RENAL-TRANSPLANTATION IN ADULTS WITH AUTOSOMAL RECESSIVE INHERITANCE OF HEMOLYTIC-UREMIC SYNDROME

When hemolytic uremic syndrome (HUS) is occasionally inherited in an a utosomal recessive mode, this occurs mainly in infants and children. W e describe four families in which two adult siblings were affected wit h HUS in each kindred, HUS first occurred between the ages of 19 to 36 years, and the intervals between the onset of HUS in each sibling pai r ranged from 6 months to 6 years, None of the patients had a typical prodrome of bloody diarrhea, and one had a recurrence of HUS before tr ansplantation. All eight patients developed renal failure requiring di alysis and transplantation, and seven patients received kidney transpl ants, Donor kidneys were from parents, siblings, and cadavers, The ini tial renal transplants were performed from 6 months to 6 years after t he onset of the syndrome. HUS recurred in six of the seven patients 2 weeks to 6.5 years after transplantation regardless of the interval be tween the onset of HUS and transplantation, the origin of the allograf t, or the use of cyclosporin A, The only marker for autosomal recessiv e HUS is the occurrence of the syndrome in a second sibling several mo nths to many years after its occurrence in the proband, In patients wi th the autosomal recessive form of HUS, the risk for a recurrence in a n allograft is high regardless of the source of the kidney. (C) 1997 b y the National Kidney Foundation, Inc.