicreconstructionsurgerywith Prosima.Clinical parameters during peri-operation were recorded and compared.Pelvic organ prolapse quantitative examiuation(POP-Q)andanatomicimprovementinthesepatientsafter surgery were analyzed.Comparisons of pelvic floor impact questionnaire-short form 7 ( PFIQ-7 ) and pelvic organ prolapse-urinary incontinence sexual questionnaire-short form 12 (PISQ-12) in these patients before and after surgery were used to evaluate quality of life and quality of sexual life.ResultsThe mean operating time was (55 ± 13) minutes,mean blood loss was (66 ± 25 ) ml.No severe intraoperative complications were observed.All patients were able to recover spontaneous mieturation within 5 days.Two cases experienced pelvic hematoma with diameters less than 7 cm,and resolved later.Another case was urinary tract infection.At the median follow-up 6 months ( 1 - 15 months),the rate of anatomic success defined as the leading vaginal edge above the hymen was 94％ (29/31).There were significant improvements in Aa,Ba,Ap,Bp,and C (P ＜0.01 ) by POP-Q.Two patients showed recurrent prolapse at 3 months and 1 year after surgery,without the need of further operation.The median score of post-operative PFIQ-7 was 0 point at 6 months and 0 point at 12 months after operation,respectively,which were significantly lower than that of 50 points pre-operation ( P ＜ 0.01 ).And there was no significant difference in the average score of PISQ-12 before and after surgery [ ( 30 ± 6) points versus (31 ± 4) points ] ( P ＞ 0.05 ).The rate of mesh exposure was 16％ ( 5/31 ),all the 5 cases occurred within 6 months and was cut in clinic.There was no case of de novo urinary incontinence and de novo dyspareunia.Conclusions Pelvic rcconstruction surgery with Prosima is safe and efficacy in treatment of POP stage Ⅲ.It could improve quality of life remarkably without influence on sexual quality of life.

Objective To investigate the ultrasonic image characteristics of Zenker diverticulum(ZD).Methods The ultrasonographic features were summarized using the analysis confirmed by surgery pathology or X-ray imaging of the ZD in six eases.Results The diverticulum of Zenker located behind the thyroid gland in all the six cases,with five cases to the left and one to the right.The internal hyperechoic foci caused by air was found for all the six cases.The diverticulum wall appeared to be semicircular,narrow-band and acoustic halo low echo with an average thinckness of 0.53 mm.The Doppler sonogram of the six diverticulums showed no vascular signal.Conclusions The ZD had several unique characterisitics for identification by ultrasonic diagnosis,which included hyperechoic foci caused by air,internal changes of echo after drinking water and the special layered structure of the five-layer ZD wall.

Enzymatic conversion is very important to produce functional rare sugars, but the conversion rate of single enzymes is generally low. To increase the conversion rate, a dual-enzyme coupled reaction system was developed. Dual-enzyme coupled reaction system was constructed using D-psicose-3-epimerase (DPE) and L-rhamnose isomerase (L-RhI), and used to convert D-fructose to D-psicose and D-allose. The ratio of DPE and L-RhI was 1:10 (W/W), and the concentration of DPE was 0.05 mg/mL. The optimum temperature was 60 degrees C and pH was 9.0. When the concentration of D-fructose was 2%, the reaction reached its equilibrium after 10 h, and the yield of D-psicose and D-allose was 5.12 and 2.04 g/L, respectively. Using the dual-enzymes coupled system developed in the current study, we could obtain sugar syrup containing functional rare sugar from fructose-rich raw material, such as high fructose corn syrup.
Aldose-Ketose Isomerases ; metabolism ; Carbohydrate Epimerases ; metabolism ; Fructose ; chemistry ; Glucose ; chemistry ; Hydrogen-Ion Concentration ; Temperature

Objective: To observe the effects of acupuncture on synovial pathology, synovial mast cell degranulation and tryptase expression and to investigate the relationship between the functions of mast cells and effects of acupuncture on early adjuvant arthritis in rats. Methods: Forty-six male Wistar rats were randomly divided into normal control group (n=16), untreated group (n=15) and acupuncture group (n=15). Adjuvant arthritis was induced by injection of 0.1 mL Freund's complete adjuvant in right hind limb footpad. Normal control group and untreated group received no acupuncture treatment, while rats in the acupuncture group were treated with sterilized disposable stainless steel needles inserted perpendicularly as deep as 2 to 3 mm at Xuanzhong (GB39), 6 mm at Shenshu (BL23) and 7 mm at Zusanli (ST36) for eight times (15 min each time) every two days. Setting the modeling day as the 0 day of the experiment, the body weight and paw volume of the rats were measured every three days from the 0 day. In the end, synovial tissues of the right hind ankles were sampled and made into paraffin sections. Then they were firstly stained with hematoxylin-eosin for observing synovial pathology to evaluate the effects of acupuncture on adjuvant arthritis, then stained with toluidine blue for observing the number and degranulation ratio of synovial mast cells and finally detected by immunohistochemical staining method to investigate the expression of tryptase in synovium. Results: Compared with the untreated group, the body weight of rats in the acpuncture group was increased significantly (P<0.05), while the paw volume decreased obviously (P<0.01). Hematoxylin-eosin staining showed that acupuncture significantly inhibited inflammatory cell infiltration, synovial cell hyperplasia, and synovial fibroplasia in synovium of rats with adjuvant arthritis as compared with the untreated group (P<0.05). Toluidine blue staining showed that acupuncture could significantly diminish the numbers of total and degranulated mast cells in rats with adjuvant arthritis (P<0.01), which were significantly higher in the untreated group than in the normal control group (P<0.01). Showing by immunohistochemical staining, the expression of tryptase in synovium in the acupuncture group was decreased as compared with the untreated group (P<0.01). Analyzed by Spearman's bivariate correlation, the number of mast cells and degranulation ratio of mast cells were positively correlated with the pathological scores (r=0.837, P<0.01; r=0.634, P<0.01). Conclusion: Acupuncture can improve pathological condition of inflammatory synovium in rats with early adjuvant arthritis by inhibiting the function of synovial mast cells, which may play an important underlying role in the immunoregulation of acupuncture on adjuvant arthritis.

A HPLC method for simultaneous determination of uridine, inosine, guanosine, adenosine and cordycepine
in Cordyceps sinensis was developed. The sample solution was prepared with 0.5% phosphoric acid solution by ultrasonic extraction. The separation was performed on a ZORBAX SB-AQ (150 mmí4.6 mm, 5 μm)column with gradient elution by 0.1% formic acid solution and acetonitrile, with column temperature 30℃, at a flow rate of 0.8 mL/min, and detected at wavelength of 260 nm. The result of method validation showed that the developed method had high accuracy and good repeatability. This method has been successfully applied for analysis of 5 kinds of nucleosides in different parts of C. sinensis. The results indicated that content of nucleosides in stroma is higher than that in insect body and whole C. sinensis.

Objective To summarize and analyze the shielding,retention and reentry works of blood donors,and to investigate the feasibility of retention and reentry strategy.Methods The samples of ELISA single reagent reactive/NAT non-reactive and ELISA non-reactive/ NAT reactive were negative by confirmatory tests.Then the blood was weeded out and the donation qualification was reserved.The donors of shielding more than 6 months could propose the reentry application at any blood station in the province,and were allowed to return to the ranks after qualified by routine detection and re-detection by Jiangsu Provincial Blood Center.The unqualified rates were compared between the donors of again blood donation after retention and reentry with the common donors by χ2 test.Results From October 2014 to June 2016,1 615 cases were ELISA single reagent reactive/NAT non-reactive,among which 67 cases were confirmed as positive,42 cases were undetermined and 1 506 cases were negative;831 cases were ELISA non-reactive/ NAT reactive,in which 809 cases were positive by confirmation and 22 cases were negative.A total of 1 528 donors were confirmed as negative and their donation qualifications were reserved,89 donors conducted blood donation again and 79 were qualified in blood detection.The unqualified rate was 11.24%,compared with that of common donors,the difference was statistically significant(P<0.001).Meanwhile,596 donors applied for reentry,among them 218 persons were weeded out by the reentry blood station.In remaining 378 samples sent to Jiangsu Provincial Blood Center,359 samples were qualified and confirmed to the reentry condition.Among them,332 donors conducted blood donation and all were qualified by blood detection.Conclusion The reentry strategy in Jiangsu Province is reasonable and feasible,but the donors retention strategy needs to be further optimized and perfected.

Objective To determine gender differences in baseline characteristics and intervention treatment in relation to prognosis in patients with non-ST segment elevation acute coronary syndrome (NSTEACS). Methods A total of 814 patients (545 men and 269 women) with NSTEACS were randomized to early intervention (coronary angiography ＜ 24 hours after randomization ) or delayed intervention (coronary angiography＞36 hours after randomization). The primary outcome was a composite of death, myocardial infarction, or stroke at 6 months. Results Women were older and more frequently had hypertension, diabtetes, and history of coronary artery disease (CAD) or chronic angina (P＜0.05 for all).Women less were smokers and had elevations in cardiac marker(P ＜ 0. 05 for both). Women who underwent angiography had no significant lesions more often, but the left main stem and/or three-vessel diseases were similar with men. In adjusted multiple logistic regression analysis,the previous myocardial infarction and severe coronary artery disease were independently associated with the risk of primary endpoint in women. On multivariate analysis for men, severe coronary artery disease delayed intervention strategy and at least 3 risk factors for CAD were independently associated with the risk of primary endpoint. Conclusions In NSTEACS patients, different gender had the different prognostic predictor. Severe coronary diseases were as an independent predictor for both male and female patients. An early intervention strategy resulted in a beneficial effect in men which was not seen in women.