With the development of biological techniques,the study on the pathogenesis of disease-causing genes of congenital cataracts has substantial progress.Some positive results of screen of mutation gene in congenital cataract family has been reported,but the report of negative result is rate.ObjectiveThe present study attempts to screen the mutation of CRYAA,CRYAB,CRYA1/A3,CRYBB2,CRYGC and CRYGD gene in a Chinese family with autosomal dominant congenital cataract. MethodsThe periphery blood samples were exacted from 8 patients of 4 generations of with congenital cataract in this family.The complete coding region and intron spliced sites of CRYAA,CRYAB,CRYA1/A3,CRYBB2,CRYGC and CRYGD were amplified with polymerase chain reaction (PCR),and the products of PCR were directly sequenced.The control blood samples were from 10 normal subjects.This study followed the Declaration of Helsinki.Written informed consent was obtained from all of the patients.ResultsThe patients were found in each generation in this family and the mode of inheritance was in accordance with the characteristic of autosomal dominant inheritance.The sequence of amplified genetic fragments of CRYAA,CRYAB,CRYA1/A3,CRYBB2,CRYGC and CRYGD genes were inaccordance with those of normal subjects and GeneBank.No any mutation loci was found in all of the patients of this family.ConclusionCRYAA,CRYAB,CRYA1/A3,CRYBB2,CRYGC and CRYGD genes is not the causing-disease genes in this family.

Objective:To analyze the pros and cons of the US strategic national stockpile ( SNS) from the per-spective of the supply chain and to put forward suitable recommendations for China’s national emergency medicine system. Methods:Literature review and a comprehensive analysis have been utilized to understand the SNS supply chain model and its differences from the Chinese model. Results:From the perspective of the supply chain, the Unit-ed State’s storage of emergency medicine consists of the forward placement of push packages, managed inventory and purchase contracts. The deployment of logistics for emergency medicine must gain approval from the federal government’s “local-state-nation” system, and be conducted at a different time. To transmit information, a large da-tabase, simulation software, and a technical advisory response unit have been established to manage information flow to distribute medicine accurately and promptly. Conclusion:Given the experiences of supply chain management in the US SNS system, it is critical for China to improve the supply chain system of emergency medicine, define the institu-tions and responsibilities within the supply chain, establish disaster relief reserve of emergency medicine and adopt fourth-party logistics for emergency medicine in order to make the China’s emergency preparedness more systemized, standardized, and information-based.

OBJECTIVE: To explore the diagnosis, treatment and surgical approaches of parapharyngeal space tumors. METHOD: This retrospective study consisted of 112 patients with parapharyngeal space tumors underwent surgeries. The data included clinical symptoms and signs, pathological types, imaging examinations, surgical approaches and postoperative complications. RESULT: Computerized tomography (CT), magnetic resonance imaging (MRD and digital subtraction angiography (DSA) made clear the tumor size, localization and its relation to adjacent structures. The postoperative histopathology varied and showed benign in 98 cases consisting of salivary gland tumors (52 cases), neurogenic tumors(33 cases) and other types (13 cases). During the 3 years follow-up period, local recurrence appeared in 3 patients with salivary pleomorphic adenoma and 1 patient with neurofibromatosis. These recurrent tumor cases were cured with second surgery. In 14 patients with malignant tumors reported, 8 cases survived for 5 years, 2 cases (1 adenoid cystic carcinoma and 1 carcinoma in pleornorphic adenoma) recurred in the two and a half years cured with second surgery affiliated radiation therapy through 3 years follow-up time and the rest were followed up 1 to 3 years without recurrence. Peripheral facial paralysis was observed in 25 patients, and 3 patients experienced hypoglossal nerve palsy. Only 1 patient encountered vagus nerve injury, and 2 patients appeared Horner's syndrome, and 4 patients endured Frey syndrome. Gills leakage was discovered in 3 cases and cavity infection was noted in 1 patient. CONCLUSION CT, MRI and DSA were important in the diagnosis and differential diagnosis of parapharyngeal space tumors. The key to successful treatment is knowing the anatomy of the parapharyngeal space, preoperative assessment and appropriate surgical approach.
Adenoma, Pleomorphic ; complications ; Carcinoma, Adenoid Cystic ; complications ; Diagnosis, Differential ; Facial Paralysis ; complications ; Horner Syndrome ; complications ; Humans ; Magnetic Resonance Imaging ; Neoplasm Recurrence, Local ; Pharyngeal Neoplasms ; diagnosis ; therapy ; Pharynx ; pathology ; Postoperative Complications ; Postoperative Period ; Retrospective Studies ; Salivary Gland Neoplasms ; complications ; Tomography, X-Ray Computed

OBJECTIVEThis study aimed to improve students' ability in practical and theoretical courses of oral health education and to promote students' learning interest and initiative.

METHODSFourth-year students of the oral medical profession from 2006 to 2008 at Weifang Medical University were chosen as research objects for oral health education to explore the experimental teaching reform. The students were divided into test and control groups, with the test group using the "speak out" way of teaching and the control group using the traditional teaching method. Results of after-class evaluation of the test group, as well as final examination and practice examination of the two groups, were analyzed and compared.

RESULTSAfter-class evaluation results of the test group showed that the "speak out" teaching method was recognized by the students and improved students' ability to understand oral health education. The final examination and practice examination results showed that the score of the test group was higher than that of the control group (P < 0.01).

CONCLUSION"Speak out" teaching methods can improve students' ability for oral health education, in accordance with the trend of teaching reform.

Objective To discuss the cause and treatment strategy for open laryngotracheal trauma.Methods The clinical data of 38 cases with open laryngotracheal trauma admitted from 1998 to 2012 were collected and analyzed.Of them,one patient died in emergency department despite energetic resuscitation,37 were hospitalized and given advanced treatment after life support measures in emergency department.They were treated with tracheotomy before or during operation.Of them,32 patients were regularly followed up for imaging studies.Result Except 1 patient died of exsanguination from carotid artery rupture,the other 37 survived after successful treatment.Twenty patients were treated with debridement and suture of the wounds and laryngoplasty (20/37,54.1％),8 patients were operated with laryngoplasty plus intraluminal stents implanted (8/37,21.6％) ; the rest 9 patients (9/37,24.3％) were separately given placement of nickel-titanium shape memory alloy stent (n =2),laryngofissure with rubber gloves throat models implanted and laryngoplasty (n =3),linear silicone tube implanted and laryngoplasty with trans-cervical approach (n =1) and tracheoesophageal fistula neoplasty with laryngoplasty and laryngofissure (n =3).The operated patients were followed-up for 0.5 to 3 years after discharge.Of them,35 patients (35/37,94.6％) had successful decannulation,breathing smoothly and swallowing normally,23 patients (23/37,62.2％) had almost normal pronunciation,12 patients (12/37,32.4％) had different degrees in hoarseness of voice,and 2 patients (2/37,5.4％) were referred to other hospital because of failure in extubation with severe stenosis of laryngotrachea.Conclusions When the patients with open laryngotracheal trauma were treated,the essential strategy was to prevent shock,hemorrhage,and asphyxia.In the case of patent respiratory tract and stable vital signs,laryngotracheal reconstruction should be carried out as soon as possible to prevent complications,thereby obtaining good therapeutic effect.

Objective:To explore the implicit attitude and explicit attitude toward death of college students,and the relationship between the two kinds of attitude.Methods:Totally 65 college studems participated in this study.The Single Category Implicit Association Test (SC-IAT,including cognition,affection and behavior) was used to exam the implicit attitude toward death of undergraduate students.The Death Attitude Profile-Revised (DAP-R,including five dimensions,i.e.,fear of death,death avoidance,death acceptance,escape acceptance,and neutral acceptance) was used to measure the explicit attitude.Results:Data of the SC-IAT indicated that the differences between the mean reaction time of the incompatible task and compatible in all three dimensions and the total SC-IAT were significant (Ps ＜0.01),and both the D scores of the total SC-IAT and three dimensions were larger than zero.The mean score of neutral acceptance was the highest in the five factors of SC-IAT.Relationships between the implicit effect of SC-IAT and the Mean scores of the DAP-R's five factors were not significant(Ps ＞ 0.05).Conclusion:The implicit attitude toward death of college students is negative,the explicit attitude toward death of college students is neutral.The implicit and explicit attitude toward death of college students is incongruent,and they are relatively independent.

Purpose To exp1ore the c1inicopatho1ogica1 features,diagnosis and differentia1 diagnosis of mu1ti1ocu1ar cystic rena1 ce11 carcinoma( MCRCC). Methods 18 cases of MCRCC were reported by microscopy,immunohistochemistry,differentia1 diagnosis and were fo11owed-up. Results A11 patients were adu1ts inc1uding twe1ve ma1es and six fema1es who aged from 26 to 68 years(mean 55. 6 years). Imaging studies revea1ed a po1ycystic mass,with c1ear boundary. Gross1y,the cut surface of the tumors had more cysts of va-rying sizes,containing serous or b1oody f1uid. Microscopica11y,the cyst wa11s of tumors were often covered with a few simp1e c1ear ce11s,stratified epithe1ium or devoid of epithe1ium. The septa contained aggregates of epithe1ia1 ce11s with transparent cytop1asm which showed gradeⅠ nuc1ear features,these characteristics were diagnostic c1ues of MCRCC. Immunohistochemica11y the c1ear ce11 was positive for CD10,vimentin,EMA and Ki-67 showed 1ow pro1iferative activity. 18 case were fo11owed up,mean fo11ow-up 43 months, no case recurred or with metastasis. Conclusion MCRCC is a rare histo1ogica1 subtype of rena1 ce11 carcinoma with more favorab1e prognosis. It shou1d be distinguished from cystic change of c1ear ce11 rena1 carcinoma and cysts of kidney 1esion.

Objective To evaluate the efficacy of splenectomy plus selective pericardial devascularization under endoscope in the treatment of advanced schistosomiasis patients with portal hypertension and hypersplenism so as to explore the minimally in-vasive and safer surgical treatment. Methods A secure splenectomy was performed with laparoscope and its supporting devices, and at the same time,the ligation of the left gastric vein and the ligation of esophageal vein perforating vertically into the esopha-gus were also performed in 14 advanced schistosomiasis patients with portal hypertension and hypersplenism. Results Among the 14 patients,the splenic artery was separated and clipped before the treatment of splenic pedicle. One patient was of conversion to open laparotomy for the splenic vein rupture bleeding in the separation. There was no death. Conclusion The operation of sple-nectomy plus selective pericardial devascularization under endoscope is effective,truly minimally invasive,and safe in the treat-ment of advanced schistosomiasis patients with portal hypertension and hypersplenism.

Objective To explore the value of joint detection of serum cystatin C (Cys C) ,retinol‐binding protein(RBP) ,urea ni‐trogen(BUN) and creatinine(Cr) in early diagnosis of nephropathy .Methods A total of 230 cases of inpatients with nephropathy form April 2013 to June 2014 were collected and divided into diabetic nephropathy group (130 cases)and hypertensive nephropathy group(100 cases) .Other 200 healthy individuals were collected as healthy control group .The serum levels of Cys C ,RBP ,BUN and Cr were detected by using the Hitachi 7180 automatic biochemistry analyzer .Results Compared with the healthy control group ,the sertum levels of Cys C ,RBP ,BUN and Cr were significantly increased in the diabetic nephropathy group ,and the serum levels of Cys C and RBP were significantly increased in hypertensive nephropathy group ,there were statistically significant differences (P<0 .05);while no statistically significant differences in serum levels of BUN and Cr were found between the hypertensive nephropathy group and healthy control group(P>0 .05) .Positive rates(81 .9% ) of the four indicators in the two nephropathy groups were high‐er than those in the healthy control group ,and the positive rate of joint detection of the four indicators was higher than single detec‐tion in patients with nephropathy .Conclusion Serum RBP and Cys C are sensitive indicators for early renal damage ,and combined with BUN and Cr could increase sensitivity of early diagnosis of nephropathy ,which has significant clinical value .

Objective To explore a medical insurance model for rare diseases and orphan drugs applicable in China.Methods Eleven rare diseases from six disease systems and thirty-five orphan drugs covered were pinpointed for comparative analysis of their present coverage in various medical insurance systems in China.Results The coverage of 35 orphan drugs in the national essential drug list(2009)and the national essential drug list(2012)is 0 and 37.1％ respectively; the coverage in the national essential insurance medicine list is 54.3％ (19 types),and the average coverage in the medicine list for the New Rural Cooperative Medical Insurance Scheme is 33.6％.The study found insufficient rare diseases included in the Basic Medical Insurance Schemes in general.Conclusion The coverage of basic medical insurance for rare disease patients is low in China.In this regard,such measures should be taken to mitigate the economic burden for these patients,namely expanding the coverage of reimbursement list of orphan drugs,building the special reimbursement system of rare diseases and orphan drugs and improving the medical rescue system for rare diseases in China and their medical care level.