Thin basement membrane disease biopsy: Difference between revisions
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{{Thin basement membrane disease}} | {{Thin basement membrane disease}} | ||
{{CMG}} | |||
{{AE}} {{MMT}} | |||
==Overview== | ==Overview== | ||
The WHO guideline for normal GBM thickness is 250nm for adult and 180nm for children of 2-11 years of age. Diffuse thinning of 50% of glomerular basement membrane in glomerular capillaries is the criteria for diagnosing TBMD on Electron microscopy. | The [[WHO]] guideline for normal [[GBM]] thickness is 250nm for adult and 180nm for children of 2-11 years of age. [[Diffuse]] thinning of 50% of [[glomerular]] [[basement membrane]] in [[glomerular]] [[capillaries]] is the criteria for diagnosing [[TBMD]] on [[Electron microscopy]]. | ||
==Renal Biopsy== | ==Renal Biopsy== | ||
[[File:Thin_Basement_Membrane_Disease.jpg| | |||
*Light | [[File:Thin_Basement_Membrane_Disease.jpg|thumb|500px|right|none|1(A) and 1(B) showing light microscopy of Thin basement membrane disease with cystic dilation. 1(C), 1(D), 1(E) showing GBM using different Immunofluoroscence 1(F), 1(G) showing thin GBM without electron deposition using Electron microscopy Courtesy: https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-019-1451-6/figures/2|]] | ||
*Electron microscopy of renal samples shows diffuse thinning of 50% of glomerular basement membrane in glomerular capillaries. GBM thickness should be measured according to age.The WHO guideline for normal GBM thickness is 250nm for adult and 180nm for children of 2-11 years of age. <ref name="pmid3449814">{{cite journal |vauthors=Vogler C, McAdams AJ, Homan SM |title=Glomerular basement membrane and lamina densa in infants and children: an ultrastructural evaluation |journal=Pediatr Pathol |volume=7 |issue=5-6 |pages=527–34 |date=1987 |pmid=3449814 |doi=10.3109/15513818709161416 |url=}}</ref> Early stages of X-linked alport syndrome is similar to TBMD, so differential features including lamellation/thickening must be ruled out on electron microscopy. <ref name="urlThin Basement Membrane Nephropathy | American Society of Nephrology">{{cite web |url=https://jasn.asnjournals.org/content/17/3/813#sec-3 |title=Thin Basement Membrane Nephropathy | American Society of Nephrology |format= |work= |accessdate=}}</ref> | |||
*Immunohistochemistry of renal samples evaluates Type 4 collagen alpha 3-alpha5 chains to differentiate TBMD and early stage of alport syndrome.<ref name="pmid7783412">{{cite journal |vauthors=Gubler MC, Knebelmann B, Beziau A, Broyer M, Pirson Y, Haddoum F, Kleppel MM, Antignac C |title=Autosomal recessive Alport syndrome: immunohistochemical study of type IV collagen chain distribution |journal=Kidney Int |volume=47 |issue=4 |pages=1142–7 |date=April 1995 |pmid=7783412 |doi=10.1038/ki.1995.163 |url=}}</ref> | *[[Light microscopy]] of [[renal]] [[samples]] shows usually normal [[glomerular]] [[histopathology]] with minimally showing nonspecific findings including [[matrix]] expansion, [[Mesangial cell|mesangial]] cellular proferation, [[RBC]]<nowiki/>s in [[glomerular]] spaces with [[focal segmental glomerulosclerosis]] or [[fibrosis]] on rare occasions.<ref name="pmid15880325">{{cite journal |vauthors=Foster K, Markowitz GS, D'Agati VD |title=Pathology of thin basement membrane nephropathy |journal=Semin Nephrol |volume=25 |issue=3 |pages=149–58 |date=May 2005 |pmid=15880325 |doi=10.1016/j.semnephrol.2005.01.006 |url=}}</ref> | ||
*[[Electron microscopy]] of [[renal]] samples shows [[diffuse]] thinning of 50% of [[glomerular]] [[basement membrane]] in [[glomerular]] [[capillaries]]. [[GBM]] thickness should be measured according to age.The [[WHO]] guideline for normal [[GBM]] thickness is 250nm for adult and 180nm for children of 2-11 years of age. <ref name="pmid3449814">{{cite journal |vauthors=Vogler C, McAdams AJ, Homan SM |title=Glomerular basement membrane and lamina densa in infants and children: an ultrastructural evaluation |journal=Pediatr Pathol |volume=7 |issue=5-6 |pages=527–34 |date=1987 |pmid=3449814 |doi=10.3109/15513818709161416 |url=}}</ref> Early stages of [[X-linked]] [[alport syndrome]] is similar to [[TBMD]], so differential features including [[Lamella|lamellation]]/thickening must be ruled out on [[electron microscopy]]. <ref name="urlThin Basement Membrane Nephropathy | American Society of Nephrology">{{cite web |url=https://jasn.asnjournals.org/content/17/3/813#sec-3 |title=Thin Basement Membrane Nephropathy | American Society of Nephrology |format= |work= |accessdate=}}</ref> | |||
*[[Immunohistochemistry]] of [[renal]] samples evaluates [[Type-IV collagen|Type 4 collagen]] alpha 3-alpha5 chains to differentiate [[TBMD]] and early stage of [[alport syndrome]].<ref name="pmid7783412">{{cite journal |vauthors=Gubler MC, Knebelmann B, Beziau A, Broyer M, Pirson Y, Haddoum F, Kleppel MM, Antignac C |title=Autosomal recessive Alport syndrome: immunohistochemical study of type IV collagen chain distribution |journal=Kidney Int |volume=47 |issue=4 |pages=1142–7 |date=April 1995 |pmid=7783412 |doi=10.1038/ki.1995.163 |url=}}</ref> | |||
==References== | ==References== | ||
Latest revision as of 09:39, 21 December 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Marufa Marium, M.B.B.S[2]
Overview
The WHO guideline for normal GBM thickness is 250nm for adult and 180nm for children of 2-11 years of age. Diffuse thinning of 50% of glomerular basement membrane in glomerular capillaries is the criteria for diagnosing TBMD on Electron microscopy.
Renal Biopsy
- Light microscopy of renal samples shows usually normal glomerular histopathology with minimally showing nonspecific findings including matrix expansion, mesangial cellular proferation, RBCs in glomerular spaces with focal segmental glomerulosclerosis or fibrosis on rare occasions.[1]
- Electron microscopy of renal samples shows diffuse thinning of 50% of glomerular basement membrane in glomerular capillaries. GBM thickness should be measured according to age.The WHO guideline for normal GBM thickness is 250nm for adult and 180nm for children of 2-11 years of age. [2] Early stages of X-linked alport syndrome is similar to TBMD, so differential features including lamellation/thickening must be ruled out on electron microscopy. [3]
- Immunohistochemistry of renal samples evaluates Type 4 collagen alpha 3-alpha5 chains to differentiate TBMD and early stage of alport syndrome.[4]
References
- ↑ Foster K, Markowitz GS, D'Agati VD (May 2005). "Pathology of thin basement membrane nephropathy". Semin Nephrol. 25 (3): 149–58. doi:10.1016/j.semnephrol.2005.01.006. PMID 15880325.
- ↑ Vogler C, McAdams AJ, Homan SM (1987). "Glomerular basement membrane and lamina densa in infants and children: an ultrastructural evaluation". Pediatr Pathol. 7 (5–6): 527–34. doi:10.3109/15513818709161416. PMID 3449814.
- ↑ "Thin Basement Membrane Nephropathy | American Society of Nephrology".
- ↑ Gubler MC, Knebelmann B, Beziau A, Broyer M, Pirson Y, Haddoum F, Kleppel MM, Antignac C (April 1995). "Autosomal recessive Alport syndrome: immunohistochemical study of type IV collagen chain distribution". Kidney Int. 47 (4): 1142–7. doi:10.1038/ki.1995.163. PMID 7783412.