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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.
Abstract:
Diffuse mesangial sclerosis (DMS) is a rare cause of nephrotic syndrome in the infantile and childhood period. DMS is a phenotypic expression of syndromic entities such as WAGR syndrome (Wilms' tumor, aniridia, genitourinary anomalies and mental retardation), Denys Drash syndrome, Pierson syndrome, Frasier syndrome, or Galloway-Mowat syndrome. We report two cases of DMS, one presenting in first year of life and another in second decade of life. Both of them had fatal outcome. Recognition of the disease is very important in modifying the management of patient and active surveillance of family members.
Insights
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.
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.
