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Cystic kidney diseases (view source)

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[[Image:Polycystic kidneys, gross pathology 20G0027 lores.jpg|thumb|Polycystic kidneys. (WC/CDC)]]
'''Cystic kidney diseases''', also '''cystic renal diseases''', are a group of [[medical kidney diseases]] characterized by multiple cysts.   
'''Cystic kidney diseases''', also '''cystic renal diseases''', are a group of [[medical kidney diseases]] characterized by multiple cysts.   


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*[[Acquired renal cystic disease]].
*[[Acquired renal cystic disease]].
*Cystic [[renal cell carcinoma]].
*Cystic [[renal cell carcinoma]].
*[[Localized cystic disease of the kidney]].


===Pediatric===
===Pediatric===
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=Specific diseases=
=Specific diseases=
==Benign cortical cyst of the kidney==
*[[AKA]] ''benign cortical cyst''.
{{Main|Benign cortical cyst of the kidney}}
==Autosomal dominant polycystic kidney disease==
==Autosomal dominant polycystic kidney disease==
*Abbreviated ''ADPKD''.
*Abbreviated ''ADPKD''.
===General===
{{Main|Autosomal dominant polycystic kidney disease}}
====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.
*Associated 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==
==Acquired renal cystic disease==
===General===
{{Main|Acquired cystic disease of the kidney}}
*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:
*[[Papillary renal cell carcinoma]].<ref>{{Ref DARP|438}}</ref>
*[[Acquired cystic disease-associated renal cell carcinoma]].
 
===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.
 
DDx:
*[[Autosomal dominant polycystic kidney 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> see ''Microscopic'' in [[ADPKD]].
*[[Acquired cystic disease-associated renal cell carcinoma]].


==Autosomal recessive polycystic kidney disease==
==Autosomal recessive polycystic kidney disease==
*Abbreviated ''ARPKD''.
*Abbreviated ''ARPKD''.
===General===
{{Main|Autosomal recessive polycystic kidney disease}}
*Uncommon.
*Homogenous for mutated ''PKHD1 gene'' (polycystic kidney and hepatic disease).<ref name=omim263200>{{OMIM|263200}}</ref>
**The same gene is implicated in [[Caroli disease]].
*Associated with congenital hepatic fibrosis
 
Subdivided into:<ref name=Ref_Klatt235>{{Ref Klatt|235}}</ref>
*Neonatal.
*Infantile.
*Juvenile.
 
===Gross===
Features:<ref name=Ref_Klatt235>{{Ref Klatt|235}}</ref>
*Marked bilateral enlargement - may almost fill the abdomen.
*Smooth cortical surface.
*Poorly demarcated corticomedullary junction.
 
DDx:
*[[Multicystic renal dysplasia]] - has larger variability of cyst size.
 
Images:
*[http://radiographics.rsna.org/content/20/3/837/F18.expansion ARPKD (radiographics.rsna.org)].<ref name=pmid10835131>{{Cite journal  | last1 = Lonergan | first1 = GJ. | last2 = Rice | first2 = RR. | last3 = Suarez | first3 = ES. | title = Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. | journal = Radiographics | volume = 20 | issue = 3 | pages = 837-55 | month =  | year =  | doi =  | PMID = 10835131 }}</ref>
*[http://radiology.uchc.edu/eAtlas/GU/529.htm ARPKD (radiology.uchc.edu)].


===Microscopic===
==Localized cystic disease of the kidney==
Features:<ref name=Ref_Klatt236>{{Ref Klatt|236}}</ref>
*[[AKA]] ''segmental cystic disease of the kidney''.
*Numerous cysts of the collecting ducts.
*[[AKA]] ''unilateral cystic disease of the kidney''.
**Typically radially arranged.
*[[AKA]] ''benign multilocular cyst of the kidney''.
**Lined by cuboidal cells.
{{Main|Localized cystic disease of the kidney}}
*Abnormally low number of glomeruli.


=See also=
=See also=
*[[Medical kidney diseases]].
*[[Medical kidney diseases]].
*[[Kidney tumours]].
*[[Kidney tumours]].
*[[Bosniak classification]].
*[[Multicystic dysplastic kidney]].


=References=
=References=
Michael
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