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结节性硬化症相关肾脏疾病的临床与分子研究进展

Clinical and molecular insights into tuberous sclerosis complex renal disease.

机构信息

Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, MLC 7022, 3333 Burnet Avenue, Cincinnati, OH, 45229-3039, USA.

出版信息

Pediatr Nephrol. 2011 Jun;26(6):839-52. doi: 10.1007/s00467-010-1689-5. Epub 2010 Dec 9.

Abstract

Patients with tuberous sclerosis complex are at great risk of developing renal lesions as part of their disease. These lesions include renal cysts and tumors. Significant advances in understanding the cell biology of these renal lesions has already led to clinical trials demonstrating that pharmacological interventions are likely possible. This review focuses on the pathology of these renal lesions, their underlying cell biology, and the possible therapeutic strategies that may prove to significantly improve care for these patients.

摘要

结节性硬化症患者存在发生肾脏病变的巨大风险,这些病变包括肾囊肿和肿瘤。对这些肾脏病变的细胞生物学的深入理解已经取得了重大进展,从而开展了临床试验,表明药物干预很可能是可行的。本综述重点关注这些肾脏病变的病理学、其潜在的细胞生物学以及可能的治疗策略,这些策略可能会显著改善对这些患者的治疗。

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