Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Clinical journal of the American Society of Nephrology: CJASN

Alexandra Bruel¹ 

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关键词: hemolytic uremic syndrome;    complement;    pregnancy;    thrombotic microangiopathy;    Antibodies, Monoclonal, Humanized;    Atypical Hemolytic Uremic Syndrome;    Complement Pathway, Alternative;    Female;    Follow-Up Studies;    France;    Humans;    Italy;    Kidney Failure, Chronic;    kidney transplantation;    Plasma Exchange;    Postpartum Period;    Pregnancy;    Recurrence;    renal dialysis;    Retrospective Studies;    Thrombotic Microangiopathies;    United Kingdom;    chemotactic factor inactivator;    eculizumab;   

DOI  :  10.2215/CJN.00280117

学科分类：泌尿医学

来源: American Society of Nephrology

【 摘 要 】

Background Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation.Design, setting, participants, & measurements We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome.Results Eighty-seven patients with pregnancy-associated hemolytic uremic syndrome were included. Hemolytic uremic syndrome occurred mainly during the first pregnancy (58%) and in the postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) patients. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%).Conclusions Pregnancy-associated hemolytic uremic syndrome and atypical hemolytic uremic syndrome nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.

【 授权许可】

CC BY