Sequential liver–kidney transplantation from a living-related donor in primary hyperoxaluria type 1 (oxalosis)

Sato, Shuichi; Fuchinoue, Shohei; Kimikawa, Masaaki; Tojimbara, Tamotsu; Nakajima, Ichiro; Teraoka, Satoshi; Saito, Hiroshi; Ito, Katsuhiko

doi:10.1016/s0041-1345(02)03911-8

Cited by 23 publications

(22 citation statements)

References 5 publications

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“…The disease is caused by a genetic defect in the hepatic enzyme alanine-glyoxylate aminotransferase (AGT), which transforms glyoxylate into glycine (7). Abnormal AGT function leads to the metabolism of glyoxylate to oxalate and hyperproduction of the latter.…”

Section: Discussionmentioning

confidence: 99%

Cadaveric Orthotopic Auxiliary Split Liver Transplantation and Kidney Transplantation: An Alternative for Type 1 Primary Hyperoxaluria

et al. 2005

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Liver transplantation (LTX) corrects the enzymatic defect responsible for type 1 primary hyperoxaluria (PH1). It has been advocated in combination with kidney transplantation (KTX) in patients with renal failure from PH1 because KTX alone can result in early graft loss. A 58-year-old male patient with PH1 on hemodialysis underwent resection of the left lateral segment of the liver followed by orthotopic auxiliary left lateral segment liver transplantation and kidney transplantation from a deceased donor. The serum oxalate dropped from 34.8 micromol/L before transplant to 3.6-8.3 in the first months posttransplant to <1 micromol/L (normal range 0.4-3.0). One year after posttransplant, the patient has an iothalamate glomerular filtration rate of 58 ml/min. Orthotopic auxiliary LTX is an alternative to whole LTX in PH1. By using a split deceased donor liver, it does not deprive the donor pool and protects the recipient from liver failure in case of graft loss.

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Section: Discussionmentioning

confidence: 99%

Cadaveric Orthotopic Auxiliary Split Liver Transplantation and Kidney Transplantation: An Alternative for Type 1 Primary Hyperoxaluria

et al. 2005

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“…All but one of the organs were from deceased donors; the exception was one living-related kidney in a sequential transplantation [39]. A few cases of sequential LKT from the same living-related donor have been reported from Japan, with liver and kidney transplants separated from 51 days to 10 months [40]. Given the shortage of deceased-donor organs, such an alternative may be an attractive option, although operative morbidity to the donor is not inconsequential.…”

Section: Combined Liver-kidney Transplantationmentioning

confidence: 99%

Hyperoxaluria and systemic oxalosis: current therapy and future directions

Bobrowski¹,

Langman²

2006

Expert Opinion on Pharmacotherapy

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Excessive urinary oxalate excretion, termed hyperoxaluria, may arise from inherited or acquired diseases. The most severe forms are caused by increased endogenous production of oxalate related to one of several inborn errors of metabolism, termed primary hyperoxaluria. Recurrent kidney stones and progressive medullary nephrocalcinosis lead to the loss of kidney function, requiring dialysis or transplantation, accompanied by systemic oxalate deposition that is termed systemic oxalosis. For most primary hyperoxalurias, accurate diagnosis leads to the use of therapies that include pyridoxine supplementation, urinary crystallisation inhibitors, hydration with enteral fluids and, in the near future, probiotic supplementation or other innovative therapies. These therapies have varying degrees of success, and none represent a cure. Organ transplantation results in reduced patient and organ survival when compared with national statistics. Exciting new approaches under investigation include the restoration of defective enzymatic activity through the use of chemical chaperones and hepatocyte cell transplantation, or recombinant gene therapy for enzyme replacement. Such approaches give hope for a future therapeutic cure for primary hyperoxaluria that includes correction of the underlying genetic defect without exposure to the life-long dangers associated with organ transplantation.

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“…Although some Japanese cases of PH1 were reported [19,20,21,22,23], PH1 has not adequately been surveyed in Japan. Japanese patients with PH reported in the literature were reviewed in the present investigation.…”

Section: Introductionmentioning

confidence: 99%

Primary Hyperoxaluria Type 1 in Japan

et al. 2005

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Background/Aims: Current status of primary hyperoxaluria (PH) has not been surveyed in Japan. Methods: Japanese patients with PH were reviewed in the published literature. Results: Fifty-nine patients were diagnosed as PH from 1962 to 2003. The median ages both at diagnosis and at the onset of initial symptoms were 17 (range: 0.02–63) and 13 (range: 0–58) years, respectively. Twenty-nine (49%) patients were older than 20 years at diagnosis, among whom 26 (90%) already presented end-stage renal failure (ESRF) or soon evolved into ESRF. Among 30 (51%) diagnosed as PH under 20 years old, only 13 (43%) were already in a terminal stage of renal insufficiency. Ten patients were diagnosed as PH1 by liver biopsy. We identified two types of enzymatic phenotypes in 3 of those patients examined. In 1 case, immunoreactive SPT/AGT protein level was very low due to accelerated proteolysis, while in other 2 cases, the immunoreactivity was detected on mitochondria due to mistargeting. Of 9 cases having been subjected to kidney transplantation at a median age of 20 years (range 7.3–40.0), it was only 2 cases that were reported to be successful, while the median survival time of the kidney grafts being 1.4 years (range 0–7). Of 4 patients having undergone combined liver/kidney transplantations (at the ages of 1.3, 1.4, 9 and 41 years, respectively), the surgery was successful in 3 cases; in the remaining one case, however, rejection required removal of the transplanted kidney was observed. The overall survival ratio of all the 59 PH cases accounted for 77, 71 and 55% at 5, 10 and 20 years, respectively. Conclusion: Assuming that the majority of the 59 patients with PH reported was classified as PH1, it is postulated that morbidity of violent infantile PH1 in Japan might be less than those in the USA and Europe, and symptoms of elderly Japanese PH1 patients seem to be milder than those of Western patients. Establishment of an early detection system of PH1 and more popular application of combined liver/kidney transplantation deserve further study.

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Sequential liver–kidney transplantation from a living-related donor in primary hyperoxaluria type 1 (oxalosis)

Cited by 23 publications

References 5 publications

Cadaveric Orthotopic Auxiliary Split Liver Transplantation and Kidney Transplantation: An Alternative for Type 1 Primary Hyperoxaluria

Cadaveric Orthotopic Auxiliary Split Liver Transplantation and Kidney Transplantation: An Alternative for Type 1 Primary Hyperoxaluria

Hyperoxaluria and systemic oxalosis: current therapy and future directions

Primary Hyperoxaluria Type 1 in Japan

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