Symptoma

Diffuse Mesangial Sclerosis

Abstract Diffuse mesangial sclerosis (DMS) is a renal disease that usually presents as a nephrotic syndrome. [ncbi.nlm.nih.gov]

  • Sepsis

    Seven patients died, with sepsis being the main cause of death. Diffuse mesangial sclerosis must be suspected in a child that presents with early onset proteinuria and/or rapidly progressive renal failure. [ncbi.nlm.nih.gov]

  • Atrial Septal Defect

    However, neither megalocornea, Dandy-Walker malformation, postaxial hexadactyly, rocker-bottom feet, nor atrial septal defect, as observed in our patient, has been reported previously in association with DMS. [ncbi.nlm.nih.gov]

  • Turkish

    Abstract Clinical and pathological findings in four Turkish infants with isolated diffuse mesangial sclerosis (DMS) are presented. [ncbi.nlm.nih.gov]

  • Dolichocephaly

    Pregnancy was terminated because of elevated maternal serum alpha-fetoprotein and ultrasonographic evidence of severe oligohydramnios, intrauterine growth retardation, non-visualization of the bladder, dolichocephaly and possible mild hydrocephalus. [ncbi.nlm.nih.gov]

  • Suggestibility

    Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in WT1 could also be operative in isolated diffuse mesangial sclerosis. [ncbi.nlm.nih.gov]

  • Kidney Failure

    Diffuse mesangial sclerosis is a kidney condition that begins early in childhood and rapidly progresses to kidney failure. It is usually associated with other conditions such as Denys-Drash Syndrome. [patientslikeme.com]

If this observation is confirmed in a larger study, mutations in PLCE1 may serve as a biomarker for selecting patients with IDMS who may benefit from treatment. [ncbi.nlm.nih.gov]

Immunology[edit] It has been reported that DMS cases show no oligoclonal bands, being therefore distinct from standard MS.[7] Treatments[edit] Management Corticosteroids may be effective in some patients. [en.wikipedia.org]

It has a poor prognosis as this nephropathy is resistant to treatment with corticosteroids and rapidly progresses to end-stage renal failure. [kidneypathology.com]

This nephropathy has a poor prognosis, but the survival rate has improved in the last decade. [ncbi.nlm.nih.gov]

Prognosis[edit] The prognosis of this disease is very variable and can take three different courses: a monophasic, not remitting;[2][8] remitting;[9][10][11] and finally, progressive, with increase in deficits.[12] History[edit] It was first described [en.wikipedia.org]

It has a poor prognosis as this nephropathy is resistant to treatment with corticosteroids and rapidly progresses to end-stage renal failure. [kidneypathology.com]

The etiology and pathogenesis of DMS is not understood. We recently identified by positional cloning recessive mutations in the gene PLCE1/NPHS3 as a novel cause of IDMS. We demonstrated a role of PLCE1 in glomerulogenesis. [ncbi.nlm.nih.gov]

The etiology and pathogenesis of DMS is not understood, but the three mutations described in this glomerular disease involve the genes WT1, LAMB2 or PLCE1. Mutations in PLCE1 have been associated to isolated DMS. [kidneypathology.com]

Gonadectomy and/or nephrectomy is also advised prophylactically for prevention of tumor development.[10] The phenotypic finding of a DMS is not a single entity but is associated with many genotypic disorders including the WAGR syndrome (Wilms’ tumor, [ncbi.nlm.nih.gov]