Abstract |
This case study describes a domino liver transplantation in which a patient with familial amyloid polyneuropathy received a cadaveric liver, and the explanted liver was in turn transplanted into a second recipient. Familial amyloid polyneuropathy is an autosomal dominant inherited disease associated with a mutant form of the protein transthyretin. Liver transplantation is the only definitive treatment for this disease. Transplantation removes the source of mutant transthyretin, halts the progression of this otherwise fatal disease, and significantly palliates many underlying symptoms. This case study illustrates that domino transplantation is a practical option to provide a liver transplant for a patient with this disease and a second listed patient from a single cadaveric liver organ, thus alleviating the organ donor shortage. Transplantation offers the only cure for the genetic defect that causes familial amyloid polyneuropathy, appears to result in subjective and objective improvement in neurological function, and eliminates the mortality associated with the disease. A signed informed consent was provided for publication of this case study.
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Authors | Maria T Carrera, Eileen Heanue Bogue, Thomas D Schiano |
Journal | Progress in transplantation (Aliso Viejo, Calif.)
(Prog Transplant)
Vol. 13
Issue 2
Pg. 151-3
(Jun 2003)
ISSN: 1526-9248 [Print] United States |
PMID | 12841523
(Publication Type: Case Reports, Journal Article)
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Topics |
- Age Factors
- Aged
- Amyloid Neuropathies, Familial
(genetics, surgery)
- Cadaver
- Carcinoma, Hepatocellular
(surgery)
- Female
- Hepatitis B
(surgery)
- Humans
- Liver Neoplasms
(surgery)
- Liver Transplantation
(methods)
- Living Donors
- Middle Aged
- Tissue and Organ Procurement
(methods)
- Waiting Lists
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