Long-term survival on renal replacement therapy for primary hyperoxaluria type I.
We describe the case of a patient in end-stage renal failure due to primary hyperoxaluria type I (PH1) who started hemodialysis in 1977 and is still alive and active. The diagnosis of PH1 was first suspected after a bone biopsy performed in 1981 to investigate hyperparathyroidism. Oxalosis recurred as early as 3 months after transplantation in a cadaver kidney grafted in 1987; nevertheless, graft function remained good enough to make possible the discontinuation of dialysis treatment for 5 months and thereafter to have only 1 dialysis a week for 17 months. The diagnosis of PH1 has been recently confirmed despite the patient being already anuric by means of the determination of plasma oxalate and glycolate levels as well as by determining hepatic alanine:glyoxylate amino-transferase.
Calzavara
P
P
Transplant Center, General Hospital, Treviso, Italy.
Marangella
M
M
Petrarulo
M
M
Ballanti
P
P
Bonucci
E
E
Calconi
G
G
Maresca
M C
MC
da Porto
A
A
Vianello
A
A
D000328:Adult; D000409:Alanine / Q000032:analysis; D006016:Glycolates / Q000097:blood; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000097:blood / Q000150:complications / Q000601:surgery*; D007676:Kidney Failure, Chronic / Q000097:blood / Q000601:surgery; D016030:Kidney Transplantation; D008099:Liver / Q000201:enzymology; D008297:Male; D010070:Oxalates / Q000097:blood; D006435:Renal Dialysis; D013997:Time Factors; D000637:Transaminases / Q000032:analysis
D006016:Glycolates; D010070:Oxalates; D000637:Transaminases; C023067:glyoxylate aminotransferase; D000409:Alanine