000 | nab a22 7a 4500 | ||
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999 |
_c17131 _d17131 |
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003 | PC17131 | ||
005 | 20221222134108.0 | ||
008 | 221222b xxu||||| |||| 00| 0 eng d | ||
040 | _cH12O | ||
041 | _aspa | ||
100 |
_9559 _aMarcacuzco Quinto, Alberto _eCirugía General y Aparato Digestivo |
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100 |
_9360 _aManrique Municio, Alejandro _eCirugía General y del Aparato Digestivo |
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100 |
_9358 _aJiménez Romero, Carlos _eCirugía General y del Aparato Digestivo |
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100 |
_9558 _aLoinaz Segurola, Carmelo _eCirugía General y del Aparato Digestivo |
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100 |
_9557 _aCalvo Pulido, Jorge _eCirugía General y del Aparato Digestivo |
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100 |
_9282 _aJusto Alonso, Iago _eCirugía General y del Aparato Digestivo |
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100 |
_9556 _aGarcía-Sesma Pérez-Fuentes, Álvaro _eCirugía General y del Aparato Digestivo |
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100 |
_9560 _aAbradelo de Usera, Manuel _eCirugía General y Aparato Digestivo |
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100 |
_9552 _aCambra Molero, Félix _eCirugía General y del Aparato Digestivo |
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100 |
_9554 _aCaso Maestro, Óscar _eCirugía General y del Aparato Digestivo |
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100 |
_9510 _aMoreno González, Enrique _eCirugía General y del Aparato Digestivo |
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245 | 0 | 0 |
_aTrasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años. _h[artículo] |
260 |
_bMedicina clínica, _c2015 |
||
300 | _a144(9):385-8. | ||
500 | _aFormato Vancouver: Marcacuzco Quinto AA, Manrique Municio A, Jiménez Romero LC, Loinaz Segurola C, Calvo Pulido J, Justo Alonso I et al. Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años. Med Clin (Barc). 2015 May 8;144(9):385-8. | ||
501 | _aPMID: 24746275 | ||
504 | _aContiene 30 referencias | ||
520 | _aBackground and objective: Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. Material and method: Six patients with PAF underwent liver transplantation between April 1986 and December 2012. Results: The mean age was 57.7+16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart-liver transplants sequentially were performed. Patient survival and graft was 80% at one, 3 and 5 years. Conclusions: The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage. | ||
710 |
_9271 _aServicio de Cirugía General y del Aparato Digestivo |
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856 |
_uhttp://pc-h12o-es.m-hdoct.a17.csinet.es/pdf/pc/1/pc17131.pdf _ySolicitar documento |
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942 |
_2ddc _cART _n0 |