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Deficiencia familiar de lipoproteinlipasa - Overview

Nombres alternativos

Hiperlipoproteinemia tipo I; Quilomicronemia familiar

Definición:

Es un grupo de extraños trastornos genéticos en los cuales la persona carece de una proteína necesaria para descomponer las moléculas de grasa. Este trastorno hace que se acumulen grandes cantidades de grasa en la sangre.

Causas:

La causa de la deficiencia familiar de lipoproteinlipasa generalmente es un gen defectuoso que se transmite de padres a hijos.

Las personas con esta afección carecen de una enzima llamada lipoproteinlipasa, sin la cual el cuerpo no puede descomponer la grasa de los alimentos digeridos. Las partículas de grasa, llamadas quilomicrones, se acumulan en la sangre.

Los factores de riesgo abarcan antecedentes familiares de deficiencia de lipoproteinlipasa.

El trastorno afecta a 1 de cada 1,000,000 de personas. Esta enfermedad por lo general se observa primero en la lactancia o la infancia.

  • Reviewed last on: 5/29/2011
  • David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Gennest J, Libby P. Lipoprotein disorders and cardiovascular disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 47.

Semenkovich CF. Disorders of lipid metabolism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007: chap 217.

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