Reversal by liver transplantation of the complications of primary hyperoxaluria as well as the metabolic defect
Article Abstract:
In some individuals genetic errors present in every cell of the body result in the inability to process certain important chemical reactions because of the absence of specific enzymes. In some cases the reactions which these enzymes enable are located in a single organ such as the liver. Liver transplantation has now been shown to be an effective means of providing an individual who has been congenitally deprived of a specific enzyme with the necessary genetic information to produce that enzyme and complete the missing metabolic step. A case report is presented of a patient unable to effectively control the blood level of oxalic acid who suffered from elevated levels of urinary oxalic acid (hyperoxaluria). The patient suffered from repeated inflammation of the kidney as a result of high levels of oxalic acid in his blood, which led to the formation of calcium oxalate kidney stones. The stones in turn led to pyelonephritis, a serious infection of the kidney's collecting duct system, and this ultimately necessitated removal of both kidneys. Patients suffering from primary hyperoxaluria often require removal of their kidneys and kidney transplantation. This is usually accomplished with no difficulty, but in this case a severe crisis occurred, namely the amputation of limbs affected with gangrene, and an emergency kidney transplant was performed to save the patient's life. The new kidney soon began to fail and biopsy again showed the presence of deposits of calcium oxalate. Ultimately a decision was made to replace the patient's liver. This procedure resulted in a complete cure of the primary hyperoxaluria and the patient is considered completely cured of his metabolic congenital disorder. The replacement of the individual's liver with a normal liver obtained from a cadaver completely cured both the metabolic defect and the secondary problems, kidney stones and hyperoxaluria.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1989
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Donor-derived acute promyelocytic leukemia in a liver-transplant recipient
Article Abstract:
Researchers describe a case of a 57-year-old woman who developed leukemia two years after receiving a liver transplant. The donor was a 16-year-old boy who had died of a head injury. Genetic analysis of some of the cancer cells revealed that they were clearly from the donor. The liver did not contain obvious cancer at the time of transplant, but some cancerous cells may have been transplanted.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1999
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Influence of donor C3 allotype on late renal-transplantation outcome
Article Abstract:
A study was carried out to examine the association between the C3 allotypes of renal donors and recipients and graft function after transplantation. The findings suggest that donor C3F and C3S alleles might confer functional differences.
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2006
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