Hemolytic Uremic Syndrome in Pregnancy and Postpartum.
Clin J Am Soc Nephrol
; 12(8): 1237-1247, 2017 Aug 07.
Article
en En
| MEDLINE
| ID: mdl-28596415
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.
Palabras clave
Antibodies, Monoclonal, Humanized; Atypical Hemolytic Uremic Syndrome; Complement Pathway, Alternative; Female; Follow-Up Studies; France; Humans; Italy; Kidney Failure, Chronic; Plasma Exchange; Postpartum Period; Pregnancy; Recurrence; Retrospective Studies; Thrombotic Microangiopathies; United Kingdom; chemotactic factor inactivator; complement; eculizumab; hemolytic uremic syndrome; kidney transplantation; pregnancy; renal dialysis; thrombotic microangiopathy
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Complicaciones del Embarazo
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Periodo Posparto
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
País/Región como asunto:
Europa
Idioma:
En
Revista:
Clin J Am Soc Nephrol
Asunto de la revista:
NEFROLOGIA
Año:
2017
Tipo del documento:
Article