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多囊肾的病理变化

来源:华人健康网 | 发布时间:2011-08-20 16:03:59 | www.chinesejk.com

  ADPKD患者早期肾脏大小正常,后期则增大,并出现形态异常,如肾盂肾盏的异形,明乳头及肾锥体的完整结构受到破坏等。囊肿呈球形,大小不一。初起时肾内可仅有少数囊肿,随病程进展而渐增多,最终令肾均由囊肿所占,肾脏可达足球大小。在光镜下,囊肿间尚可见到完整肾结构,从正常表现到肾小球硬化,小管萎缩、间质纤维化等不等,这些改变均为囊肿压迫所致肾缺血所为。在电镜下,囊肿上皮细胞显示为二种形态:一种与近端小管上皮细胞相似,另一种则类似于远端小管。囊液一般较清晰,当出现囊内感染或出血时则可为脓性或血性。

  ARPKD双肾体积和重量明显增大,约为正常的10倍左右。外形光滑,切面可见梭状或柱状囊肿,呈放射状分布。上皮细胞呈柱形,与集合管皮皮细胞一致。肾盂和肾盏被膨胀的肾实质压迫而变窄、变小。肝脏病变局限于门脉区,呈弥漫性,胆管扩张伴结缔组织增生,引起门脉周围纤维化,随时间进展,出现门脉高压及肝脾肿大。

(编辑:小鱼 )

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