MARC details
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04446nmi a22003857a 4500 |
| 003 - CONTROL NUMBER IDENTIFIER |
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OSt |
| 005 - DATE AND TIME OF LATEST TRANSACTION |
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20191105162603.0 |
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180124d2019 sp ||||| |||| 00| 0 spa d |
| 040 ## - CATALOGING SOURCE |
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| Transcribing agency |
Salus Infirmorum |
| 100 1# - MAIN ENTRY--PERSONAL NAME |
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| Personal name |
Díaz Castillo, Esther |
| 9 (RLIN) |
3345 |
| 245 13 - TITLE STATEMENT |
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| Title |
La terapia de reemplazo enzimático en la enfermedad de Fabry / |
| Statement of responsibility, etc. |
Esther Díaz Castillo; tutor: Alicia Del Prado Díaz |
| Medium |
[Trabajo Fin de Grado] |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Date of publication, distribution, etc. |
2019 |
| 300 ## - PHYSICAL DESCRIPTION |
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| Extent |
1 archivo (pdf.) ; |
| Dimensions |
766 KB |
| 500 ## - GENERAL NOTE |
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| General note |
Trabajo fin de grado. Defendido en 2019 |
| 520 3# - SUMMARY, ETC. |
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| Summary, etc. |
Introducción: La Enfermedad de Fabry es un trastorno hereditario recesivo, ligado al cromosoma X debido a una mutación en el gen GLA. Un paciente afectado por la Enfermedad de Fabry presenta una actividad deficiente de la enzima ∝-Galactosidasa A, conllevando al almacenamiento lisosomal de globotrisosilceramida en diferentes células.<br/>Es una enfermedad multiorgánica que afecta principalmente al sistema cardiovascular y renal, aunque también puede afectar a nivel cerebrovascular, oftalmológico y gastrointestinal entre otros.<br/>El tratamiento actual es la Terapia de Reemplazo Enzimático (ERT) mediante la administración de dos proteínas recombinantes de la ∝-Galactosidasa A; agalsidasa alfa y agalsidasa beta, cuyo objetivo es reemplazar a la enzima deficiente. El objetivo principal es analizar la eficacia del tratamiento de ERT de la Enfermedad de Fabry.<br/>Material y métodos: para realizar este trabajo se llevó a cabo una revisión bibliográfica de estudios, sobre la ERT en la Enfermedad de Fabry, publicados en Pubmed, SciELO, EBSCOhost, LILACS/VHL portal regional y DIALNETplus.<br/>Resultados: se utilizaron un total de 18 artículos de los cuales cinco tratan sobre la ERT con agalsidasa beta, ocho sobre la ERT con agalsidasa alfa y cinco sobre el cambio de agalsidasa beta a agalsidasa alfa.<br/>Conclusiones: Se ha demostrado que tanto la agalsidasa beta como la agalsidasa alfa son eficaces y seguras como enzimas de la ERT. A su vez se ha observado que la agalsidasa beta es segura como tratamiento en embarazadas y la agalsidasa alfa es eficaz y segura en pacientes pediátricos. Por otro lado, se ha mostrado la eficacia y seguridad del cambio de agalsidasa beta a agalsidasa alfa. |
| 520 8# - SUMMARY, ETC. |
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| Summary, etc. |
Fabry Disease is a recessive hereditary disorder, it’s matched to X chromosome due to a mutation of GLAN gen. An FD’s patient shows a deficient activity in ∝-Galactosidase A-enzym, which leads to a lysosomal warehousing of Globotriaosylceramide in different cells.<br/>FD is a multi organic disease, it affects the cardiovascular and renal system, but it can also interfere on cerebrovascular, ophthalmologic and gastrointestinal levels.<br/>The Enzymatic Replacement Therapy (ERT) is the actual treatment, it consists in giving two recombinant proteins of the ∝-Galactosidase A: agalsidase alpha and agalsidase beta; they’re mission is to replace ∝-Galactosidase A. The objective is to analyse ERT´s efficiency in Fabry Disease.<br/>Material and methods: To make possible this project, a bibliographic review was conducted on many studies published on Pubmed, SciELO, EBSCOhost, LILACS/VHL portal regional y DIALNETplus.<br/>Results: 18 articles were chosen from the bibliographic review, it divided in five artcs about agalsidase beta, eight artcs about agalsidase alpha and other five artcs related to the exchange from agalsidase beta to alpha.<br/>Conclusions: The efficience and safety of agalsidase alpha and beta has been proved for ERT. Also, agalsidase beta has been checked as safe for pregnant women and the agalsidase alpha has been designated for children treatment as safe and efficient. And finally, the project show the effience and safety on the exchange from agalsidase beta to alpha. |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
enfermedad de Fabry |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
terapia de Reemplazo Enzimático |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
agalsidasa beta |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
agalsidasa alfa |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
tratamiento |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
enzima |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
Fabry Disease |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
enzymatic Replacement Therapy |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
agalsidase beta |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
agalsidase alpha |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
treatment |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
enzyme |
| 653 ## - INDEX TERM--UNCONTROLLED |
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| Uncontrolled term |
phenotype |
| 700 1# - ADDED ENTRY--PERSONAL NAME |
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| Personal name |
Prado Díaz, Alicia Del |
| 9 (RLIN) |
3346 |
| 710 2# - ADDED ENTRY--CORPORATE NAME |
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| Corporate name or jurisdiction name as entry element |
Universidad Pontificia de Salamanca. |
| Subordinate unit |
Facultad de Enfermería y Fisioterapia Salus Infirmorum. |
| -- |
Grado en Enfermería. |
| 9 (RLIN) |
3338 |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) |
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| Source of classification or shelving scheme |
Universal Decimal Classification |
| Koha item type |
TFG/TFM |
