Shimada T, Nakayama M, Matsuda H, Kanai T, Kimura Y, Saitou A, Inomata I, Yoshida H, Takazoe K, Kitajima T
Department of Internal Medicine, Kashiwa Hospital.
Nihon Jinzo Gakkai Shi. 1991 Feb;33(2):167-72.
A 19-year-old female patient with renovascular hypertension accompanied by focal segmental glomerulosclerosis like lesion in the contralateral kidney is described. She was admitted to our hospital for uncontrollable hypertension which was first pointed out at check-up when she was 18 years old. On admission physical examination revealed remarkable hypertension and a bruit around the umbilical region. Laboratory findings disclosed renal dysfunction and slight proteinuria (0.8-1.1 g/day). Arteriography revealed severe narrowing of the right renal artery suggesting fibromuscular dysplasia. Taken together, renovascular hypertension was diagnosed. She underwent an operation to reconstruct the artery with autotransplantation of the ipsilateral kidney in the pelvic cavity, and sections were taken from both kidney for histological evaluation during the procedure. After the operation her blood pressure normalized and proteinuria decreased. And interestingly, the kidney specimens revealed that the left side consisted of focal segmental glomerulosclerosis like lesion whereas the right was only of ischemic change. Hyperfiltration theory has recently been highlighted experimentally. And the etiology of the renal findings in this case may be based on such hemodynamic alteration as has been described in animal models. We present the case so that it may be of some help to understand how focal segmental glomerulosclerosis is brought about in humans.
本文描述了一名19岁的女性患者，患有肾血管性高血压，对侧肾脏伴有局灶节段性肾小球硬化样病变。她因无法控制的高血压于我院就诊，高血压在其18岁体检时首次被发现。入院时体格检查显示显著高血压，脐周有血管杂音。实验室检查发现肾功能不全及轻度蛋白尿（0.8 - 1.1克/天）。血管造影显示右肾动脉严重狭窄，提示纤维肌性发育不良。综合判断，诊断为肾血管性高血压。她接受了手术，将同侧肾脏自体移植到盆腔以重建动脉，并在手术过程中从双侧肾脏取材进行组织学评估。术后她的血压恢复正常，蛋白尿减少。有趣的是，肾脏标本显示左侧为局灶节段性肾小球硬化样病变，而右侧仅有缺血性改变。近年来，高滤过理论在实验中受到关注。该病例中肾脏表现的病因可能基于动物模型中所描述的此类血流动力学改变。我们呈现此病例，希望有助于理解人类局灶节段性肾小球硬化是如何发生的。
