Abstract
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.
Similar content being viewed by others
References
Kashtan CE (1999) Alport syndrome. An inherited disorder of renal, ocular, and cochlear basement membranes. Medicine (Baltimore) 78(5):338–360
Tryggvason K, Zhou J, Hostikka SL, Shows TB (1993) Molecular genetics of Alport syndrome. Kidney Int 43(1):38–44
Coppo R, Gianoglio B, Porcellini MG, Maringhini S (1998) Frequency of renal diseases and clinical indications for renal biopsy in children (report of the Italian National Registry of Renal Biopsies in Children). Group of Renal Immunopathology of the Italian Society of Pediatric Nephrology and Group of Renal Immunopathology of the Italian Society of Nephrology. Nephrol Dial Transplant 13(2):293–297
Hasstedt SJ, Atkin CL (1983) X-linked inheritance of Alport syndrome: family P revisited. Am J Hum Genet 35(6):1241–1251
Matsuo K, Tudor EL, Baschat AA (2007) Alport syndrome and pregnancy. Obstet Gynecol 109(2 Pt2):531–532
Nakano Y, Yuhara C, Saito J, Miyazaki R, Yoshida K, Kuwabara Y (1993) Pregnancy with Alport syndrome: a case report. Nissanpu Tokyo Kaishi 42:302–304
Omori H, Wa S, Mikami S et al (2004) A case of Alport syndrome with deteriorating nephrosis during pregnancy. Nihon Jinzou Gakkaishi 46:532
Crovetto F, Moroni G, Zaina B, Acaia B, Ossola MW, Fedele L (2013) Pregnancy in women with Alport syndrome. Int Urol Nephrol 45(4):1223–1227
Matsubara S, Ueda Y, Takahashi H, Nagai T, Kuwata T, Muto S et al (2009) Pregnancy complicated with Alport syndrome: a good obstetric outcome and failure to diagnose an infant born to a mother with Alport syndrome by umbilical cord immunofluorescence staining. J Obstet Gynaecol Res 35(6):1109–1114
Alessi M, Fabris A, Zambon A, Cremasco D, Muraro E, Dosa L et al (2014) Pregnancy in Alport syndrome: a report of two differently-evolving cases. J Obstet Gynaecol 34(1):98–100
Matsubara S, Muto S (2012) Good obstetric outcome of consecutive pregnancies in a woman with Alport syndrome. Arch Gynecol Obstet 286(1):261–262
Mehta S, Saifan C, Abdellah M, Choueiry R, Nasr R, El Sayegh S (2013) Alport’s syndrome in pregnancy. Case Rep Med 2013:374020
Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group (2013) KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int (Suppl 3):1–150
Alper AB, Yi Y, Rahman M, Webber LS, Magee L, von Dadelszen P et al (2011) Performance of estimated glomerular filtration rate prediction equations in preeclamptic patients. Am J Perinatol 28(6):425–430
Smith MC, Moran P, Davison JM (2011) EPI-CKD is a poor predictor of GFR in pregnancy (abstract). Arch Dis Child Fetal Neonatal Ed 96(1):Fa99
Abe S (1991) An overview of pregnancy in women with underlying renal disease. Am J Kidney Dis 17(2):112–115
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Details of ethics approval
Informed consent was obtained from the patient treated at our institution. All the information regarding the patients in this study is presented anonymously. This study was approved by the ethics committee.
Conflict of interest
The authors declare that they have no conflict of interest.
Rights and permissions
About this article
Cite this article
Yefet, E., Tovbin, D. & Nachum, Z. Pregnancy outcomes in patients with Alport syndrome. Arch Gynecol Obstet 293, 739–747 (2016). https://doi.org/10.1007/s00404-015-3893-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00404-015-3893-9