Cystic Renal Disease - Kidney Disease

Cystic Renal Disease

Adult Polycystic Kidney

·        Autosomal dominant: PKD1 loci on chromosome 16 (worse), PKD2 on chromosome 4 (better)

·        1 in 500

·        Pathogenesis: 

o   Whole nephron blows up ® squashes other nephrons ® progressive renal failure

o   Cystic lesions in other organs: liver, pancreas, lung

·        Presentation: 

o   Present with hypertension around 50 ® IHD, CVA

o   Vary in severity and onset

o   Usually only moderate proteinuria

o   Kidney‟s can get very large ® impair respiration

·        Diagnose with US or CT

·        Treatment: slow progression through aggressive blood pressure control

Infantile Polycystic Kidney

·        Autosomal recessive

·        In mild forms that escape renal failure, is associated with congenital hepatic fibrosis

Cystic Renal Dysplasia

·        Due to obstruction of urinary outflow tract prior to the union of metanephric blastema and the ascending ureteral bud 

·        ® Disordered kidney development.  Contains bone, smooth muscle, etc but is not a tumour

Simple Cortical Cyst

·        Dilation of a single nephron, usually to 5 mm – 1 cm.  Most people usually have 3 or 4

·        Usually asymptomatic

·        If large and rupture ® urinary peritonitis

Other

·        Infection: Tb and hydatids can present as cystic dilation on US

·        Medullary Sponge Kidney: Rare.  Dilated collecting ducts