[Sequential combined liver-kidney transplantation for a one-year-old boy with infantile primary hyperoxaluria type 1].
We present the case of a one-year-old male patient with infantile primary hyperoxaluria type 1 (PH1). The patient visited hospital because of growth delay and poor feeding when he was six months old, and was diagnosed as PH1 with chronic renal failure. He underwent peritoneal dialysis until receiving a living-related liver transplantation when he was seventeen months old, and after the operation, underwent hemodialysis or hemodiafiltration four times per week. Six months after the liver transplantation, his serum oxalate level decreased to around 20 micromol/l and a living-related kidney transplantation was successfully performed. Nine months have passed since the kidney transplantation, and the patient's liver and kidney functions have been good and his growth and development much better than before the sequential liver and kidney transplantation. However, his serum and urine oxalate levels remained high and he has required high dose hydration to prevent deposition of calcium oxalate crystals in his grafted kidney. The key-points for treating infantile PHI patients are summarized as follows; 1) make a precise diagnosis as soon as possible, 2) perform a combined liver-kidney transplantation successfully, 3) conduct careful monitoring of the serum and urine oxalate levels and continue adequate hydration after kidney transplantation until the serum and urine oxalate levels normalize. Furthermore, cooperation between the medical staff and the patient's family seems to be essential.
Department of Pediatric Nephrology, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
Yaeko
Y
Motoyoshil
Motoshi
M
Hattori
Hiroko
H
Chikamoto
Hyogo
H
Nakakura
Takeki
T
Furue
Sanpei
S
Miyakawa
Miyuki
M
Kohno
Katsumi
K
Ito
Kohtaro
K
Kai
Ichiro
I
Nakajima
Shohei
S
Fuchinoue
Satoshi
S
Teraoka
Takashi
T
Akiba
Hirotsugu
H
Kitayama
Naohiro
N
Wada
Yoshihide
Y
Ogawa
D006801:Humans; D006960:Hyperoxaluria, Primary / Q000145:classification / Q000601:surgery* / Q000652:urine; D007223:Infant; D007676:Kidney Failure, Chronic / Q000628:therapy; D016030:Kidney Transplantation; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D010530:Peritoneal Dialysis