RENAL ALLOTRANSPLANTATION IN MAN .2. TRANSPLANTATION IN CYSTINOSIS, A METABOLIC DISEASE

Two patients with cystinosis in end stage renal failure received renal transplants from parental (heterozygous) donors. Although initial renal function was excellent, rapid glomerular insufficiency ensued between the third and sixth post-transplant months, without the reappearance of the Fanconi syndrome. Biopsies disclosed nephron loss, interstitial edema and fibrosis, and moderate infiltration with lymphocytes, plasma cells, and blast cells. The glomeruli showed mild to marked membranous and proliferative changes. One biopsy specimen demonstrated intracellular deposits of cystine crystals. These changes do not permit an assessment of the relative roles of systemic cytinosis, cell-mediated immune rejection, or antibody-mediated damage. The implications of these findings for future transplants in cystinotic children are discussed. © 1969.