title: Two-step transplantation for primary hyperoxaluria: a winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases.
abstract: Several transplant strategies for PH1 have been proposed, and LT is performed to correct the metabolic defects. The patients with PH1 often suffer from ESRD and require simultaneous LKT, which leads to a long wait due to the shortage of suitable organ donors. Five patients with PH1 underwent LDLT at our institute. Three of the five patients were under dialysis before LDLT, while the other two patients were categorized as CKD stage 3. An isolated LDLT was successfully performed in all but our first case, who had complicated postoperative courses and consequently died due to sepsis after retransplantation. The renal function of the patients with CKD stage 3 was preserved after LDLT. On the other hand, our second case with ESRD underwent successful LDKT six months after LDLT, and our infant case is waiting for the subsequent KT without any post-LDLT complications after the early establishment of PD. In conclusion, a two-step transplant strategy may be needed as a life-saving option for patients with PH1 and may be possible even in small infants with systemic oxalosis. While waiting for a subsequent KT, an early resumption of PD should be considered from the perspective of the long-term requirement of RRT.
authors.affiliation: Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Kengo
authors.initials: K
authors.lastname: Sasaki
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Hajime
authors.initials: H
authors.lastname: Uchida
authors.forename: Takanobu
authors.initials: T
authors.lastname: Shigeta
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Matsunami
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanazawa
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Mai
authors.initials: M
authors.lastname: Sato
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ito
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yokoi
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Azuma
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
mesh_terms: D000293:Adolescent; D002648:Child; D018450:Disease Progression; D005260:Female; D006801:Humans; D006959:Hyperoxaluria / Q000209:etiology* / Q000517:prevention & control*; D006960:Hyperoxaluria, Primary / Q000150:complications* / Q000601:surgery*; D007223:Infant; D016030:Kidney Transplantation / Q000379:methods*; D008297:Male; D051437:Renal Insufficiency / Q000150:complications*
keywords: infantile oxalosis; living donor liver transplantation; primary hyperoxaluria type 1