diffuse Mesangial sclerosis

Also Known As:

Mesangial sclerosis, diffuse; Diffuse isolated mesangial sclerosis; Diffuse mesangial sclerosis; Familial mesangial sclerosis; Isolated diffuse mesangial sclerosis; Mesangial Sclerosis, Familial; Mesangial Sclerosis, Isolated Diffuse; Nephrotic Syndrome, Early-Onset, With Diffuse Mesangial Sclerosis; Nephrotic syndrome, early onset with diffuse mesangial sclerosis

Networked: 30 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Lipoid Nephrosis (Glomerulonephritis, Minimal Change)
2.	Nephrotic Syndrome (Syndrome, Nephrotic)
3.	Denys-Drash Syndrome
4.	Chronic Kidney Failure (Chronic Renal Failure)
5.	Focal Segmental Glomerulosclerosis (Glomerulosclerosis, Focal)

Experts

1.	Azocar, Marta: 2 articles (10/2017 - 05/2014)
2.	Bogdanovic, Radovan: 2 articles (10/2017 - 05/2014)
3.	Borzecka, Halina: 2 articles (10/2017 - 05/2014)
4.	Erdogan, Ozlem: 2 articles (10/2017 - 05/2014)
5.	Gellermann, Jutta: 2 articles (10/2017 - 05/2014)
6.	Jankauskiene, Augustina: 2 articles (10/2017 - 05/2014)
7.	Melk, Anette: 2 articles (10/2017 - 05/2014)
8.	Ozaltin, Fatih: 2 articles (10/2017 - 05/2014)
9.	PodoNet Consortium: 2 articles (10/2017 - 05/2014)
10.	Ranchin, Bruno: 2 articles (10/2017 - 05/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to diffuse Mesangial sclerosis:

1.	SteroidsIBA 01/01/1999 - "In conclusion, the homozygous nephrotic mouse (ICGN strain) is believed to be a good model for investigating not only nephrotic conditions but also cellular and molecular pathogenesis of diffuse mesangial sclerosis in steroid-resistant infantile nephrotic syndrome." 04/01/2005 - "Nephrotic syndrome (NS) in infancy includes NS of Finnish type (mutation of the nephrin gene), diffuse mesangial sclerosis (idiopathic or linked to WT1 mutation), idiopathic NS, most often steroid resistant, and NS related to infections during pregnancy (virus, syphilis, toxoplasmosis). "
2.	ChondroitinIBA 08/01/1991 - "To evaluate the specificity of a raised heparan sulphate (HS) excretion previously reported in four children with congenital nephrotic syndrome (CNS), we measured the urinary excretion of HS and chondroitin sulphate (CS) in seven children with Finnish-type congenital nephrotic syndrome (CNSF), seven with diffuse mesangial sclerosis (DMS), nine with focal segmental glomerulosclerosis (FSGS), 14 with steroid-sensitive nephrotic syndrome of whom eight had a biopsy confirming minimal change histology (SSNS), and 17 controls. " 01/01/1992 - "These biochemical and immunohistochemical results are in contrast to the decrease in anionic sites (by polyethyleneimine staining) and the replacement of GBM HS by chondroitin sulphate, observed by others in CNS of the diffuse mesangial sclerosis type."
3.	TRPC6 Cation ChannelIBA 03/10/2022 - "The missense variant of the TRPC6 gene probably underlay the diffuse mesangial sclerosis in this patient. " 03/10/2022 - "[A child with diffuse mesangial sclerosis caused by a missense mutation of TRPC6 gene]." 01/01/2021 - "Heterozygous missense variant in TRPC6 in a boy with rapidly progressive infantile nephrotic syndrome associated with diffuse mesangial sclerosis."
4.	phospholipase C epsilonIBA 02/01/2009 - "Mutations in phospholipase C epsilon 1 are not sufficient to cause diffuse mesangial sclerosis." 07/01/2010 - "Mutations in the PLCE1 gene encoding phospholipase C epsilon 1 (PLCepsilon1) have been recently described in patients with early onset nephrotic syndrome (NS) and diffuse mesangial sclerosis (DMS). "
5.	Intercellular Signaling Peptides and Proteins (Growth Factors)IBA 05/01/2001 - "Glomerular extracellular matrix and growth factors in diffuse mesangial sclerosis."
6.	Collagen Type IV (Type IV Collagen)IBA 06/01/1997 - "Diffuse mesangial sclerosis associated with Kawasaki disease: an analysis of alpha chains (alpha 1-alpha 6) of human type IV collagen in the renal basement membrane."
7.	CytokinesIBA 01/01/1999 - "Our results suggest that diffuse mesangial sclerosis, a hereditary glomerular disease, may be genetically generated through early myofibroblast formation, which occurs and develops probably independently of up-regulation of these fibrogenic cytokines. "
8.	Transcription Factors (Transcription Factor)IBA 04/01/1998 - "Constitutional mutations of the WT1 gene, encoding a zinc-finger transcription factor involved in renal and gonadal development, are found in most patients with Denys-Drash syndrome (DDS), or diffuse mesangial sclerosis (DMS) associated with pseudohermaphroditism and/or Wilms tumor (WT). "
9.	Serum AlbuminIBA 10/01/2017 - "IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. "
10.	Proteins (Proteins, Gene)FDA Link 10/01/1998 - "Advances in pediatric nephrology has been mainly characterized during the last years by a burst of knowledge in the area of genetic renal diseases: 1/almost complete understanding of Alport syndrome related to mutations of COL4A5 or COL4A3/A4 genes of collagene; 2/the mapping and cloning of the nephronophthisis gene which is deleted in 75% of cases; 3/the mapping and cloning of the cystinosis gene coding for a protein of the lysosomal membrane; 4/the mapping and cloning of the Finnish-type congenital nephrotic syndrome gene; 5/the linkage to the SNR 1 gene on chromosome 1 of a large number of familial corticoresistant nephrotic syndromes, and the disclosure of mutations of the WT1 gene in diffuse mesangial sclerosis and in Frazier syndrome. "

Therapies and Procedures

1.	Therapeutics 05/01/2019 - "Combination therapy improves pathology indices in diffuse mesangial sclerosis." 03/01/2003 - "We observed three cases in a series of 50 children with NS nonresponsive to therapy, corresponding to one case each of minimal change disease, Finnish-type glomerulopathy, and diffuse mesangial sclerosis--two histopathologic studies were performed in each case. " 03/01/1980 - "Ultrastructural examinations of renal glomeruli in a case of diffuse mesangial sclerosis revealed a hitherto unknown type of alteration of the basement membranes ("cloudy pattern") which suggests a disturbance of glomerular basement membrane development as the basic mechanism which leads to therapy resistent infantile nephrotic syndrome."
2.	Nephrectomy 04/01/1995 - "Do children with diffuse mesangial sclerosis in association with mutations of the Wilm's tumour suppressor gene (WT1) require bilateral nephrectomy?" 05/01/1992 - "Should bilateral nephrectomy be carried out in all children with diffuse mesangial sclerosis prior to renal transplantation in view of the connection with Drash syndrome and therefore the risk of a Wilms' tumour developing?" 07/18/2017 - "At the time of bilateral nephrectomy five months after her initial biopsy, she had diffuse mesangial sclerosis, typical of Denys-Drash syndrome, without evidence of thrombotic microangiopathy. " 06/01/2005 - "In Denys-Drash syndrome, nephrectomy is required for associated Wilms' tumor or after progression to end stage renal disease secondary to diffuse mesangial sclerosis because of risk of development of Wilms' tumor. "
3.	Kidney Transplantation 08/01/1992 - "A lethal polyadenopathic and visceral KS occurred in a 5-year-old Caribbean boy who had undergone an allogenic renal transplantation for diffuse mesangial sclerosis with end-stage renal failure 4 months previously. " 05/01/1992 - "Should bilateral nephrectomy be carried out in all children with diffuse mesangial sclerosis prior to renal transplantation in view of the connection with Drash syndrome and therefore the risk of a Wilms' tumour developing?"