Department of Internal Medicine, Center of Expertise for Tuberous Sclerosis Complex, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Obstetrics and Gynecology, University Medical Center Utrecht, Utrecht, The Netherlands.
BMC Nephrol. 2024 Mar 22;25(1):113. doi: 10.1186/s12882-024-03483-4.
Women are counseled preconceptionally about the potential risks of rAML progression and chance of complications during and due to pregnancy. However, a systematic search investigating the evidence on which this advice is based does not exist. The aim of this systematic review is to determine the effect of pregnancy on renal angiomyolipoma (rAML) size and risk of haemorrhage in patients with tuberous sclerosis complex (TSC).
We searched PubMed, EMBASE, Medline and ClinicalTrials.gov using terms for "renal angiomyolipoma" and "pregnancy". English-language articles published between January 1st 2000, and December 31st 2020 of which full-text was available were included. The initial search resulted in 176 articles. After the screening process we included 45 case reports and 1 retrospective study. For the retrospective study we assessed the risk of bias using the Newcastle-Ottawa Scale. We included articles about renal AML and pregnancy with and without an established diagnosis of TSC. From these articles we recorded the rAML sizes and rAML complications.
Seven case reports, from a total of 45 case reports, provided follow-up data on renal AML size (these were all cases of renal AML without a known diagnosis of TSC). Of these cases, renal AML size decreased in one patient, was stable in one patient, increased in three patients and fluctuated in two others. Renal AML size of women who suffered a haemorrhage were significantly larger (12.1 ± 4.6 cm) than rAMLs of women who did not suffer a haemorrhage (8.3 ± 3.2 cm). Data from the retrospective study showed no difference in renal complications between the women with and without a history of pregnancy. Haemorrhage occurred in 30% of the women with a history of pregnancy (n = 20) and in 11% in the patients without a history of pregnancy (n = 2), however this retrospective study had methodological limitations.
The effect of pregnancy on renal AML size and complications in patients with TSC is unclear. More research is needed to determine the risk of pregnancy on TSC-associated kidney disease in TSC patient.
女性在受孕前就被告知，肾血管平滑肌脂肪瘤（rAML）进展的潜在风险以及妊娠期间和妊娠后发生并发症的可能性。然而，目前尚未进行系统检索来调查这一建议的依据。本系统评价的目的是确定妊娠对结节性硬化症（TSC）患者的 rAML 大小和出血风险的影响。
我们使用“肾血管平滑肌脂肪瘤”和“妊娠”的术语在 PubMed、EMBASE、Medline 和 ClinicalTrials.gov 中进行了检索。纳入了 2000 年 1 月 1 日至 2020 年 12 月 31 日发表的英文文章，且全文可用。初始检索得到了 176 篇文章。经过筛选过程，我们纳入了 45 篇病例报告和 1 项回顾性研究。对于回顾性研究，我们使用纽卡斯尔-渥太华量表评估了偏倚风险。我们纳入了有和没有 TSC 明确诊断的 rAML 和妊娠的文章。从这些文章中，我们记录了 rAML 的大小和 rAML 并发症。
在总共 45 篇病例报告中，有 7 篇病例报告提供了 rAML 大小的随访数据（这些病例均为无 TSC 已知诊断的 rAML）。在这些病例中，1 例 rAML 大小减小，1 例 rAML 大小稳定，3 例 rAML 大小增大，2 例 rAML 大小波动。发生出血的女性 rAML 大小明显大于未发生出血的女性（12.1±4.6cm 比 8.3±3.2cm）。回顾性研究的数据显示，有妊娠史和无妊娠史的女性之间肾脏并发症无差异。有妊娠史的女性中有 30%（n=20）发生出血，无妊娠史的女性中有 11%（n=2）发生出血，但该回顾性研究存在方法学局限性。
妊娠对 TSC 患者 rAML 大小和并发症的影响尚不清楚。需要进一步的研究来确定妊娠对 TSC 相关肾脏疾病的风险。
