脾脏进行性增大者。
它以双肾多发性囊肿为特征，囊肿不断形成和进行性增大，导致肾功能下降，到60岁时大约有50％的患者进展至终末期肾衰。
Progressive cyst formation and enlargement leads to loss of renal function, hypertension, and in 50% of cases, end-stage renal disease by the age of 60 year.
肾血管平滑肌脂肪瘤的继发性出血、心脏横纹肌瘤伴发的心脏增大，进行性肺脏损害都可以导致死亡。
Hemorrhage secondary to renal angiomyolipomas, cardiomyopathy associated with cardiac rhabdomyomas or progressive pulmonary impairment may also result in death.
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