Utilize este identificador para referenciar este registo:
https://hdl.handle.net/10316/108961
Título: | Ocular Manifestations and Therapeutic Options in Patients with Familial Amyloid Polyneuropathy: A Systematic Review | Autor: | Martins, A. C. Rosa, A. M. Costa, E. Tavares, C. Quadrado, M. J. Murta, J. N. |
Data: | 2015 | Editora: | Hindawi | Título da revista, periódico, livro ou evento: | BioMed Research International | Volume: | 2015 | Resumo: | This paper aims to review the morphological and functional characteristics of patients affected by familial amyloid polyneuropathy (FAP), with greater focus on type I and its progression after liver transplantation. We also analyse therapeutic options for the ophthalmic manifestations. Methods.The literature from 2002 through 2015 was reviewed, with a total of 45 articles studied, using the key terms related to amyloidosis and its therapeutic approaches. Information was collated, evaluated, critically assessed, and then summarised in its present form. Pathophysiology and Treatment. FAP results from mutation of the transthyretin gene, withVal30Met being themost frequent substitution.Thesymptoms are those typical of a sensorimotor autonomic neuropathy and can be halted with liver transplantation. Nowadays there are new medical therapies that delay the progression of the systemic neuropathy. However, there are still no options to avoid ocular disease. Conclusion.Themain ocularmanifestations in patients with FAP type I are amyloid deposition in the vitreous, dry eye, and secondary glaucoma. Despite liver transplantation, eye synthesis of amyloid persists and is associated with progressive ocular manifestations, which require continued ophthalmologic follow-up. New therapeutic strategies are therefore needed, particularly to target the ocular synthesis of the abnormal protein. | URI: | https://hdl.handle.net/10316/108961 | ISSN: | 2314-6133 2314-6141 |
DOI: | 10.1155/2015/282405 | Direitos: | openAccess |
Aparece nas coleções: | FMUC Medicina - Artigos em Revistas Internacionais |
Ficheiros deste registo:
Ficheiro | Descrição | Tamanho | Formato | |
---|---|---|---|---|
Ocular-Manifestations-and-Therapeutic-Options-in-Patients-with-Familial-Amyloid-Polyneuropathy-A-Systematic-ReviewBioMed-Research-International.pdf | 1.78 MB | Adobe PDF | Ver/Abrir |
Visualizações de página
61
Visto em 28/ago/2024
Downloads
23
Visto em 28/ago/2024
Google ScholarTM
Verificar
Altmetric
Altmetric
Este registo está protegido por Licença Creative Commons