Peces Ramón, Cuesta-López Emilio, Peces Carlos, Selgas Rafael
Servicio de Nefrología, Hospital Universitario La Paz, IdiPAZ, 28046 Madrid, Spain.
ScientificWorldJournal. 2011;11:2115-23. doi: 10.1100/2011/263137. Epub 2011 Nov 2.
We report the case of a 25-year-old woman who presented with abdominal and flank pain with two successive pregnancies and was diagnosed of giant bilateral renal AMLs and pulmonary LAM associated with TSC in the post-partum of her second pregnancy. This case illustrates that in women with TSC rapid growth from renal AMLs and development of LAM may occur with successive pregnancies. It also stresses the potential for preservation of renal function despite successive bilateral renal surgery of giant AMLs. Moreover, the treatment with a low-dose rapamycin may be an option for LAM treatment. Finally, a low-dose rapamycin may be considered as an adjuvant treatment together to kidney-sparing conservative surgery for renal AMLs.
我们报告了一名25岁女性的病例,该女性在连续两次怀孕期间出现腹部和侧腹疼痛,在第二次怀孕产后被诊断为双侧巨大肾血管平滑肌脂肪瘤(renal AMLs)以及与结节性硬化症(TSC)相关的肺淋巴管肌瘤病(LAM)。该病例表明,对于患有TSC的女性,肾AMLs可能会随着连续怀孕而快速生长,LAM也可能会发展。它还强调了尽管对巨大AMLs进行了连续双侧肾脏手术,但仍有保留肾功能的可能性。此外,低剂量雷帕霉素治疗可能是LAM治疗的一种选择。最后,低剂量雷帕霉素可被视为与保留肾脏的保守性肾AMLs手术联合使用的辅助治疗方法。