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Síndrome de Alport - Overview

Nombres alternativos

Hematuria, nefropatía y sordera; Nefritis hereditaria; Sordera hereditaria y nefropatía; Nefritis hemorrágica familiar

Definición:

Es un trastorno hereditario que causa daño a los diminutos vasos sanguíneos en los riñones.

Causas, incidencia y factores de riesgo:

El síndrome de Alport es una forma hereditaria de inflamación del riñón (nefritis) y es causado por una mutación en un gen para una proteína en el tejido conectivo, llamada colágeno.

El trastorno es poco común y afecta con mayor frecuencia a los hombres. Las mujeres pueden transmitir el gen del trastorno a sus hijos incluso si no tienen síntomas.

Entre los factores de riesgo están:

Enfermedad renal terminal en parientes masculinos
Antecedente familiar del síndrome de Alport
Glomerulonefritis
Hipoacusia antes de los 30 años
Nefritis

Sistema urinario masculino

Reviewed last on: 11/30/2009
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Herbert Y. Lin, MD, PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.

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