Liver retransplantation in patients with acquired familial amyloid polyneuropathy: a Portuguese center experience.

In 1995 Furtado et al performed the first domino transplantation using a donor liver with familial amyloid polyneuropathy (FAP), thereby increasing the pool of donors. Our experience showed that the onset of FAP symptoms occurs earlier in some patients. Patients with FAP acquired by transplantation are candidates for liver retransplantation to minimize the progression of symptoms. Liver retransplantation is considered to be a high-risk procedure and has lower survival compared with the first transplantation. We evaluated the risk of liver retransplantation in patients with acquired FAP. We did a retrospective analysis of these patients based on the records of perioperative data. From 1995 to 2004 we carried out 81 domino transplantations, of which 10 were submitted to liver retransplantation because of acquired FAP. The better outcomes in this group lead us to think that the liver retransplantation in patients with acquired FAP is not associated with the same risks of liver retransplantation in candidates with graft failure.

Evaluation of operative risk in de novo familial amyloid polyneuropathy retransplantation.

Familial amyloid polyneuropathy (FAP) is the most common hereditary amyloidosis, characterized by progressive peripheral sensory and motor neuropathy. The livers of patients with FAP are used in domino liver transplantation in selected cases to increase the number of grafts available. In our department 10 patients underwent liver retransplantation (ReLTx) in the absence of liver dysfunction by de novo FAP after domino liver transplantation. Our aim was to compare the differences in the consumption of blood products and intraoperative hemodynamic support among patients with FAP undergoing liver transplantation (LTx) and patients with de novo FAP undergoing ReLTx in the same time frame. The anesthetic records of all patients who underwent LTx for FAP and ReLTx for de novo FAP were analyzed, from January 2009 to May 2014. Patients were divided into 2 groups: group 1 patients with FAP, and group 2 patients with de novo FAP. Statistical differences in the value of preoperative creatinine were found. Hemoglobin levels, preoperative international normalized ratio (INR), use of blood products, aminergic support, and surgical time showed no statistical difference. Major bleeding rates would be expected in patients undergoing ReLTx. Changes in renal function, chronic immunosuppressive therapy, and age may contribute to the increase in intraoperative complications. We did not find statistically significant differences, leading us to the conclusion that de novo FAP does not seem to be a predictor of perioperative risk.

Incidentally discovered hepatocellular carcinoma in explanted liver: clinical, histopathologic features and outcome.

BACKGROUND: Incidental hepatocellular carcinomas (iHCCs) are tumors discovered on the explanted liver that were not present on imaging before transplantation. The natural history, histopathologic characteristics, and prognosis are not clearly defined. METHODS: We compared the characteristics of iHCC and previously known hepatocellular carcinoma (pkHCC) in patients who underwent liver transplantation from 1998 to 2012 in a retrospective study. RESULTS: During this period a total of 675 patients were transplanted; 56 patients (9%) had pkHCC and 12 (2%) had iHCC. The sex and age distributions were similar. The median Model for End-Stage Liver Disease score in iHCC patients was 17.0 versus 13.0 in patients with pkHCC (P = .001). Thirty-three percent of iHCC patients had multiple tumors, and 25% had bilobar involvement. The median cumulative tumor size in iHCC was 1.8 cm, and 5.5 cm in pkHCC (P = .005). Incidence of microvascular invasion was not different (16.7% vs 38.9%; P = .191). American Joint Committee on Cancer T1 stage was found in 58.3% of patients with iHCC and in 22.2% of pkHCC patients. Patients with iHCC had 1-, 3-, and 5-year survivals, respectively, of 100%, 83% and 64%, compared with 80%, 66%, and 38% for patients with pkHCC (P = .138). None of the patients with iHCC had recurrence of HCC, whereas incidence of recurrence in pkHCC patients was 12.5%. CONCLUSIONS: iHCC occurred in patients with more advanced liver disease. The cumulative tumor size of iHCC was smaller but one-third were multifocal. Survival was similar to patients with pkHCC, and recurrence was not noted in patients with iHCC.

Churg-Strauss syndrome: case series.

Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma. This is a rare syndrome of unknown etiology, affecting both genders and all age groups. CSS patients usually respond well to steroid treatment, although relapses are common after it ends. Timely diagnosis and treatment generally lead to a good prognosis with a 90% survival rate at one year. A brief review of CSS is presented, with particular attention to diagnosis, therapy and recent developments in this area. The authors then report and discuss the clinical, laboratory and imaging characteristics of four patients admitted to an Internal Medicine Department with this diagnosis. The treatment, response and follow-up of the cases are also described.