View source for Cystic kidney diseases

'''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:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref> 
*Encodes polycystin.
*Death at ~53 years.
*Assoc. with cerebral aneurysms.

PKD2 related disease:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref> 
*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:<ref name=pmid9186898>{{cite journal |author=Perrone RD |title=Extrarenal manifestations of ADPKD |journal=Kidney Int. |volume=51 |issue=6 |pages=2022–36 |year=1997 |month=June |pmid=9186898 |doi= |url=http://www.nature.com/ki/journal/v51/n6/pdf/ki1997276a.pdf}}</ref>
**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:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref>
#Infected cyst.
#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:<ref name=Ref_DARP426>{{Ref DARP|426}}</ref>
*Cysts lined by simple flattened epithelium.
*Normal renal tubules interspersed between cysts.
*+/-Fibrosis (late-stage).

DDx: 
*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 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 name=rj_oct2010>RJ. 20 October 2010.</ref><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= 87 |edition=2nd |isbn=978-0865774933 |oclc= |doi= |accessdate=}}</ref>

==Acquired renal cystic disease==
===General===
*Thought to arise due to uremia,<ref name=pmid7861721>{{cite journal |author=Fick GM, Gabow PA |title=Hereditary and acquired cystic disease of the kidney |journal=Kidney Int. |volume=46 |issue=4 |pages=951–64 |year=1994 |month=October |pmid=7861721 |doi= |url=http://www.nature.com/ki/journal/v46/n4/pdf/ki1994354a.pdf}}</ref> not specific to the type of renal dialysis, i.e. hemodialysis vs. peritoneal dialysis.
*Presence of cysts dependent on duration of dialysis:<ref name=pmid7861721/>
**< 3 years ~44%.
**>4 years ~80%.
**>10 years ~90%.
*Associated with [[renal cell carcinoma]] (papillary subtype).<ref>{{Ref DARP|438}}</ref>

===Microscopic===
Features:<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>
*Cysts - location: cortex and medulla.
**Lined by simple flattened epithelium.

=See also=
*[[Medical kidney diseases]].
*[[Kidney tumours]].

=References=
{{Reflist|2}}

[[Category:Genitourinary pathology]]