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Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases

Aceruloplasminemia is a rare autosomal recessive disease in which a mutation leads to the absence or dysfunction of ceruloplasmin. Deficiency of this enzyme leads to the accumulation of iron in various organs; aceruloplasminemia is usually characterized by diabetes, retinal degeneration and neurolog...

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Detalles Bibliográficos
Autores principales: Roberti, Maria do Rosário Ferraz, Borges Filho, Handel Meireles, Gonçalves, Cláudio Humberto, Lima, Flávio Leão
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Associação Brasileira de Hematologia e Hemoterapia 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3415789/
https://www.ncbi.nlm.nih.gov/pubmed/23049345
http://dx.doi.org/10.5581/1516-8484.20110104
Descripción
Sumario:Aceruloplasminemia is a rare autosomal recessive disease in which a mutation leads to the absence or dysfunction of ceruloplasmin. Deficiency of this enzyme leads to the accumulation of iron in various organs; aceruloplasminemia is usually characterized by diabetes, retinal degeneration and neurological disorders. Diagnosis is suspected by the presence of elevated levels of ferritin, anemia, decreased serum copper and absence of ceruloplasmin in serum. Treatment of aceruloplasminemia is mainly based on the control of iron overload.