雷帕霉素靶蛋白抑制剂治疗后结节性硬化症复杂型肾囊肿疾病的改善
Improvement in Renal Cystic Disease of Tuberous Sclerosis Complex After Treatment with Mammalian Target of Rapamycin Inhibitor.
作者信息
Siroky Brian J, Towbin Alexander J, Trout Andrew T, Schäfer Hannah, Thamann Anna R, Agricola Karen D, Tudor Cynthia, Capal Jamie, Dixon Bradley P, Krueger Darcy A, Franz David N
机构信息
Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
出版信息
J Pediatr. 2017 Aug;187:318-322.e2. doi: 10.1016/j.jpeds.2017.05.015. Epub 2017 Jun 7.
Renal cysts occur in approximately 50% of patients with tuberous sclerosis complex, but their clinical significance and response to treatment are unknown. Abdominal imaging of 15 patients with tuberous sclerosis complex-associated renal cystic disease who had received mammalian target of rapamycin inhibitor therapy for other tuberous sclerosis complex-related indications was evaluated. Reductions in cyst number, sum diameter, and volume were observed.
肾囊肿发生于约50%的结节性硬化症患者中，但其临床意义及对治疗的反应尚不清楚。对15例患有结节性硬化症相关肾囊性疾病且因其他结节性硬化症相关指征接受雷帕霉素靶蛋白抑制剂治疗的患者进行腹部成像评估。观察到囊肿数量、总直径和体积均有所减少。
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