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.

Humans ; Infant, Newborn ; Infant, Premature ; Infant, Premature, Diseases ; microbiology ; prevention & control ; Mycoses ; prevention & control

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

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

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