Fig. 1.

Cardiovascular autonomic testing and gastrointestinal biopsy in a patient with acquired amyloid polyneuropathy after domino liver transplant. a–d Representative test results of a 76-year-old female who, as a result of autoimmune liver failure, had a liver transplant at age 55 years. She suffered a liver transplant rejection and required a second liver transplant at age 69. The liver she received was from a donor with hereditary transthyretin amyloidosis (Val71Ala mutation), who had recently died after several years of severe sensory and autonomic neuropathy (i.e., domino liver transplant). Approximately 1 year after receiving the second liver transplant, the patient developed severe diarrhea, exercise intolerance and painful tingling in the feet. Two years after the liver transplant her tingling had worsened significantly and she developed dry mouth and neurogenic bladder. At age 75 she developed severe weight loss (15–20 lb), recurrent orthostatic dizziness and lightheadedness and suffered frequent episodes of loss of consciousness upon standing. She became wheelchair bound at age 76. a Cardiovascular autonomic testing showing absent blood pressure overshoot after release of the Valsalva strain (dashed arrow), indicating impaired baroreflex-mediated sympathetic activation. b Tilt-table test showing a supine blood pressure of 133/70 mmHg with a heart rate of 79 bpm. After 15 min of head-up tilt, her blood pressure had fallen to 76/40 mmHg and her heart rate was 88 bpm, consistent with severe neurogenic orthostatic hypotension. To confirm that her sensory and autonomic neuropathy was caused by amyloid, she underwent a upper gastrointestinal endoscopy and biopsies from the stomach and duodenum were obtained. c and d Congo red stain in upper gastrointestinal tissue showing abundant amyloid deposition in the muscularis mucosae (arrows)