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Multiple Sclerosis l: Introduction01:19

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Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...

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Diffuse mesangial sclerosis - Report of two cases.

M Vankalakunti1, P K Jha, R M Madraki

  • 1Department of Pathology, Manipal Hospital, Bangalore, India.

Indian Journal of Nephrology
|October 23, 2012
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Summary
This summary is machine-generated.

Diffuse mesangial sclerosis (DMS), a rare nephrotic syndrome cause, presents in infancy and childhood. Early recognition is crucial for patient management and family surveillance, as illustrated by two fatal cases.

Keywords:
Diffuse mesangial sclerosisIndianephrotic syndrome in childhood

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Area of Science:

  • Pediatric Nephrology
  • Rare Genetic Diseases

Background:

  • Diffuse mesangial sclerosis (DMS) is a rare but serious cause of nephrotic syndrome, predominantly affecting infants and children.
  • DMS often manifests as a key feature of several syndromic genetic disorders, including WAGR, Denys Drash, Pierson, Frasier, and Galloway-Mowat syndromes.

Observation:

  • This report details two cases of DMS with distinct age of onset: one in infancy and another in the second decade of life.
  • Both presented cases unfortunately resulted in fatal outcomes, highlighting the aggressive nature of the condition.

Findings:

  • Diffuse mesangial sclerosis (DMS) is a significant cause of nephrotic syndrome in pediatric populations.
  • Syndromic associations are common, underscoring the genetic basis of many DMS cases.
  • The disease course can be severe, leading to fatal outcomes even with late-onset presentation.

Implications:

  • Timely diagnosis of DMS is critical for optimizing patient care and prognosis.
  • Genetic counseling and active surveillance of family members are essential due to the syndromic nature of DMS.
  • Further research into the pathogenesis and therapeutic strategies for DMS is warranted.