renal ARPKD
Image Gallery
[ (||image_reduire{0,60}|inserer_attribut{alt,Renal ARPKD (34 weeks)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Renal ARPKD (34 weeks)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney at 21 weeks}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney at 21 weeks}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney in a neonate (1 month)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney in a neonate (1 month)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney in a neonate (1 month)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,ARPKD kidney in a neonate (1 month)}) ]
Synopsis
bilateral nephromegaly 9symetrically enlarged kidneys)
progressive dilatation of medullary collecting ducts (medullary cysts formnation) (early) then dilatation of cortical collecting ducts
- tubular cysts (diameter: 1 to 3 mm)
- radially arranged cysts
- uniform distribution
normal nephronogenesis
no hydronephrosis
- normal renal pelvis
- normal ureteres
- normal urinary bladder
preserved reniform shape of the kidneys
corticomedullary differenciation
- early preservation of corticomedullary demarcation
- late loss of corticomedullary differenciation
preservation of calyces and renal pelvis
no significant interstitial fibrosis, inflammation and obstruction
conservation of renal shape
normal glomeruli and tubules between the cysts
oligohydramnios sequence (Potter sequence)
tendency of the kidneys to collapse on sectionings as fluid drains from the cut surface
NB: All forms of renal ARPKD are associated with hepatic ARPKD (congenital hepatic fibrosis and ductal plate malformations).
Differential diagnosis
fetal cystic kidneys
- bilateral non-obstructive renal dysplasia (BNORD)
- non-syndromal BNORD
- polycystic kidney diseases (PKDs)
- autosomal dominant polycystic kidney disease (ADPKD)
See also:
multicystic kidneys/polycystic kidneys
- polycystic kidney diseases (PKDs)
- autosomal dominant polycystic kidney disease (ADPKD)
- autosomal recessive polycystic kidney disease (ARPKD)
- hepatic ARPKD
- pancreatic ARPKD
ductal plate malformations (DPM)
References
Strayer DS, Kissane JM. Dysplasia of the kidneys, liver, and pancreas: report of a variant of Ivemark’s syndrome. Hum Pathol. 1979 Mar;10(2):228-34. PMID: #422192#