传单2013

Infectious mononucleosis,Xie minDepartment of Pediatrics , Xiangya Hospital,男，7岁，发热5天，发现颈部包块3天入院。入院前5天，患儿无诱因出现发热，体温最高达39，不伴畏寒、寒颤，无咳嗽、流涕等不适，自服退热药，病情反复。入院前3天，家长发现患儿左颈部一包块，并出现双眼睑浮肿，晨起为重，不伴双下肢水肿。患儿自述咽痛 。体查：T 38，P 110次/分，R 26次/分，BP 95/60mmHg，W 23左侧颈部扪及一约23大小质韧淋巴结，光滑、活动，轻触痛，局部皮肤不红，皮温不高，右侧颈部扪及数个黄豆大小质韧淋巴结，光滑 双睑浮肿，球结膜无充血，咽充血，双侧扁桃体大，表面可见白色膜样物覆盖。心、肺（-）腹平软，肝肋下3触及，质软边锐，无触痛，脾肋下1，质软边锐，无触痛。WBC 13.5109/L, N 0.24, L 0.60, 异常细胞0.16, Hb 115g/L, Plt 210109/L尿常规：隐血（），尿蛋白（） 该患儿可能诊断？,病例一,男，13 岁，发热、咽痛3天入院。伴右上腹痛、尿黄。体查：T 37.8 ，皮肤巩膜黄染，双侧颈部及双侧腹股沟触及数个黄豆大小淋巴结，质软，活动可。咽部充血, 双侧扁桃体I度大，表面无脓性渗出，心肺未见异常，右上腹压痛明显，无反跳痛，肝肋下未及,脾肋下3 cm，质软，无触痛。肝区叩痛（+）。血常规:WBC 9.6109/L，Hb149/L, N 0.389，L 0.589。肝功能：ALT 328U/L，AST 233 U/L，TBIL 75mol/ L。,病例二,入院第3 天双扁桃体 II度肿大, 表面出现白色渗出物, 第7 天颜面部、躯干、四肢出现弥漫性红色斑丘疹, 第8 天右上腹痛消失, 黄疸加重,第10 天皮疹消退, 出现皮肤瘙痒及陶土便, 扁桃体表面白色渗出物消失, 第12天体温正常。WBC22.0109/L，Hb149/L, N 0.25，L 0.70，异型淋巴细胞0.04骨髓涂片: 增生活跃, 异型淋巴细胞0.165 该患儿可能的诊断？,学龄期儿童 发热、咽痛 扁桃体肿大有白色渗出物 颈淋巴结肿大 肝脾肿大 伴随有其他系统损伤：肾脏、肝脏 血常规：白细胞升高，分类以淋巴细胞为主， 可见异型淋巴细胞,Company Logo,,Introduction,s,Infectious mononucleosis (IM) is a very common illness caused by the Epstein-Barr virus (EBV ) IM is a clinical syndrome consisting of fever, pharyngitis, lymphadenopathy,splenomegaly and hepatomegaly Blood tests show an increase in atypical lymphocytes IM is most often diagnosed in adolescents and young adults, with a peak incidence at ages 15-17. it is also seen in children and less severe in young children,Company Logo,,四川大学华西第二医院414例传单,郭霞，中华儿科杂志，2008，46（11）：870-871,婴儿组 5例 1.21%幼儿组 93例 22.46%学龄前组 224例 54.11%学龄组 77例 18.60%青春期 15例 3.62%,s,Company Logo,,s,IM was first described in 1920 The association between the disease syndrome and a positive heterophile antibody test was recognized in 1932 The association between IM and EBV was described in 1968,Company Logo,,Epstein-Barr virus,s,EBV is a member of the herpes family Human herpesvirus 4 (HHV-4) EBV is named after Epstein and Barr discovered in 1964 EBV is associated with IM, T/NK lymphoproliferative diseases, Burkitt lymphoma, non-Hodgkin lymphoma , oral hairy leukoplakia post-transplant lymphoproliferative disorder（PTLD)， nasopharyngeal cancers,Company Logo,,s,EBV antigen,viral capsid antigen VCA Early antigens EA nuclear antigens EBNA lymphocyte determinant membrane antigen LYDMA memberane antigen MA,EA-D antibodiesare prominent in patients with EBV-associated nasopharyngeal carcinomas.,Johnson DH, Cunha BA. Infect Dis Pract. 1995;19:26-27.,Anti-VCA,Anti-EA,EBV Antibody Responses,Company Logo,,In the U.S., up to 95% of adults 35-40 years of age have antibodies directed against EBV. In developing countries, 90% of children experience an asymptomatic Epstein-Barr virus infection when younger than 5 years,EBV occurs worldwide, most people become infected with EBV and gain adaptive immunity,s,儿童EB病毒感染279 例临床分析,黄洋，华中医学杂志，2006，30（3）：247-248,急性上呼吸道感染 43.4 % 急性支气管炎 10.8 % 肺炎 20.1 % 扁桃体炎 11.8 % 传染性单核细胞增多症 6.5 % 川崎病 0.4 %特发性血小板减少性紫癜 0.4 % 病毒性心肌炎 2.5 % 淋巴结炎 0.4 % 过敏性紫癜 1.8 % 粒细减少症 1.4 %婴儿肝炎综合征 0.4 %急性出血性胃炎 0.4 %,Company Logo,,Pathogenesis,s,EBV infects epithelial cells of the oropharynx and salivary glands.B lymphocytes become infected through exposure to these cells or may be directly infected in the tonsillar crypts. The proliferation of infected B cells results in massive activation and proliferation of cytotoxic T lymphocytes(CD8)Infected B-cell transformated to immortal plasmacytoid cells,tonsillitislymphadenopathy hepatosplenomegalyatypical lymphocytes,a wide variety antibodies,Company Logo,,s,Prevalence,Epidemiology,population without immunity,Company Logo,,Clinical features,s,fever pharyngitisis and tonsillitis lymphadenopathy splenomegaly hepatomegaly maculopapular rash petechiae, periorbital edema, Jaundice ,uvular edema,中山大学附属第二医院儿科238例儿童IM特点,发热 94.96%咽峡炎 93.70%鼻塞 84%淋巴结肿大 94.12%肝肿大 89.50%脾肿大 50.84%眼睑水肿 57.98%,岑丹阳，中国热带医学，2008，8（4）：594-596,Company Logo,,四川大学华西第二医院414例IM,发热 92.5%咽峡炎 90.1%淋巴结肿大 94.2%肝肿大 79.2%脾肿大 62.8%眼睑水肿 38.6%皮疹 19.1%,郭霞，中华儿科杂志，2008，46（11）：870-871,fever lymphadenopathy pharyngitis rash periorbital edema,early signs,later physical findings,hepatomegaly palatal petechiae jaundice uvular edema splenomegaly,Company Logo,,Complications,s,CNS complications,meningitis, encephalitis, hemiplegia, psychosis, cranial nerve palsies, Guillain-Barr syndrome, transverse myelitis, multiple sclerosis,autoimmune hemolytic anemia, pancytopenia, aplastic anemia , severe thrombocytopenia, agranulocytopenia,hematologic complications,urinary system complications,hematuria,proteinuria,other complications,pericarditis, myocarditis, splenic rupture,Company Logo,,Laboratory studies,s,White blood cell (WBC) count and differential,elevated WBC with a peak of 10,000-20,000 during the second or third week of the illness an elevated lymphocyte count with greater than 10% atypical lymphocytes.,Heterophil agglutination test,EBV specific antibodies,EBV-DNA,VCA-IgG , VCA-IgM,Other tests,型异型淋巴细胞(泡沫型、浆细胞型),II型异型淋巴细胞(不规则型、单核细胞型),III型异型淋巴细胞(幼稚型),Company Logo,,Laboratory studies,s,White blood cell (WBC) count and differential,elevated WBC with a peak of 10,000-20,000 during the second or third week of the illness an elevated lymphocyte count with greater than 10% atypical lymphocytes.,Heterophil agglutination test,EBV specific antibodies,EBV-DNA,VCA-IgG , VCA-IgM,Other tests,Company Logo,,EA-D antibodiesare prominent in patients with EBV-associated nasopharyngeal carcinomas.,Johnson DH, Cunha BA. Infect Dis Pract. 1995;19:26-27.,s,Company Logo,,Diagnosis,s,The diagnosis of IM is suspected based on the symptoms and signs and confirmed by blood tests and EBV specific antibodies tests,Company Logo,,Differential diagnoses,s,streptococcal pharyngitis pseudolymphoma lymphoma SLE sarcoidosis TB Kawasaki disease allergic reactions anicteric hepatitis,上呼吸道感染 4例化脓性扁桃体炎 3例病毒性肝炎 3例疑似败血症 3例病毒性心肌炎 2例 血液病待查 2例链球菌性咽峡炎 1例,传染性单核细胞增多症误诊18例分析,杨菊萍，中国误诊学杂志，2008，8（33）：8186-7,传单和急淋、传淋的鉴别,传单 急淋 传淋发热 常持续13周 持续不规则发热 无或暂短发热淋巴结肿大 有 有 无脾肿大 2575%有 有 无传染性 小 无 大白细胞计数 中等度增多 从减少到极度 显著增多 增多有诊断价值细胞 异型淋巴细胞 原淋、幼淋 正常成熟小淋巴贫血 无 有 无血小板减少 一般无 有 无骨髓象 有异型淋巴 原淋+幼淋显著 正常小淋巴细胞 细胞 增多 增多嗜异性凝集试验 阳性 阴性 阴性预后 良好 不良 良好,传染性单核细胞增多综合症,临床症状和体征与IM类似 病因,感染性因素,非感染因素,腺病毒、 CMV、 Hepatitis A、 HIV 、Rubella、Human herpes virusToxoplasma gondii、TB链球菌、支原体等,苯妥英、磺胺药、巴比妥心脏手术输血后、恶性肿瘤等,X-联淋巴增生综合症（X-linked lymphoproliferative syndrome),严重或致死性传单获得性低球蛋白血症恶性淋巴瘤,临床特点,Company Logo,,s,EBV相关的噬血细胞性淋巴组织细胞增多症(EBV-HLH)慢性活动性EBV感染（CAEBV),s,患儿，男，反复发热7天，体温最高41度，无明显伴随症状当地血常规示全血细胞减少，予抗感染治疗效果不佳转我院。入院体查： T 37.8 ，贫血貌，皮肤未见出血点，浅表淋巴结不大，咽部无充血，扁桃体炎不大，心肺无异常，腹软，肝肋5cm，质韧，脾肋下3 cm，质中，无触痛。血常规:WBC 1.6109/L，Hb84g/L,Plt 50109/L，N 0.5109/L; 肝功能：ALT 164U/L，AST 259 U/L，心肌酶：乳酸脱氢酶 726U/L,Company Logo,,Treatment,s,When coexisting complications may indicate the use of corticosteroids,Treatm