RT Journal Article
T1 A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome.
A1 Huerta, Ana
A1 Arjona, Emilia
A1 Portoles, Jose
A1 Lopez-Sanchez, Paula
A1 Rabasco, Cristina
A1 Espinosa, Mario
A1 Cavero, Teresa
A1 Blasco, Miquel
A1 Cao, Mercedes
A1 Manrique, Joaquin
A1 Cabello-Chavez, Virginia
A1 Suñer, Marta
A1 Heras, Manuel
A1 Fulladosa, Xavier
A1 Belmar, Lara
A1 Sempere, Amparo
A1 Peralta, Carmen
A1 Castillo, Lorena
A1 Arnau, Alvaro
A1 Praga, Manuel
A1 Rodriguez de Cordoba, Santiago
K1 cesarean section
K1 complement
K1 eculizumab
K1 hemolytic uremic syndrome
K1 postpartum
AB 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.
YR 2017
FD 2017-09-12
LK http://hdl.handle.net/10668/11581
UL http://hdl.handle.net/10668/11581
LA en
DS RISalud
RD Apr 19, 2025