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Line 566: |
Line 566: |
| *Usually associated with kappa clone. | | *Usually associated with kappa clone. |
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| ==Polycystic kidney disease== | | ==Cystic kidney diseases== |
| Types:
| | {{Main|Cystic kidney diseases}} |
| *Adult onset - autosomal dominant polycystic kidney disease (ADPKD).
| | These are discussed in a separate article and include: |
| *Childhood onset - autosomal recessive polycystic kidney disease (ARPKD).
| | *Autosomal dominant polycystic kidney disease (ADPKD). |
| | | *Adult-onset medullary cystic disease. |
| ==Autosomal dominant polycystic kidney disease (ADPKD)==
| | *Acquired renal cystic disease. |
| ===General===
| | *Autosomal recessive polycystic kidney disease (ARPKD). |
| ====Etiology====
| | *Medullary sponge kidney. |
| *Mutation in ''PKD1'' gene or ''PKD2'' gene.
| | *Nephronophthisis. |
| *Is classified in a large group of diseases - ''ciliopathies''.
| | *Cystic [[renal cell carcinoma]]. |
| | |
| PKD1 related disease:<ref name=Ref_MacSween174-5>{{Ref MacSween|174-5}}</ref>
| |
| *Encodes polycystin.
| |
| *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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| *Assoc. with colonic diverticula, aortic aneurysm, mitral valve prolapse.
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| | |
| ====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:
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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. | |
| *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.
| |
| #Cholangiocarcinoma.
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| | |
| =====Microscopic=====
| |
| Features:
| |
| *''Von Meyenburg complexes'':
| |
| **Cluster of dilated ducts with "altered" bile.
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| **Surrounded by collagenous stroma.
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| | |
| See: ''[[Medical liver disease]]''.
| |
| | |
| ===Gross===
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| Features:
| |
| *Thin walled cysts.
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| **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.
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| *+/-Fibrosis (late-stage).
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| | |
| 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>RJ. 20 October 2010.</ref>
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| | |
| ==Acquired renal cystic disease==
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| ===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%.
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| **>4 years ~80%.
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| **>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.
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| ==Transplant== | | ==Transplant== |