Difference between revisions of "Cystic kidney diseases"

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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===
*Autosomal recessive polycystic kidney disease (ARPKD).
*[[Autosomal recessive polycystic kidney disease]] (ARPKD).
*Medullary sponge kidney.
*Medullary sponge kidney.
*Nephronophthisis.
*Nephronophthisis.


=Specific diseases=
==Benign cortical cyst of the kidney==
*[[AKA]] ''benign cortical cyst''.
{{Main|Benign cortical cyst of the kidney}}


=Specific diseases=
==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>
 
===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]].


==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]].


=References=
=References=

Latest revision as of 14:43, 21 June 2016

Polycystic kidneys. (WC/CDC)

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

Pediatric

Specific diseases

Benign cortical cyst of the kidney

  • AKA benign cortical cyst.
Main article: Benign cortical cyst of the kidney

Autosomal dominant polycystic kidney disease

  • Abbreviated ADPKD.
Main article: Autosomal dominant polycystic kidney disease

Acquired renal cystic disease

Main article: Acquired cystic disease of the kidney

Autosomal recessive polycystic kidney disease

  • Abbreviated ARPKD.
Main article: Autosomal recessive polycystic kidney disease

Localized cystic disease of the kidney

  • AKA segmental cystic disease of the kidney.
  • AKA unilateral cystic disease of the kidney.
  • AKA benign multilocular cyst of the kidney.
Main article: Localized cystic disease of the kidney

See also

References

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