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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Associação Brasileira de Hematologia e Hemoterapia
2011
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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 |
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author | Roberti, Maria do Rosário Ferraz Borges Filho, Handel Meireles Gonçalves, Cláudio Humberto Lima, Flávio Leão |
author_facet | Roberti, Maria do Rosário Ferraz Borges Filho, Handel Meireles Gonçalves, Cláudio Humberto Lima, Flávio Leão |
author_sort | Roberti, Maria do Rosário Ferraz |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-3415789 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Associação Brasileira de Hematologia e Hemoterapia |
record_format | MEDLINE/PubMed |
spelling | pubmed-34157892012-10-04 Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases Roberti, Maria do Rosário Ferraz Borges Filho, Handel Meireles Gonçalves, Cláudio Humberto Lima, Flávio Leão Rev Bras Hematol Hemoter Case Report 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. Associação Brasileira de Hematologia e Hemoterapia 2011 /pmc/articles/PMC3415789/ /pubmed/23049345 http://dx.doi.org/10.5581/1516-8484.20110104 Text en http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Roberti, Maria do Rosário Ferraz Borges Filho, Handel Meireles Gonçalves, Cláudio Humberto Lima, Flávio Leão Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title | Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title_full | Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title_fullStr | Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title_full_unstemmed | Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title_short | Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
title_sort | aceruloplasminemia: a rare disease - diagnosis and treatment of two cases |
topic | Case Report |
url | 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 |
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