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2015 ; 94
(31
): e1267
Nephropedia Template TP
gab.com Text
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English Wikipedia
Left Lateral Sectionectomy of the Native Liver and Combined Living-Related
Liver-Kidney Transplantation for Primary Hyperoxaluria Type 1
#MMPMID26252291
Chen GY
; Wei SD
; Zou ZW
; Tang GF
; Sun JJ
; Zhou ST
Medicine (Baltimore)
2015[Aug]; 94
(31
): e1267
PMID26252291
show ga
Primary hyperoxaluria type I (PH1), the most severe form of primary
hyperoxalurias, is a liver disease of the metabolic defect in glyoxylate
detoxification that can be corrected by liver transplantation. A 21-year-old man
presented to our center after 4 months of regular hemodialysis for kidney failure
caused by nephrolithiasis. A diagnosis of PH1 was confirmed by mutations of the
AGXT gene. Left lateral sectionectomy of the native liver was performed; and
auxiliary partial orthotopic liver transplantation (APOLT) and kidney
transplantation were carried out synchronously using a living donor. After
transplantation, the patient's plasma oxalate and creatinine levels substantially
decreased and the patient recovered well with good dual grafts function. APOLT
and kidney transplantation can compensate the liver deficient in liver enzyme
production and aid the renal elimination of oxalate, thus serving as an effective
treatment option for patients with PH1. In conclusion, left lateral sectionectomy
of the native liver and combined living-related liver-kidney transplantation can
be a surgical option for PH1.