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renal ARPKD

 

Synopsis

-  bilateral nephromegaly 9symetrically enlarged kidneys)
-  progressive dilatation of medullary collecting ducts (medullary cysts formnation) (early) then dilatation of cortical collecting ducts

-  normal nephronogenesis
-  no hydronephrosis

-  preservation of calyces
-  no significant interstitial fibrosis, inflammation and obstruction
-  conservation of renal shape
-  normal glomeruli and tubules between the cysts
-  loss of corticomedullary differenciation
-  oligohydramnios sequence (Potter sequence)

NB: All forms of renal ARPKD are associated with hepatic ARPKD (congenital hepatic fibrosis and ductal plate malformations).

See also:

-  multicystic kidneys/polycystic kidneys

-  ductal plate malformations (DPM)


Renal ARPKD (34 weeks) - Note the associate congenital hepatic fibrosis (ductal plate malformation).

Renal ARPKD (34 weeks)

ARPKD kidney at 21 weeks - Slight multicystic appearance

ARPKD kidney at 21 weeks - Slight multicystic appearance

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