Pediatric Radiology > Genitorinary > Cystic Renal Disease > Autosomal Recessive Polycystic Kidney Disease

Autosomal Recessive Polycystic Kidney Disease

Autosomal recessive polycystic kidney (ARPKD) disease is inherited in a recessive fashion with varying degrees of clinical presentations. There is medullary ductal ectasia and loss of renal function. Most children will die shortly after birth. US demonstrates renal enlargement and increased renal echogenicity. No macroscopic cysts are seen, in contradistinction to autosomal dominant polycystic disease. ARPKD is also associated with periportal fibrosis. The degree of periportal fibrosis is inversely proportional to the degree of renal involvement. ARPKD can be classified into perinatal, neonatal, infantile, and juvenile forms.
Renal enlargement and increased renal echogenicity	Renal enlargement and increased renal echogenicity