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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]]. |
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| ===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}} |
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| ==Autosomal dominant polycystic kidney disease== | | ==Autosomal dominant polycystic kidney disease== |
| *Abbreviated ''ADPKD''. | | *Abbreviated ''ADPKD''. |
| ===General===
| | {{Main|Autosomal dominant polycystic kidney disease}} |
| ====Etiology====
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| *Mutation in ''PKD1'' gene or ''PKD2'' gene.
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| *Is classified in a large group of diseases - ''ciliopathies''.
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| PKD1 related disease:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref>
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| *Encodes polycystin.
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| *Death at ~53 years.
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| *Assoc. with cerebral aneurysms.
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| PKD2 related disease:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref>
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| *Death at ~69 years.
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| *Associated with colonic diverticula, [[aortic aneurysm]], [[mitral valve prolapse]].
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| ====Liver cysts and PKD====
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| =====General=====
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| Features:
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| *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>
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| **Age dependence:
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| ***10-17% <40 years old have liver cysts.
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| ***70-75% >60 years old have liver cysts.
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| **Renal function:
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| ***60-70% of patients with [[end-stage renal disease]] (ESRD) and near-ESRD.
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| **Females more often affected.
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| *Hepatic function usu. preserved.
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| Complications:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref>
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| #Infected cyst.
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| #[[Cholangiocarcinoma]].
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| =====Microscopic=====
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| Features:
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| *''Von Meyenburg complexes'':
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| **Cluster of dilated ducts with "altered" bile.
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| **Surrounded by collagenous stroma.
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| See: ''[[Medical liver disease]]''.
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| ===Gross===
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| Features:
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| *Thin walled cysts.
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| **Number of cysts:
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| ***If you can count 'em it favours acquired renal cystic disease... if you can't it favours the genetic condition.
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| ===Microscopic===
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| Features:<ref name=Ref_DARP426>{{Ref DARP|426}}</ref>
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| *Cysts lined by simple flattened epithelium.
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| *Normal renal tubules interspersed between cysts.
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| *+/-Fibrosis (late-stage).
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| DDx:
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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>
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| **Morphologically similar to acquired renal cystic disease.<ref name=rj_oct2010>RJ. 20 October 2010.</ref>
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| **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>
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| ==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.
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| *Presence of cysts dependent on duration of dialysis:<ref name=pmid7861721/>
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| **< 3 years ~44%.
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| **>4 years ~80%.
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| **>10 years ~90%.
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| *Associated with [[papillary renal cell carcinoma]].<ref>{{Ref DARP|438}}</ref>
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| ===Microscopic===
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| 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>
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| *Cysts - location: cortex and medulla.
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| **Lined by simple flattened epithelium.
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| DDx:
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| *[[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]].
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| *[[Acquired cystic disease-associated renal cell carcinoma]].
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| ==Autosomal recessive polycystic kidney disease== | | ==Autosomal recessive polycystic kidney disease== |
| *Abbreviated ''ARPKD''. | | *Abbreviated ''ARPKD''. |
| ===General===
| | {{Main|Autosomal recessive polycystic kidney disease}} |
| *Uncommon.
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| *Homogenous for mutated ''PKHD1 gene'' (polycystic kidney and hepatic disease).<ref name=omim263200>{{OMIM|263200}}</ref>
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| **The same gene is implicated in [[Caroli disease]].
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| *Associated with congenital hepatic fibrosis
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| Subdivided into:<ref name=Ref_Klatt235>{{Ref Klatt|235}}</ref>
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| *Neonatal.
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| *Infantile.
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| *Juvenile.
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| ===Gross===
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| Features:<ref name=Ref_Klatt235>{{Ref Klatt|235}}</ref>
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| *Marked bilateral enlargement - may almost fill the abdomen.
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| *Smooth cortical surface.
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| *Poorly demarcated corticomedullary junction.
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| DDx:
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| *[[Multicystic renal dysplasia]] - has larger variability of cyst size.
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| Images:
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| *[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>
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| *[http://radiology.uchc.edu/eAtlas/GU/529.htm ARPKD (radiology.uchc.edu)].
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| ===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.
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| =See also= | | =See also= |
| *[[Medical kidney diseases]]. | | *[[Medical kidney diseases]]. |
| *[[Kidney tumours]]. | | *[[Kidney tumours]]. |
| | *[[Bosniak classification]]. |
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| =References= | | =References= |
