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ISSN 0185-3252 (Impreso)
Revista de la Asociación Médica del Centro Médico ABC

2007, Número 3

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An Med Asoc Med Hosp ABC 2007; 52 (3)


Porfirias: Consideraciones anestésicas

Fernández DPL, Esquinca CMT, Vanesa RM, Valdespin PR

Idioma: Español
Referencias bibliográficas: 83
Paginas: 130-142
Archivo PDF: 145.26 Kb.


RESUMEN

Las porfirinas juegan un papel esencial en las secuencias enzimáticas relacionadas con la formación del oxígeno, así como con su transporte, almacenamiento y consumo. Todas las formas de vida requieren de oxígeno para su supervivencia y, con este fin, adaptaron vías metabólicas para emplear la energía en procesos biológicos. Las porfirias pueden clasificarse en agudas y no agudas. En ambos casos se trata de enfermedades metabólicas causadas por una falla autonómica heredada, la cual produce defectos enzimáticos en la vía de la formación del hem. Un defecto en una de estas enzimas genera una acumulación en los sustratos precursores que anteceden a dicha falla, con lo cual se desarrolla una forma específica de porfiria aguda. El cuadro clásico de la porfiria aguda se caracteriza por dolor abdominal intenso, náusea, vómito, constipación severa y alteraciones siquiátricas y neurológicas que pueden progresar hasta una parálisis bulbar. Esta crisis es desencadenada por una serie de factores de riesgo, como son: algunos medicamentos, ingesta de alcohol, dietas hipocalóricas, ayuno, infecciones y ciclo menstrual. En esta revisión describiremos brevemente la síntesis de las porfirinas y el cuadro clínico de los pacientes porfíricos, con énfasis en las implicaciones anestésicas, particularmente el potencial peligro que representa la anestesia en términos de mayor morbilidad y mortalidad.


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