Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report with MCP Gene Mutation and Successful Eculizumab Treatment

被引:1
|
作者
Dominguez-Vargas, Alex [1 ,2 ]
Arino, Fanny [2 ]
Silva, Diana [2 ]
Gonzalez-Torres, Henry J. [2 ]
Aroca-Martinez, Gustavo [1 ,2 ,3 ]
Egea, Eduardo
Musso, Carlos G. [2 ]
机构
[1] Univ Norte, Div Ciencias Salud, Barranquilla, Colombia
[2] Univ Simon Bolivar, Fac Ciencias Salud, Barranquilla, Colombia
[3] Clin Costa, Dept Nefrol, Barranquilla, Colombia
来源
AJP REPORTS | 2024年 / 14卷 / 01期
关键词
aHUS; pregnancy; thrombotic microangiopathy; eculizumab; HELLP syndrome; COMPLEMENT;
D O I
10.1055/a-2164-8438
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia and kidney injury from thrombotic microangiopathy. P-aHUS occurs in approximately 1 in 25,000 pregnancies and is strongly related to complement dysregulation and pregnancy-related disorders, such as preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome, resulting in adverse perinatal and fetal outcomes. Complement dysregulation in P-aHUS is commonly attributed to genetic mutations or autoantibodies affecting complement factors, including CFH , CFI , and MCP. We present a case of a 25-year-old primigravida who experienced severe preeclampsia and HELLP syndrome followed by the development of complicated P-aHUS during the early postpartum period. The patient exhibited severe clinical manifestations, including hypertensive emergency, central nervous system involvement, renal impairment, and microangiopathic hemolytic anemia. Timely initiation of eculizumab therapy resulted in successful disease remission. Further genetic analysis revealed a likely rare pathogenic MCP gene variant.
引用
收藏
页码:e96 / e100
页数:5
相关论文
共 50 条
  • [41] Preservation of Renal Function in Atypical Hemolytic Uremic Syndrome by Eculizumab: A Case Report
    Giordano, Mario
    Castellano, Giuseppe
    Messina, Giovanni
    Divella, Claretta
    Bellantuono, Rosa
    Puteo, Flora
    Colella, Vincenzo
    Depalo, Tommaso
    Gesualdo, Loreto
    PEDIATRICS, 2012, 130 (05) : E1385 - E1388
  • [42] Atypical hemolytic uremic syndrome in a patient with HIV treated with eculizumab: A case report
    Gonzalez, Danilo E. Trujillo
    Misol, Dagoberto Duarte
    Ordonez, Nicolas Ariza
    Zamora, Fabian Andres Salgado
    Prada, Henry A. Millan
    Zunga, R. Eduardo
    Trivino, Alejandra Molano
    IDCASES, 2023, 31
  • [43] A SUCCESSFUL KIDNEY TRANSPLANTATION WITH PROPHYLACTIC ECULIZUMAB TREATMENT IN A PATIENT WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME
    KasapogLu, Umut
    Ruhi, Caglar
    TugCu, Murat
    BoynuegRi, BasAk
    Titiz, IZzet
    Hancer, Veysel Sabri
    ApaydiN, Suheyla
    TRANSPLANT INTERNATIONAL, 2015, 28 : 641 - 641
  • [44] Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation and IgA Nephropathy: A Case Report Successfully Treated with Eculizumab
    Nakamura, Hironori
    Anayama, Mariko
    Makino, Mutsuki
    Makino, Yasushi
    Tamura, Katsuhiko
    Nagasawa, Masaki
    NEPHRON, 2018, 138 (04) : 324 - 327
  • [45] Successful Use of Eculizumab for Prevention of Atypical Hemolytic Uremic Syndrome Recurrence in a Pregnant Kidney Transplant Recipient with CFH Gene Mutation: A Case Report and Literature Review
    Zahran, Somaya
    Nodzynski, Thomas
    Matte, Catherine
    Sandal, Shaifali
    Podymow, Tiina
    Cantarovich, Marcelo
    JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2024, 35 (10):
  • [46] Acute Discontinued Eculizumab Treatment In Atypical Hemolytic-uremic Syndrome Due To Mcp Mutation. May Be A Therapeutic Option?
    Garcia-Martinez, Elena
    Anton-gamero, Montserrat
    Azpilicueta-idarreta, Maria
    Yebenes-cano, Sonia
    Sanchez-corral, Pilar
    LOpez-trascasa, Margarita
    PEDIATRIC NEPHROLOGY, 2014, 29 (09) : 1838 - 1838
  • [47] Hepatotoxicity associated with eculizumab in a patient with atypical hemolytic uremic syndrome
    Oruc, Aysegul
    Ayar, Yavuz
    Vuruskan, Berna Aytac
    Yildiz, Abdulmecit
    Aktas, Nimet
    Yavuz, Mahmut
    Gullulu, Mustafa
    Dilek, Kamil
    Ersoy, Alparslan
    NEFROLOGIA, 2018, 38 (04): : 448 - 450
  • [48] A CASE OF ATYPICAL HEMOLYTIC UREMIC SYNDROME SUCCESSFULLY TREATED WITH ECULIZUMAB
    Thajudeen, Bijin
    Kamel, Mahmoud
    Bracamonte, Erika
    Sussman, Amy
    AMERICAN JOURNAL OF KIDNEY DISEASES, 2013, 61 (04) : A94 - A94
  • [49] Discontinuation of Eculizumab Treatment in Atypical Hemolytic Uremic Syndrome: An Update
    Ardissino, Gianluigi
    Possenti, Ilaria
    Tel, Francesca
    Testa, Sara
    Salardi, Stefania
    Ladisa, Vito
    AMERICAN JOURNAL OF KIDNEY DISEASES, 2015, 66 (01) : 172 - 173
  • [50] A Case of Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab
    Thajudeen, B.
    Sussman, A.
    Bracamonte, E.
    CASE REPORTS IN NEPHROLOGY AND DIALYSIS, 2013, 3 (02): : 139 - 146