Kidney Int 1999; 56: 1094

Transplantation for primary hyperoxaluria in the United States

Saborio P, Scheinman JI

Department of Pediatrics, Medical College of Virginia, Virginia Commonwealth
University, Richmond, USA

Abstract
BACKGROUND: Transplantation (TX) has become an acceptable treatment for renal
failure in primary hyperoxaluria (PH). We have analyzed data from three U.S.
sources to estimate the success or failure of different modes of management in
PH patients. METHODS: The United States Renal Data System (USRDS) tapes provided
coded medical record data, with PH assigned to 235 patients from 1974 to 1996.
Another 45 patients were found from USRDS hospitalization records. We limited
patients to those developing end-stage renal disease at <55 years of age after
1984 (95 PH patients). The North American Pediatric Renal Transplantation
Cooperative Study (NAPRTCS) identified 34 (11 new) PH patients, and the United
Network for Organ Sharing (UNOS) database identified PH in 34 (16 new, 5 more in
both UNOS and NAPRTCS) patients. These secondary sources were used to correct
some data from the USRDS and to add 32 more patients, with a total of 128 PH
patients. Considering kidney TX (KTX) prior to combined kidney/liver TX (K/LTX)
as a separate record for some calculations, the total "cases" were 138. RESULTS:
By life table analysis, the 94 total TX patient survival was better than for the
34 NoTX patients (P<0.001). The 52 KTX patients' survival was better than either
the 32 primary K/LTX (P<0.001) or the 10 K/LTX that following KTX (P<0.001). The
62 KTX cases' survival was better than the 42 K/LTX cases (P<0.005), which did
not differ from the 34 NoTX (P<0.67). The overall survival of these 62 KTX
patients was 76%. The survival of 42 K/LTX was 69%, and the survival of 34 NoTX
patients was 44%. Kidney graft life table projected survival curves for TX
patients did not differ between K/LTX (56% at 6 years) and isolated KTX (51% at
6 years, 35% at 10 years, P<0.91). CONCLUSION: KTX offers better patient
survival in the United States then either K/LTX or NoTX. Graft survival does not
differ between KTX and K/LTX. Because K/LTX can still follow a failed KTX,
isolated living related donor KTX is still a reasonable first option for PH type
1 if a strictly managed protocol is followed