2013年我国克雅氏病监测病例特征分析及随访合作程度影响因素的研究
发布时间：2018-08-07 19:22
【摘要】：可传播性海绵状脑病(Transmissible Spongiform Encephalopathies, TSEs)是由朊病毒引起的可导致人类及多种动物中枢神经系统发生不可逆退行性病变的一类疾病,潜伏期长,致死率为100%。目前认为其致病因子是一种不含核酸、具有自我复制能力的感染性朊蛋白粒子,因此,此类疾病又称朊病毒病。目前已经在人类以及20余种动物中发现有自然发生或感染的TSEs。人类的TSEs,包括克雅氏病(Creutzfeldt-Jakob disease, CJD)、致死性家族型失眠症(Fatal Familial Insomnia, FFI)、吉斯特曼-施特劳斯综合征(Gerstmann-Straussler-Scheinker syndrome, GSS)和库鲁病(Kuru)。动物的朊病毒病包括牛海绵状脑病(Bovine Spongiform Encephalopathy, BSE)、羊瘙痒病(Scrapie)、骡鹿和麇鹿的慢性消耗性疾病(Chronic Wasting Disease, CWD)、可传播性貂脑病(Transmissible Mink Encephalopathy, TME)、猫科动物海绵状脑病(Feline Spongiform Encephalopathy, FSE)等。CJD根据发病机制的不同可以分为4类：散发型CJD (sporadic CJD, sCJD)、家族遗传型CJD (genetic CJD or familial CJD, gCJD or fCJD)、医源型CJD(iatrogenic CJD, iCJD)及变异型CJD (variant CJD, vCJD)。 本文分为两部分,第一部分是对我国2013年CJD监测病例进行特征分析,研究我国CJD的发病情况、流行病学及临床特征。第二部分是根据我国CJD监测系统的数据,对2013年1-9月监测系统中收集的CJD病人通过电话随访的方式获取病人和受访者的信息,探寻这些因素对受访者接受随访合作程度的影响。 第一部分：2013年我国克雅氏病监测病例特征分析 为了解我国(未包括香港、澳门和台湾地区,以下同)克雅氏病(CJD)的发病情况、流行病学及临床特征,对2013年我国克雅氏病监测网络获得的可疑CJD病例的临床及流行病学资料进行分析,收集患者脑脊液及血液样品,利用Western blot方法检测脑脊液中14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对PRNP基因进行129位和219位氨基酸多态性及基因是否存在突变进行分析。 2013年,共有299份送检病例,其中诊断为sCJD临床诊断病例116例(38.80%),疑似sCJD病例25例(8.36%),gCJD病例9例(3.01%),FFI病例3例(1.00%)。病例报告无季节聚集性,长久居住地呈散在分布,职业分布广泛。临床诊断病例年龄中值为61岁(32,86),男女比例为0.87：1；疑似诊断病例年龄中值为53岁(21,78),男女性别比例为0.79：1。在病例出现的临床症状中,快速进行性痴呆为最常见的首发症状。另外同时出现3种以上典型临床表现的病例所占的比例在临床诊断病例组中明显高于疑似诊断病例组。分析比较脑脊液14-3-3蛋白、脑电图(electroencephalograph, EEG)以及头颅核磁共振(Magnetic Resonance Imaging, MRI)三项检测结果,出现阳性结果越多的病例表现出更多的典型症状。对283份病例血液样品PRNP基因多态性检测发现,278例129位氨基酸为甲硫氨酸纯合子(M/M),5例129位氨基酸为甲硫氨酸/缬氨酸杂合子(M/V)；277例219位氨基酸为赖氨酸纯合子(E/E),6例219位氨基酸为赖氨酸/谷氨酸杂合子(E/K)。 2013年我国监测系统中克雅氏病的发病时间、长久居住地、职业、性别比例以及年龄分布均符合散发型克雅氏病的发病特点。临床诊断病例表现出更多的典型临床症状和临床实验室阳性检测结果。2013年共发现12例遗传型朊病毒病病例,其中D178N、E200K和T188K为中国汉族人群中最常见的PRNP基因突变类型。 第二部分：克雅氏病病例随访合作程度影响因素的研究 中国自2006年开展了人类朊病毒病监测以来,随访作为CJD监测日常工作的一部分,对于明确该病的诊断一直发挥着很重要的作用。在随访的方式中,电话随访相对于其它随访方式以其可行性强的特点,成为最常采用的一种随访方式。尽管电话随访是由中国疾病预防控制中心CJD豁测中心拥有多年监测经验的专业技术人员进行,但是受访对象在接受随访时的合作程度各有差异,不合作的情况也时有发生。本研究共纳入2013年监测系统1-9月份收集到的199份病例,分别从13个涉及患者和5个涉及受访者方面的信息设计了调查问卷,目的在于探索影响受访对象合作程度的因素。受访者的合作程度作为因变量被分为不合作,基本合作,非常合作三个等级。在经过单因素和有序回归两步分析后发现患者的性别、CJD诊断结果、出现典型临床表现个数、治疗方案、是否坚持治疗、受访者与患者关系以及受访者对CJD的认识等七个因素对合作程度有显著性影响。这是全国在CJD的监测随访过程中,首次对影响受访者合作程度的因素开展研究,该研究有助于在监测CJD病例发病特征过程中,进一步提高随访工作的质量。
[Abstract]:Transmissible Spongiform Encephalopathies (TSEs) is a kind of disease caused by prion causing irreversible degenerative diseases in the central nervous system of human and various animals. It has a long latent period and the mortality rate is 100%.. It is considered as a kind of non nucleic acid and has the ability of self replicating. The infectious prion particles, therefore, are also known as prion diseases. TSEs of TSEs. humans, including Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (Fatal Familial Insomnia, FFI), and gilltman Strauss heald, have been found in humans and more than 20 species of animals. Gerstmann-Straussler-Scheinker syndrome, GSS and Kuru. Prion diseases in animals include bovine spongiform encephalopathy (Bovine Spongiform Encephalopathy, BSE), sheep pruritus (Scrapie), mule deer and deer's chronic wasting disease (Chronic Wasting Disease,), and transmissible mink encephalopathy Athy, TME), cat spongiform encephalopathy (Feline Spongiform Encephalopathy, FSE) and other.CJD can be divided into 4 categories according to the different pathogenesis: scattered CJD (sporadic CJD, sCJD), familial genetic CJD, and variant forms.
This article is divided into two parts. The first part is the characteristic analysis of CJD monitoring cases in 2013 in China, the study of the incidence, epidemiology and clinical features of CJD in China. The second part is based on the data of the CJD monitoring system in China, to obtain patients and interviewees through the telephone follow-up of the CJD patients collected in the 1-9 month monitoring system in 2013. Information to explore the impact of these factors on the degree of interviewees' follow-up cooperation.
Part one: characteristics of surveillance of Creutzfeldt Jakob disease in China in 2013
In order to understand the incidence, epidemiology and clinical features of CJD (CJD), the clinical and epidemiological data of suspected CJD cases obtained from the 2013 surveillance network in China were analyzed, the cerebrospinal fluid and blood samples were collected, and the Western blot method was used to detect the disease. 14-3-3 protein in cerebrospinal fluid (CSF) was used to extract whole blood genomic DNA and to analyze the polymorphism of 129 and 219 bit amino acids and the existence of mutations in the PRNP gene by PCR and sequencing.
In 2013, there were 299 cases of examination, of which 116 cases (38.80%) were diagnosed as sCJD clinical cases, 25 cases (8.36%), 9 cases (3.01%) of gCJD cases and 3 cases (1%) of FFI cases. The cases reported no seasonal aggregation, the long residence was scattered and the occupational distribution was wide. The median age of clinical diagnosis was 61 years (32,86), the ratio of men and women was to the ratio of men and women. 0.87:1, the median age of suspected cases is 53 years (21,78), and the sex ratio of men and women is 0.79:1. in the clinical symptoms of the case, and rapid progressive dementia is the most common first symptom. In addition, the proportion of cases with more than 3 typical clinical manifestations is significantly higher than the suspected diagnosis case in the clinical diagnosis case group. Analysis and comparison of three items of 14-3-3 protein in cerebrospinal fluid, electroencephalograph (EEG) and Magnetic Resonance Imaging (MRI), and more cases with more positive results showed more typical symptoms. The polymorphism of PRNP gene of blood samples in 283 cases was found to be 278 cases of 129 amino acids. Methionine homozygote (M/M), 129 amino acids in 5 cases were methionine / valine heterozygote (M/V), 277 cases of 219 amino acids were lysine homozygote (E/E), and 6 cases of 219 amino acids were lysine / glutamic heterozygote (E/K).
In 2013, the time of the disease, the place of residence, the occupation, the sex ratio and the age distribution were all conformed to the characteristics of the disease. The clinical diagnosis cases showed more typical clinical symptoms and clinical laboratory test results, and 12 cases of hereditary prion disease were found in.2013 years. Middle D178N, E200K and T188K were the most common PRNP gene mutation types in Chinese Han population.
The second part: Research on the factors influencing the degree of cooperation in patients with Creutzfeldt Jakob disease.
Since the monitoring of human prion disease in 2006, follow up as part of the daily work of CJD monitoring has been playing an important role in the diagnosis of the disease. In the follow-up mode, telephone follow-up is the most frequently used follow-up mode compared with other follow up methods. The telephone follow-up was carried out by professional technicians who had years of monitoring experience at the CJD center of China Center for Disease Control and prevention. However, there were differences in the degree of cooperation between the respondents and the cases of non cooperation. This study included 199 cases collected in the 2013 monitoring system from 13, respectively, from 13. A questionnaire was designed for patients and 5 interviewees. The purpose of the questionnaire was to explore the factors affecting the degree of cooperation among the respondents. The degree of cooperation among the respondents was divided into non cooperation, basic cooperation, and very cooperative three levels. The gender of the patient was found after the two step analysis by the single factor and the sequential regression, CJD The seven factors, such as the results of the diagnosis, the number of typical clinical manifestations, the treatment plan, the adherence to the treatment, the relationship between the interviewees and the patients, and the awareness of the interviewees on the CJD have a significant impact on the degree of cooperation. This is the first time to study the factors affecting the degree of cooperation of the respondents in the follow-up process of the CJD monitoring. In the course of monitoring the characteristics of CJD cases, the quality of follow-up work is further improved.
【学位授予单位】：中国疾病预防控制中心
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R742
本文编号：2171101
[Abstract]:Transmissible Spongiform Encephalopathies (TSEs) is a kind of disease caused by prion causing irreversible degenerative diseases in the central nervous system of human and various animals. It has a long latent period and the mortality rate is 100%.. It is considered as a kind of non nucleic acid and has the ability of self replicating. The infectious prion particles, therefore, are also known as prion diseases. TSEs of TSEs. humans, including Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (Fatal Familial Insomnia, FFI), and gilltman Strauss heald, have been found in humans and more than 20 species of animals. Gerstmann-Straussler-Scheinker syndrome, GSS and Kuru. Prion diseases in animals include bovine spongiform encephalopathy (Bovine Spongiform Encephalopathy, BSE), sheep pruritus (Scrapie), mule deer and deer's chronic wasting disease (Chronic Wasting Disease,), and transmissible mink encephalopathy Athy, TME), cat spongiform encephalopathy (Feline Spongiform Encephalopathy, FSE) and other.CJD can be divided into 4 categories according to the different pathogenesis: scattered CJD (sporadic CJD, sCJD), familial genetic CJD, and variant forms.
This article is divided into two parts. The first part is the characteristic analysis of CJD monitoring cases in 2013 in China, the study of the incidence, epidemiology and clinical features of CJD in China. The second part is based on the data of the CJD monitoring system in China, to obtain patients and interviewees through the telephone follow-up of the CJD patients collected in the 1-9 month monitoring system in 2013. Information to explore the impact of these factors on the degree of interviewees' follow-up cooperation.
Part one: characteristics of surveillance of Creutzfeldt Jakob disease in China in 2013
In order to understand the incidence, epidemiology and clinical features of CJD (CJD), the clinical and epidemiological data of suspected CJD cases obtained from the 2013 surveillance network in China were analyzed, the cerebrospinal fluid and blood samples were collected, and the Western blot method was used to detect the disease. 14-3-3 protein in cerebrospinal fluid (CSF) was used to extract whole blood genomic DNA and to analyze the polymorphism of 129 and 219 bit amino acids and the existence of mutations in the PRNP gene by PCR and sequencing.
In 2013, there were 299 cases of examination, of which 116 cases (38.80%) were diagnosed as sCJD clinical cases, 25 cases (8.36%), 9 cases (3.01%) of gCJD cases and 3 cases (1%) of FFI cases. The cases reported no seasonal aggregation, the long residence was scattered and the occupational distribution was wide. The median age of clinical diagnosis was 61 years (32,86), the ratio of men and women was to the ratio of men and women. 0.87:1, the median age of suspected cases is 53 years (21,78), and the sex ratio of men and women is 0.79:1. in the clinical symptoms of the case, and rapid progressive dementia is the most common first symptom. In addition, the proportion of cases with more than 3 typical clinical manifestations is significantly higher than the suspected diagnosis case in the clinical diagnosis case group. Analysis and comparison of three items of 14-3-3 protein in cerebrospinal fluid, electroencephalograph (EEG) and Magnetic Resonance Imaging (MRI), and more cases with more positive results showed more typical symptoms. The polymorphism of PRNP gene of blood samples in 283 cases was found to be 278 cases of 129 amino acids. Methionine homozygote (M/M), 129 amino acids in 5 cases were methionine / valine heterozygote (M/V), 277 cases of 219 amino acids were lysine homozygote (E/E), and 6 cases of 219 amino acids were lysine / glutamic heterozygote (E/K).
In 2013, the time of the disease, the place of residence, the occupation, the sex ratio and the age distribution were all conformed to the characteristics of the disease. The clinical diagnosis cases showed more typical clinical symptoms and clinical laboratory test results, and 12 cases of hereditary prion disease were found in.2013 years. Middle D178N, E200K and T188K were the most common PRNP gene mutation types in Chinese Han population.
The second part: Research on the factors influencing the degree of cooperation in patients with Creutzfeldt Jakob disease.
Since the monitoring of human prion disease in 2006, follow up as part of the daily work of CJD monitoring has been playing an important role in the diagnosis of the disease. In the follow-up mode, telephone follow-up is the most frequently used follow-up mode compared with other follow up methods. The telephone follow-up was carried out by professional technicians who had years of monitoring experience at the CJD center of China Center for Disease Control and prevention. However, there were differences in the degree of cooperation between the respondents and the cases of non cooperation. This study included 199 cases collected in the 2013 monitoring system from 13, respectively, from 13. A questionnaire was designed for patients and 5 interviewees. The purpose of the questionnaire was to explore the factors affecting the degree of cooperation among the respondents. The degree of cooperation among the respondents was divided into non cooperation, basic cooperation, and very cooperative three levels. The gender of the patient was found after the two step analysis by the single factor and the sequential regression, CJD The seven factors, such as the results of the diagnosis, the number of typical clinical manifestations, the treatment plan, the adherence to the treatment, the relationship between the interviewees and the patients, and the awareness of the interviewees on the CJD have a significant impact on the degree of cooperation. This is the first time to study the factors affecting the degree of cooperation of the respondents in the follow-up process of the CJD monitoring. In the course of monitoring the characteristics of CJD cases, the quality of follow-up work is further improved.
【学位授予单位】：中国疾病预防控制中心
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R742
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