过敏性紫癜邬惊雷课件

过 敏 性 紫 癜 Anaphylactoid Purpura 许兰-亨诺血管炎(综合征) Schonlein-Henoch vasculitis(syndrome) 儿科医院 邬惊雷 概 貌 Outline 是一种小血管炎 A kind of vasculitis of small vessels 以皮肤紫癜、关节痛、积液、腹痛、便血、血 尿和蛋白尿为主要临床特征 Characterized clinically by purpura, arthralgia ,articular effusion, abdominal pain, hemafecia, hematuria and proteinuria 学龄前和学龄期儿童多见 More frequent in preschoolers and school age children 男孩发病率高于女孩 Boys are affected more frequently than girls 估计人群总体发病率约为9/10万 The overall incidence estimated to be 9/100,000 population 病 因 Etiology 病因不清楚，可能与下列因素有关: The etiology is unknown, maybe related with the follows: 食物过敏：海鲜、蛋、牛奶 Food allergy: sea food, eggs, milk 感染：细菌、病毒、寄生虫 Infection: bacteria, virus , parasites 病 因 Etiology 药物：抗生素、阿司匹林 Drugs: antibiotics, aspirin 其它：虫咬、预防接种 Others: insect bite , immunization 病理改变 Histopathological findings 白细胞碎裂性血管炎 Leukocytoclastic vasculitis IgA为主的免疫复合物沉积 Deposition of immune complexes of IgA(in the small vessels and glomeruli) 病理改变 Histopathological findings 肾脏病变：微小、局灶、新月体形成等 箭头所指处为新月体 临床表现 Clinical manifestations 常随上呼吸道感染后 Usaually follows an upper respiratory tract infection 可有低热、纳差、乏力 Low-grade fever, losing appetite and fatigue may occur 皮肤紫癜 Purpura 高出皮面，初期压之退色 Palpable purpura that initially blanch on pressure 反复出现，呈紫红色 Appear at intervals and evolve from red to purple 对称分布于腰以下 Occur symmetrically below the waist 尚可伴有血管神经性水肿 May have angioedema 1 岁半女孩患过敏性紫癜： 注意：局限于半下身，明显的、边缘清楚分布的皮疹！ 5 岁男孩患过敏性紫癜：典型的臀部皮疹 5 岁男孩患过敏性紫癜: 典型的皮疹和双踝关节肿胀 4 岁女孩患过敏性紫癜： 下肢伸侧显著、部分融合的皮疹 8 岁半男孩患过敏性紫癜： 皮肤出血几乎仅限于双足背和 大足趾；关节轻度肿胀 消化道症状 Symptoms of gastrointestinal tract 间歇性腹痛，位于脐周或下腹部 Intermittent abdominal pain localized to periomphalic area and lower abdomen 呕吐、便血 Vomiting, hemafecia 可有肠套叠、肠梗阻和肠穿孔 Intussusception, obstruction and bower perforation may occur 关节症状 主要累及膝、踝关节 Mainly affects the knees and ankles 伴有肿胀和积液 Concomitant with edema and effusion 能在一定时间内消退，不留畸形 May resolve after a few days without residual deformity 4 岁男孩患紫癜性风 湿病： 所见： 双关节明显肿胀 （左 右) 肾脏症状 Renal involvement 约2550病例累及肾脏 Renal involvement occurs in 25-50% cases 多数在起病一个月内出现 Most will develop renal symptoms within 1 month 表现为血尿、尿蛋白和高血压 Presented with hematuria、proteinuria and hypertension 少数呈肾病综合征表现 Few may appear as nephrotic syndrome 其他表现 Other clinical findings 颅内出血：惊厥、瘫痪、失语、昏迷等 Intracranial hemorrhage：convulsion、paralysis(paresis)、 aphasia、coma et al. 可有胰腺炎、睾丸出血、肺出血等。 May have pancreatitis、testicular or pulmonary hemorrhage 实验室检查 Laboratory findings 无敏感和特异诊断指标 Routine tests are neither specific nor diagnostic 可有血小板、白细胞升高,血沉增快 May have thrombocytosis and leukocytosis. The erythrocyte sedimentation rate may be elevated. 约50病例IgA升高 About 50% of patients have elevated concentration of IgA 重症者可出现高凝状态 Hypercoagulable state may occur in severe cases 鉴别诊断 Differential diagnosis 突发性血小板减少性紫癜 Idiopathic thrombocytopenic purpura 外科急腹症 （急性阑尾炎、肠套叠） Surgical abdomen(acute appendicitis,intussusception 细菌感染（败血症，感染性心内膜炎，流脑） Bacterial infection(septicemia, IBE, meningococcal meningitis 风湿性关节炎 Rheumatic arthritis 治疗：对症治疗 Symptomatic treatment 去除诱因：感染、食品、药物 Excluding induction factors（infections,foods,drugs) 对症治疗： Symptomatic treatment 休息、饮食、抗组胺药物、钙剂、维生素C、 非甾体类抗炎药 Rest, bland diet, antihistamine drugs,calcium, vitamine C and NSAID 肾上腺皮质激素：能明显改善消化道症状和关节 痛，但不能阻止肾脏病变 Costicosteroids: Often associated with dramatic improvement of both gastrointestinal symptoms and arthralgia.But, the renal involvement can not be stoped 其它免疫抑制剂：严重病例可选用 Other immunosuppressive drugs: used in severe cases 肾脏病变的治疗 Management of renal involvement 与急性肾小球肾炎相同 The same as for acute glomerulonnephritis 抗凝治疗：用以肾脏病变为主要表现者 Anticosgulant therapy: used for the cases mainly with renal damage 肝素 Heparin 阿司匹林 Aspirin