Clinical manifestations in classically affected hemizygotes (with absent α-gal A activity), include pain and paresthesias in the extremities, vessel ectasia (angiokeratoma) in skin and mucous membranes, and hypohidrosis during childhood or adolescence. Corneal and lenticular opacities are early findings. With increasing age, proteinuria and renal impairment lead to hypertension and uremia. Renal failure typically occurs in the third-fifth decades of life, and these patients need chronic hemodialysis and/or renal transplantation.