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您的位置: 中华康网 >> 常见疾病 >> 糖原累积?、蛐?/div>

糖原累积?、蛐?/h1>
www.jms88.com  2011-2-9  中国康网    

核心提示:糖原累积?、蛐?   【病因和病理生理】  本病是由于α-l,4-葡糖苷酶(酸性麦芽糖酶)缺乏所致,该酶是一溶酶体酶,能使低聚糖和糖原分解出葡萄糖,酶缺乏时……

   【病因和病理生理】

  本病是由于α-l,4-葡糖苷酶(酸性麦芽糖酶)缺乏所致,该酶是一溶酶体酶,能使低聚糖和糖原分解出葡萄糖,酶缺乏时,大量糖原累积在溶酶体内,溶酶体膨胀造成细胞受损。体内各种组织均受累,病理检查可见肝、肌肉、心、脑干运动核和脊髓前角细胞中有大量糖原累积。

    【临床表现】

  本病可分为三型;婴儿型常在出生后6个月以内发病,以肌力、肌张力减低,心脏极度肥大和充血性心力衰竭为主要表现,常在周岁左右死亡;幼年型起病稍晚,以乏力、肌张力低下为主,无心脏侵及,病程进展缓慢,但常在早年死于肺炎;青、少年期发病者的症状类似其他肌病,预后亦多不佳。造成临床表现差异的原因尚有待阐明。

    【诊断】

  对本病需作肌肉活检组织的糖原和酶活性测定。检测培养羊水细胞中的酶活性可进行产前诊断。

 

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