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Title: A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome.
Authors: Huerta, Ana
Arjona, Emilia
Portoles, Jose
Lopez-Sanchez, Paula
Rabasco, Cristina
Espinosa, Mario
Cavero, Teresa
Blasco, Miquel
Cao, Mercedes
Manrique, Joaquin
Cabello-Chavez, Virginia
Suñer, Marta
Heras, Manuel
Fulladosa, Xavier
Belmar, Lara
Sempere, Amparo
Peralta, Carmen
Castillo, Lorena
Arnau, Alvaro
Praga, Manuel
Rodriguez de Cordoba, Santiago
Keywords: cesarean section;complement;eculizumab;hemolytic uremic syndrome;postpartum
metadata.dc.subject.mesh: Adult
Antibodies, Monoclonal, Humanized
Atypical Hemolytic Uremic Syndrome
Cesarean Section
Complement Activation
Complement C3b Inactivator Proteins
Complement Factor H
Gene Conversion
Immunosuppressive Agents
Plasma Exchange
Postpartum Period
Pregnancy Complications
Renal Dialysis
Retrospective Studies
Risk Factors
Thrombotic Microangiopathies
Treatment Outcome
Issue Date: 12-Sep-2017
Abstract: Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis. Identification of inherited complement abnormalities explained nine of the 22 cases, with CFH mutations and CFH to CFHR1 gene conversion events being the most prevalent genetic alterations associated with this disorder (66%). In thirteen of the cases, pregnancy complications were sufficient to trigger a thrombotic microangiopathy in the absence of genetic or acquired complement alterations. The postpartum period was the time with highest risk to develop the disease and the group shows an association of cesarean section with pregnancy-associated aHUS. Seventeen patients underwent plasma treatments with a positive renal response in only three cases. In contrast, ten patients received eculizumab with an excellent renal response in all, independent of carrying or not inherited complement abnormalities. Although the cohort is relatively small, the data suggest that pregnancy-associated aHUS is not different from other types of aHUS and suggest the efficacy of eculizumab treatment over plasma therapies. This study may be useful to improve prognosis in this group of aHUS patients.
metadata.dc.identifier.doi: 10.1016/j.kint.2017.06.022
Appears in Collections:Producción 2020

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