Partial-Liver Transplantation To Treat Familial Amyloid Polyneuropathy: Follow-up of 11 Patients

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Abstract
Recently, liver transplantation has been used to treat patients with familial amyloid polyneuropathy (FAP). To describe the clinical course of patients with FAP who received partial-liver transplantation from living donors. Case series. University hospital in Matsumoto, Japan. 11 patients with FAP who underwent partial-liver transplantation. The transthyretin gene abnormality in all 11 patients was the substitution of methionine for valine at position 30. Partial liver transplantation from living donors. Preoperative and follow-up (3 to 64 months) clinical data, including routine laboratory data, nerve conduction velocity tests, and sural nerve histology. All 7 patients who had severe gastrointestinal autonomic disorders or polyneuropathy localized to the lower limbs for less than 4 years showed improvement. Three of 4 patients with polyneuropathy involving both the upper and lower limbs had adverse outcomes, including two deaths. The preoperative duration of their illness was more than 6 years. These 3 patients also had marked decreases in creatinine clearance and nerve conduction velocities and severe loss of myelinated fibers in sural nerves. Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.