Impact of liver transplantation on familial amyloidotic polyneuropathy (FAP) patients' symptoms and complications.
Liver transplantation for transthyretin (TTR) amyloidosis is today an accepted treatment for the disease. However, the long-term outcome of the procedure remains to be elucidated, especially for non-Val30Met TTR mutations. Using serum amyloid P (SAP) component scintigraphy, amyloid deposits have been shown to decrease in approximately 50% of the patients, but this finding has not been confirmed in biopsy specimens. However, free radical activity has been reduced after transplantation. Conflicting results regarding neurological disturbances have emerged, but some improvement may take place, especially in patients transplanted at an early stage of the disease. For heart complications, recent reports indicate that increased cardiomyopathy after transplantation is a common finding, and that the development of arrhythmia is also common. Even though an improvement of the patients' nutritional status is observed after transplantation, gastrointestinal disturbances appear to be unchanged after the procedure. Kidney function deteriorates initially after transplantation, but thereafter it remains stable. Since TTR is produced locally in the eye, development of amyloid deposits in the eye has been reported in several cases after transplantation. Transplantation for FAP should be performed early on after onset of the disease, since the majority of symptoms will remain unchanged after the procedure. The patients should be followed closely after transplantation to detect the development of heart arrhythmia.
Suhr
Ole B
OB
Department of Medicine, Ume University Hospital, SE 901 85 Ume, Sweden. ole.suhr@medin.umu.se
D028227:Amyloid Neuropathies, Familial / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D009202:Cardiomyopathies / Q000150:complications*; D005128:Eye Diseases / Q000150:complications; D005500:Follow-Up Studies; D005767:Gastrointestinal Diseases / Q000150:complications*; D006801:Humans; D007674:Kidney Diseases / Q000150:complications*; D016031:Liver Transplantation / Q000009:adverse effects*; D011228:Prealbumin / Q000235:genetics / Q000378:metabolism; D000683:Serum Amyloid P-Component / Q000378:metabolism
D011228:Prealbumin; D000683:Serum Amyloid P-Component