Renal cystic diseases

Renal cystic diseases

A. INHERITED

•     Autosomal-recessive polycystic disease(ARPKD)
•     Autosomal-dominant polycystic disease(ADPKD)
•     Glomerulocystic kidney disease
•     Medullary cystic disease

B.NON-GENETIC

•  Renal dysplasia

                  Multicystic dysplastic disease
                   Obstuctive cystic dysplasia

•  Nondysplastic nongenetic cysts

                  Simple cysts
                  Multilocular cysts
                 Medullary sponge kidney

• The most common cause of noninherited renal cysts ------parenchymal dysplasia
• The most common cause of inherited cystic disease------- Autosomal-dominant polycystic disease(1 in 800 live birth)

ARPKD  

• Marked elongation of the collecting tubules that expand into multiple tubules that expand into multiple small cysts.Outer cotex is spared because it does not contain tubules
• Markedly enlarged hyperechoic kidneys without CMD
• Cortical rim sign (a hypoechoic outer cortical rim)
• Reversed CMD
• oligohydramnios

ADPKD

• Presence of multiple cysts that develop on the wall of collecting tubules and nephrons
• Cysts are scattered within the cortex and medulla
• Typically identified in early adulthood
• Moderately enlarged kidneys with hyperechoic cortices and relatively hypoechoic medullae
• normal amniotic fluid