Objective To evaluate the sensitivity and specificity of international classification criteria （2002） for primary Sjogren＇s syndrome （pSS） and the role of lower lip biopsy in diagnosis of pSS in Chinese patients....Objective To evaluate the sensitivity and specificity of international classification criteria （2002） for primary Sjogren＇s syndrome （pSS） and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Mothoda Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease （GVHD）, and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies （including anti-SSA/SSB antibodies） test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched （47.8 ± 10.9 years vs. 46.2±13.6 years, P 〉 0.05）. The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB antibodies were diagnosed as pSS by lower lip biopsy. Coadwion The international classification criteria （2002） for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.展开更多
SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The cl...SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The clinical manifestations of SS are complex. When the digestive system is involved,展开更多
KIDNEY involvement is common in non-Hodgkin＇s lymphoma （NHL） with incidence up to 30%-40% in autopsy studies. However, it us- ually occurs late in the course of the diseaseand is clinically silent. Clinically overt...KIDNEY involvement is common in non-Hodgkin＇s lymphoma （NHL） with incidence up to 30%-40% in autopsy studies. However, it us- ually occurs late in the course of the diseaseand is clinically silent. Clinically overt renal disease including acute kidney injury （AKI） as its primary manifestation is rarely reported, moreover, Fanconi syndrome （FS） is extremely rare as the main manifestation in NHL. In this report, we presented a case of NHL primarily presenting with FS and AKI due to diffuse interstitial infiltration of NHL cells and emphasized the important role of renal biopsy, especially renal immunohistochemical analysis in the diagnosis of renal diffuse lymphoma.展开更多
A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated in...A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.展开更多
It was not until 1979 that Bayes de Ltma described atrial conduction blocks and classified them as interatrial and intra- atrial.Interatrial blocks refer to conduction disorders lo- cated between the atria, while intr...It was not until 1979 that Bayes de Ltma described atrial conduction blocks and classified them as interatrial and intra- atrial.Interatrial blocks refer to conduction disorders lo- cated between the atria, while intraalxial blocks occur within the same atrium. This work motivated several authors, in- cluding Bayes himself, to further investigate atrial conduc- tion and interatrial block （IAB）.展开更多
This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy ...This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.展开更多
Horner＇s syndrome （HS） results from interruption of sympathetic nervous supply to the eye and manifests clinically with partial ptosis, miosis and enophthalmos, along with anhidrosis of face on the affected side.
信号转导和转录激活因子3(signal transducer and activator of transcription 3,STAT3)是一种细胞内信号转导因子,在细胞的生长、分化、凋亡和免疫应答等方面发挥重要作用。辅助性T细胞17(T helper cell 17,Th17)的异常活化与多种自身...信号转导和转录激活因子3(signal transducer and activator of transcription 3,STAT3)是一种细胞内信号转导因子,在细胞的生长、分化、凋亡和免疫应答等方面发挥重要作用。辅助性T细胞17(T helper cell 17,Th17)的异常活化与多种自身免疫性疾病的发生和发展密切相关。STAT3通过诱导Th17细胞过度增殖、异常分化并影响淋巴细胞浸润外分泌腺等机制参与干燥综合征(Sjogren syndrome)的疾病进展,因此阻断STAT3信号转导通路是治疗干燥综合征的潜在药物治疗靶点。本文综述了STAT3通过调节Th17细胞参与干燥综合征发病及进展的研究,并聚焦于STAT3信号通路抑制剂的最新研究进展,为该靶点在干燥综合征的治疗药物相关研究提供依据。展开更多
目的建立孕早期产前筛查孕妇血清解整合素金属蛋白酶12分泌型(a disintegrin and metalloprotease,ADAM12-S)中位数倍数的校正方法。方法随机选择2008年在本中心自愿参加孕早期唐氏综合征(DS)产前筛查并经随访证实分娩健康儿的孕妇孕8...目的建立孕早期产前筛查孕妇血清解整合素金属蛋白酶12分泌型(a disintegrin and metalloprotease,ADAM12-S)中位数倍数的校正方法。方法随机选择2008年在本中心自愿参加孕早期唐氏综合征(DS)产前筛查并经随访证实分娩健康儿的孕妇孕8～13周血清样本577份,用时间分辨荧光免疫分析法检测血清ADAM12-S浓度。用SPSS 15.0软件构建ADAM12-S与孕龄间的回归关系,选最优模型计算中位浓度,得到ADAM12-S中位数倍数(multiple of the median,MoM)值。探讨MoM值与体重的关系,选取最优模型求出经体重调整后的MoM值。将ADAM12-S初始浓度与消除孕周和体重影响校正后的最终MoM值进行曲线拟合,选取最优模型最终得到ADAM12-S初始浓度与校正后MoM值的关系方程。结果 ADAM12-S中位数(Y)与孕周(t)呈正相关,经s曲线模型拟合后的中位数方程为:Y=e8.412-24.534/t;ADAM12-S MoM值中位数(Y)与体重中位数(z)存在负相关,经指数模型拟合后的方程为:Y=1.603*e-0.009*z;ADAM12-S初始浓度(X)与孕周和体重校正后的最终MoM值(Y)的关系方程为Y=0.036*X0.552。结论 ADAM12-S初始浓度与最终MoM值的关系方程的建立,对临床开展ADAM12-S筛查DS妊娠的研究有重要意义。展开更多
基金Supported by the National Natural Sciences Foundation of China(30300164 ).
文摘Objective To evaluate the sensitivity and specificity of international classification criteria （2002） for primary Sjogren＇s syndrome （pSS） and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Mothoda Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease （GVHD）, and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies （including anti-SSA/SSB antibodies） test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched （47.8 ± 10.9 years vs. 46.2±13.6 years, P 〉 0.05）. The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB antibodies were diagnosed as pSS by lower lip biopsy. Coadwion The international classification criteria （2002） for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.
文摘SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The clinical manifestations of SS are complex. When the digestive system is involved,
文摘KIDNEY involvement is common in non-Hodgkin＇s lymphoma （NHL） with incidence up to 30%-40% in autopsy studies. However, it us- ually occurs late in the course of the diseaseand is clinically silent. Clinically overt renal disease including acute kidney injury （AKI） as its primary manifestation is rarely reported, moreover, Fanconi syndrome （FS） is extremely rare as the main manifestation in NHL. In this report, we presented a case of NHL primarily presenting with FS and AKI due to diffuse interstitial infiltration of NHL cells and emphasized the important role of renal biopsy, especially renal immunohistochemical analysis in the diagnosis of renal diffuse lymphoma.
文摘A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.
文摘It was not until 1979 that Bayes de Ltma described atrial conduction blocks and classified them as interatrial and intra- atrial.Interatrial blocks refer to conduction disorders lo- cated between the atria, while intraalxial blocks occur within the same atrium. This work motivated several authors, in- cluding Bayes himself, to further investigate atrial conduc- tion and interatrial block （IAB）.
文摘This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.
文摘Horner＇s syndrome （HS） results from interruption of sympathetic nervous supply to the eye and manifests clinically with partial ptosis, miosis and enophthalmos, along with anhidrosis of face on the affected side.
文摘目的建立孕早期产前筛查孕妇血清解整合素金属蛋白酶12分泌型(a disintegrin and metalloprotease,ADAM12-S)中位数倍数的校正方法。方法随机选择2008年在本中心自愿参加孕早期唐氏综合征(DS)产前筛查并经随访证实分娩健康儿的孕妇孕8～13周血清样本577份,用时间分辨荧光免疫分析法检测血清ADAM12-S浓度。用SPSS 15.0软件构建ADAM12-S与孕龄间的回归关系,选最优模型计算中位浓度,得到ADAM12-S中位数倍数(multiple of the median,MoM)值。探讨MoM值与体重的关系,选取最优模型求出经体重调整后的MoM值。将ADAM12-S初始浓度与消除孕周和体重影响校正后的最终MoM值进行曲线拟合,选取最优模型最终得到ADAM12-S初始浓度与校正后MoM值的关系方程。结果 ADAM12-S中位数(Y)与孕周(t)呈正相关,经s曲线模型拟合后的中位数方程为:Y=e8.412-24.534/t;ADAM12-S MoM值中位数(Y)与体重中位数(z)存在负相关,经指数模型拟合后的方程为:Y=1.603*e-0.009*z;ADAM12-S初始浓度(X)与孕周和体重校正后的最终MoM值(Y)的关系方程为Y=0.036*X0.552。结论 ADAM12-S初始浓度与最终MoM值的关系方程的建立,对临床开展ADAM12-S筛查DS妊娠的研究有重要意义。
