title: Sustained pyridoxine response in primary hyperoxaluria type 1 recipients of kidney alone transplant.
abstract: Combined liver kidney transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) given that it removes the hepatic source of oxalate production and improves renal allograft survival. However, PH1 patients homozygous for the G170R mutation can develop normal urine oxalate levels with pyridoxine therapy and may be candidates for kidney alone transplant (KTx). We examined the efficacy of pyridoxine therapy following KTx in five patients homozygous for G170R transplanted between September 1999 and July 2013. All patients were maintained on pyridoxine posttransplant. Median age at transplant was 39 years (range 33-67 years). Median follow-up posttransplant was 8.5 years (range 0.2-13.9 years). At the end of follow-up, four grafts were functioning. One graft failed 13.9 years posttransplant due to recurrent oxalate nephropathy following an acute medical illness. After tissue oxalate stores had cleared, posttransplant urine oxalate levels were <0.5 mmol/24 h the majority of times checked. Calcium oxalate crystals were noted in only 3/13 allograft biopsies. This series suggests that a subgroup of PH1 patients demonstrate sustained response to pyridoxine therapy following KTx. Therefore, pyridoxine combined with KTx should be considered for PH1 patients with a homozygous G170R mutation.
authors.affiliation: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: E C
authors.initials: EC
authors.lastname: Lorenz
authors.forename: J C
authors.initials: JC
authors.lastname: Lieske
authors.forename: B M
authors.initials: BM
authors.lastname: Seide
authors.forename: A M
authors.initials: AM
authors.lastname: Meek
authors.forename: J B
authors.initials: JB
authors.lastname: Olson
authors.forename: E J
authors.initials: EJ
authors.lastname: Bergstralh
authors.forename: D S
authors.initials: DS
authors.lastname: Milliner
mesh_terms: D000328:Adult; D002648:Child; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000188:drug therapy* / Q000503:physiopathology / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D011736:Pyridoxine / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D011736:Pyridoxine
keywords: Kidney disease; kidney transplantation; living donor; pathology; recurrent disease