Kidney nontumor / medical renal
Glomerular disease
Inherited glomerular disease
Diffuse mesangial sclerosis
Author: Nikhil Sangle, M.D.
Last author update: 1 April 2012
Last staff update: 30 June 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Diffuse mesangial sclerosis [title]
Table of Contents
Definition / general | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Electron microscopy descriptionCite this page: Sangle N. Diffuse mesangial sclerosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneycongenitalnephroticsyndromediffusemesangialsclerosis.html. Accessed April 18th, 2024.
Definition / general
- Clinically and genetically heterogeneous group of disorders in non-Finnish patients (J Am Soc Nephrol 2010;21:1209)
- Early onset of severe proteinuria (50% within months 0 - 2, 50% in months 3 - 9 months), with rapid progression to end stage renal disease by age 3 years
- May be associated with WT1 abnormalities and Denys-Drash syndrome (OMIM: Nephrotic Syndrome, Type 4; NPHS4), PLCE1 mutations (Nephrol Dial Transplant 2008;23:1291) or be isolated (Pediatr Nephrol 2009;24:1013)
- Normal placenta, no premature births but is associated with cataracts and corneal clouding, aniridia, microencephaly, developmental disability and hypertelorism
- Does not recur after transplantation
Case reports
- Two cases of isolated diffuse mesangial sclerosis with WT1 mutations (J Korean Med Sci 2006;21:160)
Microscopic (histologic) description
- Diffuse mesangial sclerosis; tubular atrophy with focal tubular dilatation and interstitial fibrosis
Microscopic (histologic) images
Images hosted on other servers:
Mesangial sclerosis
WT1 associated
Immunofluorescence description
- Nonspecific staining with mesangial deposits of IgM, C3 and C1q
Electron microscopy description
- Obliteration of foot processes, basement membrane thickening and increase in mesangial matrix
