M. Akhtar et E. Al-sabban, Alport-like glomerular changes in a patient with nephrotic syndrome: report of a case, PED NEPHROL, 14(10-11), 2000, pp. 973-975
We report a 17-year-old Saudi girl who presented with nephrotic syndrome at
the age of 7 years. A renal biopsy revealed a mildly proliferative immune
complex-mediated glomerulonephritis, which on ultrastructural examination r
evealed prominent thickening of the capillary basement membranes, along wit
h marked splitting and lamellation of lamina densa resembling those seen in
Alport syndrome. These changes were even more pronounced in renal biopsies
performed 1 and 3 years later, respectively. Thorough clinical evaluations
and follow-up of more than 10 years failed to reveal any evidence of Alpor
t syndrome. Review of the literature revealed four similar cases reported p
reviously. Diffuse and prominent Alport-like glomerular changes may rarely
be seen in patients with nephrotic syndrome in the absence of Alport syndro
me. Pathogenesis of these changes, however, remains to be understood.