Literature DB >> 30659006

Recurrent case of pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS).

Dileep Kumar1, Mary King2, Belinda Jim1, Anjali Acharya1.   

Abstract

Pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS) is a rare condition characterised by microangiopathic haemolytic anaemia, thrombocytopenia and renal failure. It accounts for approximately 7% of total HUS cases. Here, we present a case of recurrent P-aHUS in a 25-year-old Hispanic woman. Pregnancy was the clear trigger in both instances, and the disease manifested in first week of the postpartum period. Because of her significant obstetric history, a multidisciplinary approach was adopted to monitor her second pregnancy antepartum and post partum. As the patient developed recurrence of P-aHUS 4 days after her delivery, she was immediately administered eculizumab within few hours of disease manifestation. The patient normalised her haematological parameters within 1 week but sustained dialysis-requiring renal failure for a total of 6 weeks. This case highlights the advances as well as the ongoing uncertainties, especially with respect to the use of eculizumab, in this rare but morbid disease. © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.

Entities:  

Keywords:  acute renal failure; chronic renal failure; dialysis; genetic screening / counselling; pregnancy

Mesh:

Substances:

Year:  2019        PMID: 30659006      PMCID: PMC6340516          DOI: 10.1136/bcr-2018-226571

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  18 in total

1.  Eculizumab cessation in atypical hemolytic uremic syndrome.

Authors:  Samuel A Merrill; Zachary D Brittingham; Xuan Yuan; Alison R Moliterno; C John Sperati; Robert A Brodsky
Journal:  Blood       Date:  2017-05-01       Impact factor: 22.113

2.  Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.

Authors:  Marina Noris; Jessica Caprioli; Elena Bresin; Chiara Mossali; Gaia Pianetti; Sara Gamba; Erica Daina; Chiara Fenili; Federica Castelletti; Annalisa Sorosina; Rossella Piras; Roberta Donadelli; Ramona Maranta; Irene van der Meer; Edward M Conway; Peter F Zipfel; Timothy H Goodship; Giuseppe Remuzzi
Journal:  Clin J Am Soc Nephrol       Date:  2010-07-01       Impact factor: 8.237

3.  Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab.

Authors:  Aude Servais; Nadège Devillard; Véronique Frémeaux-Bacchi; Aurélie Hummel; Laurent Salomon; Cécile Contin-Bordes; Hélène Gomer; Christophe Legendre; Yahsou Delmas
Journal:  Nephrol Dial Transplant       Date:  2016-09-01       Impact factor: 5.992

Review 4.  Eculizumab in pregnancy: a narrative overview.

Authors:  Laura Sarno; Antonella Tufano; Giuseppe Maria Maruotti; Pasquale Martinelli; Mario M Balletta; Domenico Russo
Journal:  J Nephrol       Date:  2018-08-29       Impact factor: 3.902

5.  The management of pregnancy in paroxysmal nocturnal haemoglobinuria on long term eculizumab.

Authors:  Richard Kelly; Louise Arnold; Stephen Richards; Anita Hill; Charlotte Bomken; John Hanley; Andrew Loughney; Jon Beauchamp; Gus Khursigara; Russell P Rother; Elizabeth Chalmers; Andrew Fyfe; Edward Fitzsimons; Ryotaro Nakamura; Anna Gaya; Antonio M Risitano; Jörg Schubert; Derek Norfolk; Nigel Simpson; Peter Hillmen
Journal:  Br J Haematol       Date:  2010-02-11       Impact factor: 6.998

6.  FBN1 mutation screening of patients with Marfan syndrome and related disorders: detection of 46 novel FBN1 mutations.

Authors:  M Attanasio; I Lapini; L Evangelisti; L Lucarini; B Giusti; Mc Porciani; R Fattori; C Anichini; R Abbate; Gf Gensini; G Pepe
Journal:  Clin Genet       Date:  2008-04-22       Impact factor: 4.438

7.  Novel C3 mutation p.Lys65Gln in aHUS affects complement factor H binding.

Authors:  Elena Volokhina; Dineke Westra; Xiaoguang Xue; Piet Gros; Nicole van de Kar; Lambert van den Heuvel
Journal:  Pediatr Nephrol       Date:  2012-06-06       Impact factor: 3.714

Review 8.  HUS and atypical HUS.

Authors:  T Sakari Jokiranta
Journal:  Blood       Date:  2017-04-17       Impact factor: 25.476

9.  Post-partum atypical haemolytic-uraemic syndrome treated with eculizumab: terminal complement activity assessment in clinical practice.

Authors:  Yahsou Delmas; Cécile Bordes; Chantal Loirat; Véronique Frémeaux-Bacchi; Christian Combe
Journal:  Clin Kidney J       Date:  2013-04

Review 10.  Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab.

Authors:  Arif Asif; Ali Nayer; Christian S Haas
Journal:  J Nephrol       Date:  2016-11-15       Impact factor: 3.902

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  1 in total

1.  An Unusual cause of Atypical Hemolytic Uremic Syndrome Relapse.

Authors:  Mehmet Mert; Mevlut Ceri; Selcuk Yuksel; Belda Dursun
Journal:  Indian J Nephrol       Date:  2022-01-05
  1 in total

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