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Difference between revisions of "Medical kidney diseases"
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Medical kidney diseases (view source)
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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- | *PR3-ANCA (proteinase 3 antineutrophil cytoplasmic autoantibody). | ||
**Previously c-ANCA. | **Previously c-ANCA. | ||
**Seen in ANCA-[[vasculitides]], esp. [[ | **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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|- | |- | ||
| [[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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**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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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. | *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. | *[[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== | ||