Phenylketonuria,an autosomal recessive inherited disease characterized by amino acid metabolism abnormality,has been listed as a screening program for neonatal genetic metabolic diseases in China.Eearly screening and treatment can reduce or even avoid the disease,However,delayed intervention can influence social functioning and psychological development.The research of life quality including psychological and social functioning in phenylketonuria patients has atlracted the increasing attention along with the medical model transformation.In order to study quality of life in patients with phenylketonuria,life quality questionnaires have been developed.It reveals that early and long-term treatments can improve the quality of life.The assessment and present condition as well as the impact factors of quality of life in phenylketonuria patients have been reviewed in this article,which have important value to evaluate the quality of life and guide clinical treatments as well as judge the prognosis of patients with phenylketonuria.

OBJECTIVE: To prevent medication errors during drug subpackage and ensure patients' medication safety. METHODS: Healthcare failure mode and effect analysis (HFMEA) was applied to evaluate the potential failure modes and effects during drug repackaging and dispensing. Thereafter rectification measures was analyzed and carried out in order to prevent the recurrence of failure modes. Risk priority numbers (RPNs) before and after the implication of rectification measures were analyzed using paired t tests. RESULTS: After the application of healthcare failure mode and effect analysis, RPN was statistically significantly (P<0.05) reduced by 60% (P=0.000 2), which affecting the incidences of medication errors during drug unit-dose repackaging. CONCLUSION: The application of healthcare failure mode and effect analysis can effectively prevent medication errors during drug subpackage and improve patients' medication safety.

Objective To analyze the value of three-dimensional computed tomography (CT) reconstruction imaging for the diagnosis and treatment of congenital scoliosis. Methods 76 patients with congenital scoliosis were examined with X-ray of total spine and three-dimensional CT reconstruction, and the results were analyzed. Compared with X-ray film, the findings with the CT imaging were classified into three groups: Group Ⅰ, no supplementary useful information was obtained; Group Ⅱ, further confirmation of findings which were unclear or ambiguous on X-ray film, with improved visualization and comprehension of the type of the deformity; Group Ⅲ, substantial new information was obtained. Results Group Ⅰ: 11 (14.5%) patients, without supplementary useful information obtained with three-dimensional CT reconstruction imaging. The patients aged from 2 to 16 years(11.00?10.09 years), and the Cobb angle ranged from 28? to 102?(55.60??21.06?). Group Ⅱ: 49 (64.5%) patients, with confirmatory finding or improved visualization and comprehension of the previously identified deformities obtained with CT imaging. The 49 patients aged from 4 to 28 years (13.53?4.47 years), and the Cobb angle ranged from 20? to 115? (55.41??23.44?). Group Ⅲ: 16 (21%) patients, with substantial new information obtained with CT reconstruction images which was unrecognized with X-ray film. The patients aged from 6 to 16 years (12.88?2.68 years),and the Cobb angle ranged from 37? to 145?(73.19??28.18?). The difference of age of patients between 3 groups was of no significance (P=0.052), but the difference of Cobb angle between 3 groups was significant statistically (P=0.039). Conclusion Three-dimensional and multiplanar reformatted CT imaging allows better visualization and understanding of the deformities of scoliosis, and supplementary useful information may be obtained which was obscure or unrecognized with X-ray film, especially for patients with severe deformities. Its application in clinical practice may contribute to the diagnosis of the type of deformity and the segments involved, as well as the individualized operative planning.

Objective: To optimize the preparation process of tetrandrine dropping pills (TDP) and investigate the in vitro dissolution rate. Methods: Plackett-Burman experimental design was used to screen the critical factors in the preparation process of TDP from the ratio of matrix, ratio of matrix to drug, dropping temperature, dropping rate, dropping distance, and condensate temperature. The forming rate and weight variation of TDP were used as the evaluation index, the parameters in the preparation process of TDP were optimized by using the Box-Behnken response surface method. Moreover, the in vitro dissolution rate of TDP was compared with tetrandrine tablets by rotating basket method. Results: The Plackett-Burman experimental design results showed that the ratio of matrix, dropping temperature and condensate temperature had a significant effect on the forming rate of TDP. The optimum preparation parameters by Box-Behnken response surface method were as follows: the ratio of matrix was 2.6∶1, dropping temperature was 82.4 ℃ and condensation temperature was 7.5 ℃ with high forming rate, good roundness, stable weight, and fast drug dissolution rate of TDP. Conclusion: The quality of TDP by experimental design method can meet the requirements and can be further amplified.

ObjectiveTo evaluate the reasonableness of total mesorectal excision (TME) in the management of rectal cancer. Methods The carcinoembryonic antigen (CEA) level of surgically removed distal mesorectum (3?cm below the tumor),tumor tissue and normal mesocolon was detected by microparticle enzyme immunoassay in 26 rectal cancer patients. Results The CEA level in normal mesocolon was (1.6?1.0)?ng/g, and (62.5?85.2)?ng/g in distal mesorectum(P1?000?ng/g).ConclusionsIn patients with rectal cancer, much higher CEA level in distal mesorectum than in normal mesocolon might indicate distal mesorectal dissemination. It is necessary to routinely perform TME in the surgical management of middle and lower rectal cancer.

Aim To investigate the relationship be-tween the depression-like behaviors of rats induced by chronically unpredictable stress( CUS) and the protein expression of tumor necrosis factor alpha( TNF-α) , in-dole-2 , 3-dioxygenase ( IDO ) and 3-hydroxyl amino acid oxygenase( HAAO) . Methods Thirty male Spra-gue Dawley( SD) rats were randomly divided into con-trol group and model group. CUS plus solitary condi-tion were used to establish the depression model. The open field test ( OFT ) and the force swimming test ( FST ) were used to evaluate the depression-like behaviors of rats ;Western blot was used to determine the protein expressions of TNF-α, IDO and HAAO in the prefrontal cortex and hippocampus of rats. Results Model group rats showed obvious depression-like be-haviors with increasing immobile time in FST ( P <0. 01) and decreasing locomotive activity in OFT( P<0. 01 ) , and up-regulating the protein expression of TNF-α, IDO and HAAO in the prefrontal cortex and hippocampus significantly ( P <0. 05 ) , compared with control group rats. Conclusion The depression-like behaviors of rats induced by CUS may be associated with the activation of IDO-HAAO pathway mediated by TNF-α.

BACKGROUND:Surface marker molecules and differential ability of human adipose tissues-derived mesenchymal stem cells can change with the prolonged culture time.OBJECTIVE:To analyze the morphological characteristics and colony forming capacity of early cultured rabbit adipose tissues-derived mesenchymal stem cells in vitro.DESIGN,TIME AND SETTING:The cytological in vitro study was performed at the Institute of Ophthalmology,and Central Laboratory,Eye and ENT Hospital,Fudan University from January to March 2008.MATERIALS:Nine female New Zealand rabbits aged three months were obtained from the Shanghai Yingenyang Rabbit Room.METHODS:Adipose tissue was harvested from the nape fat pad of the New Zealand rabbits following anesthesia.Primary cultured cells were established using type I collagenase and cell cultures were maintained with DMEM containing 0.1 volume fraction of fetal bovine serum.Cells were passaged when 80% was confluent.The second,third and fourth passages of cells were utilized for the study.MAIN OUTCOME MEASURES:The morphology of adipose tissues-derived mesenchymal stem cells was observed using inverted microscope.Cell phenotype was detected utilizing flow cytometry.The cloning capacity was determined.RESULTS:Adipose tissues-derived mesenchymal stem cells were fibroblast-like and proliferated rapidly in vitro at the second,third and fourth passage.Flow cytometry showed that adipose tissues-derived mesenchymal stem cells expressed CD29 and PCNA.The colony-forming rates were respectively(8.0?0.6)%,(6.7?0.4)% and(4.6?0.5)%(F=12.18,P

Objective To analyze the epidemic characteristics of brucellosis in Nangang District of Harbin City,and to provide a scientific basis for making strategies in prevention and control of the disease.Methods Descriptive epidemiological methods were used,epidemic characteristics (time distribution,regional distribution and population distribution) of the disease in Nangang District of Harbin City in 2006-2015 were studied,and brucellosis cases were reported online through the national epidemic network.Results A total of 105 cases of brucellosis were reported,the average annual incidence rate was 7.84/100 thousand and no death case.The number of cases was the highest in 2014,it was 21 cases,the incidence rate was 1.57/100 thousand;the number of cases was the least in 2010,it was 3 cases,the incidence rate was 0.22/100 thousand;in 2006,the number of cases was 9,the incidence rate was 0.67/100 thousand;in 2015,the number of cases was 17,the incidence rate was 1.27/100 thousand.The incidence of the disease was mainly concentrated in summer and autumn,accounting for 65.71％ (69/105),the number of summer onset was 38,accounting for 36.19％ (38/105);the incidence of the autumn was 31,accounting for 29.52％ (31/105).In 2006-2012,the incidence of the disease was mainly concentrated in Wanggang and Hongqi townships,accounting for 60.00％ (30/50);after 2013,the cases in urban areas were on the rise,Wanggang and Hongqi townships accounted for 34.55％ (19/55).Incidence of male youth was the highest,male and female incidence ratio was 2.18:1.00 (72:33);incidence peak was 30-60 years old,accounting for 60.95％ (64/105);after analyzing the incidence of occupational groups,farmer was the highest,accounting for 43.81％ (46/105).Transmission route was contact with cattle and sheep and lived in the environment cattle and sheep were breed,the number of cases was 34.Conclusions The incidence of brucellosis in Nangang District of Harbin City is rising after 2013.Disease season focus in summer and autumn,occupation focus in farmers,and the scope of the outbreak has been expanded.We should further strengthen publicity and education in disease focus season,improve the awareness of protection,in order to effectively control the epidemic of brucellosis.

·AIM: To study the tear function changes in patients with pterygium before and after pterygium transposition.·METHODS: Twenty eyes of 20 patients were enrolled in this study.Schirmer I test and tear break-up time (BUT) were evaluated in patients before and after pterygium transposition.·RESULTS: There was no significant difference in the results of Schirmer I test before and after the surgery.The results of BUT were not significantly different before and up to 4 weeks after surgery. However, BUT prolonged significantly 6 weeks after surgery (P<0.05).·CONCLUSION: Pterygium transposition can improve the tear film function in patients with pterygium.

Objective To investigate the optimal ultrasound exposure parameter on H22 neoplasms of mice meditated by ultrasound exposure combined with self‐made nanobubbles ,and then observe their therapeutic effect combined with cisplatin and their possible mechanism of anti‐tumor . Methods Thirty mice engrafts models with subcutaneous H22 neoplasms were established and divided into 6 groups randomly ,which received ultrasound exposure at different intensity and exposure time . The contrast enhanced ultrasound imaging ( CEUS ) was performed in every group at the four time points of before treatment and at 0 h ,24 h ,72 h after treatment . To obtain the optimal ultrasound parameters ,the tumor inhibitory effect was assessed by enhanced intensity ( EI) and microvascular density ( MVD) . The H22 tumor were treated by ultrasound exposure nanobubbles combined with cisplatin to observe their tumor growth inhibition rate ,and the microvessels density and nuclear associated antigen Ki‐67 proliferation index were measured by immunohistochemical staining . Results There was a statistically difference in enhanced intensity (EI) between the experimental groups and control group ( P < 0 .05) . With the increasing of ultrasound intensity and exposure time ,the tumor inhibitory effect was more obvious ,with an increasing side reactions . Except the simple ultrasound group ,there was a statistical difference in tumor inhibition ,the mean MVD and the tumor cell proliferation index (KI‐67) between control group and the other ultrasound therapy groups ( P<0 .05) . The tumor inhibitory rate was the highest ( tumor inhibition rate 70 .0% ) and the mean MVD and KI‐67 expression were the lowest ( P <0 .05) in the combination group comparing with the others . Conclusions The ideal ultrasound exposure parameter of tumor inhibition showed that exposure intensity chose 1 W/cm2 and exposure time chose 1 min or 3 min intermittence . The ultrasound exposure self‐made nanobubbles combined with cisplatin could enhance the tumor inhibitory effect .Its mechanism may be related to the decrease of microvascular density ,the inbition of tumor cell proliferation and the increase of tumor cell necrosis .