Bonsib S M, Fallon B, Mitros F A, Anuras S
J Urol. 1984 Dec;132(6):1112-6. doi: 10.1016/s0022-5347(17)50054-3.
Of 10 patients with familial or nonfamilial visceral myopathy studied for urological manifestations only 3 had symptoms of urinary retention. Excretory urography revealed megacystis in all 3 symptomatic patients, while the 7 asymptomatic patients had normal-sized bladders. Cystometrograms performed in 4 asymptomatic patients also were normal. Bladder tissue from 5 patients was studied. Of 3 autopsy cystectomy specimens 2 were dilated severely and thin-walled without trabeculations or diverticula suggestive of mechanical obstruction. Microscopic sections showed segmental intrafascicular fibrosis in 4 patients, which was associated with elastosis in 3. Electron microscopy of leiomyocytes in abnormal fascicles revealed myofilament disorganization, lipid accumulation and increased numbers of cytoplasmic organelles. We conclude that pathological changes in the bladder occur commonly but urinary symptoms occur in less than half of the patients with visceral myopathy available for study. An abnormal excretory urogram was seen in only patients who had symptoms suggestive of lower urinary tract dysfunction.
在仅针对泌尿系统表现进行研究的10例家族性或非家族性内脏肌病患者中，只有3例出现尿潴留症状。排泄性尿路造影显示，所有3例有症状的患者均存在巨膀胱，而7例无症状患者的膀胱大小正常。对4例无症状患者进行的膀胱测压图也正常。研究了5例患者的膀胱组织。在3例尸检膀胱切除标本中，2例严重扩张且壁薄，无提示机械性梗阻的小梁或憩室。显微镜切片显示4例患者存在节段性束内纤维化，其中3例伴有弹性组织变性。对异常束中的平滑肌细胞进行电子显微镜检查发现肌丝紊乱、脂质蓄积和细胞质细胞器数量增加。我们得出结论，膀胱的病理改变很常见，但在可供研究的内脏肌病患者中，不到一半的患者出现泌尿系统症状。仅在有提示下尿路功能障碍症状的患者中观察到异常的排泄性尿路造影。
