Focal segmental glomerulosclerosis is one of the most serious acquired
chronic renal disorders of children [1]. The lesion, originally descr
ibed in 1957 by Rich as ''a hitherto undescribed vulnerability of juxt
amedullary glomeruli in lipoid nephrosis'' [2], has become a therapeut
ic challenge to nephrologists in general and pediatric nephrologists i
n particular, since the lesion is more commonly seen in children and i
nvariably leads to end-stage renal disease. Currently no specific ther
apy is available to control the proteinuria, and it has been shown tha
t the progression of the disease is related to the profusion of protei
nuria [3]. In view of the seriousness of the disease controversial tre
atments are now being proposed to control the proteinuria. This review
will deal with the magnitude of the problem, current therapeutic moda
lities, the pathogenesis of the disease process, possible therapeutic
interventions and their antecedent complications. The review will also
provide the design of a clinical trial organized to determine if two
potential modalities of therapy are comparable in efficacy and/or seri
ous side effects.