Difference between revisions of "Cystic kidney diseases"

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*Acquired renal cystic disease - rarely.<ref name=pmid1819219>{{cite journal |author=Kessler M, Testevuide P, Aymard B, Huu TC |title=Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis |journal=Am. J. Nephrol. |volume=11 |issue=6 |pages=513–7 |year=1991 |pmid=1819219 |doi= |url=}}</ref>
*Acquired renal cystic disease - rarely.<ref name=pmid1819219>{{cite journal |author=Kessler M, Testevuide P, Aymard B, Huu TC |title=Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis |journal=Am. J. Nephrol. |volume=11 |issue=6 |pages=513–7 |year=1991 |pmid=1819219 |doi= |url=}}</ref>
**Morphologically similar to acquired renal cystic disease.<ref>RJ. 20 October 2010.</ref>
**Morphologically similar to acquired renal cystic disease.<ref name=rj_oct2010>RJ. 20 October 2010.</ref>
**It is said that "... if you can count 'em it's simple cysts; if you can't it's polycystic (renal disease)."<ref>{{cite book |author= Barbaric, Zoran L.  |title=[http://books.google.com/books?id=uABgezxjhYwC&pg=PA87&lpg=PA87&dq=cystic+renal+disease,+%22if+you+can+count+them%22&source=bl&ots=GYyIdR9YmS&sig=DBYzT8LO2MltLA-wwW-0QwnQ6Lk&hl=en&ei=0hF-Taz0IsG3twe5yfG6BQ&sa=X&oi=book_result&ct=result&resnum=1&ved=0CBoQ6AEwAA#v=onepage&q=cystic%20renal%20disease%2C%20%22if%20you%20can%20count%20them%22&f=false Principles of Genitourinary Radiology] |publisher=Thieme |location= |year=1994 |pages= {{{1|}}} |edition=2nd |isbn=978-0865774933 |oclc= |doi= |accessdate=}}</ref><ref name=rj_oct2010>RJ. 20 October 2010.</ref>


==Acquired renal cystic disease==
==Acquired renal cystic disease==

Revision as of 13:07, 14 March 2011

Cystic kidney diseases, also cystic renal diseases, are a group of medical kidney diseases characterized by multiple cysts.

Renal neoplasms, e.g. renal cell carcinoma, may be cystic. They are dealt with in kidney tumours.

Overview

Adult

  • Autosomal dominant polycystic kidney disease (ADPKD).
  • Adult-onset medullary cystic disease.
  • Acquired renal cystic disease.
  • Cystic renal cell carcinoma.

Pediatric

  • Autosomal recessive polycystic kidney disease (ARPKD).
  • Medullary sponge kidney.
  • Nephronophthisis.


Specific diseases

Autosomal dominant polycystic kidney disease

  • Abbreviated ADPKD.

General

Etiology

  • Mutation in PKD1 gene or PKD2 gene.
  • Is classified in a large group of diseases - ciliopathies.

PKD1 related disease:[1]

  • Encodes polycystin.
  • Death at ~53 years.
  • Assoc. with cerebral aneurysms.

PKD2 related disease:[1]

  • Death at ~69 years.
  • Assoc. with colonic diverticula, aortic aneurysm, mitral valve prolapse.

Liver cysts and PKD

General

Features:

  • Most common extra-renal manifestation of PKD; dependent on age, sex and renal function:[2]
    • Age dependence:
      • 10-17% <40 years old have liver cysts.
      • 70-75% >60 years old have liver cysts.
    • Renal function:
      • 60-70% of patients with end-stage renal disease (ESRD) and near-ESRD.
    • Females more often affected.
  • Hepatic function usu. preserved.

Complications:[1]

  1. Infected cyst.
  2. Cholangiocarcinoma.
Microscopic

Features:

  • Von Meyenburg complexes:
    • Cluster of dilated ducts with "altered" bile.
    • Surrounded by collagenous stroma.

See: Medical liver disease.

Gross

Features:

  • Thin walled cysts.
    • Number of cysts:
      • If you can count 'em it favours acquired renal cystic disease... if you can't it favours the genetic condition.

Microscopic

Features:[3]

  • Cysts lined by simple flattened epithelium.
  • Normal renal tubules interspersed between cysts.
  • +/-Fibrosis (late-stage).

DDx:

  • Acquired renal cystic disease - rarely.[4]
    • Morphologically similar to acquired renal cystic disease.[5]
    • It is said that "... if you can count 'em it's simple cysts; if you can't it's polycystic (renal disease)."[6][5]

Acquired renal cystic disease

General

  • Thought to arise due to uremia,[7] not specific to the type of renal dialysis, i.e. hemodialysis vs. peritoneal dialysis.
  • Presence of cysts dependent on duration of dialysis:[7]
    • < 3 years ~44%.
    • >4 years ~80%.
    • >10 years ~90%.
  • Associated with renal cell carcinoma (papillary subtype).[8]

Microscopic

Features:[4]

  • Cysts - location: cortex and medulla.
    • Lined by simple flattened epithelium.

See also

References

  1. ↑ 1.0 1.1 1.2 Burt, Alastair D.;Portmann, Bernard C.;Ferrell, Linda D. (2006). MacSween's Pathology of the Liver (5th ed.). Churchill Livingstone. pp. 174-5. ISBN 978-0-443-10012-3.
  2. ↑ Perrone RD (June 1997). "Extrarenal manifestations of ADPKD". Kidney Int. 51 (6): 2022–36. PMID 9186898. http://www.nature.com/ki/journal/v51/n6/pdf/ki1997276a.pdf.
  3. ↑ Fogo, Agnes B.; Kashgarian, Michael (2005). Diagnostic Atlas of Renal Pathology: A Companion to Brenner and Rector's The Kidney 7E (1st ed.). Saunders. pp. 426. ISBN 978-1416028710.
  4. ↑ 4.0 4.1 Kessler M, Testevuide P, Aymard B, Huu TC (1991). "Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis". Am. J. Nephrol. 11 (6): 513–7. PMID 1819219.
  5. ↑ 5.0 5.1 RJ. 20 October 2010.
  6. ↑ Barbaric, Zoran L. (1994). Principles of Genitourinary Radiology (2nd ed.). Thieme. ISBN 978-0865774933.
  7. ↑ 7.0 7.1 Fick GM, Gabow PA (October 1994). "Hereditary and acquired cystic disease of the kidney". Kidney Int. 46 (4): 951–64. PMID 7861721. http://www.nature.com/ki/journal/v46/n4/pdf/ki1994354a.pdf.
  8. ↑ Fogo, Agnes B.; Kashgarian, Michael (2005). Diagnostic Atlas of Renal Pathology: A Companion to Brenner and Rector's The Kidney 7E (1st ed.). Saunders. pp. 438. ISBN 978-1416028710.
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