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      蚕豆病是什么

      【导读】在长江以南的地区里面,蚕豆病并不是一种生僻的病,这是一种6-磷酸葡萄糖脱氢酶(G-6-PD)缺乏所导致的疾病,主要表现为在遗传性葡萄糖-6-磷酸脱氢酶(G-6-PD)缺陷的情况下,食用新鲜蚕豆后突然发生的急性血管内溶血。在吞食蚕豆后的1-2天会发病,早期症状有厌食、疲劳、腹痛等,继而出现黄疸、贫血、体温升高,可持续3日左右。如果病情严重者如果抢救不及时可在一至二天内死亡。因此在发病后一定要及时送往医院救治。

      蚕豆病是对于北方人来说并不常见,它常见于发生于长江以南地区,主要是由于遗传性红细胞六磷酸葡萄糖脱氢酶(G6PD酶)缺乏者进食蚕豆后,随后发生的急性溶血性疾病,又名胡豆黄,蚕豆黄。常见于小儿,特别是5岁以下男童多见,约占90%,常发生在蚕豆成熟的季节,进食蚕豆或蚕豆制品(如粉丝、酱油)均可致病。

      患者通常于进食蚕豆后24~48小时内发病,表现为急性血管内溶血,有全身不适,发热、恶心、呕吐、迅速贫血、黄疸、血红蛋白尿,溶血严重者出现休克、少尿、无尿、酸中毒和急性肾功能衰竭症状。其发病与否,病情轻重与吃蚕豆的数量无关,有时吃1~2粒也难幸免,有时吸入或接触蚕豆花粉即可发病,甚至有时乳母吃蚕豆也可通过乳汁而致病。本病的贫血程度和症状大多很严重。一般病例症状持续2~6天。如果不及时纠正贫血、缺氧和电解质平衡失调,可以致死;但如果及时得到救治,能摆脱危险而康复。在低发地区,本病易被忽视而漏。目前为止没有特效根治的药物,发病后主要是根据病情对症治疗,这就要求蚕豆病患者平时要避免接触诱发的因素。

      蚕豆病是我国南方最常见的遗传病之一,呈X性连锁不完全显性遗传,一般男性多于女性。因此,家庭中有蚕豆病史者,应禁食蚕豆。那么蚕豆病的症状是怎样的呢?我们一起来了解一下。

      蚕豆病在儿童身上出现的几率比较高,在每年3-5月蚕豆成熟季节时发病最多。一般是在进食蚕豆后12~24小时突然发病,出现急性溶血危象症状:寒战、高热、黄疸,严重者可致昏迷、惊厥、休克、心功能不全和急性肾功能衰竭。贫血严重者,如不及时抢救,可于发病后1~2日内死亡。所以在发现病情发作的时候就要及时送往医院,防止病情的恶化。

      导致蚕豆病孩子的死亡原因多为贫血、严重酸中毒以及并发症如急性心肾功能衰竭、肺炎、脑实质出血等,因此各位家长万不可掉以轻心。

      目前来说,蚕豆病的致病机制尚未十分明了。已知有遗传缺陷的敏感红细胞,因G-6-PD的缺陷不能提供足够的NADPH以维持还原型谷胱甘肽(GSH)的还原性(抗氧化作用),在遇到蚕豆和某种因子后更诱发了红细胞膜被氧化,产生溶血反应。G-6-PD有?;ふ:煜赴庠庋趸苹档淖饔?,新鲜蚕豆是很强的氧化剂,当G-6-PD缺乏时则红细胞被破坏而致病。

      另外一种是伴性隐性遗传,也有人认为是伴性不完全显性遗传,突变基因位于x染色体上。蚕豆中可提出多种活性物质,如蚕豆嘧啶核苷、蚕豆嘧啶、多巴、多巴糖苷和异脲咪等,认为可能与蚕豆病的发病有关。

      蚕豆病是能够遗传的,所以3岁以下儿童,第一次吃蚕豆要谨慎,一旦发现儿童吃蚕豆后出现低热、腹痛、皮肤发黄、尿色加深等症状,须立即送正规医院就医。如果家有蚕豆病史的孩子,不但要禁食蚕豆,还要避免接触蚕豆花粉、樟脑丸、龙胆紫(紫药水),以及磺胺类药物,因为这些东西都含有氧化剂。

      蚕豆病是一种重在预防,根治的可能性小的病,也就是说蚕豆病是不能治好的。那么在中医与西医当中,是怎么治疗蚕豆病的呢?

      西医对蚕豆病的治疗:

      1、输血:本病为急性溶血,且贫血严重,输血或输浓集红细胞是最有效的治疗措施。严重者可反复输血。但对血液来源应行葡萄糖-6-磷酸脱氢酶快速筛选检查,以避免葡萄糖-6-磷酸脱氢酶缺乏者供血,使患者发生第二次溶血。

      2、肾上腺皮质激素:主要是免疫抑制作用,应争取早期,大量,短程用药。

      3、纠正酸中毒:蚕豆病溶血期常有不同程度的酸中毒,严重者单纯输血无效,应积极纠正,这是抢救危重患者的关键措施。

      4、补液:应多饮水或输入液体,以改善微循环,维持有效血循环,促进肾脏排酸及排血红蛋白尿功能,但应防止急性肾功能衰竭。并发急性肾功能衰竭时,应注意维持水,电解质平衡

      5、对症处理:蚕豆病如合并感染可加重溶血,高热、缺氧和心率加快可出现心力衰竭等,故应积极处理并发症,避免使用对肾脏有损害作用的药物。

      中医对蚕豆病治疗:

      症状:身目黄色鲜明,发热口渴,心烦不宁,恶心呕吐,脘腹胀满,纳呆食少,大便秘结,小便短赤或呈酱油色尿,舌质红,苔黄腻或黄糙,脉弦数或滑数。

      病机:食蚕豆后,中毒较重,毒入营血,血毒相搏,毒重血旺,则高热;毒热伤及肝胆,而使肝胆失于疏泄,以致胆汁不循常道而泛于肌肤发为黄疸;因热为阳邪,热重于湿,则身目黄色鲜明;热邪内盛,灼伤津液,则口渴;湿热蕴结中焦,运化失常,则纳呆食少;胃失和降,浊气上犯,则恶心呕吐,心烦不宁;胃腑热盛,腑气不通,则脘腹胀满,大便秘结;湿热下注,邪扰膀胱,气化失利,则小便短赤或呈酱油色尿;舌质红,苔黄腻或黄糙,脉弦数或滑数,此湿热蕴结之象。

      治法:清热解毒,利湿退黄。

      方药:方用茵陈五苓散加减。药用茵陈15克、猪苓12克、茯苓10克、泽泻10克、白术12克、黄芩15克、栀子12克、板蓝根18克、郁金10克、甘草6克。

      加减:大便秘结,加大黄、枳实;呕吐严重,加半夏、竹茹。

      蚕豆病是一种重在预防的病情,能够完全治愈的机会不大,所以应应避免接触蚕豆花粉。另外蚕豆病不能吃珍珠末,金银花,川莲,?;?,腊梅花,熊胆,保婴丹,切记禁食蚕豆或蚕豆生加工品,避免在蚕豆开花、结果或收获季节去蚕豆地。至于其他水果蔬菜的话,一般没有禁止。

      另外已患有蚕豆病的患者应该注意一以下几项事情:

      1、禁用品少接触

      禁止使用的日用品:樟脑、臭丸、冬青油、颜料、曼秀雷敦薄荷膏、比膏、平安膏、跌打酒有?;频?。

      2、小心杀虫剂喷雾

      有些杀虫剂的喷雾则肯定可以使血液溶解,因此在使用的时候也要格外注意。

      3、勿用氧化性药物

      不宜服用氧化性药物,如伯氨喹啉、磺胺类、呋喃类、维生素K3、K4、非那西丁、氨基比林、砜类等药物。

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