Transthyretin amyloidosis is a progressive and eventually fatal disease primarily characterized by sensory, motor, and autonomic neuropathy and/or cardiomyopathy. Given its phenotypic unpredictability and variability, transthyretin amyloidosis can be difficult to recognize and manage. Misdiagnosis is common, and patients may wait several years before accurate diagnosis, risking additional significant irreversible deterioration. This article aims to help physicians better understand transthyretin amyloidosis—and, specifically, familial amyloidotic polyneuropathy—so they can recognize and manage the disease more easily and discuss it with their patients. We provide guidance on making a definitive diagnosis, explain methods for disease staging and evaluation of disease progression, and discuss symptom mitigation and treatment strategies, including liver transplant and several pharmacotherapies that have shown promise in clinical trials.

Risk factors for invasive fungal infection in patients undergoing orthotopic liver transplantation were examined. Thirty-four of 168 transplants were complicated within 100 days after transplantation by documented invasive fungal infection (Candida species, 28 patients; mycelial fungi, 5; both Candida and Aspergillus species, 1). In the multivariate Cox proportional hazards model, three baseline and two posttransplant variables were independently significant risk factors for infection: level of creatinine (hazard ratio = 1.4), length of transplant operation (HR = 1.2), retransplantation (HR = 3.2), abdominal or intrathoracic reoperations (HR = 2.5), and cytomegalovirus infection (HR = 8.5). Four predictors (creatinine of > 3.0 mg/dL, operative time of > or = 11 h, retransplantation, and early colonization) assessable at the time of transplantation or shortly thereafter were incorporated into a simple predictive model for risk stratification. The risk of invasive fungal infection ranged from 1% in patients with no predictors to 67% in patients with two or more predictors. Strategies to prevent invasive fungal infections after liver transplantation should be targeted to these high-risk groups.

Liver regeneration continues throughout the first postoperative year. Only one donor achieved complete liver regeneration during this time period; however, all donors have maintained normal liver function without long-term complications. Longer follow-up is needed to determine whether donors ever achieve original TLV.

Autoimmune hepatitis (AIH) after liver transplantation (LT) may recur and is difficult to diagnose. Our aims were to define the histopathology of and factors related to AIH recurrence. Fourteen of 475 patients received LT for AIH; 2 died perioperatively. Liver specimens (native and post-LT biopsies) from 12 other patients were reviewed and correlated with pre-and post-LT clinical course and outcome. Recurrent AIH was seen in 5 of 12 patients, 35 to 280 days post-LT as lobular hepatitis with acidophil bodies and lymphoplasmacytic infiltrate. Portal/interface hepatitis was seen with disease progression and 2 of 5 patients developed cirrhosis. Of 7 nonrecurrent patients, 1 had acquired hepatitis C with lobular/portal hepatitis and none developed cirrhosis. Histology suggestive of overlap syndrome was seen in 3 of 12 native livers with no effect on post-LT course or pathology. High-grade necroinflammation was present in native livers at LT in 5 of 5 cases with recurrent AIH and in 1 of 7 without recurrence (P < .01). Pre-LT disease duration, donor/recipient gender distribution, HLA studies, and rejection episodes did not correlate with AIH recurrence. We conclude that (1) recurrent AIH is not uncommon and was seen in 42% of patients with lymphoplasmacytic lobular, portal, and interface hepatitis; (2) acidophil bodies with lymphoplasmacytic cells are seen in early recurrent AIH; (3) recurrent AIH appears at variable time periods post-LT, and the progression is slow; and (4) high-grade inflammation in native liver at LT is a strong predictor of recurrent AIH. (HEPATOLOGY 2000;32:185-192.)