A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab.

BMC Nephrology
Prianka PuriMirna Vucak-Dzumhur

Abstract

Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy. A 27-year-old female of Lebanese descent presented at 36 weeks' gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2 weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2 months she became dialysis independent. This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her ris...Continue Reading

References

Mar 6, 2010·Journal of the American Society of Nephrology : JASN·Fadi FakhouriVeronique Frémeaux-Bacchi
Nov 26, 2010·Nature Reviews. Nephrology·Julien ZuberChristophe Legendre
Dec 12, 2012·Clinical Journal of the American Society of Nephrology : CJASN·Phyllis August
Jan 12, 2013·Clinical Journal of the American Society of Nephrology : CJASN·Véronique Fremeaux-BacchiChantal Loirat
Sep 14, 2013·Journal of the American Society of Nephrology : JASN·Fengxiao BuRichard J H Smith
Mar 25, 2015·Molecular Immunology·Jean F RegalRichard M Burwick
Sep 10, 2015·The New England Journal of Medicine·Richard J KellyRegis Peffault de Latour
Jun 10, 2017·Clinical Journal of the American Society of Nephrology : CJASN·Alexandra BruelFadi Fakhouri
Sep 16, 2017·Kidney International·Ana HuertaSantiago Rodriguez de Cordoba

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Methods Mentioned

BETA
biopsy
exome sequencing

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