Multidisciplinary Cooperation in a Simultaneous Combined Liver and Kidney Transplantation Patient of Primary Hyperoxaluria

Chen Maogen,Guo Zhiyong,He Xiaoshun,Ju Weiqiang,Ren Qingqi,Wang Dongping

doi:10.31729/jnma.2671

Abstract

Primary hyperoxaluria type 1 is an autosomal recessive hereditary glyoxylate metabolism disorder characterized by excessive production of oxalate, caused by the deficiency of liver specific peroxisomal enzyme: alanineglyoxylate aminotransferase. For patients with end-stage renal disease, combined liver and kidney transplantation was needed. This report describes one patient, with a diagnosis of end-stage renal disease and primary hyperoxaluria 1 confirmed by PCR and direct sequencing with genomic DNA, received the simultaneous combined liver and kidney transplantation after seven months' waiting. However, there were several complications observed post surgery, such as protracted bleeding, common bile duct anastomotic stenosis, biliary calculi and recurrence of urolithiasis. All these were well solved by relevant department, and finally a satisfactory outcome was achieved. Multidisciplinary cooperation plays an important role on the PH1 patient management, especially when multiple complications are encountered.

Highlights

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive hereditary disorder
We report a case with multiple complications and successful multidisciplinary cooperation on its managements
Percutaneous transhepatic cholangical drainage (PTCD) was done under the guidance of ultrasound 12 days’ post-surgery when the total bilirubin reached 339.6 μmol/L and it decreased to normal level one month later

Summary

Introduction

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive hereditary disorder. Ren et al Multidisciplinary Cooperation in a Simultaneous Combined Liver and Kidney Transplantation Patient of Primary Hyperoxaluria 1 for consecutive 5 days to reduce the circulating oxalate maximally.

Results

Conclusion