Síndrome hemolítico urémico atípico en tratamiento con eculizumab: casos clínicos / Atypical hemolytic uremic syndrome: report of two cases treated with Eculizumab
Rev. méd. Chile
;
146(2): 254-259, feb. 2018. graf
Artículo
en Español
| LILACS
| ID: biblio-961385
ABSTRACT
Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy where organic damage predominates in the kidney. Atypical HUS (aHUS) is a rare disease that affects young adults and causes terminal chronic renal failure ending in dialysis, in most cases. It also recurs after kidney transplantation. aHUS is associated with genetic defects of the alternative complement pathway or its activation by other factors such as drugs, autoimmune diseases, infections, malignant hypertension and ischemia-reperfusion. We report two women aged 17 and 25 years old with catastrophic aHUS. In both cases, complement amplifying factors (drugs and infections) were added and acted on a genetic vulnerability to precipitate complement activation and produce aHUS. Both patients developed terminal renal failure and had to undergo hemodialysis. Fortunately, after a broad etiological study, it was possible to make the diagnosis of aHUS and start treatment with Eculizumab, a monoclonal antibody that changed the natural history of aHUS. It inhibits complement activity controlling microangiopathy and preventing the development of end-stage renal disease. It also improves the success rate in kidney transplantation. In the case of our patients, both discontinued dialysis after chronic treatment with Eculizumab.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Anticuerpos Monoclonales Humanizados
/
Síndrome Hemolítico Urémico Atípico
Tipo de estudio:
Estudio diagnóstico
/
Estudio observacional
/
Estudio pronóstico
/
Factores de riesgo
Límite:
Adolescente
/
Adulto
/
Femenino
/
Humanos
Idioma:
Español
Revista:
Rev. méd. Chile
Asunto de la revista:
Medicina
Año:
2018
Tipo del documento:
Artículo
País de afiliación:
Chile
Institución/País de afiliación:
Pontificia Universidad Católica de Chile/CL
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