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renal ARPKD

Monday 6 December 2004

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

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

Portfolio

  • Renal ARPKD (34 weeks)
  • Renal ARPKD (34 weeks)
  • ARPKD kidney at 21 weeks
  • ARPKD kidney at 21 weeks
  • ARPKD kidney in a neonate (1 month)
  • ARPKD kidney in a neonate (1 month)
  • ARPKD kidney in a neonate (1 month)
  • ARPKD kidney in a neonate (1 month)