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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==Overview==
==Overview==
*Autosomal dominant polycystic kidney disease (ADPKD).
===Adult===
*Adult-onset medullary cystic disease.
*[[Autosomal dominant polycystic kidney disease]] (ADPKD).
*Acquired renal cystic disease.
*[[Adult-onset medullary cystic disease]].
*Autosomal recessive polycystic kidney disease (ARPKD).
*[[Acquired renal cystic disease]].
*Cystic [[renal cell carcinoma]].
*[[Localized cystic disease of the kidney]].
 
===Pediatric===
*[[Autosomal recessive polycystic kidney disease]] (ARPKD).
*Medullary sponge kidney.
*Medullary sponge kidney.
*Nephronophthisis.
*Nephronophthisis.
*Cystic [[renal cell carcinoma]].


=Specific diseases=
=Specific diseases=
==Autosomal dominant polycystic kidney disease (ADPKD)==
==Benign cortical cyst of the kidney==
===General===
*[[AKA]] ''benign cortical cyst''.
====Etiology====
{{Main|Benign cortical cyst of the kidney}}
*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:
==Autosomal dominant polycystic kidney disease==
*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>
*Abbreviated ''ADPKD''.
**Morphologically similar to acquired renal cystic disease.<ref>RJ. 20 October 2010.</ref>
{{Main|Autosomal dominant polycystic kidney disease}}


==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 [[renal cell carcinoma]] (papillary subtype).<ref>{{Ref DARP|438}}</ref>


===Microscopic===
==Autosomal recessive polycystic kidney disease==
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>
*Abbreviated ''ARPKD''.
*Cysts - location: cortex and medulla.
{{Main|Autosomal recessive polycystic kidney disease}}
**Lined by simple flattened epithelium.


==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|Localized cystic disease of the kidney}}


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


=References=
=References=
Michael
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