geoscience.net logo
+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Outcome of liver transplantation for transthyretin amyloidosis: Follow-up of Japanese familial amyloidotic polyneuropathy patients


, : Outcome of liver transplantation for transthyretin amyloidosis: Follow-up of Japanese familial amyloidotic polyneuropathy patients. Journal of the Neurological Sciences 171(1): 19-23, Dec 1

Since 1990, liver transplantation for familial amyloidotic polyneuropathy (FAP) has been carried out world-wide, and the outcome of the procedure seems to be promising. FAP is inherited systemic disease caused by mutated transthyretin. The most common cause is the valine to methionine substitution at position 30 (Met30). We have developed a scoring system for FAP Met30 that takes into account a variety of clinical symptoms of the disease. Six patients with FAP Met30 underwent extensive examinations according to our scoring system before and after transplantation. All patients survived the procedure and are alive after transplantation. Improvements of sensory and autonomic disturbances were observed during the initial 12 months after the procedure only, thereafter the patients' status remained unchanged. Following transplantation, no improvement of motor function and visceral organ damage were observed, but the modified body mass index improved in four of six patients after the operation. These results suggest that liver transplantation of FAP patients stops the progress of the disease, and that minor improvements are noted in several patients after the procedure. However, transplantation should be performed early after the onset of the disease in order to preserve the patients' functional status.

US$19.90

PMID: 10567045

DOI: 10.1016/s0022-510x(99)00231-2


Other references

Rodriquez, C., 1969: Some species of promising fora. Revista cafetalera: 86 28-29

Dixon, R.M., 1964: Obtaining aerial photographs for photo interpretation in forestry. A summary of advice and information on the timing, scale, materials, equipment, contracts, etc. for aerial photography for forestry purposes in Chile.

Gutiérrez, J.P.; Berkowitz, R.G.; Robertson, C.F., 1999: Vallecular cysts in newborns and young infants. Vallecular cyst is a rare cause of stridor and respiratory distress in infancy and has been associated with sudden airway obstruction resulting in death. In a retrospective review of eight cases over a 20-year period all infants developed symptoms...

D.L.o; Vallerini, A.; Magni, G., 1984: Eysenck Personality Inventory and choice of psychiatry as a career. The case of a patient who refused to give the analyst his name for over a year and a half is presented. Analysis of his resistances and transference reactions points to the fact that he unconsciously wanted to be raped. Analysis of other secrets r...

Clever, Ulrich, 1967: Control of chromosome puffing. Annual Meeting of the Society of General Physiologists: Symposium on cytoplasmic and environmental influences on nuclear behavior, 31 August-3 September, 1966, Woods Hole, Mass 161-186

Jalenques, I.; Coudert, A.J., 1992: A new therapeutic approach tp drug-resistant schizophrenia: clozapine. Long-term prospective study in 16 patients. Clozapine, a dibenzodiazepine derivative, has potent antipsychotic activity; but bone marrow suppression resulting in agranulocytosis has been associated with clozapine treatment and has restricted the administration of this drug to treatment-resi...

Machala, O., 1965: On the detection of microbial aerosols with the membrane filter method. Zentralblatt für Biologische Aerosolforschung 12(6): 519-529

Datta A.K.; Fleming J.R.; Hortobagyi T.; Stephens J.A., 1985: Synchronization of motor unit firing in different muscles recorded during voluntary isometric contractions in man. Society for Neuroscience Abstracts 11(1): 702

Hartog C.D., 1972: Influence of nutrition in youth on health in later years and some other matters. Voeding (The Hague) 33(11): 529-541

Bennis-Bryan, K.; Miles, RS., 1983: Corrigendum: renaming of Kimberia Dennis-Bryan & Miles, 1983. Zoological Journal of the Linnean Society, 784: 397