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什么是先天性肌营养不良?

发布时间:2016-07-06   问大夫  责任编辑:红豆

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说到先天性疾病,大家会想到胎儿在母体的时候就已经患病,或者是父母遗传,致使孩子也有这种病。那对于先天性肌营养不良又是由于什么原因造成的呢?先天性肌营养不良与遗传有关吗?下面小编带大家了解一下什么是先天性肌营养不良,以及该怎么治疗。

什么是先天性肌营养不良

专家讲,先天性肌营养不良是属于一种隐性的遗传疾病,而且是常染色体造成的遗传。它主要的内容就是包括婴儿在出生的时候出现的肌无力,肌张力低或者是在出生几个月以后出现的肌无力,肌张力低的情况。

先天性肌营养不良从新生儿开始发病,表现为脊柱后突,肌张力下降,常伴发髋关节脱位,近端关节挛缩,斜颈,而远端关节表现出惊人的松弛,弹性过度增高。然而在一些严重的病例中,远端关节过度松弛也可以不存在,同时这种关节挛缩是可以进行性发生、发展,最终影响到以前表现为松弛的踝、腕及手指。病人最大的运动能力变异很大,有些患者可以自由活动,而另一些从未能独立行走。后期病人运动功能受限的原因大多与关节挛缩有关,病人可因关节挛缩而失去已具有的行走能力。UCMD病人特征性的面容为:下眼睑轻度下垂,圆脸,招风耳。典型的皮肤损害为滤泡性过度角化。

关于先天性肌营养不良的病因,症状都有了相应的介绍。而先天性肌营养不良给患者的带来的伤害是相当的大的,有的患者会出现不能行走的情况,所以说必须对先天性肌营养不良的疾病,进行及时的有效的治疗,才能缓解患者的症状。

肌营养不良的治疗方法

病员家族应作详细家谱分析和血清CPK测定及基因分析,极早发现携带者,做好婚姻、遗传和优生的宣传教育。对拟诊为携带者的妇女,妊娠后期应作羊水检查,并以PCR方法发现胎儿是否异常。该项工作既可达到宫内诊断,又可及时处理并预防本病出现之目的。 肌营养不良症中医治疗方法   

一、辨证治疗

1、脾胃虚弱:治宜益气健脾,调和脾胃。方用四君子汤加味。药用:党参1015g、白术12g茯苓1015g、炙甘草610g。脾虚痰湿盛者,加苍术、半夏、陈皮等;气虚明显者,可以改党参为人参,加用黄芪   

2、气血亏虚:治宜气血双补,益气养血。方用八珍汤加减。药用:党参12g、白术12g茯苓15g当归12g白芍12g熟地10g、怀牛膝15g、炙甘草6g。气血亏虚明显者,可加用一些羊肉、牛肉、阿胶、鹿角胶等血肉有情之品;气虚明显者,加黄芪   

3、肝肾不足:治宜滋补肝肾,强筋壮骨。方用虎潜丸加减。药用:龟板20g、怀牛膝15g当归12g、枸杞10g、党参12g熟地10g白芍12g知母10g、陈皮6g。阴虚有热者,加黄柏、地骨皮、鳖甲等;肝风内动,筋惕肉跳者,加天麻、钩藤、石决明等;兼有瘀象者,加鸡血藤、丹参、赤芍等。   

4、湿热侵淫:治宜清利湿热,健脾和中。方用三妙丸加味。药用:黄柏12g、苍术12g、牛膝15g、薏苡仁15g扁豆12g、茯苓12g、陈皮6g、白术12g。脾虚者;加党参、黄精;湿盛者可加藿香、佩兰、竹叶等。   

二、体疗:

临床实践表明,适当的体育锻炼及各关节的充分活动虽然不能治愈本病,但至少可以延缓更严重的肌肉萎缩、肌无力和关节挛缩的发生。因此,鼓励病人进行尽量多的活动在本病的治疗中起着较为重要的作用。   针灸严格来讲,本病患者应慎用针灸治疗,尤其是要慎用体针治疗。在必需应用针灸治疗时,一定要注意掌握针刺的刺激强度,本病患者不宜使用强刺激。   

1、体针:可以选取脾俞、肾俞、中脘、足三里、腰阳关、膈俞、曲池、合谷、环跳、肩井、风池、阳陵泉、大椎、承山、公孙等穴,每次选取610穴,不宜取穴过多。留针20分钟。隔日针灸1次。   

2、耳针:选取肝、脾、肾、内分泌、皮质下等穴。可以针刺;可以埋针;亦可以埋豆按压刺激。   

3、头针:取运动区的前1/5,中2/5处作头针及相应治疗。   

推拿   

按摩按摩对于本病的按摩大体包括用适当的手法刺激有关的经穴,起到调整机体及脏腑功能的作用;和协助病人对各个关节进行必要的功能锻炼,以延缓患者肌肉萎缩的发生和防止病人的关节发生挛缩等。在许多时候,后者比前者还要重要。

肌营养不良症西医治疗方法   

用药治疗   

各型肌营养不良症均可见到肌原性受损的肌电图表现。包括运动单位平均时限缩短,运动单位电位平均幅度下降,多相电位增加,重收缩时出现干扰相,运动单位范围缩小,运动单位电位最大幅度下降等,此外还可见到纤颤电位,正相电位等等。据统计,100例假性肥大型肌营养不良症四头肌的肌电图,肌强直电活动出现率为10%,纤颤或正相电位出现率为70%,运动单位电位时限缩短,多相电位增加95%,重收缩时出现干扰相及病理干扰相占96%  

目前,虽然很多学者对肌营养不良症的病因及发病机理进行了探索,但至今仍不清楚,由于本病的病程缓慢,某些类型长达正常生命跨度,这对本病的治疗以及疗效估计带来很大困难,现在虽然有上百种药物的临床应用,但至今仍无肯定疗效的药物,以至于此病仍在发展状态中,造成患者病累终生。   

下面是几种常用的药物介绍。   

三磷酸腺苷(ATP):肌注药,常能暂时缓解症状。但肌注后,尿酸排泄增加,代谢活跃,停药后症状发展迅速。   

三磷酸苷(UPT):肌注药,通过糖代谢而改善症状。停药后瘫痪发展更快。   

维生素E:通过细胞膜功能的改善提高受累骨骼肌的肌力。   

另外还有:增肌注射兴高采烈、地塞来松、葡萄糖、胰岛素、心可定、等等

肌营养不良症食疗方法

肌营养不良症食疗方法(以下资料仅供参考,详细需咨询医生)  

130克黄芪煎取药汁,50克牛肉切成小块,用黄芪汁和适量清水煮,将熟时放入100克大米煮粥,每日服2次。   

2、母鸡1只,枸杞子30克、菟丝子30克放入鸡肚内,炖熟后食鸡饮汤。   

3、蹄胖1只、蹄筋、50克。将杜仲30克、牛膝30克水煎,然后用药什炖蹄胖和蹄筋,放入盐、味精适量,食肉喝汤。   

4芝麻25克、胡桃肉25克共研碎,与100克猪骨髓粉一起炒熟,每次服10克,每日服2次。   

肌营养不良吃什么好  

1、饮食宜清淡、营养丰富;   

2、可多食鱼类、蛋类、鸡肉、瘦猪肉等,但不可太过,以免损伤脾胃。   

3、白菜、豆芽、西红柿、山楂、广柑、枣子之类的蔬菜水果可以适当多食一些。  

4、饮食宜高蛋白,富含维生素、钙、锌、瘦肉、鸡蛋、鱼、虾仁、动物肝脏、排骨、木耳、蘑菇、豆腐、黄花菜等可适当多食。  

肌营养不良不能吃什么  

1、忌食或少食油腻厚味过热、伤津耗液及损伤脾胃之品;   

2、少吃或忌食过辣、过咸、生冷等不宜消化和有刺激性食品。

看了以上的介绍,大家对关于先天性肌营养不良的病因,症状都有了相应的介绍。而先天性肌营养不良给患者的带来的伤害是相当的大的,有的患者会出现不能行走的情况,所以说必须对先天性肌营养不良的疾病,进行及时的有效的治疗,才能缓解患者的症状。

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