Difference between revisions of "Cystic kidney diseases"

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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.
*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===
Features:<ref name=Ref_Klatt236>{{Ref Klatt|236}}</ref>
*Numerous cysts of the collecting ducts.
**Typically radially arranged.
**Lined by cuboidal cells.
*Abnormally low number of glomeruli.


=See also=
=See also=
Retrieved from "https://librepathology.org/wiki/Cystic_kidney_diseases"