蚕豆病的病因及治疗方法(蚕豆病怎么来的)

植物图片 2022-05-01 20:21

蚕豆病的病因及治疗方法

蚕病是由葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏引起的疾病，其特征是在遗传性葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏的情况下，食用新鲜蚕豆后发生急性血管内溶血。

蚕豆病

病因

致病机制尚不完全清楚。已知遗传缺陷的敏感红细胞由于G-6-PD的缺陷，不能提供足够的NADPH维持还原型谷胱甘肽(GSH)的还原性(抗氧化作用)，甚至在遇到蚕豆和某些因素后诱发红细胞膜氧化和溶血反应。G-6-帕金森病可以保护正常红细胞免受氧化损伤。新鲜蚕豆是强氧化剂。当G-6-帕金森病缺乏时，红细胞会受损并导致疾病。

蚕豆病

临床表现

早期有畏寒、微热、头晕、乏力、食欲不振、腹痛，其次为黄疸、贫血、血红蛋白尿，尿呈酱油色。之后体温升高，疲劳和劳累加重，持续3天左右。在溶血性贫血的同时，呕吐、腹泻、腹痛加重，出现肝脏肿大、肝功能异常，约50%的患者出现脾肿大。严重者可见昏迷、惊厥、急性肾功能衰竭。如果急救不够，往往会在1 ~ 2天内死亡。

他们大多在吃蚕豆后1-2天生病。早期症状包括厌食、乏力、低热、恶心和不确定的腹痛，随后是由于溶血、酱油尿和贫血引起的黄眦感染和全身性黄疸。严重者有尿量、休克、心功能、肾功能衰竭，严重缺氧者可见双眼固定偏差。如果此时不及时抢救，一两天内就可能死亡。

蚕豆病

检查

1.血像

血红蛋白急剧下降。重量降到10克/升以下.

红细胞最低降至0.51012个/升以下。

网织红细胞增加0.20。

外周血涂片可见有核红细胞增多。

白细胞数增至(10 ~ 20)109/升，甚至出现白血病样反应。

血小板计数正常或升高。

2.骨髓图像

红细胞和粒细胞增殖明显，年龄越小，颗粒细胞系增殖越明显。

红细胞系统以中晚期幼红细胞增多为特征。

3.尿液检查

尿液呈酱油色、浓茶色、红葡萄酒色、水洗肉色、黄色等。

尿潜血试验阳性率可达60% ~ 70%。

尿检显示蛋白、红细胞和管型、尿胆汁和尿胆汁阳性。

血清游离血红蛋白升高。结合珠蛋白减少。

葡萄糖-6-磷酸脱氢酶活性下降。

蚕豆病

诊断

这种疾病是由性别不完全显性遗传的。G-6-帕金森病基因位于X染色体上，患者多为男性，男女比例约为71。食蚕豆生后数小时至数天(1-3天)内，病情突然发展为发热、头晕、烦躁、恶心，尿液呈酱油样或酒色，一般发作2-6天后自行恢复。但如果不及时抢救，会有循环衰竭的危险。可通过病史和高铁血红蛋白还原试验(还原率大于75%)诊断，尤其是荧光点试验。

蚕豆病

治疗

G-6-PD缺乏症和正常人一样，在无诱因、无发病的情况下，不需要特殊治疗。防治的关键在于预防，严格遵循蚕豆患者的健康处方，防止发作。其次，孕晚期孕妇或新生儿服用小剂量苯巴比妥可有效降低新生儿核黄疸的发生率。

输血是这种疾病急性发作最有效的治疗方法，其次是纠正酸中毒和治疗肾衰竭。轻度至中度溶血患者一般采用补液治疗。

专题： 蚕豆病
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