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部分年幼婴儿,水痘肺炎可出现在皮疹之前,极易误诊和漏诊。
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因而有明确水痘接触史者,如发生肺炎,亦应考虑本病,并予以隔离。
[ { "end_idx": 6, "entity": "水痘", "start_idx": 5, "type": "dis" }, { "end_idx": 16, "entity": "肺炎", "start_idx": 15, "type": "dis" } ]
二、供肾的选择儿童肾移植具有更广泛的供肾来源,成人供肾移植给10岁以上的儿童,技术操作与成人肾移植无差别;成人肾移植给婴幼儿需克服手术操作中的一些困难;体重7~8kg以下的婴幼儿宜使用儿童供肾;取自5岁以下小儿的尸体肾移植给年龄较大的儿童可获满意的肾功能。
[ { "end_idx": 3, "entity": "肾", "start_idx": 3, "type": "bod" }, { "end_idx": 11, "entity": "儿童肾移植", "start_idx": 7, "type": "pro" }, { "end_idx": 19, "entity": "肾", "start_idx": 19, "type": "bod" }, { "end_idx": 26, "entity": "肾", "start_idx": 26, "type": "bod" }, { "end_idx": 28, "entity": "移植", "start_idx": 27, "type": "pro" }, { "end_idx": 48, "entity": "成人肾移植", "start_idx": 44, "type": "pro" }, { "end_idx": 57, "entity": "肾移植", "start_idx": 55, "type": "pro" }, { "end_idx": 66, "entity": "手术", "start_idx": 65, "type": "pro" }, { "end_idx": 95, "entity": "肾", "start_idx": 95, "type": "bod" }, { "end_idx": 110, "entity": "肾移植", "start_idx": 108, "type": "pro" }, { "end_idx": 124, "entity": "肾", "start_idx": 124, "type": "bod" } ]
儿童肾移植更容易从亲属中获取活体肾移植,无论在组织相容性还是缩短缺血时间上都更具优越性,从而降低排斥反应的发生率。
[ { "end_idx": 4, "entity": "儿童肾移植", "start_idx": 0, "type": "pro" }, { "end_idx": 18, "entity": "肾移植", "start_idx": 16, "type": "pro" }, { "end_idx": 24, "entity": "组织", "start_idx": 23, "type": "bod" }, { "end_idx": 33, "entity": "缺血", "start_idx": 32, "type": "sym" }, { "end_idx": 33, "entity": "血", "start_idx": 33, "type": "bod" }, { "end_idx": 51, "entity": "排斥反应", "start_idx": 48, "type": "sym" } ]
另有成功病例报道将新生儿尸体双肾连同主动脉、腔静脉一并切下并移植给先天性马蹄肾肾衰竭的患儿。
[ { "end_idx": 15, "entity": "双肾", "start_idx": 14, "type": "bod" }, { "end_idx": 20, "entity": "主动脉", "start_idx": 18, "type": "bod" }, { "end_idx": 24, "entity": "腔静脉", "start_idx": 22, "type": "bod" }, { "end_idx": 41, "entity": "先天性马蹄肾肾衰竭", "start_idx": 33, "type": "dis" } ]
三、葡萄糖-6-磷酸脱氢酶缺陷症红细胞葡萄糖-6-磷酸脱氢酶(glucose-6-phosphatedehydrogenase,G-6-PD)缺陷症是一种常见伴性不完全显性遗传性红细胞膜酶缺陷病,此类缺陷遍布全世界,据统计约有2亿多人具有此类缺陷。
[ { "end_idx": 15, "entity": "葡萄糖-6-磷酸脱氢酶缺陷症", "start_idx": 2, "type": "dis" }, { "end_idx": 73, "entity": "红细胞葡萄糖-6-磷酸脱氢酶(glucose-6-phosphatedehydrogenase,G-6-PD)缺陷症", "start_idx": 16, "type": "dis" }, { "end_idx": 96, "entity": "伴性不完全显性遗传性红细胞膜酶缺陷病", "start_idx": 79, "type": "dis" }, { "end_idx": 101, "entity": "缺陷", "start_idx": 100, "type": "dis" }, { "end_idx": 122, "entity": "缺陷", "start_idx": 121, "type": "dis" } ]
编码该酶蛋白的基因位于X染色体长臂2区8带(Xq28)。
[ { "end_idx": 8, "entity": "编码该酶蛋白的基因", "start_idx": 0, "type": "bod" }, { "end_idx": 20, "entity": "X染色体长臂2区8带", "start_idx": 11, "type": "bod" }, { "end_idx": 25, "entity": "Xq28", "start_idx": 22, "type": "bod" } ]
目前已证实至少有90种G-6-PD突变基因存在,大多为编码区内单个或多个碱基置换的突变,少数为缺失型。
[ { "end_idx": 20, "entity": "G-6-PD突变基因", "start_idx": 11, "type": "bod" }, { "end_idx": 42, "entity": "编码区内单个或多个碱基置换的突变", "start_idx": 27, "type": "sym" }, { "end_idx": 48, "entity": "缺失", "start_idx": 47, "type": "sym" } ]
出现慢性非球形红细胞溶血性贫血(CNSHA)者的基因突变发生于G-6-PD基因羧基末端第1089~1361核苷酸序列中,突变发生于氨基末端者病情较轻。
[ { "end_idx": 14, "entity": "慢性非球形红细胞溶血性贫血", "start_idx": 2, "type": "dis" }, { "end_idx": 20, "entity": "CNSHA", "start_idx": 16, "type": "dis" }, { "end_idx": 27, "entity": "基因突变", "start_idx": 24, "type": "sym" }, { "end_idx": 25, "entity": "基因", "start_idx": 24, "type": "bod" }, { "end_idx": 58, "entity": "G-6-PD基因羧基末端第1089~1361核苷酸序列中", "start_idx": 31, "type": "bod" }, { "end_idx": 61, "entity": "突变", "start_idx": 60, "type": "sym" }, { "end_idx": 68, "entity": "氨基末端", "start_idx": 65, "type": "bod" } ]
我国已发现12种G-6-PD突变基因,均为点突变。
[ { "end_idx": 17, "entity": "G-6-PD突变基因", "start_idx": 8, "type": "bod" }, { "end_idx": 23, "entity": "点突变", "start_idx": 21, "type": "sym" } ]
多种G-6-PD突变基因所表达的产物为各种变异酶,它们的活性大多降低或稳定性降低。
[ { "end_idx": 11, "entity": "G-6-PD突变基因", "start_idx": 2, "type": "bod" }, { "end_idx": 23, "entity": "变异酶", "start_idx": 21, "type": "bod" }, { "end_idx": 39, "entity": "它们的活性大多降低或稳定性降低", "start_idx": 25, "type": "sym" } ]
到目前为止,已发现400多种G-6-PD变异酶,其中可引起各种溶血症者有100种以上。
[ { "end_idx": 22, "entity": "G-6-PD变异酶", "start_idx": 14, "type": "bod" }, { "end_idx": 33, "entity": "溶血症", "start_idx": 31, "type": "dis" } ]
根据酶的活性和是否出现临床表现,可将它们分为五大类:①严重酶缺陷伴CNSHA;②酶活性严重缺乏,活性<10%;③酶活性轻~中度缺陷,活性为10%~60%;④酶活性轻度降低或正常,活性为60%~100%;⑤酶活性增加,高于正常的4~5倍。
[ { "end_idx": 5, "entity": "酶的活性", "start_idx": 2, "type": "ite" }, { "end_idx": 37, "entity": "严重酶缺陷伴CNSHA", "start_idx": 27, "type": "dis" }, { "end_idx": 53, "entity": "酶活性严重缺乏,活性<10%", "start_idx": 40, "type": "sym" }, { "end_idx": 42, "entity": "酶活性", "start_idx": 40, "type": "ite" }, { "end_idx": 75, "entity": "酶活性轻~中度缺陷,活性为10%~60%", "start_idx": 56, "type": "sym" }, { "end_idx": 58, "entity": "酶活性", "start_idx": 56, "type": "ite" }, { "end_idx": 99, "entity": "酶活性轻度降低或正常,活性为60%~100%", "start_idx": 78, "type": "sym" }, { "end_idx": 80, "entity": "酶活性", "start_idx": 78, "type": "ite" }, { "end_idx": 116, "entity": "酶活性增加,高于正常的4~5倍", "start_idx": 102, "type": "sym" }, { "end_idx": 104, "entity": "酶活性", "start_idx": 102, "type": "ite" } ]
其中②③类多数表现为新生儿高胆红素血症及急性溶血性贫血,少数伴有CNSHA。
[ { "end_idx": 18, "entity": "新生儿高胆红素血症", "start_idx": 10, "type": "dis" }, { "end_idx": 26, "entity": "急性溶血性贫血", "start_idx": 20, "type": "dis" }, { "end_idx": 36, "entity": "CNSHA", "start_idx": 32, "type": "dis" } ]
根据发病种族的不同,将G-6-PD分为G-6-PDA和G-6-PDB两种变异型。
[ { "end_idx": 16, "entity": "G-6-PD", "start_idx": 11, "type": "bod" }, { "end_idx": 25, "entity": "G-6-PDA", "start_idx": 19, "type": "bod" }, { "end_idx": 33, "entity": "G-6-PDB", "start_idx": 27, "type": "bod" } ]
在我国广东,还发现另一种变异型,称为G-6-PDCanton(广东型),区域发病率可高达5%。
[ { "end_idx": 13, "entity": "变异", "start_idx": 12, "type": "sym" }, { "end_idx": 29, "entity": "G-6-PDCanton", "start_idx": 18, "type": "bod" } ]
【发病机制】G-6-PD在磷酸戊糖旁路中是6-磷酸葡萄糖(G-6-P)转变为6-磷酸葡萄糖酸(G-6-PG)反应中必需的酶。
[ { "end_idx": 11, "entity": "G-6-PD", "start_idx": 6, "type": "bod" }, { "end_idx": 16, "entity": "磷酸戊糖", "start_idx": 13, "type": "bod" }, { "end_idx": 27, "entity": "6-磷酸葡萄糖", "start_idx": 21, "type": "bod" }, { "end_idx": 33, "entity": "G-6-P", "start_idx": 29, "type": "bod" }, { "end_idx": 45, "entity": "6-磷酸葡萄糖酸", "start_idx": 38, "type": "bod" }, { "end_idx": 52, "entity": "G-6-PG", "start_idx": 47, "type": "bod" }, { "end_idx": 60, "entity": "酶", "start_idx": 60, "type": "bod" } ]
G-6-PD缺乏时,使还原型三磷酸吡啶核苷(NADPH)减少,不能维持生理浓度的还原型谷胱甘肽(GSH),从而使红细胞膜蛋白和酶蛋白中的巯基遭受氧化,改变了红细胞膜的完整性。
[ { "end_idx": 7, "entity": "G-6-PD缺乏", "start_idx": 0, "type": "sym" }, { "end_idx": 5, "entity": "G-6-PD", "start_idx": 0, "type": "bod" }, { "end_idx": 29, "entity": "还原型三磷酸吡啶核苷(NADPH)减少", "start_idx": 11, "type": "sym" }, { "end_idx": 20, "entity": "还原型三磷酸吡啶核苷", "start_idx": 11, "type": "ite" }, { "end_idx": 26, "entity": "NADPH", "start_idx": 22, "type": "ite" }, { "end_idx": 51, "entity": "不能维持生理浓度的还原型谷胱甘肽(GSH)", "start_idx": 31, "type": "sym" }, { "end_idx": 46, "entity": "还原型谷胱甘肽", "start_idx": 40, "type": "bod" }, { "end_idx": 50, "entity": "GSH", "start_idx": 48, "type": "bod" }, { "end_idx": 73, "entity": "红细胞膜蛋白和酶蛋白中的巯基遭受氧化", "start_idx": 56, "type": "sym" }, { "end_idx": 61, "entity": "红细胞膜蛋白", "start_idx": 56, "type": "bod" }, { "end_idx": 65, "entity": "酶蛋白", "start_idx": 63, "type": "bod" }, { "end_idx": 69, "entity": "巯基", "start_idx": 68, "type": "bod" }, { "end_idx": 85, "entity": "改变了红细胞膜的完整性", "start_idx": 75, "type": "sym" }, { "end_idx": 81, "entity": "红细胞膜", "start_idx": 78, "type": "bod" } ]
此外,NADPH减少时,使高铁血红蛋白(MHb)不能转变为氧合血红蛋白,MHb增加致红细胞内不可溶性变性珠蛋白小体(Heinz小体)形成明显增加,红细胞膜变硬,通过脾脏时Heinz小体被巨噬细胞摘出,随之丢失部分的红细胞膜,致表面积减少,可塑性降低,而易破裂溶血。
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【临床表现】本病一般可分为四种类型:(一)新生儿高胆红素血症新生儿红细胞G-6-PD缺乏时可受外源性或内源性氧化剂作用诱发或自发发生新生儿溶血症,导致高胆红素血症。
[ { "end_idx": 29, "entity": "新生儿高胆红素血症", "start_idx": 21, "type": "dis" }, { "end_idx": 43, "entity": "新生儿红细胞G-6-PD缺乏", "start_idx": 30, "type": "sym" }, { "end_idx": 41, "entity": "红细胞G-6-PD", "start_idx": 33, "type": "bod" }, { "end_idx": 56, "entity": "氧化剂", "start_idx": 54, "type": "dru" }, { "end_idx": 71, "entity": "新生儿溶血症", "start_idx": 66, "type": "dis" }, { "end_idx": 80, "entity": "高胆红素血症", "start_idx": 75, "type": "dis" } ]
我国新生儿G-6-PD缺乏发生率约3.0%~8.7%,其中严重黄疸发生率为20%~50%。
[ { "end_idx": 12, "entity": "G-6-PD缺乏", "start_idx": 5, "type": "sym" }, { "end_idx": 10, "entity": "G-6-PD", "start_idx": 5, "type": "bod" }, { "end_idx": 32, "entity": "严重黄疸", "start_idx": 29, "type": "dis" } ]
感染或药物可使症状加重。
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黄疸多出现在生后48~96小时内,也可迟至2周才出现黄疸。
[ { "end_idx": 1, "entity": "黄疸", "start_idx": 0, "type": "dis" }, { "end_idx": 27, "entity": "黄疸", "start_idx": 26, "type": "dis" } ]
进展迅速,严重的于1周后发生胆红素脑病甚至死亡。
[ { "end_idx": 18, "entity": "胆红素脑病", "start_idx": 14, "type": "dis" } ]
(二)蚕豆病(favism)本病最初因食用蚕豆、及其制品后发生急性溶血性贫血、黄疸而得名,多见于我国南方以及地中海沿岸国家的某些人群。
[ { "end_idx": 5, "entity": "蚕豆病", "start_idx": 3, "type": "dis" }, { "end_idx": 12, "entity": "favism", "start_idx": 7, "type": "dis" }, { "end_idx": 37, "entity": "急性溶血性贫血", "start_idx": 31, "type": "dis" }, { "end_idx": 40, "entity": "黄疸", "start_idx": 39, "type": "dis" } ]
溶血的轻重与食用蚕豆的量也无关系。
[ { "end_idx": 1, "entity": "溶血", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "血", "start_idx": 1, "type": "bod" } ]
目前认为,蚕豆中含有大量左旋多巴,在酪氨酸酶的作用下,可变成多巴醌,后者可使GSH含量减少而发生溶血。
[ { "end_idx": 21, "entity": "酪氨酸酶", "start_idx": 18, "type": "bod" }, { "end_idx": 44, "entity": "GSH含量减少", "start_idx": 38, "type": "sym" }, { "end_idx": 42, "entity": "GSH含量", "start_idx": 38, "type": "ite" }, { "end_idx": 49, "entity": "溶血", "start_idx": 48, "type": "sym" }, { "end_idx": 49, "entity": "血", "start_idx": 49, "type": "bod" } ]
本病大多发生在每年产新鲜蚕豆季节,在食用蚕豆或其制品、吸入蚕豆花粉、或吸吮乳母吃过蚕豆的乳汁后数小时至数天内发生不同程度的急性血管内溶血。
[ { "end_idx": 67, "entity": "急性血管内溶血", "start_idx": 61, "type": "sym" }, { "end_idx": 64, "entity": "血管", "start_idx": 63, "type": "bod" }, { "end_idx": 67, "entity": "血", "start_idx": 67, "type": "bod" } ]
血红蛋白迅速下降,多有黄疸。
[ { "end_idx": 7, "entity": "血红蛋白迅速下降", "start_idx": 0, "type": "sym" }, { "end_idx": 3, "entity": "血红蛋白", "start_idx": 0, "type": "ite" }, { "end_idx": 12, "entity": "黄疸", "start_idx": 11, "type": "dis" } ]
极重者甚至出现惊厥、休克、急性肾衰竭;如不及时治疗可于1~2天内死亡。
[ { "end_idx": 8, "entity": "惊厥", "start_idx": 7, "type": "dis" }, { "end_idx": 11, "entity": "休克", "start_idx": 10, "type": "dis" }, { "end_idx": 17, "entity": "急性肾衰竭", "start_idx": 13, "type": "dis" } ]
溶血期大多持续1~2天,最长1周左右,然后进入恢复期。
[ { "end_idx": 17, "entity": "溶血期大多持续1~2天,最长1周左右", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "血", "start_idx": 1, "type": "bod" } ]
(三)药物诱发溶血性贫血服用具有氧化作用的药物如:①乙酰苯胺(acetanild)、氨基比林等退热药;②伯氨喹、扑疟喹等抗疟药;③磺胺类药物;④其他如呋喃唑酮、呋喃坦丁、萘(naphthalene)、苯肼等可引起溶血。
[ { "end_idx": 11, "entity": "溶血性贫血", "start_idx": 7, "type": "dis" }, { "end_idx": 22, "entity": "具有氧化作用的药物", "start_idx": 14, "type": "dru" }, { "end_idx": 29, "entity": "乙酰苯胺", "start_idx": 26, "type": "dru" }, { "end_idx": 39, "entity": "acetanild", "start_idx": 31, "type": "dru" }, { "end_idx": 45, "entity": "氨基比林", "start_idx": 42, "type": "dru" }, { "end_idx": 49, "entity": "退热药", "start_idx": 47, "type": "dru" }, { "end_idx": 54, "entity": "伯氨喹", "start_idx": 52, "type": "dru" }, { "end_idx": 58, "entity": "扑疟喹", "start_idx": 56, "type": "dru" }, { "end_idx": 62, "entity": "抗疟药", "start_idx": 60, "type": "dru" }, { "end_idx": 69, "entity": "磺胺类药物", "start_idx": 65, "type": "dru" }, { "end_idx": 78, "entity": "呋喃唑酮", "start_idx": 75, "type": "dru" }, { "end_idx": 83, "entity": "呋喃坦丁", "start_idx": 80, "type": "dru" }, { "end_idx": 85, "entity": "萘", "start_idx": 85, "type": "dru" }, { "end_idx": 97, "entity": "naphthalene", "start_idx": 87, "type": "dru" }, { "end_idx": 101, "entity": "苯肼", "start_idx": 100, "type": "dru" }, { "end_idx": 107, "entity": "溶血", "start_idx": 106, "type": "sym" }, { "end_idx": 107, "entity": "血", "start_idx": 107, "type": "bod" } ]
具体某一药物对G-6-PD缺乏者诱发溶血作用并不一致,如服用氯霉素可诱发地中海沿岸重度G-6-PD缺乏者出现溶血,而在A-型或广东型的G-6-PD缺乏患者则不引起溶血。
[ { "end_idx": 14, "entity": "G-6-PD缺乏", "start_idx": 7, "type": "sym" }, { "end_idx": 12, "entity": "G-6-PD", "start_idx": 7, "type": "bod" }, { "end_idx": 19, "entity": "溶血", "start_idx": 18, "type": "sym" }, { "end_idx": 19, "entity": "血", "start_idx": 19, "type": "bod" }, { "end_idx": 32, "entity": "氯霉素", "start_idx": 30, "type": "dru" }, { "end_idx": 50, "entity": "地中海沿岸重度G-6-PD缺乏", "start_idx": 36, "type": "sym" }, { "end_idx": 48, "entity": "G-6-PD", "start_idx": 43, "type": "bod" }, { "end_idx": 55, "entity": "溶血", "start_idx": 54, "type": "sym" }, { "end_idx": 55, "entity": "血", "start_idx": 55, "type": "bod" }, { "end_idx": 74, "entity": "A-型或广东型的G-6-PD缺乏", "start_idx": 59, "type": "sym" }, { "end_idx": 72, "entity": "G-6-PD", "start_idx": 67, "type": "bod" }, { "end_idx": 82, "entity": "溶血", "start_idx": 81, "type": "sym" }, { "end_idx": 82, "entity": "血", "start_idx": 82, "type": "bod" } ]
急性溶血期持续1~2周,血红蛋白多于1周末降至最低点,此时出现血红蛋白尿,高铁血红蛋白血症,血浆GSH和结合珠蛋白下降,周围血涂片可见红细胞轻度大小不等,红细胞碎片,异型或嗜多染红细胞增多等。
[ { "end_idx": 3, "entity": "急性溶血", "start_idx": 0, "type": "sym" }, { "end_idx": 3, "entity": "血", "start_idx": 3, "type": "bod" }, { "end_idx": 25, "entity": "血红蛋白多于1周末降至最低点", "start_idx": 12, "type": "sym" }, { "end_idx": 15, "entity": "血红蛋白", "start_idx": 12, "type": "ite" }, { "end_idx": 35, "entity": "血红蛋白尿", "start_idx": 31, "type": "dis" }, { "end_idx": 44, "entity": "高铁血红蛋白血症", "start_idx": 37, "type": "dis" }, { "end_idx": 58, "entity": "血浆GSH和结合珠蛋白下降", "start_idx": 46, "type": "sym" }, { "end_idx": 50, "entity": "血浆GSH", "start_idx": 46, "type": "ite" }, { "end_idx": 56, "entity": "结合珠蛋白", "start_idx": 52, "type": "ite" }, { "end_idx": 64, "entity": "血涂片", "start_idx": 62, "type": "ite" }, { "end_idx": 75, "entity": "红细胞轻度大小不等", "start_idx": 67, "type": "sym" }, { "end_idx": 69, "entity": "红细胞", "start_idx": 67, "type": "ite" }, { "end_idx": 81, "entity": "红细胞碎片", "start_idx": 77, "type": "sym" }, { "end_idx": 79, "entity": "红细胞", "start_idx": 77, "type": "bod" }, { "end_idx": 93, "entity": "异型或嗜多染红细胞增多", "start_idx": 83, "type": "sym" }, { "end_idx": 91, "entity": "异型或嗜多染红细胞", "start_idx": 83, "type": "ite" } ]
肝、脾可轻度增大。
[ { "end_idx": 7, "entity": "肝、脾可轻度增大", "start_idx": 0, "type": "sym" }, { "end_idx": 0, "entity": "肝", "start_idx": 0, "type": "bod" }, { "end_idx": 2, "entity": "脾", "start_idx": 2, "type": "bod" } ]
2周后进入恢复期,网状红细胞、血红蛋白逐渐上升至正常,病情也明显好转,此时即使服用同类药物也无明显溶血,于4周后血红蛋白恢复正常。
[ { "end_idx": 25, "entity": "网状红细胞、血红蛋白逐渐上升至正常", "start_idx": 9, "type": "sym" }, { "end_idx": 13, "entity": "网状红细胞", "start_idx": 9, "type": "ite" }, { "end_idx": 18, "entity": "血红蛋白", "start_idx": 15, "type": "ite" }, { "end_idx": 50, "entity": "溶血", "start_idx": 49, "type": "sym" }, { "end_idx": 50, "entity": "血", "start_idx": 50, "type": "bod" }, { "end_idx": 59, "entity": "血红蛋白", "start_idx": 56, "type": "ite" } ]
此后进入代偿稳定期,由于骨髓代偿功能良好,病人无贫血或黄疸,网织红细胞正常。
[ { "end_idx": 17, "entity": "骨髓代偿功能", "start_idx": 12, "type": "ite" }, { "end_idx": 25, "entity": "贫血", "start_idx": 24, "type": "dis" }, { "end_idx": 28, "entity": "黄疸", "start_idx": 27, "type": "dis" }, { "end_idx": 34, "entity": "网织红细胞", "start_idx": 30, "type": "ite" } ]
但若于2~3月后再服此药物可再次诱发溶血。
[ { "end_idx": 19, "entity": "溶血", "start_idx": 18, "type": "sym" }, { "end_idx": 19, "entity": "血", "start_idx": 19, "type": "bod" } ]
(四)感染诱发的溶血性贫血G-6-PD缺乏患儿,在有些病毒和细菌感染如急性传染性肝炎、传染性单核细胞增多症、上呼吸道感染、肺炎、败血症、肠炎、菌痢、伤寒等情况下均可诱发与伯氨喹型药物相似的溶血性贫血,主要机制是由于急性感染过程中体内氧化产物如H2</sub>O2</sub>等堆积,造成红细胞破坏所致。
[ { "end_idx": 4, "entity": "感染", "start_idx": 3, "type": "dis" }, { "end_idx": 12, "entity": "溶血性贫血", "start_idx": 8, "type": "dis" }, { "end_idx": 20, "entity": "G-6-PD缺乏", "start_idx": 13, "type": "sym" }, { "end_idx": 18, "entity": "G-6-PD", "start_idx": 13, "type": "bod" }, { "end_idx": 33, "entity": "病毒和细菌感染", "start_idx": 27, "type": "dis" }, { "end_idx": 41, "entity": "急性传染性肝炎", "start_idx": 35, "type": "dis" }, { "end_idx": 52, "entity": "传染性单核细胞增多症", "start_idx": 43, "type": "dis" }, { "end_idx": 59, "entity": "上呼吸道感染", "start_idx": 54, "type": "dis" }, { "end_idx": 62, "entity": "肺炎", "start_idx": 61, "type": "dis" }, { "end_idx": 66, "entity": "败血症", "start_idx": 64, "type": "dis" }, { "end_idx": 69, "entity": "肠炎", "start_idx": 68, "type": "dis" }, { "end_idx": 72, "entity": "菌痢", "start_idx": 71, "type": "dis" }, { "end_idx": 75, "entity": "伤寒", "start_idx": 74, "type": "dis" }, { "end_idx": 90, "entity": "伯氨喹型药物", "start_idx": 85, "type": "dru" }, { "end_idx": 98, "entity": "溶血性贫血", "start_idx": 94, "type": "dis" }, { "end_idx": 110, "entity": "急性感染", "start_idx": 107, "type": "dis" }, { "end_idx": 139, "entity": "体内氧化产物如H2</sub>O2</sub>等堆积", "start_idx": 114, "type": "sym" }, { "end_idx": 119, "entity": "氧化产物", "start_idx": 116, "type": "bod" }, { "end_idx": 122, "entity": "H2", "start_idx": 121, "type": "bod" }, { "end_idx": 147, "entity": "红细胞破坏", "start_idx": 143, "type": "sym" }, { "end_idx": 145, "entity": "红细胞", "start_idx": 143, "type": "bod" } ]
【辅助检查】1.血象溶血发作时红细胞与血红蛋白迅速下降。
[ { "end_idx": 9, "entity": "血象", "start_idx": 8, "type": "ite" }, { "end_idx": 11, "entity": "溶血", "start_idx": 10, "type": "sym" }, { "end_idx": 11, "entity": "血", "start_idx": 11, "type": "bod" }, { "end_idx": 26, "entity": "红细胞与血红蛋白迅速下降", "start_idx": 15, "type": "sym" }, { "end_idx": 17, "entity": "红细胞", "start_idx": 15, "type": "ite" }, { "end_idx": 22, "entity": "血红蛋白", "start_idx": 19, "type": "ite" } ]
出现变形和嗜多色性红细胞及红细胞碎片,网织红细胞增多,大多在5%~15%之间,最高可达20%。
[ { "end_idx": 11, "entity": "变形和嗜多色性红细胞", "start_idx": 2, "type": "sym" }, { "end_idx": 11, "entity": "红细胞", "start_idx": 9, "type": "bod" }, { "end_idx": 17, "entity": "红细胞碎片", "start_idx": 13, "type": "sym" }, { "end_idx": 15, "entity": "红细胞", "start_idx": 13, "type": "bod" }, { "end_idx": 45, "entity": "网织红细胞增多,大多在5%~15%之间,最高可达20%", "start_idx": 19, "type": "sym" }, { "end_idx": 23, "entity": "网织红细胞", "start_idx": 19, "type": "ite" } ]
白细胞可以增高,达10×109</sup>~20×109</sup>/L,甚至出现类白血病反应,血小板通常正常或偏高。
[ { "end_idx": 35, "entity": "白细胞可以增高,达10×109</sup>~20×109</sup>/L", "start_idx": 0, "type": "sym" }, { "end_idx": 2, "entity": "白细胞", "start_idx": 0, "type": "ite" }, { "end_idx": 44, "entity": "白血病", "start_idx": 42, "type": "dis" }, { "end_idx": 57, "entity": "血小板通常正常或偏高", "start_idx": 48, "type": "sym" }, { "end_idx": 50, "entity": "血小板", "start_idx": 48, "type": "ite" } ]
2.骨髓象粒细胞、红细胞系均增生,粒细胞增生程度与发病年龄呈负相关。
[ { "end_idx": 15, "entity": "骨髓象粒细胞、红细胞系均增生", "start_idx": 2, "type": "sym" }, { "end_idx": 7, "entity": "骨髓象粒细胞", "start_idx": 2, "type": "ite" }, { "end_idx": 12, "entity": "红细胞系", "start_idx": 9, "type": "ite" }, { "end_idx": 32, "entity": "粒细胞增生程度与发病年龄呈负相关", "start_idx": 17, "type": "sym" }, { "end_idx": 19, "entity": "粒细胞", "start_idx": 17, "type": "ite" } ]
3.尿常规检查根据溶血程度的不同,肉眼观察可见尿呈酱油色、浓茶色、茶色或黄色,尿隐血试验60%~70%病例呈阳性。
[ { "end_idx": 6, "entity": "尿常规检查", "start_idx": 2, "type": "ite" }, { "end_idx": 10, "entity": "溶血", "start_idx": 9, "type": "sym" }, { "end_idx": 10, "entity": "血", "start_idx": 10, "type": "bod" }, { "end_idx": 18, "entity": "肉眼", "start_idx": 17, "type": "bod" }, { "end_idx": 37, "entity": "尿呈酱油色、浓茶色、茶色或黄色", "start_idx": 23, "type": "sym" }, { "end_idx": 23, "entity": "尿", "start_idx": 23, "type": "bod" }, { "end_idx": 55, "entity": "尿隐血试验60%~70%病例呈阳性", "start_idx": 39, "type": "sym" }, { "end_idx": 43, "entity": "尿隐血试验", "start_idx": 39, "type": "pro" } ]
严重时可导致肾功能损害,出现蛋白尿、红细胞尿及管型尿,尿胆原和尿胆红素增加。
[ { "end_idx": 10, "entity": "肾功能损害", "start_idx": 6, "type": "dis" }, { "end_idx": 16, "entity": "尿", "start_idx": 16, "type": "bod" }, { "end_idx": 21, "entity": "尿", "start_idx": 21, "type": "bod" }, { "end_idx": 25, "entity": "管型尿", "start_idx": 23, "type": "sym" }, { "end_idx": 25, "entity": "尿", "start_idx": 25, "type": "bod" }, { "end_idx": 36, "entity": "尿胆原和尿胆红素增加", "start_idx": 27, "type": "sym" }, { "end_idx": 29, "entity": "尿胆原", "start_idx": 27, "type": "ite" }, { "end_idx": 34, "entity": "尿胆红素", "start_idx": 31, "type": "ite" } ]
4.血清游离血红蛋白增加,结合珠蛋白降低。
[ { "end_idx": 11, "entity": "血清游离血红蛋白增加", "start_idx": 2, "type": "sym" }, { "end_idx": 9, "entity": "血清游离血红蛋白", "start_idx": 2, "type": "ite" }, { "end_idx": 19, "entity": "结合珠蛋白降低", "start_idx": 13, "type": "sym" }, { "end_idx": 17, "entity": "结合珠蛋白", "start_idx": 13, "type": "ite" } ]
5.变性珠蛋白小体试验用结晶紫将Heinz小体染成紫色,然后计数含Heinz小体的红细胞的百分比。
[ { "end_idx": 10, "entity": "变性珠蛋白小体试验", "start_idx": 2, "type": "pro" }, { "end_idx": 22, "entity": "Heinz小体", "start_idx": 16, "type": "ite" }, { "end_idx": 39, "entity": "Heinz小体", "start_idx": 33, "type": "ite" }, { "end_idx": 43, "entity": "红细胞", "start_idx": 41, "type": "ite" } ]
在发病48小时内均可检出Heinz小体,溶血停止后即呈阴性。
[ { "end_idx": 18, "entity": "Heinz小体", "start_idx": 12, "type": "ite" }, { "end_idx": 21, "entity": "溶血", "start_idx": 20, "type": "sym" }, { "end_idx": 21, "entity": "血", "start_idx": 21, "type": "bod" } ]
但此试验并非特异性,因不稳定血红蛋白病、血红蛋白H病和其他酶缺陷也可呈阳性。
[ { "end_idx": 36, "entity": "不稳定血红蛋白病、血红蛋白H病和其他酶缺陷也可呈阳性", "start_idx": 11, "type": "sym" }, { "end_idx": 17, "entity": "血红蛋白", "start_idx": 14, "type": "bod" }, { "end_idx": 24, "entity": "血红蛋白H", "start_idx": 20, "type": "bod" }, { "end_idx": 29, "entity": "其他酶", "start_idx": 27, "type": "bod" } ]
6.高铁血红蛋白还原试验是目前常用的筛选试验。
[ { "end_idx": 11, "entity": "高铁血红蛋白还原试验", "start_idx": 2, "type": "pro" }, { "end_idx": 21, "entity": "筛选试验", "start_idx": 18, "type": "pro" } ]
G-6-PD缺陷的患者由于NADPH生成减少,MHb的还原速度显著减慢,以此来间接测定G-6-PD活性。
[ { "end_idx": 7, "entity": "G-6-PD缺陷", "start_idx": 0, "type": "sym" }, { "end_idx": 5, "entity": "G-6-PD", "start_idx": 0, "type": "bod" }, { "end_idx": 21, "entity": "NADPH生成减少", "start_idx": 13, "type": "sym" }, { "end_idx": 17, "entity": "NADPH", "start_idx": 13, "type": "bod" }, { "end_idx": 34, "entity": "MHb的还原速度显著减慢", "start_idx": 23, "type": "sym" }, { "end_idx": 25, "entity": "MHb", "start_idx": 23, "type": "bod" }, { "end_idx": 50, "entity": "G-6-PD活性", "start_idx": 43, "type": "ite" } ]
定量法还原率大于75%为正常,74%~31%为中等度(杂合子)缺陷,小于31%为重度缺陷。
[ { "end_idx": 2, "entity": "定量法", "start_idx": 0, "type": "pro" }, { "end_idx": 5, "entity": "还原率", "start_idx": 3, "type": "ite" }, { "end_idx": 32, "entity": "中等度(杂合子)缺陷", "start_idx": 23, "type": "sym" }, { "end_idx": 29, "entity": "杂合子", "start_idx": 27, "type": "bod" }, { "end_idx": 43, "entity": "重度缺陷", "start_idx": 40, "type": "sym" } ]
7.高铁血红蛋白洗脱法本试验是利用组织化学方法以观察每个红细胞中MHb被还原的能力,从而间接反映G-6-PD活性。
[ { "end_idx": 10, "entity": "高铁血红蛋白洗脱法", "start_idx": 2, "type": "pro" }, { "end_idx": 22, "entity": "组织化学方法", "start_idx": 17, "type": "pro" }, { "end_idx": 34, "entity": "红细胞中MHb", "start_idx": 28, "type": "bod" }, { "end_idx": 55, "entity": "G-6-PD活性", "start_idx": 48, "type": "ite" } ]
G-6-PD缺陷的红细胞中的MHb不易还原,可被H2</sub>O2</sub>洗脱成为不着色的红细胞空影。
[ { "end_idx": 7, "entity": "G-6-PD缺陷", "start_idx": 0, "type": "sym" }, { "end_idx": 5, "entity": "G-6-PD", "start_idx": 0, "type": "bod" }, { "end_idx": 16, "entity": "红细胞中的MHb", "start_idx": 9, "type": "bod" }, { "end_idx": 41, "entity": "H2</sub>O2</sub>洗脱", "start_idx": 24, "type": "pro" }, { "end_idx": 52, "entity": "不着色的红细胞空影", "start_idx": 44, "type": "sym" }, { "end_idx": 50, "entity": "红细胞", "start_idx": 48, "type": "bod" } ]
正常人空影红细胞小于2%,若超过80%为显著缺陷;杂合子在50%左右。
[ { "end_idx": 7, "entity": "空影红细胞", "start_idx": 3, "type": "bod" }, { "end_idx": 27, "entity": "杂合子", "start_idx": 25, "type": "bod" } ]
8.荧光斑点试验正常红细胞在紫外线波长340nm照射下可发生荧光,而患者的红细胞不发生荧光。
[ { "end_idx": 7, "entity": "荧光斑点试验", "start_idx": 2, "type": "pro" }, { "end_idx": 12, "entity": "红细胞", "start_idx": 10, "type": "bod" }, { "end_idx": 39, "entity": "红细胞", "start_idx": 37, "type": "bod" }, { "end_idx": 44, "entity": "红细胞不发生荧光", "start_idx": 37, "type": "sym" } ]
9.G-6-PD活性定量测定是一种直接测定法,对诊断有特异性。
[ { "end_idx": 13, "entity": "G-6-PD活性定量测定", "start_idx": 2, "type": "pro" }, { "end_idx": 21, "entity": "直接测定法", "start_idx": 17, "type": "pro" } ]
患者G-6-PD活性多在正常人的10%以下。
[ { "end_idx": 9, "entity": "G-6-PD活性", "start_idx": 2, "type": "ite" } ]
【诊断】根据急性溶血、血红蛋白尿、病史中找到明确的诱因,并有阳性家族史和既往发作史,再结合以上实验室检查,多可作出诊断。
[ { "end_idx": 9, "entity": "急性溶血", "start_idx": 6, "type": "sym" }, { "end_idx": 9, "entity": "血", "start_idx": 9, "type": "bod" }, { "end_idx": 15, "entity": "血红蛋白尿", "start_idx": 11, "type": "sym" }, { "end_idx": 14, "entity": "血红蛋白", "start_idx": 11, "type": "bod" }, { "end_idx": 15, "entity": "尿", "start_idx": 15, "type": "bod" }, { "end_idx": 51, "entity": "实验室检查", "start_idx": 47, "type": "pro" } ]
继发于感染时,应结合各种感染的特点加以综合考虑。
[ { "end_idx": 4, "entity": "感染", "start_idx": 3, "type": "dis" }, { "end_idx": 13, "entity": "感染", "start_idx": 12, "type": "dis" } ]
本病需与不稳定血红蛋白病(uHb病)如HbH病、免疫性溶血性贫血等鉴别,前者在血红蛋白电泳时,可出现异常区带;后者Coombs试验阳性。
[ { "end_idx": 11, "entity": "不稳定血红蛋白病", "start_idx": 4, "type": "dis" }, { "end_idx": 16, "entity": "uHb病", "start_idx": 13, "type": "dis" }, { "end_idx": 22, "entity": "HbH病", "start_idx": 19, "type": "dis" }, { "end_idx": 31, "entity": "免疫性溶血性贫血", "start_idx": 24, "type": "dis" }, { "end_idx": 53, "entity": "在血红蛋白电泳时,可出现异常区带", "start_idx": 38, "type": "sym" }, { "end_idx": 44, "entity": "血红蛋白电泳", "start_idx": 39, "type": "pro" }, { "end_idx": 64, "entity": "Coombs试验", "start_idx": 57, "type": "pro" }, { "end_idx": 66, "entity": "Coombs试验阳性", "start_idx": 57, "type": "sym" } ]
慢性溶血型应与其他遗传性溶血性疾病鉴别,可通过G-6-PD活性直接测定以资鉴别。
[ { "end_idx": 3, "entity": "慢性溶血", "start_idx": 0, "type": "sym" }, { "end_idx": 3, "entity": "血", "start_idx": 3, "type": "bod" }, { "end_idx": 16, "entity": "遗传性溶血性疾病", "start_idx": 9, "type": "dis" }, { "end_idx": 34, "entity": "G-6-PD活性直接测定", "start_idx": 23, "type": "pro" } ]
【治疗和预后】在高发病地区进行普查,凡证实有G-6-PD缺陷的,应避免食用蚕豆或具有氧化作用的药物,并应积极预防感染,以防止急性发作。
[ { "end_idx": 29, "entity": "G-6-PD缺陷", "start_idx": 22, "type": "sym" }, { "end_idx": 27, "entity": "G-6-PD", "start_idx": 22, "type": "bod" }, { "end_idx": 48, "entity": "避免食用蚕豆或具有氧化作用的药物", "start_idx": 33, "type": "pro" }, { "end_idx": 57, "entity": "预防感染", "start_idx": 54, "type": "pro" } ]
急性发作时,应尽快去除诱因,贫血严重者(Hb<40g/L)应输血或浓缩红细胞1~2次,注意水和电解质紊乱,纠正酸中毒,防止高钾血症,以及碱化尿液防止急性肾衰竭。
[ { "end_idx": 15, "entity": "贫血", "start_idx": 14, "type": "dis" }, { "end_idx": 41, "entity": "输血或浓缩红细胞1~2次", "start_idx": 30, "type": "pro" }, { "end_idx": 51, "entity": "水和电解质紊乱", "start_idx": 45, "type": "dis" }, { "end_idx": 57, "entity": "酸中毒", "start_idx": 55, "type": "dis" }, { "end_idx": 64, "entity": "高钾血症", "start_idx": 61, "type": "dis" }, { "end_idx": 71, "entity": "碱化尿液", "start_idx": 68, "type": "pro" }, { "end_idx": 78, "entity": "急性肾衰竭", "start_idx": 74, "type": "dis" } ]
新生儿时发病应注意同时防治高胆红素血症以及胆红素性脑病。
[ { "end_idx": 18, "entity": "高胆红素血症", "start_idx": 13, "type": "dis" }, { "end_idx": 26, "entity": "胆红素性脑病", "start_idx": 21, "type": "dis" } ]
急性发作多呈自限性,因新生的红细胞G-6-PD活性正常,病情多于1周后逐渐稳定。
[ { "end_idx": 22, "entity": "红细胞G-6-PD", "start_idx": 14, "type": "bod" } ]
第六章胃炎胃炎(gastritis)是由多种病因引起的胃黏膜炎症,根据病程分为急性和慢性两类,前者多为继发性,后者以原发性多见。
[ { "end_idx": 4, "entity": "胃炎", "start_idx": 3, "type": "dis" }, { "end_idx": 6, "entity": "胃炎", "start_idx": 5, "type": "dis" }, { "end_idx": 16, "entity": "gastritis", "start_idx": 8, "type": "dis" }, { "end_idx": 31, "entity": "胃黏膜炎症", "start_idx": 27, "type": "dis" } ]
近几年随着胃镜在儿科的普及应用,儿童胃炎的检出率明显增高。
[ { "end_idx": 6, "entity": "胃镜", "start_idx": 5, "type": "equ" }, { "end_idx": 9, "entity": "儿科", "start_idx": 8, "type": "dep" }, { "end_idx": 19, "entity": "儿童胃炎", "start_idx": 16, "type": "dis" } ]
第一节急性胃炎急性胃炎(acutegastritis)系由不同病因引起的胃黏膜急性炎症。
[ { "end_idx": 6, "entity": "急性胃炎", "start_idx": 3, "type": "dis" }, { "end_idx": 10, "entity": "急性胃炎", "start_idx": 7, "type": "dis" }, { "end_idx": 25, "entity": "acutegastritis", "start_idx": 12, "type": "dis" }, { "end_idx": 42, "entity": "胃黏膜急性炎症", "start_idx": 36, "type": "dis" } ]
病变严重者可累及黏膜下层与肌层,甚至深达浆膜层。
[ { "end_idx": 22, "entity": "累及黏膜下层与肌层,甚至深达浆膜层", "start_idx": 6, "type": "sym" }, { "end_idx": 11, "entity": "黏膜下层", "start_idx": 8, "type": "bod" }, { "end_idx": 14, "entity": "肌层", "start_idx": 13, "type": "bod" }, { "end_idx": 22, "entity": "浆膜层", "start_idx": 20, "type": "bod" } ]
儿童中以单纯性与糜烂性多见。
[ { "end_idx": 9, "entity": "糜烂", "start_idx": 8, "type": "sym" } ]
【病因】(一)微生物感染或细菌感染进食污染微生物和细菌毒素的食物后引起的急性胃炎中,多见沙门菌属、嗜盐杆菌及某些病毒等。
[ { "end_idx": 11, "entity": "微生物感染", "start_idx": 7, "type": "dis" }, { "end_idx": 16, "entity": "细菌感染", "start_idx": 13, "type": "dis" }, { "end_idx": 23, "entity": "污染微生物", "start_idx": 19, "type": "mic" }, { "end_idx": 28, "entity": "细菌毒素", "start_idx": 25, "type": "mic" }, { "end_idx": 39, "entity": "急性胃炎", "start_idx": 36, "type": "dis" }, { "end_idx": 47, "entity": "沙门菌属", "start_idx": 44, "type": "mic" }, { "end_idx": 52, "entity": "嗜盐杆菌", "start_idx": 49, "type": "mic" }, { "end_idx": 57, "entity": "病毒", "start_idx": 56, "type": "mic" } ]
(二)化学因素1.药物水杨酸盐类药物如阿司匹林及吲哚美辛等。
[ { "end_idx": 17, "entity": "水杨酸盐类药物", "start_idx": 11, "type": "dru" }, { "end_idx": 22, "entity": "阿司匹林", "start_idx": 19, "type": "dru" }, { "end_idx": 27, "entity": "吲哚美辛", "start_idx": 24, "type": "dru" } ]
2.误食强酸(如硫酸、盐酸和硝酸)及强碱(如氢氧化钠和氢氧化钾)引起胃壁腐蚀性损伤。
[ { "end_idx": 40, "entity": "胃壁腐蚀性损伤", "start_idx": 34, "type": "dis" } ]
3.误食毒蕈、砷、灭虫药及杀鼠剂等化学毒物,均可刺激胃黏膜引起炎症。
[ { "end_idx": 28, "entity": "刺激胃黏膜", "start_idx": 24, "type": "sym" }, { "end_idx": 28, "entity": "胃黏膜", "start_idx": 26, "type": "bod" }, { "end_idx": 32, "entity": "炎症", "start_idx": 31, "type": "dis" } ]
(三)物理因素进食过冷、过热的食品或粗糙食物均可损伤胃黏膜,引起炎症。
[ { "end_idx": 28, "entity": "损伤胃黏膜", "start_idx": 24, "type": "dis" }, { "end_idx": 33, "entity": "炎症", "start_idx": 32, "type": "dis" } ]
(四)应激状态某些危重疾病如新生儿窒息、颅内出血、败血症、休克及大面积灼伤等使患儿处于严重的应激状态是导致急性糜烂性胃炎的主要原因。
[ { "end_idx": 18, "entity": "新生儿窒息", "start_idx": 14, "type": "dis" }, { "end_idx": 23, "entity": "颅内出血", "start_idx": 20, "type": "dis" }, { "end_idx": 27, "entity": "败血症", "start_idx": 25, "type": "dis" }, { "end_idx": 30, "entity": "休克", "start_idx": 29, "type": "dis" }, { "end_idx": 36, "entity": "大面积灼伤", "start_idx": 32, "type": "dis" }, { "end_idx": 49, "entity": "处于严重的应激状态", "start_idx": 41, "type": "sym" }, { "end_idx": 59, "entity": "急性糜烂性胃炎", "start_idx": 53, "type": "dis" } ]
【发病机制】1.外源性病因可严重破坏胃黏液屏障,导致氢离子及胃蛋白酶的逆向弥散,引起胃黏膜的损伤而发生糜烂、出血。
[ { "end_idx": 22, "entity": "严重破坏胃黏液屏障", "start_idx": 14, "type": "sym" }, { "end_idx": 22, "entity": "胃黏液屏障", "start_idx": 18, "type": "bod" }, { "end_idx": 28, "entity": "氢离子", "start_idx": 26, "type": "bod" }, { "end_idx": 38, "entity": "氢离子及胃蛋白酶的逆向弥散", "start_idx": 26, "type": "sym" }, { "end_idx": 33, "entity": "胃蛋白酶", "start_idx": 30, "type": "bod" }, { "end_idx": 47, "entity": "胃黏膜的损伤", "start_idx": 42, "type": "sym" }, { "end_idx": 44, "entity": "胃黏膜", "start_idx": 42, "type": "bod" }, { "end_idx": 47, "entity": "损伤", "start_idx": 46, "type": "dis" }, { "end_idx": 52, "entity": "糜烂", "start_idx": 51, "type": "sym" }, { "end_idx": 55, "entity": "出血", "start_idx": 54, "type": "sym" }, { "end_idx": 55, "entity": "血", "start_idx": 55, "type": "bod" } ]
2.应激状态使去甲肾上腺素和肾上腺素大量分泌,内脏血管收缩,胃血流量减少,缺血、缺氧进一步使黏膜上皮的线粒体功能降低,影响氧化磷酸化过程,使胃黏膜的糖原贮存减少。
[ { "end_idx": 5, "entity": "应激状态", "start_idx": 2, "type": "sym" }, { "end_idx": 21, "entity": "去甲肾上腺素和肾上腺素大量分泌", "start_idx": 7, "type": "sym" }, { "end_idx": 12, "entity": "去甲肾上腺素", "start_idx": 7, "type": "bod" }, { "end_idx": 17, "entity": "肾上腺素", "start_idx": 14, "type": "bod" }, { "end_idx": 28, "entity": "内脏血管收缩", "start_idx": 23, "type": "sym" }, { "end_idx": 26, "entity": "内脏血管", "start_idx": 23, "type": "bod" }, { "end_idx": 35, "entity": "胃血流量减少", "start_idx": 30, "type": "sym" }, { "end_idx": 31, "entity": "胃血", "start_idx": 30, "type": "bod" }, { "end_idx": 38, "entity": "缺血", "start_idx": 37, "type": "sym" }, { "end_idx": 38, "entity": "血", "start_idx": 38, "type": "bod" }, { "end_idx": 41, "entity": "缺氧", "start_idx": 40, "type": "sym" }, { "end_idx": 41, "entity": "氧", "start_idx": 41, "type": "bod" }, { "end_idx": 67, "entity": "黏膜上皮的线粒体功能降低,影响氧化磷酸化过程", "start_idx": 46, "type": "sym" }, { "end_idx": 53, "entity": "黏膜上皮的线粒体", "start_idx": 46, "type": "bod" }, { "end_idx": 79, "entity": "胃黏膜的糖原贮存减少", "start_idx": 70, "type": "sym" }, { "end_idx": 75, "entity": "胃黏膜的糖原", "start_idx": 70, "type": "bod" } ]
而胃黏膜缺血时,不能清除逆向弥散的氢离子;缺氧和去甲肾上腺素又使碳酸氢根离子分泌减少,前列腺素合成减少,削弱胃黏膜屏障功能,导致胃黏膜急性糜烂性炎症。
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【临床表现及分型】(一)急性单纯性胃炎起病较急,多在进食污染食物数小时后或24小时发病,症状轻重不一,表现上腹部不适、疼痛,甚至剧烈的腹部绞痛。
[ { "end_idx": 18, "entity": "急性单纯性胃炎", "start_idx": 12, "type": "dis" }, { "end_idx": 57, "entity": "上腹部不适", "start_idx": 53, "type": "sym" }, { "end_idx": 55, "entity": "上腹部", "start_idx": 53, "type": "bod" }, { "end_idx": 60, "entity": "疼痛", "start_idx": 59, "type": "sym" }, { "end_idx": 70, "entity": "剧烈的腹部绞痛", "start_idx": 64, "type": "sym" }, { "end_idx": 68, "entity": "腹部", "start_idx": 67, "type": "bod" } ]
若为药物或刺激性食物所致,症状则较轻,局限上腹部,体格检查有上腹部或脐周压痛,肠鸣音可亢进。
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(二)急性糜烂性胃炎多在机体处在严重疾病应激状态下诱发,起病急骤,常以呕血或黑粪为突出症状,大量出血可引起晕厥或休克,伴重度贫血。
[ { "end_idx": 9, "entity": "急性糜烂性胃炎", "start_idx": 3, "type": "dis" }, { "end_idx": 36, "entity": "呕血", "start_idx": 35, "type": "sym" }, { "end_idx": 36, "entity": "血", "start_idx": 36, "type": "bod" }, { "end_idx": 39, "entity": "黑粪", "start_idx": 38, "type": "sym" }, { "end_idx": 39, "entity": "粪", "start_idx": 39, "type": "bod" }, { "end_idx": 49, "entity": "大量出血", "start_idx": 46, "type": "sym" }, { "end_idx": 49, "entity": "血", "start_idx": 49, "type": "bod" }, { "end_idx": 54, "entity": "晕厥", "start_idx": 53, "type": "dis" }, { "end_idx": 57, "entity": "休克", "start_idx": 56, "type": "dis" }, { "end_idx": 63, "entity": "重度贫血", "start_idx": 60, "type": "dis" } ]
(三)急性腐蚀性胃炎误服强酸、强碱史,除口腔黏膜糜烂、水肿外,中上腹剧痛、绞窄感、恶心、呕吐、呕血和黑粪,并发胃功能紊乱,急性期过后可遗留贲门或幽门狭窄,出现呕吐等梗阻症状。
[ { "end_idx": 9, "entity": "急性腐蚀性胃炎", "start_idx": 3, "type": "dis" }, { "end_idx": 25, "entity": "口腔黏膜糜烂", "start_idx": 20, "type": "dis" }, { "end_idx": 28, "entity": "水肿", "start_idx": 27, "type": "sym" }, { "end_idx": 35, "entity": "中上腹剧痛", "start_idx": 31, "type": "sym" }, { "end_idx": 33, "entity": "中上腹", "start_idx": 31, "type": "bod" }, { "end_idx": 39, "entity": "绞窄感", "start_idx": 37, "type": "sym" }, { "end_idx": 42, "entity": "恶心", "start_idx": 41, "type": "sym" }, { "end_idx": 45, "entity": "呕吐", "start_idx": 44, "type": "sym" }, { "end_idx": 48, "entity": "呕血", "start_idx": 47, "type": "sym" }, { "end_idx": 48, "entity": "血", "start_idx": 48, "type": "bod" }, { "end_idx": 51, "entity": "黑粪", "start_idx": 50, "type": "sym" }, { "end_idx": 51, "entity": "粪", "start_idx": 51, "type": "bod" }, { "end_idx": 59, "entity": "胃功能紊乱", "start_idx": 55, "type": "dis" }, { "end_idx": 75, "entity": "贲门或幽门狭窄", "start_idx": 69, "type": "dis" }, { "end_idx": 80, "entity": "呕吐", "start_idx": 79, "type": "sym" }, { "end_idx": 83, "entity": "梗阻", "start_idx": 82, "type": "dis" } ]
【实验室检查】感染因素引起者其末梢血白细胞计数一般增高,中性粒细胞比例增大。
[ { "end_idx": 5, "entity": "实验室检查", "start_idx": 1, "type": "pro" }, { "end_idx": 8, "entity": "感染", "start_idx": 7, "type": "dis" }, { "end_idx": 22, "entity": "末梢血白细胞计数", "start_idx": 15, "type": "ite" }, { "end_idx": 26, "entity": "末梢血白细胞计数一般增高", "start_idx": 15, "type": "sym" }, { "end_idx": 34, "entity": "中性粒细胞比例", "start_idx": 28, "type": "ite" }, { "end_idx": 36, "entity": "中性粒细胞比例增大", "start_idx": 28, "type": "sym" } ]
【影像学检查】(一)内镜检查胃黏膜明显充血、水肿,黏膜表面覆盖厚的黏稠炎性渗出物,糜烂性胃炎则在上述病变上见到点、圆、片、线状或不规则形糜烂,中心为红色新鲜出血或棕红色陈旧性出血,伴白苔或黄苔,常为多发亦可为单个。
[ { "end_idx": 5, "entity": "影像学检查", "start_idx": 1, "type": "pro" }, { "end_idx": 13, "entity": "内镜检查", "start_idx": 10, "type": "pro" }, { "end_idx": 20, "entity": "内镜检查胃黏膜明显充血", "start_idx": 10, "type": "sym" }, { "end_idx": 16, "entity": "胃黏膜", "start_idx": 14, "type": "bod" }, { "end_idx": 20, "entity": "血", "start_idx": 20, "type": "bod" }, { "end_idx": 23, "entity": "水肿", "start_idx": 22, "type": "sym" }, { "end_idx": 39, "entity": "黏膜表面覆盖厚的黏稠炎性渗出物", "start_idx": 25, "type": "sym" }, { "end_idx": 28, "entity": "黏膜表面", "start_idx": 25, "type": "bod" }, { "end_idx": 39, "entity": "黏稠炎性渗出物", "start_idx": 33, "type": "bod" }, { "end_idx": 45, "entity": "糜烂性胃炎", "start_idx": 41, "type": "dis" }, { "end_idx": 69, "entity": "点、圆、片、线状或不规则形糜烂", "start_idx": 55, "type": "sym" }, { "end_idx": 88, "entity": "中心为红色新鲜出血或棕红色陈旧性出血", "start_idx": 71, "type": "sym" }, { "end_idx": 79, "entity": "血", "start_idx": 79, "type": "bod" }, { "end_idx": 88, "entity": "血", "start_idx": 88, "type": "bod" }, { "end_idx": 92, "entity": "白苔", "start_idx": 91, "type": "sym" }, { "end_idx": 92, "entity": "苔", "start_idx": 92, "type": "bod" }, { "end_idx": 95, "entity": "黄苔", "start_idx": 94, "type": "sym" }, { "end_idx": 95, "entity": "苔", "start_idx": 95, "type": "bod" } ]
做胃镜时应同时取胃黏膜做幽门螺杆菌检测。
[ { "end_idx": 2, "entity": "胃镜", "start_idx": 1, "type": "pro" }, { "end_idx": 10, "entity": "胃黏膜", "start_idx": 8, "type": "bod" }, { "end_idx": 18, "entity": "幽门螺杆菌检测", "start_idx": 12, "type": "pro" } ]
(二)X线检查胃肠钡餐检查病变黏膜粗糙,局部压痛,但不能发现糜烂性病变,且不能用于急性或活动性出血患者。
[ { "end_idx": 6, "entity": "X线检查", "start_idx": 3, "type": "pro" }, { "end_idx": 12, "entity": "胃肠钡餐检查", "start_idx": 7, "type": "pro" }, { "end_idx": 18, "entity": "胃肠钡餐检查病变黏膜粗糙", "start_idx": 7, "type": "sym" }, { "end_idx": 16, "entity": "病变黏膜", "start_idx": 13, "type": "bod" }, { "end_idx": 23, "entity": "局部压痛", "start_idx": 20, "type": "sym" }, { "end_idx": 34, "entity": "糜烂性病变", "start_idx": 30, "type": "dis" }, { "end_idx": 48, "entity": "急性或活动性出血", "start_idx": 41, "type": "sym" }, { "end_idx": 48, "entity": "血", "start_idx": 48, "type": "bod" } ]
【诊断与鉴别诊断】急性胃炎无特征性临床表现,诊断主要依靠病史及内镜检查,以上腹痛为主要症状者应与下列疾病鉴别。
[ { "end_idx": 12, "entity": "急性胃炎", "start_idx": 9, "type": "dis" }, { "end_idx": 34, "entity": "内镜检查", "start_idx": 31, "type": "pro" }, { "end_idx": 39, "entity": "上腹痛", "start_idx": 37, "type": "sym" }, { "end_idx": 38, "entity": "上腹", "start_idx": 37, "type": "bod" } ]
(一)急性胰腺炎有突然发作的上腹部剧烈疼痛,放射至背部及腰部,血清淀粉酶升高,B超或CT显示胰腺肿大,严重患者腹腔穿刺可抽出血性液体且淀粉酶增高。
[ { "end_idx": 7, "entity": "急性胰腺炎", "start_idx": 3, "type": "dis" }, { "end_idx": 29, "entity": "突然发作的上腹部剧烈疼痛,放射至背部及腰部", "start_idx": 9, "type": "sym" }, { "end_idx": 16, "entity": "上腹部", "start_idx": 14, "type": "bod" }, { "end_idx": 26, "entity": "背部", "start_idx": 25, "type": "bod" }, { "end_idx": 29, "entity": "腰部", "start_idx": 28, "type": "bod" }, { "end_idx": 37, "entity": "血清淀粉酶升高", "start_idx": 31, "type": "sym" }, { "end_idx": 35, "entity": "血清淀粉酶", "start_idx": 31, "type": "ite" }, { "end_idx": 49, "entity": "B超或CT显示胰腺肿大", "start_idx": 39, "type": "sym" }, { "end_idx": 40, "entity": "B超", "start_idx": 39, "type": "pro" }, { "end_idx": 43, "entity": "CT", "start_idx": 42, "type": "pro" }, { "end_idx": 47, "entity": "胰腺", "start_idx": 46, "type": "bod" }, { "end_idx": 58, "entity": "腹腔穿刺", "start_idx": 55, "type": "pro" }, { "end_idx": 65, "entity": "腹腔穿刺可抽出血性液体", "start_idx": 55, "type": "sym" }, { "end_idx": 65, "entity": "血性液体", "start_idx": 62, "type": "bod" }, { "end_idx": 69, "entity": "淀粉酶", "start_idx": 67, "type": "ite" }, { "end_idx": 71, "entity": "淀粉酶增高", "start_idx": 67, "type": "sym" } ]
(二)胆道蛔虫症骤然发生上腹部剧烈绞痛,可放射至左、右肩部及背部,发作时辗转不安,剑突下偏右压痛明显,可伴呕吐,有时吐出蛔虫,B超见胆总管内有虫体异物。
[ { "end_idx": 7, "entity": "胆道蛔虫症", "start_idx": 3, "type": "dis" }, { "end_idx": 31, "entity": "骤然发生上腹部剧烈绞痛,可放射至左、右肩部及背部", "start_idx": 8, "type": "sym" }, { "end_idx": 14, "entity": "上腹部", "start_idx": 12, "type": "bod" }, { "end_idx": 28, "entity": "左、右肩部", "start_idx": 24, "type": "bod" }, { "end_idx": 31, "entity": "背部", "start_idx": 30, "type": "bod" }, { "end_idx": 39, "entity": "发作时辗转不安", "start_idx": 33, "type": "sym" }, { "end_idx": 49, "entity": "剑突下偏右压痛明显", "start_idx": 41, "type": "sym" }, { "end_idx": 45, "entity": "剑突下偏右", "start_idx": 41, "type": "bod" }, { "end_idx": 54, "entity": "呕吐", "start_idx": 53, "type": "sym" }, { "end_idx": 61, "entity": "有时吐出蛔虫", "start_idx": 56, "type": "sym" }, { "end_idx": 64, "entity": "B超", "start_idx": 63, "type": "pro" }, { "end_idx": 74, "entity": "B超见胆总管内有虫体异物", "start_idx": 63, "type": "sym" }, { "end_idx": 69, "entity": "胆总管内", "start_idx": 66, "type": "bod" } ]
【治疗】1.单纯性胃炎以对症治疗为主,去除病因,解痉止吐,口服黏膜保护剂,对细菌感染尤其伴有腹泻者可选用小檗碱、卡那霉素及氨苄西林等抗生素。
[ { "end_idx": 10, "entity": "单纯性胃炎", "start_idx": 6, "type": "dis" }, { "end_idx": 15, "entity": "对症治疗", "start_idx": 12, "type": "pro" }, { "end_idx": 27, "entity": "解痉止吐", "start_idx": 24, "type": "pro" }, { "end_idx": 30, "entity": "口服", "start_idx": 29, "type": "pro" }, { "end_idx": 35, "entity": "黏膜保护剂", "start_idx": 31, "type": "dru" }, { "end_idx": 41, "entity": "细菌感染", "start_idx": 38, "type": "dis" }, { "end_idx": 47, "entity": "腹泻", "start_idx": 46, "type": "sym" }, { "end_idx": 46, "entity": "腹", "start_idx": 46, "type": "bod" }, { "end_idx": 54, "entity": "小檗碱", "start_idx": 52, "type": "dru" }, { "end_idx": 59, "entity": "卡那霉素", "start_idx": 56, "type": "dru" }, { "end_idx": 64, "entity": "氨苄西林", "start_idx": 61, "type": "dru" }, { "end_idx": 68, "entity": "抗生素", "start_idx": 66, "type": "dru" } ]
有幽门螺杆菌者,则应做清除治疗。
[ { "end_idx": 5, "entity": "幽门螺杆菌", "start_idx": 1, "type": "mic" }, { "end_idx": 14, "entity": "清除治疗", "start_idx": 11, "type": "pro" } ]
2.糜烂性胃炎应控制出血,去除应激因素,可用H2</sub>受体拮抗剂:西咪替丁20~40mg/(kg•d),法莫替丁0.4~0.8mg/(kg•d),或质子泵阻滞剂奥美拉唑0.6~0.8mg/(kg•d),以及应用止血药如立止血注射,凝血酶口服等。
[ { "end_idx": 6, "entity": "糜烂性胃炎", "start_idx": 2, "type": "dis" }, { "end_idx": 11, "entity": "控制出血", "start_idx": 8, "type": "pro" }, { "end_idx": 18, "entity": "去除应激因素", "start_idx": 13, "type": "pro" }, { "end_idx": 34, "entity": "H2</sub>受体拮抗剂", "start_idx": 22, "type": "dru" }, { "end_idx": 39, "entity": "西咪替丁", "start_idx": 36, "type": "dru" }, { "end_idx": 58, "entity": "法莫替丁", "start_idx": 55, "type": "dru" }, { "end_idx": 86, "entity": "质子泵阻滞剂奥美拉唑", "start_idx": 77, "type": "dru" }, { "end_idx": 110, "entity": "止血药", "start_idx": 108, "type": "dru" }, { "end_idx": 114, "entity": "立止血", "start_idx": 112, "type": "dru" }, { "end_idx": 116, "entity": "注射", "start_idx": 115, "type": "pro" }, { "end_idx": 120, "entity": "凝血酶", "start_idx": 118, "type": "dru" }, { "end_idx": 122, "entity": "口服", "start_idx": 121, "type": "pro" } ]
3.腐蚀性胃炎应根据腐蚀剂性质给予相应中和药物,如口服镁乳氢氧化铝、牛奶和鸡蛋清等治疗强酸剂腐蚀。
[ { "end_idx": 6, "entity": "腐蚀性胃炎", "start_idx": 2, "type": "dis" }, { "end_idx": 22, "entity": "相应中和药物", "start_idx": 17, "type": "dru" }, { "end_idx": 26, "entity": "口服", "start_idx": 25, "type": "pro" }, { "end_idx": 32, "entity": "镁乳氢氧化铝", "start_idx": 27, "type": "dru" }, { "end_idx": 35, "entity": "牛奶", "start_idx": 34, "type": "dru" }, { "end_idx": 39, "entity": "鸡蛋清", "start_idx": 37, "type": "dru" } ]
第十三章常见的泌尿外科疾病第一节异位肾当肾脏未能正常发育到达肾窝的位置,即为肾脏异位。
[ { "end_idx": 12, "entity": "泌尿外科疾病", "start_idx": 7, "type": "dis" }, { "end_idx": 18, "entity": "异位肾", "start_idx": 16, "type": "dis" }, { "end_idx": 21, "entity": "肾脏", "start_idx": 20, "type": "bod" }, { "end_idx": 34, "entity": "肾脏未能正常发育到达肾窝的位置", "start_idx": 20, "type": "sym" }, { "end_idx": 31, "entity": "肾窝", "start_idx": 30, "type": "bod" }, { "end_idx": 41, "entity": "肾脏异位", "start_idx": 38, "type": "sym" }, { "end_idx": 39, "entity": "肾脏", "start_idx": 38, "type": "bod" } ]
其与肾下垂的区别在于肾下垂的肾脏最初都是位于正常的位置,随后下垂而异常的,但异位肾的位置从来都是异常的。
[ { "end_idx": 4, "entity": "肾下垂", "start_idx": 2, "type": "dis" }, { "end_idx": 12, "entity": "肾下垂", "start_idx": 10, "type": "dis" }, { "end_idx": 15, "entity": "肾脏", "start_idx": 14, "type": "bod" }, { "end_idx": 40, "entity": "异位肾", "start_idx": 38, "type": "dis" } ]
随着产前超声检查的应用,异位肾的检出率有提高之势。
[ { "end_idx": 7, "entity": "产前超声检查", "start_idx": 2, "type": "pro" }, { "end_idx": 14, "entity": "异位肾", "start_idx": 12, "type": "dis" } ]
双侧异位肾很少见,仅占异位肾总数的10%。
[ { "end_idx": 4, "entity": "双侧异位肾", "start_idx": 0, "type": "dis" }, { "end_idx": 13, "entity": "异位肾", "start_idx": 11, "type": "dis" } ]
肾脏异位的原因尚未明了,其与输尿管芽发育不良、后肾发育异常、遗传异常、致畸因子作用以及母亲的某些疾病有关。
[ { "end_idx": 3, "entity": "肾脏异位", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "肾脏", "start_idx": 0, "type": "bod" }, { "end_idx": 21, "entity": "输尿管芽发育不良", "start_idx": 14, "type": "dis" }, { "end_idx": 28, "entity": "后肾发育异常", "start_idx": 23, "type": "dis" }, { "end_idx": 33, "entity": "遗传异常", "start_idx": 30, "type": "sym" }, { "end_idx": 38, "entity": "致畸因子", "start_idx": 35, "type": "bod" } ]