La insuficiencia intestinal (II) exige el uso de nutrición parenteral (NP). Entre las causas de II prolongada grave destacan el síndrome del intestino corto, trastornos de la motilidad grave (aganglionosis total o subtotal o síndrome de seudoobstrucción intestinal crónica) y enfermedades congénitas del desarrollo de los enterocitos. Puede aparecer insuficiencia hepática grave en pacientes con II como consecuencia de una enfermedad digestiva subyacente y una NP no adaptada. La sepsis relacionada con el catéter y/o la trombosis vascular extensa pueden impedir el mantenimiento de una NP segura y eficiente. Por tanto, el tratamiento de pacientes con II exige la identificación precoz del proceso y el análisis de su riego de irreversibilidad. La cronología de la derivación para trasplante intestinal continúa siendo una cuestión crucial. En consecuencia, la asistencia debe incluir tratamientos adaptados a cada una de las etapas de la II, basándose en la puesta en práctica de un enfoque multidisciplinario en centros que cuenten con gastroenterología pediátrica, experiencia en NP, programa de NP domiciliaria, cirugía pediátrica y programa de trasplantes de hígado e intestino.

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