مرکز منطقه ای اطلاع رساني علوم و فناوري -

چكيده لاتين :

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 11.5 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.