POLYCYSTIC KIDNEY DISEASE OF CHILDHOOD

Course overview

THERE ARE TWO INHERITED VARIETIES OF POLYCYSTIC KIDNEY DISEASE (PKD): AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD) AND AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE (ARPKD). THESE TWO FORMS HAVE DISTINCT CLINICAL AND GENETIC FEATURES. ADPKD IS A MULTISYSTEM PROGRESSIVE CYSTIC DISORDER CHARACTERIZED BY BILATERAL RENAL CYSTS ARISING ANYWHERE ALONG THE NEPHRON AND LEADING TO PROGRESSIVELY WORSENING FIBROSIS AND ARCHITECTURAL DISTORTION OF KIDNEYS AND ULTIMATELY RENAL FAILURE. ARPKD INVOLVES MICROCYSTIC CYSTS THAT THAT ARISE EXCLUSIVELY IN THE COLLECTING TUBULE AND CAN LEAD TO HEPATIC FIBROSIS AND CONSEQUENTLY, MANIFESTATIONS OF PORTAL HYPERTENSION. THIS ACTIVITY REVIEWS THE EVALUATION, TREATMENT, AND COMPLICATIONS OF PKD AND THE IMPORTANCE OF AN INTERPROFESSIONAL TEAM APPROACH TO ITS MANAGEMENT.