US data were sought for transplantation in primary hyperoxaluria (PH).
The USRDS recorded 194 patients since 1974. By lifetable analysis, su
rvival was better for transplanted than for non-transplanted patients
(P < 0.001), even after trimming data for age < 55 and end-stage renal
disease since 1985 (63 patients, 39 transplanted, 24 not transplanted
). Transplant survival was longer for living related donor (21) vs cad
averic (17) transplants. Twenty-nine kidney transplants in 22 children
were registered in NAPRTCS. Interview data with physicians showed tha
t eight of 17 living related donor kidneys functioned well, three were
borderline and six were lost. All six cadaver kidneys were lost. Four
of six kidney-liver transplants functioned, and two died. United Netw
ork for Organ Sharing recorded 13 kidney-liver transplants in 11 patie
nts. Six initially functioned well; two were retransplanted. Ultimatel
y seven lived and four died. Overall, transplant is better than no tra
nsplant; cadaver donation results are poor; living related kidney dona
tion can succeed; and kidney-liver transplant is still problematic in
the US, and rarely follows appropriate investigation. Until more coope
rative effort can be achieved, isolated kidney living related donor tr
ansplant is preferable, and does not preclude kidney-liver transplant
later.