Difference between revisions of "Medical kidney diseases"

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Medical kidney diseases (view source)

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[[Image:Acute thrombotic microangiopathy - pas - very high mag.jpg|thumb|[[Micrograph]] showing a glomerulus with an [[acute thrombotic microangiopathy]]. [[PAS stain]].]]  
[[Image:Acute thrombotic microangiopathy - pas - very high mag.jpg|250px|thumb|[[Micrograph]] showing a glomerulus with an [[thrombotic microangiopathy|acute thrombotic microangiopathy]]. [[PAS stain]].]]  
This article describes '''medical renal disease''' or the '''medical kidney'''.  Much in medical kidney depends on the clinical information.  Most of the disease seen by pathologists is... glomerular disease. It is one of the few areas that routinely requires [[electron microscopy]].
This article describes '''medical renal disease''' or the '''medical kidney'''.  Much in medical kidney depends on the clinical information.  Most of the disease seen by pathologists is... glomerular disease. It is one of the few areas that routinely requires [[electron microscopy]].
   
   
Medical kidney is niche area in pathology. In the context of exams for general pathologists, if in doubt... the answer to most questions is ''[[diabetes mellitus]]'' or ''[[systemic lupus erythematosus]]''.   
Medical kidney is niche area in pathology. In the context of exams for general pathologists, if in doubt... the answer to most questions is ''[[diabetes mellitus]]'' or ''[[systemic lupus erythematosus]]''.   


Kidney tumours are dealt with in the ''[[kidney tumours]]'' article, and ''[[pediatric kidney tumours]]'' article.
Kidney tumours are dealt with in the ''[[kidney tumours]]'' article, and ''[[pediatric kidney tumours]]'' article.  


=Clinical=
=Clinical=
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**Previously p-ANCA.
**Previously p-ANCA.
**Seen in ANCA-[[vasculitides]], esp. [[microscopic polyangiitis]].  
**Seen in ANCA-[[vasculitides]], esp. [[microscopic polyangiitis]].  
*PR3-ANPA (proteinase 3 antineutrophil cytoplasmic autoantibody).  
*PR3-ANCA (proteinase 3 antineutrophil cytoplasmic autoantibody).  
**Previously c-ANCA.
**Previously c-ANCA.
**Seen in ANCA-[[vasculitides]], esp. [[Wegener granulomatosis]].
**Seen in ANCA-[[vasculitides]], esp. [[granulomatosis with polyangiitis]] (Wegener granulomatosis).


===C4d===
===C4d===
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*[[Membranoproliferative glomerulonephritis]] (MPGN).
*[[Membranoproliferative glomerulonephritis]] (MPGN).


=Normal=
=Normal kidney=
===Cells of the glomerulus===
===Cells of the glomerulus===
*Podocytes.
*Podocytes.
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====Glomerular basement membrane====
====Glomerular basement membrane====
The glomerular basement membrane (GBM) should be thinner than the tubular basement membrane.
The glomerular basement membrane (GBM) should be thinner than the tubular basement membrane.
===Images===
<gallery>
Image: Benign kidney -- very low mag.jpg | Benign kidney - very low mag.
Image: Benign kidney -- low mag.jpg | Benign kidney - low mag.
Image: Benign kidney - alt -- low mag.jpg | Benign kidney - low mag.
Image: Benign kidney -- intermed mag.jpg | Benign kidney - intermed. mag.
</gallery>


=Basic approach to renal biopsy=
=Basic approach to renal biopsy=
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*Interstitium.
*Interstitium.
*Vessels.
*Vessels.
Memory device ''GI TV'': glomeruli, interstitium, tubules, vessels.


===Glomeruli===
===Glomeruli===
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| [[membranous nephropathy]], [[focal segmental glomerulosclerosis]] (FSGS)
| [[membranous nephropathy]], [[focal segmental glomerulosclerosis]] (FSGS)
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]]
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]]
| Image?
| [[Image:Benign kidney -- intermed mag.jpg|thumb|center|150px|Benign kidney (WC)]]
|-
|-
|Nodular mesangial expansion
|Nodular mesangial expansion
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|-
|-
| [[Membranoproliferative glomerulonephritis]] (MPGN)
| [[Membranoproliferative glomerulonephritis]] (MPGN)
| mesangial proliferation with thickening of the glomerular capillary loops<ref name=pmid11682680>{{Cite journal  | last1 = Smet | first1 = AD. | last2 = Kuypers | first2 = D. | last3 = Evenepoel | first3 = P. | last4 = Maes | first4 = B. | last5 = Messiaen | first5 = T. | last6 = Van Damme | first6 = B. | last7 = Vanrenterghem | first7 = Y. | title = 'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt. | journal = Nephrol Dial Transplant | volume = 16 | issue = 11 | pages = 2258-62 | month = Nov | year = 2001 | doi =  | PMID = 11682680 }}</ref>
| mesangial proliferation with thickening of the glomerular capillary loops<ref name=pmid11682680>{{Cite journal  | last1 = Smet | first1 = AD. | last2 = Kuypers | first2 = D. | last3 = Evenepoel | first3 = P. | last4 = Maes | first4 = B. | last5 = Messiaen | first5 = T. | last6 = Van Damme | first6 = B. | last7 = Vanrenterghem | first7 = Y. | title = 'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt. | journal = Nephrol Dial Transplant | volume = 16 | issue = 11 | pages = 2258-62 | month = Nov | year = 2001 | doi =  | PMID = 11682680 | URL = http://ndt.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=11682680 }}</ref>
 
| low C3, normal C4; primary vs. secondary (often hepatitis C)
| low C3, normal C4; primary vs. secondary (often hepatitis C)
|-
|-
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====Images====  
====Images====  
<gallery>
<gallery>
Image:Diabetic_glomerulosclerosis_%281%29_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Diabetic_glomerulosclerosis_(2)_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Diabetic_glomerulosclerosis_%283%29_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Nodular_glomerulosclerosis.jpeg | Nodular glomerulosclerosis. (WC)
Image:Nodular_glomerulosclerosis.jpeg | Nodular glomerulosclerosis. (WC)
</gallery>
</gallery>
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==Post-infectious glomerulonephritis==
==Post-infectious glomerulonephritis==
*Abbreviated ''PIGN''.
*Abbreviated ''PIGN''.
===General===
{{Main|Post-infectious glomerulonephritis}}
*Classically post-streptococcal infection.
**Lab test: Antistreptolysin O titer (ASOT) +ve.
 
===Microscopic===
Features:
*+/-Neutrophils - in glomerulus.
*[[Glomerular crescents]].
 
====Images====
<gallery>
Image:Post-infectious_glomerulonephritis_-_high_mag.jpg | Post-infectious GN - high mag. (WC/Nephron)
Image:Post-infectious_glomerulonephritis_-_very_high_mag.jpg | Post-infectious GN - very high mag. (WC/Nephron)
Image:Acute_Glomerulonephritis_Pathology_Diagram.svg | Post-infectious GN - schematic (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case671.html Post-infectious glomerulonephritis - several images (upmc.edu)].
 
===IF===
*Granular immune deposits.
 
===EM===
*Subepithelial deposits - hump-like.
**Larger when measured perpendicular to the basement membrane, if compared to [[membranous nephropathy]].
**Typically focal.


=Rare diseases=
=Rare diseases=
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**Associated with a specific HLA type (HLA-DRB1*1501) and two gene families.
**Associated with a specific HLA type (HLA-DRB1*1501) and two gene families.
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated.
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated.
*Oligouria - poor prognosticator.{{fact}}


Tx:
Tx:
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===IF===
===IF===
*Linear IgG deposits - '''diagnostic'''.
*Linear IgG deposits - '''diagnostic'''.
*Fibrinogen in crescents.


DDx:
DDx:
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==Fabry disease==
==Fabry disease==
===General===
{{Main|Fabry disease}}
*Rare X-linked genetic disease.
**Caused by defect in ''alpha-galactosidase A gene'' (''GLA gene'').<ref name=omim301500>{{OMIM|301500}}</ref>
**Women partially affected
*[[Lysosomal storage disorder]] - 2nd in prevalence only to [[Gaucher disease]].
*Multisystem disease affecting small vessels and [[kidney]].
 
====Presentation====
*Women: usually proteinuria.
*Men: [[angiokeratoma]]s, proteinuria.
 
====Tx====
*Symptomatic treatment.
*Enzyme replacement - ''agalsidase alpha'' (Replagal) or ''agalsidase beta'' (Fabrazyme).
 
===Microscopic===
Features:<ref name=pmid16799480>{{cite journal |author=Fischer EG, Moore MJ, Lager DJ |title=Fabry disease: a morphologic study of 11 cases |journal=Mod. Pathol. |volume=19 |issue=10 |pages=1295-301 |year=2006 |month=October |pmid=16799480 |doi=10.1038/modpathol.3800634 |url=http://www.nature.com/modpathol/journal/v19/n10/abs/3800634a.html}}</ref>
*Foamy podocyte inclusions, best visualized with ''[[toluidine blue]]''.
*Mild mesangial hypercellularity.
 
Images:
*[http://path.upmc.edu/cases/case137/micro.html Fabry disease (upmc.edu)].
*[http://path.upmc.edu/cases/case610.html Fabry disease - several images (upmc.edu)].
 
===EM===
Features:<ref name=pmid16799480/>
*Myelin-like inclusions.
**Concentric bodies with an onion-skin-like appearance.
*Zebra bodies.
**Ovoid inclusions with striped pattern.
 
Note:
*Myelin-like inclusion are not [http://en.wikipedia.org/wiki/Pathognomonic pathognomonic] for Fabry disease; they may result from drug use:<ref name=pmid16799480/>
**Amiodarone.
**Aminoglycosides.
**Chloroquine.


==Myeloma==
==Myeloma==
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Presentation:
Presentation:
*Nephrotic range proteinuria ~ 60% of cases.<ref>{{Cite journal  | last1 = Korbet | first1 = SM. | last2 = Schwartz | first2 = MM. | last3 = Lewis | first3 = EJ. | title = Immunotactoid glomerulopathy. | journal = Am J Kidney Dis | volume = 17 | issue = 3 | pages = 247-57 | month = Mar | year = 1991 | doi =  | PMID = 1996564 }}</ref>
*Nephrotic range proteinuria ~ 60% of cases.<ref name=pmid1996564>{{Cite journal  | last1 = Korbet | first1 = SM. | last2 = Schwartz | first2 = MM. | last3 = Lewis | first3 = EJ. | title = Immunotactoid glomerulopathy. | journal = Am J Kidney Dis | volume = 17 | issue = 3 | pages = 247-57 | month = Mar | year = 1991 | doi =  | PMID = 1996564 }}</ref>
*[[Hypertension]].
*[[Hypertension]].


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*[[AKA]] ''fibrillary glomerulopathy''.
*[[AKA]] ''fibrillary glomerulopathy''.
===General===
===General===
*Rare ~ 1% native kidney biopsies.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.long }}</ref>
*Rare ~ 1% native kidney biopsies.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.full }}</ref>
*Presents as [[nephrotic syndrome]].
*Presents as [[nephrotic syndrome]].
*Prognosis poor; large number progress to ESRD.
*Prognosis poor; large number progress to ESRD.
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Note:
Note:
*[[Amyloid]] between 8 and 15 nm (diameter), most often 8-12 nm.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.long }}</ref>
*[[Amyloid]] between 8 and 15 nm (diameter), most often 8-12 nm.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.full }}</ref>


==Aristolochic acid nephropathy==
==Aristolochic acid nephropathy==
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*''Reflux nephropathy'' is considered synonym in some sources.<ref name=Ref_Sternberg5_1728>{{Ref Sternberg5|1728}}</ref>
*''Reflux nephropathy'' is considered synonym in some sources.<ref name=Ref_Sternberg5_1728>{{Ref Sternberg5|1728}}</ref>
===General===
===General===
*Inflammation of the kidney (''nephritis'') and renal pelvis (''pyelo-''<ref>URL: [http://medical-dictionary.thefreedictionary.com/pyelo- http://medical-dictionary.thefreedictionary.com/pyelo-]. Accessed on: 5 June 2015.</ref>).
*May be associated with vesicoureteral reflux.
*May be associated with vesicoureteral reflux.
*Chronic pyelonephritis may be a reason for nephrectomy.<ref>URL: [https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm]. Accessed on: 26 July 2011.</ref>
*Chronic [[pyelonephritis]] may be a reason for nephrectomy.<ref>URL: [https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm]. Accessed on: 26 July 2011.</ref>


===Gross===
===Gross===
Michael
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