2012, Número 3
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Rev Mex Pediatr 2012; 79 (3)
Síndrome nefrótico asociado a mutación de la apolipoproteína-E. ¿Nueva entidad clínica?
Gordillo ARJ, Ávila CMC
Idioma: Español
Referencias bibliográficas: 31
Paginas: 137-143
Archivo PDF: 518.84 Kb.
RESUMEN
Se conoce que las mutaciones de la secuencia de los aminoácidos y de la apolipoproteína-E se expresan en la clínica como síndrome nefrótico, esta nueva entidad se ha llamado nefropatía por lipoproteína-E (NLE), la que cursa con dislipidemia severa (tipo III), edema crónico generalizado y proteinuria masiva; se trata de una enfermedad renal progresiva que conduce a los pacientes a una insuficiencia renal crónica que no responde a la terapia médica habitual; el escaso reconocimiento de esta entidad o bien debido a un diagnóstico errado hace que el médico indique al paciente medicamentos que ordinariamente emplea para el síndrome nefrótico: los corticosteroides o la ciclosporina-A, lo que agrava la dislipidemia y consecuentemente el síndrome nefrótico, ya que propicia la hipertensión arterial en los pacientes, sin obtener una respuesta terapéutica. Es así como en esta ocasión se reporta el caso de una niña con las manifestaciones de un síndrome nefrótico común, excepto por la presencia de dislipidemia tipo III (severa), con nula respuesta al tratamiento convencional, pero con buena respuesta a la dieta y a la terapia intensiva.
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