Iranian Journal of Pathology

Alport’s Syndrome: Ultra-structural Study of 26 Suspected Cases

Document Type : Original Research

Authors

Isa Jahanzad 1
Sakineh Amoueian 2
Armin Attaranzadeh 2

1 Dept. of Pathology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

2 Dept. of Pathology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Abstract
Introduction and Objective: Alport’s syndrome (hereditary nephritis with deafness) is a familial uncommon disease that ultra-structural studies are gold standard method of its diagnosis. Materials and Methods:We studied 26 Iranian patients suspicious of Alport’s syndrome by electron microscopy. We examined 19 men and 7 women (male to female ratio was 2.7) and the average age was 1.5 years (with a range of 2.5-75 years). Results:The most common findings were irregularity of the glomerular basement membrane with splitting, lamination, and foot process effacement. Thinning in basement membrane was also reported and one case had disruption of capillary walls in study by only light microscopy. All of the patients had a diagnosis of megansial hypercellularity. Immunoflourscence studies did not show any deposits of immunoglobulin and complement component. Conclusion: definite diagnosis must be confirmed by ultra-structural findings.
 

Keywords

Ultra-structural
Electron microscopy
Glomerular Basement Membrane
Alport’s syndrome

  • Receive Date 28 September 2014

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