Yefet Enav, Tovbin David, Nachum Zohar
Department of Obstetrics and Gynecology, Emek Medical Center, Afula, Israel.
Department of Nephrology, Emek Medical Center, Afula, Israel.
Arch Gynecol Obstet. 2016 Apr;293(4):739-47. doi: 10.1007/s00404-015-3893-9. Epub 2015 Sep 28.
To analyze the maternal and obstetric outcomes of patients with Alport syndrome.
We describe the pregnancy course of 8 pregnancies of three family members with the autosomal dominant (the rarest) form of Alport syndrome. We also analyzed 10 previously reported pregnancies with other Alport mutations in order to explore risk factors for unfavorable obstetric outcomes and maternal renal deterioration.
In 13 pregnancies (72 %), renal function did not deteriorate permanently. All of these women had pre-pregnancy mild chronic kidney disease (CKD stage G1). In all of them, only a transient increase in proteinuria was recorded and in one case there was a transient decrease in the estimated glomerular filtration rate. In four other pregnancies (22 %), renal function deteriorated following pregnancy. All of them were complicated with pre-eclampsia. One woman had pre-pregnancy CKD-G2A3 and chronic hypertension. Two women had CKD-G1A3 of whom one had pre-pregnancy proteinuria near the nephrotic range. In the fourth case, renal function deterioration was reported without information on the exact pre-pregnancy renal function. In the last case, CKD-G2 was reported after pregnancy without information on CKD stage prior to pregnancy. Severe proteinuria did not imply a permanent renal function deterioration if it developed during pregnancy. Ten pregnancies ended with preterm birth (56 %). Two stillbirths were reported (11 %); however, only one was attributed to maternal health deterioration.
Data regarding pregnancy outcomes in Alport syndrome is limited. The outcome seems favorable when pre-pregnancy kidney function is normal or near normal and when chronic hypertension/pre-eclampsia is absent.
分析奥尔波特综合征患者的孕产妇及产科结局。
我们描述了三名患有常染色体显性（最罕见）形式奥尔波特综合征的家庭成员的8次妊娠过程。我们还分析了之前报道的10例伴有其他奥尔波特突变的妊娠情况，以探究不良产科结局及孕产妇肾功能恶化的危险因素。
在13次妊娠（72%）中，肾功能未出现永久性恶化。所有这些女性孕前均患有轻度慢性肾脏病（CKD G1期）。在所有这些病例中，仅记录到蛋白尿短暂增加，且有1例估计肾小球滤过率短暂下降。在另外4次妊娠（22%）中，妊娠后肾功能恶化。所有这些病例均并发子痫前期。1名女性孕前患有CKD-G2A3及慢性高血压。2名女性患有CKD-G1A3，其中1名女性孕前蛋白尿接近肾病范围。在第4例中，报告了肾功能恶化，但未提供孕前确切肾功能信息。在最后1例中，妊娠后报告为CKD-G2，但未提供孕前CKD分期信息。如果蛋白尿在孕期出现，严重蛋白尿并不意味着肾功能会永久性恶化。10次妊娠以早产告终（56%）。报告了2例死产（11%）；然而，仅1例归因于孕产妇健康恶化。
关于奥尔波特综合征妊娠结局的数据有限。当孕前肾功能正常或接近正常且无慢性高血压/子痫前期时，结局似乎较好。
