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适用于儿童的胃镜有两类:①小儿胃镜:专为儿童设计的直视式胃镜,如FujinonFG-100PE及PentaxFG-24X,该镜特点是镜身纤细,外径仅7~9mm,有效工作长度是1050mm,操作灵活方便,受检者痛苦少。
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②成人细径胃镜:外径在9.0~9.8mm范围,活检孔较大,适用于年长儿童及内镜治疗。
[ { "id": 0, "entity": "成人细径胃镜", "start_offset": 1, "end_offset": 7, "label": "equ" }, { "id": 1, "entity": "内镜治疗", "start_offset": 37, "end_offset": 41, "label": "pro" } ]
对早产婴儿,文献报道,可用直径3.55mm的纤维支气管镜代替胃镜。
[ { "id": 0, "entity": "纤维支气管镜", "start_offset": 22, "end_offset": 28, "label": "equ" }, { "id": 1, "entity": "胃镜", "start_offset": 30, "end_offset": 32, "label": "equ" } ]
(二)胃镜检查(gastroscopy)适应证及禁忌证1.适应证(1)反复发作性腹痛:尤为上腹痛,脐周痛;有半夜痛醒史;腹痛伴体重减轻;腹痛伴黑便;腹痛经驱虫及一般治疗无效者。
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(3)经常性呕吐:呕吐伴出血,呕吐伴上腹痛,呕吐伴体重减轻者。
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(5)消化不良:经常性腹胀、嗳气及纳呆,经一般治疗无效。
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(7)不能用心肺疾病解释的胸闷及胸骨后疼痛。
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2.禁忌证有严重的心肺疾病不能耐受者;疑似胃、十二指肠穿孔者;处于休克、昏迷等严重状态;有发热、急性咽喉炎及肺部感染;吞食腐蚀物的急性期,癫痫反复发作等,均被列为儿童胃镜检查禁忌证。
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(三)胃镜检查的并发症及其预防纤维或电子胃镜检查是一项比较安全的技术,根据日本87万次成人胃镜检查资料,严重并发症发生率仅0.003%~0.044%。
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其发生类型主要有以下几种:①麻醉意外:多系全麻引起的药物过敏、气管痉挛及短暂性呼吸抑制等反应;②插管损伤:主要是操作者不熟练,强行插管引起;③其他如低氧血症、吸入窒息及胃肠穿孔也有报道,但罕见。
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预防的关键是尽量少使用全麻,已经有报道非麻醉下胃镜检查的时间仅是麻醉下的一半,且安全无麻醉意外。
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此外胃镜检查必须有专人进行,操作者动作必须熟练、轻柔、仔细,切不可盲目粗暴。
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在胃镜检查过程中有专人护士给予安抚。
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(四)胃镜检查术前准备及术后处理1.术前准备(1)检查所有器械是否完整,安装是否确切。
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(4)检查前一天晚8点后禁食、禁水、禁药;哺乳期婴儿术前禁奶6小时,幽门梗阻患儿禁食3天。
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2.术前用药(1)除婴儿外术前均用2%丁卡因溶液做咽部麻醉,婴幼儿或精神紧张的年长儿术前15分钟给予10%水合氯醛0.5ml/kg保留灌肠或地西泮0.1~0.3mg/kg肌内注射,也可采用全身麻醉,氯胺酮4~6mg/kg,肌内注射,但须麻醉医师参与。
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(2)术前5分钟口服祛泡剂。
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(3)阿托品0.01~0.015mg/kg,肌内注射。
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3.术后处理胃镜术后应留观半小时,适当休息1天;术后禁食1小时,取活体组织检查者进流汁饮食1天,并嘱咐家长若有消化道出血,便来院急诊。
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(五)胃镜操作方法及注意事项1.患儿左侧卧位,松开领扣及裤带,双下肢屈曲,助手扶持患儿头部,把紧患儿口中牙垫。
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2.插镜采用单人或双人插镜法,术者左手持操作部,并调节角度钮使胃镜前端稍向下弯曲,右手拿胃镜可屈部,通过牙垫轻轻插入咽部,让患者做吞咽动作,此时,右手将胃镜往下送,便可顺利到达食管上段。
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3.当镜身通过咽喉部后,即应在直视下操作。
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依次自食管、贲门、胃体、胃窦直至幽门,进入球内观察,注气应适量,必要时将过多气体吸出。
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4.在退镜过程中,应上下、左右方向依次仔细观察胃内各部,特别是胃窦小弯后壁及胃角,对胃体、胃底及贲门部至少应采用高位翻转及正面两次观察。
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5.胃镜退出贲门时,应吸出胃内气体,然后退入食管观察,直至全部退出。
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(六)上消化道常见病变的胃镜诊断1.正常胃黏膜正常小儿胃黏液较成人更为细嫩柔软呈半透明状,表面有少量晶状稀薄黏液,使其湿润而光滑,色泽视血色素高低而不同,一般呈橘红色。
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胃镜刚入胃腔时,可见到许多皱襞,呈曲折蜿蜒状态,自贲门沿着胃的长轴,走行至幽门部,注气量增多时,皱襞变平或消失。
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2.食管炎(esophagitis)儿童食管炎占胃镜检查的10%~12%,最常见的是反流性食管炎。
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据报道儿童食管炎中74%有胃食管反流。
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所以食管炎的发病机制多认为与食管下段括约肌功能不全以及酸清除能力下降有关。
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1992年消化内镜学会在通化市拟定了反流性食管炎的内镜诊断标准如下:①轻度:红色条纹和红斑,累及食管下1/3糜烂<1/2食管圆周,仅累及食管中、下段Ⅰ级:糜烂累及>1/2食管圆周,或已累及上段,或形成溃疡<1/3食管圆周,在食管任何部位;Ⅱ级:溃疡累及>1/3圆周食管,任何部位。
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3.慢性胃炎慢性胃炎内镜表现和悉尼系统。
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(1)内镜黏膜形态学:1)充血:与邻区比较,黏膜色泽呈斑块状、线状或弥漫性鲜红。
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2)水肿:黏膜肿胀,色泽较苍白,反光增强,胃小区轮廓更明显。
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4)花纹状:黏膜红白相间,红点与红点之间黏膜较苍白,似麻疹样改变。
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5)微小结节形成:胃窦平坦时,黏膜呈微细或粗颗粒状、结节状。
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6)糜烂:局散性或散在分布,可伴有新鲜或陈旧出血点。
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糜烂位于黏液层内为平坦型糜烂。
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高于黏膜面称隆起型糜烂,隆起呈圆形或椭圆形,顶部呈脐样凹陷。
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7)出血斑点:黏膜散在分布点状或小片状新鲜或陈旧出血。
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(2)胃炎的悉尼系统分类:1990年8月在澳大利亚悉尼召开的国际胃肠病学学术会上,对慢性胃炎制订了一整套的分类和诊断方法,称为悉尼系统。
[ { "id": 0, "entity": "胃炎", "start_offset": 3, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "慢性胃炎", "start_offset": 42, "end_offset": 46, "label": "dis" } ]
该系统强调内镜和病理密切结合,胃炎的诊断要包括组织学和内镜两部分。
[ { "id": 0, "entity": "内镜", "start_offset": 5, "end_offset": 7, "label": "equ" }, { "id": 1, "entity": "胃炎", "start_offset": 15, "end_offset": 17, "label": "dis" } ]
组织学的核心是解决急性胃炎、慢性胃炎还是特殊类型胃炎以及炎症的部位,并尽可能找到病因或相关的病原,以及炎症的程度、活动性、萎缩程度、肠化分级、有无幽门螺杆菌等。
[ { "id": 0, "entity": "急性胃炎", "start_offset": 9, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "慢性胃炎", "start_offset": 14, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "胃炎", "start_offset": 24, "end_offset": 26, "label": "dis" }, { "id": 3, "entity": "幽门螺杆菌", "start_offset": 73, "end_offset": 78, "label": "dis" } ]
4.十二指肠炎(duodenitis)十二指肠炎常多相伴其他部位的炎症,内镜下黏膜炎症的改变有四种类型:(1)充血型:黏膜充血、水肿,镜下反光增强。
[ { "id": 0, "entity": "十二指肠炎", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "duodenitis", "start_offset": 8, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "十二指肠", "start_offset": 19, "end_offset": 23, "label": "dis" }, { "id": 3, "entity": "内镜", "start_offset": 36, "end_offset": 38, "label": "equ" }, { "id": 4, "entity": "黏膜炎症", "start_offset": 39, "end_offset": 43, "label": "dis" }, { "id": 5, "entity": "充血型", "start_offset": 55, "end_offset": 58, "label": "dis" }, { "id": 6, "entity": "黏膜充血、水肿,镜下反光增强", "start_offset": 59, "end_offset": 73, "label": "sym" } ]
(2)肿胀型:黏膜水肿肥厚呈不规则形隆起,表面充血。
[ { "id": 0, "entity": "肿胀型", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "黏膜水肿肥厚呈不规则形隆起,表面充血", "start_offset": 7, "end_offset": 25, "label": "sym" } ]
(3)颗粒型:黏膜呈滤泡样或颗粒状或结节状隆起,大小较均匀。
[ { "id": 0, "entity": "颗粒型", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "黏膜呈滤泡样或颗粒状或结节状隆起,大小较均匀", "start_offset": 7, "end_offset": 29, "label": "sym" } ]
(4)出血糜烂型:黏膜充血处见点状、片状或蜂窝状糜烂,表面可有出血。
[ { "id": 0, "entity": "出血糜烂型", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "黏膜充血处见点状、片状或蜂窝状糜烂,表面可有出血", "start_offset": 9, "end_offset": 33, "label": "sym" } ]
5.胃十二指肠溃疡溃疡在胃镜中所见为橘红色,黏膜上有鲜明的白色及灰白色病变,一般为圆形及椭圆形,边缘清楚,周围黏液在同一平面或仅轻微隆起,溃疡底面光滑,被有白色或灰白色苔状物,有时呈褐色,表示有血色素沉着,多数为单个溃疡,少数为多发性。
[ { "id": 0, "entity": "胃十二指肠溃疡", "start_offset": 2, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "溃疡", "start_offset": 9, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "胃镜", "start_offset": 12, "end_offset": 14, "label": "equ" } ]
溃疡病分期:(1)活动期(activestage;A期):又称厚苔膜期。
[ { "id": 0, "entity": "溃疡病", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "活动期", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "activestage", "start_offset": 13, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "A期", "start_offset": 25, "end_offset": 27, "label": "dis" }, { "id": 4, "entity": "厚苔膜期", "start_offset": 31, "end_offset": 35, "label": "dis" } ]
A1:溃疡苔厚而污秽,边缘肿胀,无皱襞集中,有较多分泌物;A2:溃疡苔膜厚而清洁,边缘肿胀逐渐消失,四周出现上皮再生形成的红晕,皱襞开始向溃疡集中。
[ { "id": 0, "entity": "A1", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "溃疡苔厚而污秽,边缘肿胀,无皱襞集中,有较多分泌物", "start_offset": 3, "end_offset": 28, "label": "sym" }, { "id": 2, "entity": "A2", "start_offset": 29, "end_offset": 31, "label": "dis" }, { "id": 3, "entity": "溃疡苔膜厚而清洁,边缘肿胀逐渐消失,四周出现上皮再生形成的红晕,皱襞开始向溃疡集中", "start_offset": 32, "end_offset": 73, "label": "sym" } ]
(2)愈合期(healingstage;H期):又称薄苔膜期。
[ { "id": 0, "entity": "愈合期", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "healingstage", "start_offset": 7, "end_offset": 19, "label": "dis" }, { "id": 2, "entity": "H期", "start_offset": 20, "end_offset": 22, "label": "dis" }, { "id": 3, "entity": "薄苔膜期", "start_offset": 26, "end_offset": 30, "label": "dis" } ]
H1:苔膜变薄,溃疡缩小,周围有上皮再生;H2:接近愈合,但溃疡面仍有极少白薄苔。
[ { "id": 0, "entity": "H1", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "苔膜变薄,溃疡缩小,周围有上皮再生", "start_offset": 3, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "H2", "start_offset": 21, "end_offset": 23, "label": "dis" }, { "id": 3, "entity": "接近愈合,但溃疡面仍有极少白薄苔", "start_offset": 24, "end_offset": 40, "label": "sym" } ]
(3)瘢痕期(scarringstage;S期):又称无苔期。
[ { "id": 0, "entity": "瘢痕期", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "scarringstage", "start_offset": 7, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "S期", "start_offset": 21, "end_offset": 23, "label": "dis" }, { "id": 3, "entity": "无苔期", "start_offset": 27, "end_offset": 30, "label": "dis" } ]
S1:溃疡白苔已消失,中央充血呈红色,又称红色瘢痕期;S2:中央充血已完全消退,变为白色瘢痕,亦称白色瘢痕期。
[ { "id": 0, "entity": "S1", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "溃疡白苔已消失,中央充血呈红色", "start_offset": 3, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "红色瘢痕期", "start_offset": 21, "end_offset": 26, "label": "dis" }, { "id": 3, "entity": "S2", "start_offset": 27, "end_offset": 29, "label": "dis" }, { "id": 4, "entity": "中央充血已完全消退,变为白色瘢痕", "start_offset": 30, "end_offset": 46, "label": "sym" }, { "id": 5, "entity": "白色瘢痕期", "start_offset": 49, "end_offset": 54, "label": "dis" } ]
四周有黏液纹辐射,表示溃疡已完全愈合。
[ { "id": 0, "entity": "四周有黏液纹辐射,表示溃疡已完全愈合", "start_offset": 0, "end_offset": 18, "label": "sym" } ]
儿童期溃疡以十二指肠多见,幼小儿童多见胃溃疡。
[ { "id": 0, "entity": "溃疡", "start_offset": 3, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "十二指肠", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 2, "entity": "胃溃疡", "start_offset": 19, "end_offset": 22, "label": "dis" } ]
十二指肠球部溃疡,大多位于前壁大弯侧,特点是溃疡病灶浅,愈合快,部分患儿不需治疗,常可自愈,溃疡灶周边充血、水肿显著,消退缓慢。
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胃镜检查常可发现溃疡已愈合,但充血,水肿仍明显的球炎状态。
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所以有作者认为十二指肠球部溃疡是球炎的发展,而球炎是溃疡修复中的一个表现。
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另在十二指肠球部溃疡中可见到下述两种特殊类型:①十二指肠多发性溃疡;前后壁各有一溃疡,中间有桥状皱襞连接,也称对口(接吻)溃疡;②霜斑样溃疡:表现一片充血的黏膜上,覆盖散在的、细微的小白斑或小白苔,给人以下过霜一样的外貌。
[ { "id": 0, "entity": "二指肠球部溃疡", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "十二指肠多发性溃疡", "start_offset": 24, "end_offset": 33, "label": "dis" }, { "id": 2, "entity": "前后壁各有一溃疡,中间有桥状皱襞连接", "start_offset": 34, "end_offset": 52, "label": "sym" }, { "id": 3, "entity": "对口(接吻)溃疡", "start_offset": 55, "end_offset": 63, "label": "dis" }, { "id": 4, "entity": "霜斑样溃疡", "start_offset": 65, "end_offset": 70, "label": "dis" }, { "id": 5, "entity": "表现一片充血的黏膜上,覆盖散在的、细微的小白斑或小白苔,给人以下过霜一样的外貌", "start_offset": 71, "end_offset": 110, "label": "sym" } ]
第六节过敏性紫癜肾炎过敏性紫癜(anaphylactoidpurpura,AP)(henochschonleinpurpura,HSP)是以皮肤紫癜、出血性胃肠炎、关节炎及肾小球肾炎为主要特点的临床综合征。
[ { "id": 0, "entity": "过敏性紫癜肾炎", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "过敏性紫癜", "start_offset": 10, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "anaphylactoidpurpura", "start_offset": 16, "end_offset": 36, "label": "dis" }, { "id": 3, "entity": "AP", "start_offset": 37, "end_offset": 39, "label": "dis" }, { "id": 4, "entity": "henochschonleinpurpura", "start_offset": 41, "end_offset": 63, "label": "dis" }, { "id": 5, "entity": "HSP", "start_offset": 64, "end_offset": 67, "label": "dis" }, { "id": 6, "entity": "皮肤紫癜", "start_offset": 70, "end_offset": 74, "label": "dis" }, { "id": 7, "entity": "出血性胃肠炎", "start_offset": 75, "end_offset": 81, "label": "dis" }, { "id": 8, "entity": "关节炎", "start_offset": 82, "end_offset": 85, "label": "dis" }, { "id": 9, "entity": "肾小球肾炎", "start_offset": 86, "end_offset": 91, "label": "dis" } ]
HSP患儿中约有一半出现肾损害,此时称过敏性紫癜肾炎(henochschonleinpurpuranephritis,HSPN)。
[ { "id": 0, "entity": "HSP", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "肾损害", "start_offset": 12, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "过敏性紫癜肾炎", "start_offset": 19, "end_offset": 26, "label": "dis" }, { "id": 3, "entity": "henochschonleinpurpuranephritis", "start_offset": 27, "end_offset": 58, "label": "dis" }, { "id": 4, "entity": "HSPN", "start_offset": 59, "end_offset": 63, "label": "dis" } ]
【病因及发病机制】HSP是白细胞碎裂性小血管炎,是主要由IgA免疫复合物沉积引起的免疫复合物病,其病因仍未完全明了,可能与下列因素有关:感染、疫苗接种、虫咬、寒冷刺激、药物过敏和食物过敏等。
[ { "id": 0, "entity": "HSP", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "白细胞碎裂性小血管炎", "start_offset": 13, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "IgA免疫复合物沉积", "start_offset": 28, "end_offset": 38, "label": "sym" }, { "id": 3, "entity": "免疫复合物病", "start_offset": 41, "end_offset": 47, "label": "dis" }, { "id": 4, "entity": "感染", "start_offset": 68, "end_offset": 70, "label": "sym" }, { "id": 5, "entity": "疫苗接种", "start_offset": 71, "end_offset": 75, "label": "pro" }, { "id": 6, "entity": "药物过敏", "start_offset": 84, "end_offset": 88, "label": "sym" }, { "id": 7, "entity": "食物过敏", "start_offset": 89, "end_offset": 93, "label": "sym" } ]
尽管这些因素都可能诱发HSP,但临床上仍难明确过敏原,脱敏治疗的效果往往难以令人满意。
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鉴于HSPN免疫病理的显著特点是系膜区颗粒状IgA沉积,与IgA肾病改变极为相似,因此推测IgA在发病中有重要作用,甚至有人认为它们本质上是同一种疾病。
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进一步研究发现两者免疫发病机制确有惊人的一致性,如均有血清IgA升高、单体以及多聚体IgA升高、λ-IgA1升高,两者血清中均有循环IgA免疫复合物;沉积在肾小球上的均以多聚IgA<sub>1</sub>为主,且有J链沉积;两者都有C<sub>4a</sub>、C<sub>4b</sub>亚型缺陷,都有IgA<sub>1</sub>O型糖基化异常等等。
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华中科技大学同济医学院附属同济医院儿科对120例HSPN及31例IgA肾病进行了比较研究,发现HSPN6.3%有IgG沿肾小球毛细血管壁的线样沉积以及膜抗肾小球基底抗体阳性,而且12.5%不是以IgA为主要沉积物,因此,至少在一部分HSPN中,其发病机制与IgA肾病显著不同。
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HSPN的肾脏损伤中补体发挥重要作用,补体的激活可能是通过旁路途径实现的:①IgA无激活C<sub>1q</sub>的能力,而能直接激活C<sub>3</sub>;②在在肾小球系膜区证实有C<sub>3</sub>、备解素C<sub>3</sub>PA,而无C<sub>1q</sub>及C<sub>4</sub>;③C<sub>2</sub>缺乏的病人易患本病。
[ { "id": 0, "entity": "HSPN", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "肾脏损伤", "start_offset": 5, "end_offset": 9, "label": "dis" }, { "id": 2, "entity": "补体", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 3, "entity": "补体", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "肾小球系膜区", "start_offset": 85, "end_offset": 91, "label": "bod" }, { "id": 5, "entity": "在肾小球系膜区证实有C<sub>3</sub>、备解素C<sub>3</sub>PA,而无C<sub>1q</sub>及C<sub>4</sub>", "start_offset": 84, "end_offset": 157, "label": "sym" }, { "id": 6, "entity": "C<sub>2</sub>缺乏", "start_offset": 159, "end_offset": 174, "label": "sym" } ]
【病理】HSPN的基本病理改变为肾小球系膜增生和系膜区IgA沉积,严重时尚有新月体形成和肾小管坏死,病理改变轻重差别很大,国际小儿肾脏病研究会根据光镜下肾小球的改变将HSPN分为6型:Ⅰ:肾小球轻微改变。
[ { "id": 0, "entity": "HSPN", "start_offset": 4, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "肾小球系膜增生", "start_offset": 16, "end_offset": 23, "label": "sym" }, { "id": 2, "entity": "系膜区IgA沉积", "start_offset": 24, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "有新月体形成", "start_offset": 37, "end_offset": 43, "label": "sym" }, { "id": 4, "entity": "肾小管", "start_offset": 44, "end_offset": 47, "label": "bod" }, { "id": 5, "entity": "肾小管坏死", "start_offset": 44, "end_offset": 49, "label": "sym" }, { "id": 6, "entity": "小儿肾脏病", "start_offset": 63, "end_offset": 68, "label": "dis" }, { "id": 7, "entity": "光镜", "start_offset": 73, "end_offset": 75, "label": "equ" }, { "id": 8, "entity": "肾小球", "start_offset": 76, "end_offset": 79, "label": "bod" }, { "id": 9, "entity": "HSPN", "start_offset": 83, "end_offset": 87, "label": "dis" }, { "id": 10, "entity": "肾小球", "start_offset": 94, "end_offset": 97, "label": "bod" }, { "id": 11, "entity": "肾小球轻微改变", "start_offset": 94, "end_offset": 101, "label": "sym" } ]
Ⅱ:单纯性系膜增生,不伴毛细血管袢局灶性改变及新月体:Ⅱa:局灶节段性系膜增生;Ⅱb:弥漫性系膜增生。
[ { "id": 0, "entity": "毛细血管", "start_offset": 12, "end_offset": 16, "label": "bod" }, { "id": 1, "entity": "单纯性系膜增生,不伴毛细血管袢局灶性改变及新月体", "start_offset": 2, "end_offset": 26, "label": "sym" }, { "id": 2, "entity": "局灶节段性系膜增生", "start_offset": 30, "end_offset": 39, "label": "sym" }, { "id": 3, "entity": "弥漫性系膜增生", "start_offset": 43, "end_offset": 50, "label": "sym" } ]
Ⅲ:系膜增生并出现局灶改变(血栓、坏死、新月体及硬化):Ⅲa:局灶性改变;Ⅲb:新月体出现,但<50%。
[ { "id": 0, "entity": "系膜增生并出现局灶改变", "start_offset": 2, "end_offset": 13, "label": "sym" }, { "id": 1, "entity": "血栓", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "坏死", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "新月体", "start_offset": 20, "end_offset": 23, "label": "sym" }, { "id": 4, "entity": "硬化", "start_offset": 24, "end_offset": 26, "label": "sym" }, { "id": 5, "entity": "局灶性改变", "start_offset": 31, "end_offset": 36, "label": "sym" }, { "id": 6, "entity": "新月体出现,但<50%", "start_offset": 40, "end_offset": 51, "label": "sym" } ]
Ⅳ:系膜增生,50%~75%肾小球有新月体或局灶性改变:Ⅳa:局灶性病变;Ⅳb:新月体50%~75%。
[ { "id": 0, "entity": "系膜增生,50%~75%肾小球有新月体或局灶性改变", "start_offset": 2, "end_offset": 27, "label": "sym" }, { "id": 1, "entity": "局灶性病变", "start_offset": 31, "end_offset": 36, "label": "sym" }, { "id": 2, "entity": "新月体50%~75%", "start_offset": 40, "end_offset": 50, "label": "sym" } ]
Ⅴ:>75%肾小球有新月体或局灶病变:Ⅴa:局灶性病变;Ⅴb:新月体。
[ { "id": 0, "entity": ">75%肾小球有新月体或局灶病变", "start_offset": 2, "end_offset": 18, "label": "sym" }, { "id": 1, "entity": "局灶性病变", "start_offset": 22, "end_offset": 27, "label": "sym" }, { "id": 2, "entity": "新月体", "start_offset": 31, "end_offset": 34, "label": "sym" } ]
Ⅵ:膜增生性肾小球肾炎。
[ { "id": 0, "entity": "膜增生性肾小球肾炎", "start_offset": 2, "end_offset": 11, "label": "dis" } ]
免疫荧光检查显示以系膜区团块状IgA沉积为主,可伴有C<sub>3</sub>、IgG和备解素,但荧光强度较IgA为弱。
[ { "id": 0, "entity": "免疫荧光检查", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "系膜区", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "系膜区团块状IgA沉积", "start_offset": 9, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "荧光强度较IgA为弱", "start_offset": 49, "end_offset": 59, "label": "sym" } ]
即使光镜下病变呈局灶、节段分布,在免疫荧光镜下沉积物仍弥漫分布。
[ { "id": 0, "entity": "光镜", "start_offset": 2, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "病变呈局灶、节段分布", "start_offset": 5, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "免疫荧光镜", "start_offset": 17, "end_offset": 22, "label": "equ" }, { "id": 3, "entity": "沉积物仍弥漫分布", "start_offset": 23, "end_offset": 31, "label": "sym" } ]
严重者延伸于毛细血管壁内。
[ { "id": 0, "entity": "毛细血管壁", "start_offset": 6, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "延伸于毛细血管壁内", "start_offset": 3, "end_offset": 12, "label": "sym" } ]
皮下及肠道小血管壁也常见IgA沉积。
[ { "id": 0, "entity": "皮下及肠道小血管壁", "start_offset": 0, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "皮下及肠道小血管壁也常见IgA沉积", "start_offset": 0, "end_offset": 17, "label": "sym" } ]
电镜下可见系膜细胞增生,系膜基质增多和系膜区大小不等的细颗粒状电子致密物沉积,沉积亦可在内皮下。
[ { "id": 0, "entity": "电镜", "start_offset": 0, "end_offset": 2, "label": "equ" }, { "id": 1, "entity": "系膜细胞", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "系膜细胞增生", "start_offset": 5, "end_offset": 11, "label": "sym" }, { "id": 3, "entity": "系膜基质", "start_offset": 12, "end_offset": 16, "label": "bod" }, { "id": 4, "entity": "系膜基质增多", "start_offset": 12, "end_offset": 18, "label": "sym" }, { "id": 5, "entity": "系膜区", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 6, "entity": "系膜区大小不等的细颗粒状电子致密物沉积", "start_offset": 19, "end_offset": 38, "label": "sym" }, { "id": 7, "entity": "内皮", "start_offset": 44, "end_offset": 46, "label": "bod" } ]
免疫电镜证实沉积物中主要含IgA及少量C<sub>3</sub>和IgG。
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这些改变轻者呈局灶性分布,重者呈弥漫性分布。
[ { "id": 0, "entity": "局灶性分布", "start_offset": 7, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "弥漫性分布", "start_offset": 16, "end_offset": 21, "label": "sym" } ]
【临床表现】(一)肾外表现主要是过敏性紫癜所致的皮肤、胃肠及关节等方面的症状与体征。
[ { "id": 0, "entity": "肾", "start_offset": 9, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "过敏性紫癜", "start_offset": 16, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "皮肤", "start_offset": 24, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "胃肠", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 4, "entity": "关节", "start_offset": 30, "end_offset": 32, "label": "bod" } ]
1.皮疹对称性分布于双下肢伸侧,严重时可波及臀部、下腹及肘部。
[ { "id": 0, "entity": "皮疹", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "对称性分布于双下肢伸侧", "start_offset": 4, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "臀部", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 3, "entity": "下腹", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "肘部", "start_offset": 28, "end_offset": 30, "label": "bod" }, { "id": 5, "entity": "严重时可波及臀部、下腹及肘部", "start_offset": 16, "end_offset": 30, "label": "sym" } ]
2.关节1/2~2/3病人出现关节肿痛,以膝关节和踝关节多见,活动可受限,一般数日内即可恢复。
[ { "id": 0, "entity": "关节", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "关节", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "关节肿痛", "start_offset": 15, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "膝关节", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 4, "entity": "踝关节", "start_offset": 25, "end_offset": 28, "label": "bod" } ]
3.胃肠道症状1/3病人出现阵发性腹部绞痛,脐周为主,可伴呕吐、黑便及呕血等,个别可出现肠梗阻、肠穿孔及肠套叠等。
[ { "id": 0, "entity": "胃肠道", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "胃肠道症状", "start_offset": 2, "end_offset": 7, "label": "sym" }, { "id": 2, "entity": "腹部", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "阵发性腹部绞痛", "start_offset": 14, "end_offset": 21, "label": "sym" }, { "id": 4, "entity": "脐周", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 5, "entity": "呕吐、黑便及呕血", "start_offset": 29, "end_offset": 37, "label": "sym" }, { "id": 6, "entity": "肠梗阻", "start_offset": 44, "end_offset": 47, "label": "dis" }, { "id": 7, "entity": "肠穿孔", "start_offset": 48, "end_offset": 51, "label": "dis" }, { "id": 8, "entity": "肠套叠", "start_offset": 52, "end_offset": 55, "label": "dis" } ]
4.其他如鼻出血、咯血及心肌炎,少数伴头痛和抽搐。
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(二)肾脏表现以血尿和蛋白尿为主。
[ { "id": 0, "entity": "肾脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "血尿", "start_offset": 8, "end_offset": 10, "label": "sym" }, { "id": 2, "entity": "蛋白尿", "start_offset": 11, "end_offset": 14, "label": "sym" } ]
1.血尿约一半病人出现肉眼血尿,均有镜下血尿。
[ { "id": 0, "entity": "血尿", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "肉眼血尿", "start_offset": 11, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "镜下血尿", "start_offset": 18, "end_offset": 22, "label": "sym" } ]
2.蛋白尿程度不等。
[ { "id": 0, "entity": "蛋白尿", "start_offset": 2, "end_offset": 5, "label": "sym" } ]
3.水肿一般为轻~中度,非凹陷性,伴大量蛋白尿时可为凹陷性水肿。
[ { "id": 0, "entity": "水肿", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "大量蛋白尿", "start_offset": 18, "end_offset": 23, "label": "sym" }, { "id": 2, "entity": "凹陷性水肿", "start_offset": 26, "end_offset": 31, "label": "sym" } ]
4.高血压。
[ { "id": 0, "entity": "高血压", "start_offset": 2, "end_offset": 5, "label": "dis" } ]
(三)其他表现HSP可累及中枢神经系统、心血管系统以及胸膜外分泌腺等而出现相应症状。
[ { "id": 0, "entity": "HSP", "start_offset": 7, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "中枢神经系统", "start_offset": 13, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "心血管系统", "start_offset": 20, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "胸膜外分泌腺", "start_offset": 27, "end_offset": 33, "label": "bod" } ]
【实验室检查】血常规及出凝血试验均可正常,ESR升高;IgA可升高,并可检出IgA类风湿因子。
[ { "id": 0, "entity": "血常规", "start_offset": 7, "end_offset": 10, "label": "ite" }, { "id": 1, "entity": "出凝血试验", "start_offset": 11, "end_offset": 16, "label": "pro" }, { "id": 2, "entity": "ESR升高", "start_offset": 21, "end_offset": 26, "label": "sym" }, { "id": 3, "entity": "IgA", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "IgA可升高", "start_offset": 27, "end_offset": 33, "label": "sym" }, { "id": 5, "entity": "IgA类风湿因子", "start_offset": 38, "end_offset": 46, "label": "bod" } ]
其他如IgG、IgA及IgE均可增高或正常,血生化及肾功能可因临床表现类型的不同而正常或出现相应的异常改变。
[ { "id": 0, "entity": "IgG", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "IgA", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "IgE", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "IgG、IgA及IgE均可增高或正常", "start_offset": 3, "end_offset": 21, "label": "sym" }, { "id": 4, "entity": "血生化", "start_offset": 22, "end_offset": 25, "label": "ite" }, { "id": 5, "entity": "肾功能", "start_offset": 26, "end_offset": 29, "label": "ite" } ]
尿液检查主要为血尿和蛋白尿,如有间质小管损害,可出现小分子蛋白如RBP、β<sub>2</sub>-微球蛋白及溶菌酶等增高。
[ { "id": 0, "entity": "尿液检查", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "血尿", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "蛋白尿", "start_offset": 10, "end_offset": 13, "label": "sym" }, { "id": 3, "entity": "间质小管损害", "start_offset": 16, "end_offset": 22, "label": "dis" }, { "id": 4, "entity": "RBP", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 5, "entity": "β<sub>2</sub>-微球蛋白", "start_offset": 36, "end_offset": 54, "label": "bod" }, { "id": 6, "entity": "溶菌酶", "start_offset": 55, "end_offset": 58, "label": "bod" }, { "id": 7, "entity": "RBP、β<sub>2</sub>-微球蛋白及溶菌酶等增高", "start_offset": 32, "end_offset": 61, "label": "sym" } ]
【诊断和鉴别诊断】中华医学会儿科分会肾脏病学组于2000年11月珠海会议上制定的诊断标准为:(一)诊断标准在过敏性紫癜病程中(多数在6个月内),出现血尿和(或)蛋白尿。
[ { "id": 0, "entity": "肾脏病", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 1, "entity": "过敏性紫癜", "start_offset": 54, "end_offset": 59, "label": "dis" }, { "id": 2, "entity": "血尿", "start_offset": 74, "end_offset": 76, "label": "sym" }, { "id": 3, "entity": "蛋白尿", "start_offset": 80, "end_offset": 83, "label": "sym" } ]
(二)临床分型1.孤立性血尿或孤立性蛋白尿。
[ { "id": 0, "entity": "孤立性血尿", "start_offset": 9, "end_offset": 14, "label": "sym" }, { "id": 1, "entity": "孤立性蛋白尿", "start_offset": 15, "end_offset": 21, "label": "sym" } ]
2.血尿和蛋白尿。
[ { "id": 0, "entity": "血尿", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "蛋白尿", "start_offset": 5, "end_offset": 8, "label": "sym" } ]
5.急进性肾炎型。
[ { "id": 0, "entity": "急进性肾炎型", "start_offset": 2, "end_offset": 8, "label": "dis" } ]
【治疗】(一)一般治疗急性期应卧床休息,如有明确过敏原,应脱敏治疗。
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