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1.
Gastroenterol Hepatol ; 45(2): 146-154, 2022 Feb.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34052403

RESUMO

Wilson's disease is a sistemic genetic disease caused by the excessive accumulation of copper. The first and main involvement is in the liver, which can range from mild and transient elevation of transaminases to the onset of an overt cirrhosis or acute liver failure. It is known that up to 20-30% of these patients may evolve to liver cirrhosis during follow-up. In clinical practice, liver fibrosis is assessed mainly by using indirect and non-invasive tools (laboratory tests, liver elastography, ultrasound), similar to other prevalent chronic liver diseases. However, despite the fact that liver elastography is a valuable tool in general hepatology, the evidence of its usefulness and accuracy in Wilsons disease is scarce. This review summarizes the available scientific data and their limitations in Wilson's disease.


Assuntos
Continuidade da Assistência ao Paciente , Degeneração Hepatolenticular/diagnóstico por imagem , Cirrose Hepática/diagnóstico por imagem , Técnicas de Imagem por Elasticidade , Seguimentos , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/enzimologia , Degeneração Hepatolenticular/terapia , Humanos , Fígado/diagnóstico por imagem , Fígado/enzimologia , Cirrose Hepática/etiologia , Cooperação do Paciente
2.
Gastroenterol. hepatol. (Ed. impr.) ; 45(2): 146-154, Feb. 2022. tab
Artigo em Espanhol | IBECS (Espanha) | ID: ibc-204147

RESUMO

La enfermedad de Wilson es una patología genética multiorgánica causada por la acumulación excesiva de cobre en el organismo. La afectación hepática es la inicial y principal, y puede suponer desde una hepatitis leve y transitoria, hasta el debut en forma de cirrosis o insuficiencia hepática aguda grave. En el seguimiento, hasta un 20-30% de estos pacientes evolucionan a cirrosis hepática. En la práctica clínica, la monitorización de la fibrosis hepática se realiza mayoritariamente mediante métodos indirectos y no invasivos (analíticas, elastografía hepática, ecografías) a semejanza de otras hepatopatías crónicas más prevalentes. No obstante, a pesar de que la elastografía constituye una herramienta de gran valor, la evidencia de su utilidad en Wilson es muy limitada. En esta revisión se repasa la información disponible y sus limitaciones para el seguimiento de la enfermedad de Wilson.


Wilson's disease is a sistemic genetic disease caused by the excessive accumulation of copper. The first and main involvement is in the liver, which can range from mild and transient elevation of transaminases to the onset of an overt cirrhosis or acute liver failure. It is known that up to 20-30% of these patients may evolve to liver cirrhosis during follow-up. In clinical practice, liver fibrosis is assessed mainly by using indirect and non-invasive tools (laboratory tests, liver elastography, ultrasound), similar to other prevalent chronic liver diseases. However, despite the fact that liver elastography is a valuable tool in general hepatology, the evidence of its usefulness and accuracy in Wilsońs disease is scarce. This review summarizes the available scientific data and their limitations in Wilson's disease.


Assuntos
Humanos , Degeneração Hepatolenticular , Seguimentos , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/diagnóstico por imagem , Hepatite , Cirrose Hepática , Técnicas de Imagem por Elasticidade , Fibrose , Gastroenterologia , Pacientes Internados
3.
Rev. méd. hered ; 27(4): 223-229, oct.-dic. 2016. tab, graf
Artigo em Espanhol | LILACS, LIPECS | ID: biblio-836254

RESUMO

Objetivos: Estudiar la asociación entre el nivel de albúmina sérica y las alteraciones de los electrolitos, gasessanguíneos y compuestos nitrogenados en adultos incidentes del servicio de emergencia de un hospital general.Material y métodos: Se incluyeron 275 pacientes que acudieron a la emergencia del Hospital Cayetano Herediaen Lima, Perú entre el 2013 y el 2015 a quienes el médico tratante solicitó al ingreso electrolitos séricos, gasessanguíneos y albúmina sérica. Se contrastó la albúmina como variable ordinal contra los electrolitos, gases arteriales,urea, calcio y fósforo séricos. También se analizó mediante análisis bivariado la albúmina normal (>3,5 g/dl) y lamuy baja (<2,5 g/dl) contra la variables antes mencionadas y la existencia de correlación lineal entre los valoresnuméricos continuos de las diferentes variables con la albúmina sérica...


Objectives: To evaluate the association between serum albumin and abnormalities in serum electrolytes, bloodgases and nitrogen compounds in adult patients attending an emergency room of a general hospital. Methods: 275patients who attended the emergency room of Hospital Cayetano Heredia en Lima, Peru between 2013 and 2015were included in the study. Serum albumin (ordinal values) was contrasted with serum electrolytes, blood gases,urea, and serum calcium and phosphorus. Normal albumin (>3.5 g/dl) and low albumin (<2.5 g/dl) were contrastedwith the above-mentioned variables using bivariate analysis, and numeric values of these variables were correlatedwith serum albumin with lineal correlation...


Assuntos
Humanos , Masculino , Adulto , Feminino , Adulto Jovem , Pessoa de Meia-Idade , Albumina Sérica , Eletrólitos/efeitos adversos , Hipoalbuminemia , Hipocalcemia , Uremia , Estudo Observacional
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