Acidosis propiónica, enfermedad metabólica rara con manejo anestésico peculiar.

  • Jesús David Rubio López Residente Anestesiología y Reanimación del H.U. Reina Sofía de Córdoba
  • Rafael Muñoz Martínez FEA Anestesiología y Reanimación del H.U. Reina Sofía de Córdoba.
Palabras clave: Acidosis propiónica, Propionic Acidemia, anesthesia, anestesia

Resumen

La Acidosis Propiónica es una enfermedad metabólica mitocondrial que afecta al catabolismo de los ácidos grasos, colesterol y los aminoácidos de cadena ramificada a causa de un déficit enzimático. No existe una correlación del genotipo con la severidad de la enfermedad. Es considerada como una enfermedad rara, aunque su incidencia es variable, y dentro de los errores del metabolismo es de los más frecuentes. En el contexto de un paciente con esta patología que va a requerir ser anestesiado en varias ocasiones procedemos a la revisión de la fisiopatología de la enfermedad, sus complicaciones e investigar en las implicaciones anestésicas del manejo anestésico futuro.

ABSTRACT

Propionic acidemia, unusual metabolic disease with special anesthesic work.

Propionic Acidemia is a mitochondrial metabolic disease that affects the catabolism of fatty acids, cholesterol and branched chain amino acids due to an enzymatic deficit. There is no correlation of the genotype with the severity of the disease. It is considered a rare disease, although its incidence is variable, and within the errors of metabolism is the most frequent. In the context of a patient with this pathology who needs to be anesthetized several times, we proceed to review the pathophysiology of the disease, its complications and investigate the anesthetic implications of future anesthetic management.

Citas

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Publicado
01-06-2020
Cómo citar
Rubio López, J. D., & Muñoz Martínez, R. (2020). Acidosis propiónica, enfermedad metabólica rara con manejo anestésico peculiar. Revista Electrónica AnestesiaR, 12(5), 3. https://doi.org/https://doi.org/10.30445/rear.v12i5.822