IgG4相关性疾病的临床病例评析

目的提高对IgG4相关性疾病颅内病变的认识。 方法描述1例IgG4相关性疾病（IgG4-RD）颅内病变患者的诊治过程,总结其临床、影像学、病理特点,诊断和鉴别诊断,治疗和预后。 结果22岁女性,头痛、左眼视力下降10个月。6个月前左眼失明,眼球固定,左眼球后占位,病理示大量淋巴浆细胞浸润,IgG4＋浆细胞>50/高倍视野,IgG4（＋）/IgG比值>10%,予足量激素及小剂量免疫抑制剂治疗病情维持原状。2个月前停药。半月前突发右眼视力下降伴头痛加重,5天前右眼失明伴烦渴多尿,高钠血症。头颅MRI提示右侧视神经、鼻窦、鞍区、左侧颞叶及邻近脑膜异常信号。脑脊液压力、细胞学、生化均正常。予大剂量激素冲击治疗,序贯减量,联合环磷酰胺冲击治疗,患者右眼视力逐步恢复至0.3,头痛完全缓解,尿量改善,血钠正常。40余天后复查头颅MRI本次新发病灶明显好转。 结论IgG4-RD颅内病变可同时累及多个部位,但不伴颅外表现,可出现病情快速进展,及时治疗可避免不可逆损害。     

2007年西藏自治区碘缺乏病高危地区重点人群调查分析

Objective To propose prevention and control strategy against iodine deficiency disorders to prevent new cases of endemic cretinism from occurring. Methods In April and August 2007, all the suspected cretinism patients born after the first of January, 1997 were searched for in 16 counties from Lhasa, Shannan, Nyingchi, Qamdo and Xigaze. Meanwhile, 60 children aged 8-10 years were selected in each of the two rural villages of each county chosen. Goiter was examined using palpation and B-ultrasound, urinary iodine was determined, inteligence quotient(IQ) was tested; 30 women of childbearing age from each village chosen underwent urinary iodine test and household salt semi-quantitative detection. Results No new cases of endemic cretinism had been found; Palpation identified goiter at 4.5% (257/5680) in children, B-ultrasound revealed a rate of 4.7% (258/5433). Median of urinary iodine was 159.4 μg/L, the averaged IQ was 78.3±14.5; women of childbearing age had a median urinary iodine of 70.2 μg/L. The coverage rate of iodized salt was 52.8%. Conclusions Goiter rate in children, urinary iodine level meet the standard set for the elimination of iodine deficiency disorders; however, the low IQ children, low coverage of iodized salt and the level of urinary iodine in women of childbearing age are less than desirable. Iodine deficiency disorders prevention and control need to be strengthened.     

2007年西藏自治区碘缺乏病高危地区重点人群调查分析

Objective To propose prevention and control strategy against iodine deficiency disorders to prevent new cases of endemic cretinism from occurring. Methods In April and August 2007, all the suspected cretinism patients born after the first of January, 1997 were searched for in 16 counties from Lhasa, Shannan, Nyingchi, Qamdo and Xigaze. Meanwhile, 60 children aged 8-10 years were selected in each of the two rural villages of each county chosen. Goiter was examined using palpation and B-ultrasound, urinary iodine was determined, inteligence quotient(IQ) was tested; 30 women of childbearing age from each village chosen underwent urinary iodine test and household salt semi-quantitative detection. Results No new cases of endemic cretinism had been found; Palpation identified goiter at 4.5% (257/5680) in children, B-ultrasound revealed a rate of 4.7% (258/5433). Median of urinary iodine was 159.4 μg/L, the averaged IQ was 78.3±14.5; women of childbearing age had a median urinary iodine of 70.2 μg/L. The coverage rate of iodized salt was 52.8%. Conclusions Goiter rate in children, urinary iodine level meet the standard set for the elimination of iodine deficiency disorders; however, the low IQ children, low coverage of iodized salt and the level of urinary iodine in women of childbearing age are less than desirable. Iodine deficiency disorders prevention and control need to be strengthened.     

2007年西藏自治区碘缺乏病高危地区重点人群调查分析

目的 掌握西藏自治区碘缺乏病防治工作现况,提出有针对性的防治对策,杜绝新发地方性克汀病(简称地克病)的发生.方法 2007年4-8月在拉萨、山南、林芝、昌都、日喀则5个地区的16个县,搜索所有1997年1月1日以后出生的疑似克汀病患者,在每个乡选择2个村,每个村选取60名8～10岁儿童,采用触诊法和B超法进行甲状腺检查、尿碘测定、智商测查;每个村选择30户育龄妇女进行尿碘测定,并对其家中食盐进行半定量检测.结果 调查未发现新发克汀病患者.触诊法检查,8～10岁儿童甲状腺肿大(简称甲肿)率为4.5%(257/5680);B超法检查,甲肿率为4.7%(258/5433).8～10岁儿童尿碘中位数为159.4μg/L,智商为78.3±14.5;育龄妇女的尿碘中位数为70.2μg/L;碘盐覆盖率为52.8%.结论 儿童甲肿率、尿碘水平达到了消除碘缺乏病标准;但是儿童智商偏低,育龄妇女尿碘水平较低,碘盐覆盖率较低,碘缺乏病的防治工作需要加强.     

2009年西藏自治区饮水型氟中毒病情监测结果分析

目的 了解西藏饮水型地方性氟中毒的病情动态,评价防治措施的效果,为及时调整防治策略提供科学依据.方法 按照"2008年中央补助地方公共卫生专项资金西藏饮水型氟中毒防治项目"技术方案,于2009年9-10月选取日喀则谢通门和林芝察隅两县作为项目县,在每个项目县采用单纯随机抽样的方法选择3个项目村,以自然村为单位,对改水及改水工程运转情况、饮水含氟量、儿童氟斑牙和成人临床氟骨症进行调查.按照<生活饮用水标准检验方法非金属指标>(GB/T 5750.6-2006)测定水氟;尿中氟化物的测定采用离子选择电极法(WS/T 89-1996);氟斑牙诊断采用Dean法;成人氟骨症诊断按<地方性氟骨症临床诊断标准>(WS192-2008)进行.结果 已改水病区水氟均值在0.18～0.34 mg/L,未改水病区水氟均值在0.70～2.13 mg/L;已改水病区8～10岁儿童氟斑牙检出率为50.78%(65/128),氟斑牙指数为1.04,儿童尿氟均值为1.64 mg/L,未改水病区氟斑牙检出率为80.65%(25/31),氟斑牙指数为1.50,儿童尿氟均值为2.08;已改水病区成人氟骨症患病率为38.7%(104/269),尿氟为1.61 mg/L,未改水病区成人氟骨症患病率为15.4%(18/117),尿氟为3.54mg/L.结论 改水降氟使氟斑牙的检出率降低至控制水平,严重程度也在降低,改水病区尿氟低于未改水病区,说明改水仍对消除氟中毒的危害有重要意义,但氟骨症患病率出现了在改水病区反而高于未改水病区的现象.

Abstract：

Objective To find out the dynamics of drinking water borne endemic fluorosis in Tibet's, to evaluate the effect of control measures, and to provide a scientific basis for the timely adjustment of control strategies. Methods During september to october 2009, according to the "2008 Central Government Special Funds to Subsidize Local Public Health in Drinking Water Borne Fluorosis in Tibet", Xigaze Xietongmen and Nyingchi Zayu were selected as project counties, three project villages were selected with simple random sampling method in each county, the functioning of water improvement projects, drinking water fluoride content, children's dental fluorosis and adult skeletal fluorosis were investigated. Water fluoride was detected by the "standard examination methods for drinking water the non-metallic targets"(GB/T 5750.6-2006) determination of fluoride; urinary fluoride was tested by ion selective electrode (WS/T 89-1996); dental fluorosis was diagnosed using Deans method; adult skeletal fluorosis was diagnosed by "endemic skeletal fluorosis clinical diagnostic criteria" (WS 192-2008). Results Mean water fluoride was 0.18 - 0.34 mg/L in drinking water changed areas, and 0.70 - 2.13 mg/L in not changed areas; prevalence of dental fluorosis of children 8 - 10 was 50.78% (65/128), dental fluorosis index was 1.04,mean urinary fluoride was 1.64 mg/L in drinking water changed areas; prevalence of dental fluorosis of children 8 -10 years old was 80.65%(25/31 ) in not changed areas, dental fluorosis index was 1.50, mean urinary fluoride of children was 2.08; adult clinical skeletal fluorosis was 38.7%(104/269) in drinking water changed areas, the mean urinary fluoride was 1.61 mg/L, prevalence of skeletal fluorosis was 15.4% (18/117) in not changed areas, mean urinary fluoride was 3.54 mg/L. Conclusions The method of change the water to reduce fluoride decreases dental fluorosis to control levels, and severity is also reduced, urinary fluoride is decreased. However, the prevalence of skeletal fluorosis is higher than that of drinking water not changed areas.     

复发性多软骨炎诊疗规范

复发性多软骨炎（RP）是一种免疫介导的全身炎症性疾病,主要累及软骨及富含蛋白聚糖成分的组织,特征性表现为耳和鼻软骨炎症、畸形。为提高临床医生对RP的认识和诊疗水平,中华医学会风湿病学分会在借鉴目前国内外经验和指南的基础上,制定了本规范,总结了RP的临床表现、诊断及疾病活动评估,旨在规范RP临床治疗方案,改善RP患者预后...     

西藏自治区犬棘球绦虫感染现状调查与分析

目的分析西藏自治区犬棘球绦虫的感染情况。方法于2016年8-11月,采取分层整群随机抽样法,在西藏自治区抽取那曲、阿里、山南、拉萨、昌都、日喀则、林芝等7个地区(市)。根据不同生产类型,将7个地区(市)下辖的县按牧区、半农半牧区、农区和城镇进行分层,按比例抽取调查村数。以行政村(居委会)为单位,每村随机抽取20个养犬户,每户采集1条犬的粪样,ELISA检测犬粪棘球绦虫抗原。利用χ~2检验比较不同组间阳性率的差异。结果西藏自治区犬粪棘球绦虫抗原阳性率为6.31%(458/7 259),那曲、阿里、山南、拉萨、昌都、日喀则、林芝等7个地区(市)阳性率依次为10.08%(109/1 081)、8.04%(34/423)、6.87%(64/932)、6.30%(66/1 047)、5.74%(78/1 358)、4.64%(89/1 917)和3.59%(18/501),差异有统计学意义(χ~2=44.671,P0.01)。家犬和无主犬粪棘球绦虫抗原阳性率分别为6.28%(133/2 119)和6.32%(325/5 140),差异无统计学意义(χ~2=0.005,P0.05)。牧区犬粪棘球绦虫抗原阳性率最高,为8.41%(187/2 224)。不同生产类型地区犬粪棘球绦虫抗原阳性率的差异有统计学意义(χ~2=25.221,P0.01)。结论西藏自治区犬粪棘球绦虫抗原阳性率较高,那曲、阿里地区和山南市属于高风险地区,尤以牧区更为严重。     

我国西藏地区(高原)系统性红斑狼疮患者临床及免疫学特征分析

目的: 探讨我国西藏地区系统性红斑狼疮(systemic lupus erythematosus,SLE)患者发病特点、受累器官、免疫学特点。方法: 回顾性研究西藏自治区人民医院风湿免疫科2014年5月至2016年4月住院的SLE患者共70例,随机抽取北京大学人民医院风湿免疫科SLE数据库中120例年龄及性别匹配的住院SLE患者作为对照组,对比高原与平原地区SLE患者临床特点、受累器官、免疫学特点。结果: 西藏地区SLE患者男女比例1 :10.7,平原患者男女比例1 :11.0;高原患者发病年龄(33.2±11.4)岁,平原患者发病年龄(35.3±13.2)岁,两组发病年龄相当。首发临床症状方面,高原地区SLE患者以关节炎(78.6%)、脱发(55.7%)、颧部红斑(48.6%)为常见,其中关节炎、脱发发生率明显高于平原组(P<0.05);高原地区SLE患者70%出现血液系统受累,与平原地区类似;狼疮肾炎及狼疮脑病发生率均明显低于平原组(38.6% vs. 56.7%, 2.9% vs. 17.5%, P<0.05)。血清学方面,高原地区抗双链DNA(double-stranded DNA,dsDNA)抗体阳性率为57.1%,抗Smith(Sm)抗体(55.7%)、抗干燥综合征A(Sj?gren syndrome A, SSA)抗体(74.3%)、抗干燥综合征B(Sj?gren syndrome B, SSB)抗体(41.4%)及抗u1核糖核蛋白(u1-ribosenuclear protein,u1RNP)抗体(45.7%)阳性率均显著高于平原地区患者(P<0.05);分别有61.4%、38.6%的高原SLE患者出现补体C3、C4减低,显著低于平原患者(P<0.05)。SLE疾病活动度指数(SLE disease activity index,SLEDAI)评分在两组间差异无统计学意义(高原组为12.19±5.58,平原组为12.69±7.28)。此外,西藏地区SLE患者合并陈旧结核或者活动结核病13例(18.6%),慢性乙型肝炎或携带者7例(10%)。高原组SLE患者血清25羟维生素D3(25-dihydroxy-vitamin D3, 25-OH-VD3)减低者少于平原组(76.7% vs. 90.0%,P=0.046),血清25-OH-VD3水平高原组为(31.14±18.74) nmol/L,平原组为(26.91±14.27) nmol/L,两组间差异无统计学意义(P=0.16)。结论: 西藏地区SLE患者首发临床症状以关节炎、脱发、颧部红斑最为常见,其中关节炎、脱发发生率显著高于平原地区;狼疮肾炎及狼疮脑病发生率明显低于平原;多种自身抗体的阳性率显著高于平原地区。在年龄、性别构成、SLEDAI评分方面与平原地区类似,血清25-OH-VD3减低者平原组多于高原组,但25-OH-VD3水平在两组间无明显差异。     

继续深入开展医院管理年活动进一步发挥藏医药特色优势

加强医院管理年活动是医院工作的永恒主题.文章介绍了西藏自治区藏医院克服松懈和厌倦情绪,切实提高对医院管理年活动的认识,不断规范执业行为,完善科室医疗质量管理,坚持特色优势化的办院思想,加强核心制度执行力度,增强忧患意识、责任意识,强化措施、持续改进,注重实效,抓好贯彻落实,力求医院基础设施建设和业务建设方面取得实实在在的成效.     

以多关节炎为首发症状的非结晶型轻链κ型近端肾小管病一例

非结晶型轻链近端肾小管病（非结晶型LCPT）是一种少见的单克隆免疫球蛋白轻链相关性肾病,是肾脏意义的单克隆免疫球蛋白病一种特殊的病理类型,临床罕见。本文报告1例以多关节炎为首发症状的非结晶型LCPT,以提高临床医师对该疾病的认识。