A Case of Familial Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation in Adults / 대한신장학회지
Korean Journal of Nephrology
;
: 259-264, 2009.
Artículo
en Coreano
| WPRIM
| ID: wpr-211091
ABSTRACT
The hemolytic uremic syndrome is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure. Atypical hemolytic uremic syndrome (aHUS) which is not usually associated with prodromal symptoms, especially diarrhea, has a higher mortality rate and a stronger tendency to progress to chronic renal failure. In approximately 30-50% of patients with aHUS, mutations have been detected in complement factor H, membrane cofactor protein or factor I. Mutations in the complement regulator factor H are the most frequent and have a very poor prognosis, with most patients developing ESRD. We have experienced a 33-year-old man with a family history of renal failure diagnosed as aHUS resulted from factor H mutation, for whom we carried out hemodialysis, plasmapheresis and other conservative management.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Pronóstico
/
Trombocitopenia
/
Proteínas del Sistema Complemento
/
Fibrinógeno
/
Diálisis Renal
/
Plasmaféresis
/
Factor H de Complemento
/
Diarrea
/
Insuficiencia Renal
/
Proteína Cofactora de Membrana
Tipo de estudio:
Estudio pronóstico
Límite:
Adulto
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Nephrology
Año:
2009
Tipo del documento:
Artículo
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