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.

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.

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.

Adenoma ; metabolism ; pathology ; surgery ; Aged ; Diagnosis, Differential ; Endodermal Sinus Tumor ; pathology ; Female ; Humans ; Immunohistochemistry ; Neprilysin ; metabolism ; Ovarian Neoplasms ; metabolism ; pathology ; surgery ; Sex Cord-Gonadal Stromal Tumors ; pathology ; Stromal Cells ; metabolism ; pathology ; Vimentin ; metabolism

BACKGROUND:Due to special physiological and anatomical location, stability of the spine is very complicated during thoracolumbar fractures. It is difficult to identify the stability of the spine. It should be based on their individual circumstances, to explore more effective internal fixation repair method.
OBJECTIVE:To explore the Cobb’s angle and vertebral height of patients with thoracolumbar fracture and spinal cord compression treated with posterior laminectomy and screw fixation, and compared with anterior laminectomy.
METHODS:One hundred patients with thoracolumbar fracture and spinal cord compression, who were treated in the Panyu District Central Hospital from January 2013 to November 2014, were enroled in this study. The patients were equaly and randomly divided into posterior laminectomy fixation group and anterior laminectomy fixation group. Tactile and sports of American Spinal Injury Association scores, Cobb’s angle and vertebral height were assessed before treatment and 1 month after treatment, and fixation effects were compared between the twogroups.
RESULTS AND CONCLUSION:(1) No significant difference in each index was detected between the two groups preoperatively (P> 0.05). (2) Tactile and sports of American Spinal Injury Association scores, Cobb’s angle and vertebral height were better in the posterior laminectomy fixation group than in the anterior laminectomy fixation group at 1 month postoperatively (P< 0.05). (3) These findings indicated that compared with the anterior laminectomy fixation, posterior laminectomy fixation for thoracolumbar fracture combined with spinal cord compression obtained better outcomes, and could obviously relieve spinal cord compression. Posterior laminectomy fixation isasafe and effective treatment method for thoracolumbar fracture and spinal cord compression.

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 .

Cardiovascular Diseases ; epidemiology ; prevention & control ; Cerebrovascular Disorders ; epidemiology ; prevention & control ; Humans ; Public Health Practice

Calcium Channel Blockers ; pharmacology ; Cell Survival ; drug effects ; Drug Antagonism ; Humans ; Kidney ; cytology ; drug effects ; Lead ; toxicity