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Atypical hemolytic uremic syndrome in the Colombian Caribbean: its particular characteristics

2021; Springer Science+Business Media; Volume: 54; Issue: 6 Linguagem: Inglês

10.1007/s11255-021-03011-5

1573-2584

Omar Cabarcas-Barbosa, Gustavo Aroca, Carlos G. Musso, Elizabeth Ramos-Bolaños, Henry J. González-Tórres, Zilac Espitaleta-Vergara, Alex Domínguez-Vargas, Edufamir Ararat-Rodriguez, J Zamora Orozco, Luis Castillo-Parodi, Juan Conde-Manotas, Rodrigo Daza-Arnedo, Víctor Rodríguez-SanJuan, Liliana Gómez-Navarro, Roberto Acosta-Madiedo, Luis Barros-Camargo, Angélica Aduen-Carrillo, Francisco Ayola-Anaya, María Pulgar-Emiliani, Andrés Cadena-Bonfanti,

Adenosine and Purinergic Signaling

Atypical hemolytic uremic syndrome (aHUS) is a rare and genetically mediated systemic disease most often caused by uncontrolled and chronic complement activation that leads to systemic thrombotic microangiopathy, renal and extra-renal damage. This is descriptive, retrospective and multicenter study, which reports demographic, clinical, laboratory, and genetic characteristics, as well as their treatment response and outcome of 20 aHUS patients diagnosed between 2014 and 2018. Most patients were female adults (75%) and 30% were associated to pregnancy/postpartum, 15% to autoimmune disease, and 65% to infections. Gastrointestinal involvement (75%) was the most frequent extra-renal organ damage. Antenatal mortality and mortality rate were 5% and 10%, respectively. 25% of the patients progressed to end-stage renal disease. In 4/8 of patients treated within 1 week of presentation, eculizumab treatment restored multi-organ function after 4 weeks of treatment. CFH (37%) and CFI (25%) mutations were the most frequent. This is the first series of aHUS cases of Colombian Caribbean region which reports the clinical and epidemiological characteristics of this condition in this region.