Kidney nontumor
Inherited glomerular diseases
Congenital nephrotic syndrome - diffuse mesangial sclerosis

Topic Completed: 1 April 2012

Minor changes: 7 February 2020

Copyright: 2002-2020,, Inc.

PubMed Search: Diffuse mesangial sclerosis [title]

Nikhil Sangle, M.D.
Page views in 2019: 295
Page views in 2020 to date: 150
Cite this page: Sangle N. Congenital nephrotic syndrome - diffuse mesangial sclerosis. website. Accessed May 28th, 2020.
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
Microscopic (histologic) description
  • Diffuse mesangial sclerosis; tubular atrophy with focal tubular dilatation and interstitial fibrosis
Microscopic (histologic) images

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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
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