Objective To retrospectively analyze the screening results of phenylketonuria(PKU ) among 567 691 neonates in Gansu Province to understand the prevalence situation of PKU and provide the basic data for preventing and treating PKU in Gansu Province .Methods 567 691 samples of neonatal dried heel blood spots were collected by Gansu Province Newborn Screening Cen‐ter from 2009 to 2014 and the phenylalanine (Phe) level was quantitatively determined by the fluorescence quantification method . The identification was performed by using the urine pterine profile analysis and phenylalanine hydroxylase(PAH) gene mutation de‐tection .Results Among 567 691 neonates ,166 neonates were diagnosed as PKU ,the total detection rate was 1/3 420 ,in which 119 cases (71 .7% ) were classic PKU ,33 cases (19 .9% ) were moderate PKU and 14 cases (8 .4% ) were mild PKU .Conclusion The morbidity rate of PKU in Gansu Province is much higher than the national average incidence level ,which is dominated by classic PKU .Therefore Gansu Province should become the major area of PKU prevention and treatment .

Objective To explore the characteristics of etiology,clinical,electroencephalogram(EEG) and prognosis of infantile spasms(IS) with focal seizures(FS).Methods The significance of age onset,seizure patterns and atteration,etiology,video-EEG(VEEG) and evolution of FS correlating to epileptic spasms(ES),which occurred in 12 cases with IS by means of clinical observation,cranial CT or MRI,VEEG monitory and follow up were investigated.Twelve cases were divided into group A,B,C according to the stages of FS occurring prece-ding,coinciding and following ES.Results Ten cases with IS were identified from focal cortical dysplasia,tuberous scleroses complex,temporal lobe cyst or scleroses and hypoxic-ischemic encephalopathy and others.Clinical manifestation presented FS at certain times during the course of the disease,and other characteristics of frequent attacks,asymmetric spasms or tonic spasms,some atypical seizures in the eyes and the head,motionless staring and focal motor seizures and other.The correlation of ES to FS occurred during one ictal episode as follows:FS→ES(6 cases),ES→FS→ES(1 case),ES→FS(3 cases).FS appeared more frequently,atypical,predominantly involving ocular,facial,oral movement or generalized convulsion,migrating or alternating seizures,associated with epileptic discharges of posterior parietal-occipital and parietal-temporal-occipital origins in group A and early period of group B;whereas in the late period of group B and group C,FS occurred less frequently and more stable,presenting complex partial seizures or secondarily generalized seizures originated mainly from frontal and surrounding areas in the lateralized or bilateral hemispheres.Regarding the natural evolution and development of IS,2 cases with early FS developed into IS;6 cases with IS evolved into Lennox-Gastaut syndrome or symtomatic generalized epilepsy,4 cases got into FS,1 case maintained in a peculiarly epileptic state with FS as well as ES until the operation at 4.5 years old,and the other case was not identified clearly.Conclusions IS coincided with FS is a special kind of aged-related FS associated with secondarily generalized seizures and epileptic encephalopathy.The multiple etiology,seizure patterns,ictal-interictal EEG,clinical evolution and prognosis of the disorder,indicate a complicated interaction of the immature cortico-subcortical abnormalities in the critical developmental period and thereafter,can be identified as a peculiar form of clinical epileptic syndrome.

Objective:To compare the survival rate and adverse reactions of patients with advanced hypopharyngeal squamous cell carcinoma undergoing surgery combined with chemoradiotherapy, and to analyze the prognostic factors of patients.Methods:The clinicopathologic data of 78 patients with advanced hypopharyngeal squamous cell carcinoma admitted to the Department of Radiation Oncology of the First Affiliated Hospital of Bengbu Medical University from August 2013 to December 2018 were retrospectively analyzed. The patients were divided into surgery combined with chemoradiotherapy group ( n=27) and chemoradiotherapy group ( n=51) according to different treatment methods. The median follow-up time was 46 months (20-84 months). The main observation indicators were overall survival (OS), progression-free survival (PFS) and local control rate (LCR). Cox regression model was used to analyze the prognostic factors. Results:Until July 31, 2020, 51 of the 78 patients with advanced hypopharyngeal squamous cell carcinoma died, including 6 cases of local recurrence, 11 cases of distant metastasis, and 34 cases of other causes (15 cases of hemorrhage, 15 cases of cachexia, and 4 cases of other diseases). In the surgery combined with chemoradiotherapy group, 12 patients died, accounting for 44.44%. In the chemoradiotherapy group, 39 patients died, accounting for 76.47%. The 1-, 3- and 5-year OS rates of 78 patients were 57.7%, 36.3% and 27.2% respectively, the 1-, 2- and 3-year PFS rates were 49.5%, 38.7% and 32.6% respectively, and the 1-, 2- and 3-year LCR were 53.4%, 40.0% and 34.2% respectively. The 1-, 3- and 5-year OS rates in the surgery combined with chemoradiotherapy group were 74.1%, 50.1% and 44.6%, and those in the chemoradiotherapy group were 49.0%, 29.3% and 12.8%, with a statistically significant difference ( χ2=5.142, P=0.023). The 1-, 2- and 3-year PFS rates in the surgery combined with chemoradiotherapy group were 62.1%, 54.3% and 44.4%, and those in the chemoradiotherapy group were 43.1%, 30.6% and 26.7%, with no statistically significant difference ( χ2=3.222, P=0.073). The 1-, 2- and 3-year LCR of the surgery combined with chemoradiotherapy group were 69.8%, 54.3% and 44.4%, and those in the chemoradiotherapy group were 45.1%, 32.9% and 29.6%, with no statistically significant difference ( χ2=3.576, P=0.059). The results of univariate analysis showed that tumor T stage ( χ2=7.140, P=0.008), N stage ( χ2=4.493, P=0.034) and treatment method ( χ2=5.142, P=0.023) were all independent influencing factors of the OS of patient with advanced hypopharyngeal squamous cell carcinoma; T stage ( χ2=5.807, P=0.016) and N stage ( χ2=6.587, P=0.010) were both independent influencing factors of PFS. The results of multivariate analysis showed that tumor T stage ( HR=2.121, 95% CI: 1.142-3.938, P=0.017), N stage ( HR=2.088, 95% CI: 1.144-3.811, P=0.016) and treatment method ( HR=0.430, 95% CI: 0.226-0.815, P=0.010) were all independent prognostic factors of the OS of patients with advanced hypopharyngeal squamous cell carcinoma; T stage ( HR=1.884, 95% CI: 1.011-3.510, P=0.046) and N stage ( HR=1.904, 95% CI: 1.058-3.429, P=0.032) were both independent prognostic factors of PFS. During the treatment period, there were statistically significant differences in the incidences of radioactive pharyngitis [7.41% (2/27) vs. 39.22% (20/51), χ2=8.821, P=0.003] and radioactive dermatitis [3.70% (1/27) vs. 29.41% (15/51), χ2=7.156, P=0.007] between the surgery combined with chemoradiotherapy group and the chemoradiotherapy group. However, there were no statistically significant differences in the incidences of radioactive oral mucositis [11.11% (3/27) vs. 17.65% (9/51), χ2=0.186, P=0.666], bone marrow suppression [37.04% (10/27) vs. 50.98% (26/51), χ2=1.381, P=0.240], pharynx infection [11.11% (3/27) vs. 5.88% (3/51), χ2=0.143, P=0.706] and tracheal fistula [7.41% (2/27) vs. 0 (0/51), P=0.117] between the two groups. Conclusion:The 1-, 3- and 5-year OS rates in the surgery combined with chemoradiotherapy group are higher than those in the chemoradiotherapy group, and the incidences of adverse reactions are low. T stage, N stage and treatment method are independent prognostic factors for OS of advanced hypopharyngeal squamous cell carcinoma patients, while T stage and N stage are independent prognostic factors for PFS.

Objective To investigate the effect and side effects of flurbiprofen axetil combined with fentanyl in postoperative patient-controlled analgesia (PCA) after craniotomy.Methods 60 patients undergoing scheduled craniotomy were randomly divided into the treatment group and control group with 30 cases in each group, and received flurbiprofen axetil combined with fentanyl and only fentanyl respectively in PCA after craniotomy. The visual analogue scale (VAS), sedation score (SS) and the side effects were evaluated in 24 h after craniotomy.Results There was no significant difference in VAS between the two groups ( P>0.05). The SS, side effects such as nausea and vomiting in the treatment group were significantly less than that in the control group ( P<0.05).Conclusion Flurbiprofen axetil combined with fentanyl in PCA can reduce the side effects of fentanyl and offers overall favorable analgesic responses.

Objective To explore the characteristic of transcranial color-coded sonography(TCCS)and contrast-enhanced transcranial color-coded sonography (CE-TCCS) in patients with intracerebral hemorrhage and thiers clinical value.Methods 66 patients were randomly selected,whose preliminary clinical diagnosis were intracerebral hemorrhage (ICH).The patients were followed up by TCCS in acute phase,subacute phase and chronic phase.The changes of the echo,boundary and the hematoma volume were recorded in different stages.In acute phase,patients' complications of intracerebral structure were observed.32 patients were selected randomly to underwent CE-TCCS examination in the subacute phase.The size,shape and the perfusion situation of hematoma were observed.The results of CE-TCCS were compared with the results of TCCS.Correlation analysis was made between the results of ultrasound and CT scan.Results (1) CT results:61 patients (61/66) were confirmed ICH through CT scan.(2) TCCS results:50 patients (50/61) with ICH could show the bleeding site.TCCS showed that 33 patients with ICH accompanied by intraventricular pressure,haematoma defeats ventricle and midline shift in acute phase.The echo became lower and the boundary became more clear with time.The long diameter,wide diameter,thickness diameter and volume of hematoma in different stages on TCCS had a good correlation with that on CT scan.(3)CE-TCCS results:30 cases of intracerebral hematoma could be clearly displayed the situation of hematoma through the ipsilateral temporal window.Compared with TCCS,CE TCCS had a better correlation with CT scan on the measurement of the hematoma length,width.The images of 2 cases observed through contralateral temporal window failded to be clearly shown.In 7 cases of ICH,visible low-enhanced edema area could be seen around the hematoma.The width of the edema area had a good correlation with the CTP result.Conclusions TCCS could clearly show the bleeding sites,hematoma volume and complications and the features of ICH in different stages of disease.TCCS could be used to monitor the condition of patients with ICH and recognized the disease progression initially.CE-TCCS had a much more clear display of intracerebral hematoma location,shape,boundary.At the same time,CE-TCCS could provide blood perfusion information of surrounding tissue in hematoma so that it could observe the change of peripheral edema more convenient.

Objective To evaluate the diagnostic performance of Xpert MTB/RIF for the diagnosis of tuberculous meningitis (TBM).Methods This was a prospective, single center clinical trial.A total of 116 consecutive patients with suspected meningitis who were admitted to Xijing Hospital from October 2013 to June 2015 were recruited.Mycobacterium tuberculosis ( MTB) and rifampicin ( RIF) resistance mutations in 1 ml cerebrospinal fluid ( CSF) were detected with Xpert MTB/RIF and the remaining sample was tested by Ziehl-Neelsen staining , MGIT960 liquid culture and other laboratory tests .And the enrolled patients were grouped according to the 2010 South African expert consensus .The diagnostic performance of Xpert MTB/RIF was evaluated by comparing against clinical score >5 points and MGIT960 liquid culture as reference standards respectively .The comparison was performed using a χ2 test or Fisher′s exact test for categorical variables and a nonparametric rank sum test for continuous variables .Results Among the enrolled 116 subjects, 23 subjects were diagnosed as definite-TBM by MGIT960 liquid culture, 16 subjects were classified as probable TBM , 27 subjects were classified as possible TBM , and 50 subjects were classified as non-TBM.When clinical score >5 points was used as a reference standard , the sensitivity of Xpert MTB/RIF (39.4%) was comparable with that of MGIT960 liquid culture (34.8%) (χ2 =0.292, P=0.589), and significantly better than that of Ziehl-Neelsen staining (9.9%) (χ2 =16.500, 12.771, P<0.001). No significant differences were found among the specificities of Xpert MTB /RIF, MGIT960 liquid culture and Ziehl-Neelsen staining ( 98.0%, 100.0% vs 98.0%, χ2 =1.014, P=0.602 ) .When tested against MGIT960 liquid culture as a reference standard , the sensitivity of Xpert MTB/RIF was 91.3%. Conclusions Xpert MTB/RIF is a rapid and specific method to detect MTB and RIF resistance in CSF .It exhibits a good rule in value for the diagnosis of TBM and a comparable sensitivity with MGIT 960 liquid culture, thus it can be used as the initial method for the diagnosis of tuberculous meningitis .

Objective To compare the dosimetric difference between jaw tracking technique (JTT) and static jaw technique (SJT) in dynamic intensity-modulated radiotherapy (IMRT) for preoperative radiotherapy of rectal cancer patients.Methods Jaw tracking and static jaw were used to develope the intensity-modulated plans for 10 patients respectively.For all the patients,the dose to surrounding tissues was minimized as low as possible,the 95％ volume of the planning target volume (PTV) and planning gross target volume (PGTV) satisfy the prescribed dose.The doses of the planning target volumes,organs at risk and normal tissue were detected by dose-volume histogram.Two groups of treatment plan dose were verified by ionization chamber array 2D-Array 729 and OCTAVIUS (PTW) phantom.Results The treatment plans of two groups could satisfy the clinical requirements.There was no significant difference between the maximum and the mean dose of target.The volumes of jaw tracking dynamic intensity-modulated radiotherapy were lower,including the V5,V10,V20,V30,V40 (volumes receiving 5,10,20,30 and 40 Gy,respectively),mean dose(D) for body and V10,V20,V30,D for bilateral femoral head,bladder,and small intestine.There was significant difference for the results (t =-2.32-12.24,P ＜0.05).The verification results showed that the treatment plans were all passed the dosimetric verification.Conclusions Jaw tracking intensity-modulated radiotherapy and jaw fixed IMRT plan could achieve equal dose coverage in patients with rectal cancer,while jaw tracking techniques could reduce normal tissue dose and organs at risk dose.

Objective To find the best model parameters through Monte Carlo simulation of 6 MV flattening-filter-free (FFF) beams in TrueBeam accelerator, and establish the foundation for the further study of the clinical dosimetry on 6 MV FFF X-rays.Methods Using the BEAMnrc and DOSXYZnrc codes, the percentage depth dose (PDD) and the off-axis ratio (OAR) curves of field ranges from 4 cm ×4 cm to 40 cm × 40 cm were simulated for 6 MV FFF X-ray by adjusting the incident beam energy, radial intensity distribution and angular spread, respectively.The simulation results and measured data were compared, where the optimal Monte Carlo model input parameters were acquired.Results The simulation was most comparable to the measurement when the incident electron energy, full width at half maximum (FWHM) and the spread angle were set as 6.1 MeV, 0.75 mm and 0.9°, respectively.The deviation of 1 mm (position)/1％ (local dose) could be met by the PDD of all tested field sizes and by the OAR when the fields sizes were no larger than 30 cm ×30 cm.The OAR of 40 cm ×40 cm field sizes fulfilled criteria of 1 mm (position)/1.5％ (local dose).Conclusions Monte Carlo simulation agrees well with the measurement and the proposed model parameters, which can be used for further clinical dosimetry studies of 6 MV FFF X-rays.

Objective: To explore the clinical and anatomical characteristics of coronary slow lfow (CSF) in relevant patients.
Methods: We summarized the patients without coronary angiography (CAG) proved coronary stenosis (stenosis < 40%) while with TIMI indicated CSF in our hospital from 2013-01 to 2015-01. The patients were divided into 2 groups: CSF group, n=56 patients having at least 1 major coronary artery with TIMI frame counts > 27 and Control group,n=55 patients with normal coronary lfow. The related laboratory indexes were examined and relationship between MCV and CSF was studied by multi-logistic regression analysis.
Results: In CSF group, MCV 90.4 (87.48, 92.65) fL and RDW-CV 12.5 (12.30, 13.18) % were lower than those in Control group 92.3 (90.1, 94.3) fL and 13(12.7, 13.4) %,P<0.05; while MCHC 337 (332, 347) g/L and the number of left circumlfex distal braches involved 3 (2, 4) were higher than those in Control group 327.5 (322, 338) g/L and 2 (2, 3),P<0.05. Multi-logistic regression analysis showed that MCV was negatively related to CSF (partial regression coefficient= -0.138, P=0.015), Spearman rank correlation analysis presented that MCV was negatively related to TIMI frame counts (r= -0.201, P=0.009).
Conclusion: Deformability of red blood cells might be involved in pathogenesis of CSF in relevant patients.

OBJECTIVE To explore the protective effect of polysaccharides from Salvia Chinensis Benth. (PSSC) on lipopolysaccharide (LPS) and D- galactosamine (GalN)- provoked mouse acute liver failure (ALF) and the possible molecular mechanism. METHODS Kunming mice were randomly divided into four groups: normal control, model, model+PSSC 30 and 100 mg·kg- 1 groups. PSCC was given once a day and for a week. To establish an ALF model, mice of model and PSSC groups were ip injected with LPS 10 μg·kg-1 and GalN 700 mg·kg-1 at the end of PSSC treatment. The microscopic structure of the liver was detected by HE staining. Serum and hepatic biochemical parameters of glutamic-oxalacetic transaminase (GOT), glutamic- pyruvic transaminase (GPT), catalase (CAT), superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), malondialdehyde (MDA), and glutathione (GSH) were detected by colorimetric methods. The relative content of hepatic reactive oxygen species (ROS) was measured by DCFH-DA fluorescent probes. Levels of cytokines tumor necrosis factor-α (TNF-α), interleukin 1β(IL-1β) and IL- 6 in the serum and liver were detected by ELISA. Activity of caspase 3 in liver homogenates was detected by aspase 3 activity assay kit. RESULTS Compared with normal control group, in the liver of model group, hepatocytes were arrayed in disorder, cytoplasm of hepatocytes shrank, and boundaries between cells were fuzzy, the infiltration of a large number of inflammatory cells and tissue hemorrhage could be detected, pathological scores were elevated significantly (P<0.01), levels of MDA and ROS in the liver of ALF model mice were elevated to 2.2 and 4.3 times that of the normal control, respectively (P<0.01), the level of GSH decreased to 51% (P<0.01), and the activities of SOD, CAT and GSH- Px declined to 74%, 36% and 42%, respectively (P<0.01). Levels of TNF- α, IL- 1β and IL- 6 in the serum and liver of model group were increased (P<0.01), and caspase 3 activity was increased to 5.3 times that of the normal control (P<0.01). Compared with the model group, the number of surviving mice in PSSC groups increased, liver pathological scores declined (P<0.01), levels of MDA and ROS increased (P<0.01), levels of GSH, TNF-α, IL-1β and IL-6 in the liver and serum declined (P<0.01), and caspase 3 activity decreased (P<0.01). CONCLUSION PSSC is able to alleviate LPS and GalN-induced ALF in mice. Inhibition of hepatic oxidative stress, inflammatory response, and cell apoptosis is possibly implicated in the protective effect of PSSC.