Pregnancy outcomes in patients with Alport syndrome

Arch Gynecol Obstet. 2016 Apr;293(4):739-47. doi: 10.1007/s00404-015-3893-9. Epub 2015 Sep 28.


Purpose: To analyze the maternal and obstetric outcomes of patients with Alport syndrome.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Alport syndrome; Kidney disease; Nephritis; Pregnancy; Renal failure.

MeSH terms

  • Adult
  • Female
  • Glomerular Filtration Rate
  • Humans
  • Infant, Newborn
  • Kidney / physiopathology*
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / genetics*
  • Kidney Failure, Chronic / pathology
  • Nephritis, Hereditary / complications*
  • Nephritis, Hereditary / genetics*
  • Nephritis, Hereditary / pathology
  • Pre-Eclampsia / diagnosis
  • Pre-Eclampsia / epidemiology*
  • Pre-Eclampsia / physiopathology
  • Pregnancy
  • Pregnancy Complications / epidemiology
  • Pregnancy Complications / physiopathology
  • Pregnancy Outcome
  • Premature Birth / epidemiology*
  • Premature Birth / physiopathology
  • Proteinuria / blood
  • Risk Factors