Sequential liver transplantation: 27 cases in 25 patients
| Autor: | R Perdigoto, José Ferrão, O Mota, L Furtado, J Viana, Luís Tomé, Emanuel Couto Furtado, A. Cipriano, Fernando Oliveira, J. Geraldes |
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| Jazyk: | angličtina |
| Rok vydání: | 2001 |
| Předmět: |
Adult
Pathology medicine.medical_specialty Amyloid medicine.medical_treatment Transplantação de Fígado Liver transplantation Amyloid Neuropathies medicine Humans Transplantation biology business.industry Liver Diseases Amyloidosis Age Factors Middle Aged medicine.disease Survival Analysis Liver Transplantation Transthyretin Amyloid Neuropathy biology.protein Surgery Hepatectomy business Polyneuropathy |
| Popis: | AMILIAL amyloid polyneuropathy (FAP) is the most common form of herederitary amyloidosis. Initial clinical manifestation is a sensoriomotor polyneuropathy starting in the lower limbs and later affecting the upper extremities. The autonomic nervous system is involved (orthostatic hypotension and impotence). Severe constipation (often the first gastrointestinal symptom) is relieved with time by bouts of diarrhea that subsequently become continuous. Cardiac arrhytmias are common. Some patients develop renal failure because of accumulation of amyloid in the kidneys together with bladder denervation. 1 FAP is characterized by systemic deposition of amyloid. Amyloid fibrils are composed of variant transthyretin in which a single amino acid substitution (TTRMet30 in FAP type 1) results in the aggregation and insolubility of the protein with deposition in extracellular spaces. More than 95% of TTR is produced in the liver. 2 Apart from the production of the variant transthyretin, FAP livers are functionally and anatomically normal. The course of the disease is progressive leading to death within 14 years of the onset of symptoms. Liver transplantation is the only effective therapy. 3 FAP presents from the third to sixth decade, which suggests that it can take this long for sufficient amyloid to be deposited in the tissues. Elderly asymptomatic TTRMet30 homozygotes have been reported. 4 Among the heterozygotes, penetrance and rate of disease progression vary substantially. 5 Portugal has several hundreds of FAP type 1 patients for whom the sole hope of stopping the progression of the disease is liver transplantation. The severe shortage of cadaveric donor organs led us 6 to consider the use of the liver removed from these subjects in patients awaiting liver transplantation. The lean body habitus and the absence of portal hypertension anticipate an easy hepatectomy. A young stable donor and the possible short cold ischemic time should allow excellent conditions for the recipient. The persistent synthesis of the anomalous TTR by the donated organ creates the potential for development of neuropathy. METHODS |
| Databáze: | OpenAIRE |
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