Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome / Síndrome nefrótica associada à síndrome hemolítico-urêmica atípica primária
J. bras. nefrol
; 43(3): 440-444, July-Sept. 2021. graf
Article
in English, Portuguese
| LILACS
| ID: biblio-1340119
Responsible library:
BR1.1
ABSTRACT
Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.
RESUMO
Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Purpura, Thrombotic Thrombocytopenic
/
Atypical Hemolytic Uremic Syndrome
/
Anemia, Hemolytic
/
Nephrotic Syndrome
Type of study:
Risk factors
Limits:
Child
/
Humans
Language:
English
/
Portuguese
Journal:
J. bras. nefrol
Journal subject:
Nephrology
Year:
2021
Document type:
Article
Affiliation country:
Colombia
Institution/Affiliation country:
Universidad de Antioquia/CO
/
University Corporation Antonio Jose de Sucre/CO
