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Diffuse Mesangial Sclerosis - Report of Two Cases

Overview
Specialty Nephrology
Date 2012 Oct 23
PMID 23087559
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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.

References
1.
Buscher A, Kranz B, Buscher R, Hildebrandt F, Dworniczak B, Pennekamp P . Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2010; 5(11):2075-84. PMC: 3001773. DOI: 10.2215/CJN.01190210. View

2.
Cohen A, Turner M . Kidney in Galloway-Mowat syndrome: clinical spectrum with description of pathology. Kidney Int. 1994; 45(5):1407-15. DOI: 10.1038/ki.1994.184. View

3.
Besbas N, Bayrakci U, Kale G, Cengiz A, Akcoren Z, Akinci D . Cytomegalovirus-related congenital nephrotic syndrome with diffuse mesangial sclerosis. Pediatr Nephrol. 2006; 21(5):740-2. DOI: 10.1007/s00467-006-0051-4. View

4.
Koziell A, Grundy R . Frasier and Denys-Drash syndromes: different disorders or part of a spectrum?. Arch Dis Child. 1999; 81(4):365-9. PMC: 1718089. DOI: 10.1136/adc.81.4.365. View

5.
Niaudet P, Gubler M . WT1 and glomerular diseases. Pediatr Nephrol. 2006; 21(11):1653-60. DOI: 10.1007/s00467-006-0208-1. View