[Galloway -Mowat syndrome. about two new cases]

ABSTRACT

Galloway - Mowat syndrome is a rare autosomal recessive disorder characterized by early onset nephrotic syndrome and central nervous system anomalies. We report two boys, born to consanguineous parents, who presented respectively at 32 months and 9 years of age a steroid-resistant nephrotic syndrome. The kidney biopsy showed, in the 2 cases, a focal and segmental glomerulosclerosis. At presentation they have a microcephaly, a mental retardation. A brain magnetic resonance imaging scan showed cortical atrophy, mega citerne, and white matter anomality in a case. One boy died at 3,5 years of age from end-stage renal failure, the other had 12 year old and on chronic hemodialysis. The particularity of the two cases is the nephrotic syndrome onset after 2 years of age