Expanding donor age in liver transplantation using liver grafts from nonagenarian donors.

INTRODUCTION: Satisfactory outcomes in a series of liver transplantations (LT) with octogenarian liver grafts have been reported, as well as several cases of LT using nonagenarian liver grafts with short follow-up. METHODS: From October 2013 to December 2019, we performed 426 LT. Six LTs used nonagenarian livers (case group) and 49 used octogenarian livers (control group). A comparative analysis was performed between groups. Median donor age was significantly higher in the nonagenarian group than in the octogenarian group (90.6 years versus 83.4 years; (P < .001). There was a significant difference in LT indications (P = .026) between the groups, but not in perioperative recipient variables, morbidity, or mortality. The 1-, 3-, and 5-year patient survival rates were 67.7% in the recipients of nonagenarian livers and 85.7%, 78.0%, and 74.4%, respectively, in the recipients of octogenarian livers (P = .631). The 1-, 3-, and 5-year graft survival rates were 66.7% in the recipients of nonagenarian livers and 81.3%, 73.8%, and 70.3%, respectively, in the recipients of octogenarian livers (P = .745). CONCLUSIONS: The results of LT with nonagenarian liver grafts are not significantly different from those obtained with octogenarian donors, taking into consideration the small sample size and careful selection of donors and adequate donor-recipient matching.

T cell-mediated response to SARS-CoV-2 in liver transplant recipients with prior COVID-19.

Whether immunosuppression impairs severe acute respiratory syndrome coronavirus 2-specific T cell-mediated immunity (SARS-CoV-2-CMI) after liver transplantation (LT) remains unknown. We included 31 LT recipients in whom SARS-CoV-2-CMI was assessed by intracellular cytokine staining (ICS) and interferon (IFN)-γ FluoroSpot assay after a median of 103 days from COVID-19 diagnosis. Serum SARS-CoV-2 IgG antibodies were measured by ELISA. A control group of nontransplant immunocompetent patients were matched (1:1 ratio) by age and time from diagnosis. Post-transplant SARS-CoV-2-CMI was detected by ICS in 90.3% (28/31) of recipients, with higher proportions for IFN-γ-producing CD4+ than CD8+ responses (93.5% versus 83.9%). Positive spike-specific and nucleoprotein-specific responses were found by FluoroSpot in 86.7% (26/30) of recipients each, whereas membrane protein-specific response was present in 83.3% (25/30). An inverse correlation was observed between the number of spike-specific IFN-γ-producing SFUs and time from diagnosis (Spearman's rho: -0.418; p value = .024). Two recipients (6.5%) failed to mount either T cell-mediated or IgG responses. There were no significant differences between LT recipients and nontransplant patients in the magnitude of responses by FluoroSpot to any of the antigens. Most LT recipients mount detectable-but declining over time-SARS-CoV-2-CMI after a median of 3 months from COVID-19, with no meaningful differences with immunocompetent patients.

Donor helper innate lymphoid cells are replaced earlier than lineage positive cells and persist long-term in human intestinal grafts - a descriptive study.

Intestinal grafts carry large donor lymphoid load that is replaced by recipient cells. The dynamics of this process may influence the tolerance, rejection or graft-versus-host disease. We analysed distribution and turnover of T and B (Lin+) lymphocytes, natural killer (NK) and helper innate lymphoid cells (hILC) in intestinal epithelium (IEp) and lamina propia (LP) from a long-term cohort of eight intestinal recipients and from a single patient monitored deeply during the first 8 months post-transplant (posTx). Long-term intestinal grafts showed significantly higher %hILC than native bowels in IEp and LP until 10 years posTx and recovery to normal levels was observed afterwards. We also observed an imbalance between hILC subsets in IEp [increase of type 1 (ILC1) and decrease in type 3 (ILC3) innate lymphoid cells] that persisted along posTx time even when %hILC was similar to native bowels. Regarding hILC origin, we still detected the presence of donor cells at 13 years posTx. However, this chimerism was significantly lower than in Lin+ and NK populations. According to these findings, observation from the patient monitored in early posTx period showed that recipient hILC repopulate earlier and faster than Lin+ cells, with increase in ILC1 related to rejection and infection episodes.

Using octreotide for refractory ascites after liver transplantation.

Refractory ascites is a condition associated with a reduced survival and a poorer quality of life. Portal hyperflow after liver transplantation is one of the main causes. We report the case of a female patient with refractory ascites after liver transplantation who was treated with splenic embolization. Ascites persisted despite embolization due to splenic revascularization by short gastric vessels and repeat embolization was technically unfeasible. Based on pathophysiology data, she was treated with octreotide, a somatostatin octapeptide analog, which resulted in splanchnic vasoconstriction and a reduction of the portal flow and venous pressure. After four months of treatment with octreotide, the patient had a good clinical status without ascites.

Direct-acting antivirals are effective and safe in HCV/HIV-coinfected liver transplant recipients who experience recurrence of hepatitis C: A prospective nationwide cohort study.

Direct-acting antivirals have proved to be highly efficacious and safe in monoinfected liver transplant (LT) recipients who experience recurrence of hepatitis C virus (HCV) infection. However, there is a lack of data on effectiveness and tolerability of these regimens in HCV/HIV-coinfected patients who experience recurrence of HCV infection after LT. In this prospective, multicenter cohort study, the outcomes of 47 HCV/HIV-coinfected LT patients who received DAA therapy (with or without ribavirin [RBV]) were compared with those of a matched cohort of 148 HCV-monoinfected LT recipients who received similar treatment. Baseline characteristics were similar in both groups. HCV/HIV-coinfected patients had a median (IQR) CD4 T-cell count of 366 (256-467) cells/µL. HIV-RNA was <50 copies/mL in 96% of patients. The DAA regimens administered were SOF + LDV ± RBV (34%), SOF + SMV ± RBV (31%), SOF + DCV ± RBV (27%), SMV + DCV ± RBV (5%), and 3D (3%), with no differences between the groups. Treatment was well tolerated in both groups. Rates of SVR (negative serum HCV-RNA at 12 weeks after the end of treatment) were high and similar for coinfected and monoinfected patients (95% and 94%, respectively; P = .239). Albeit not significant, a trend toward lower SVR rates among patients with advanced fibrosis (P = .093) and genotype 4 (P = .088) was observed. In conclusion, interferon-free regimens with DAAs for post-LT recurrence of HCV infection in HIV-infected individuals were highly effective and well tolerated, with results comparable to those of HCV-monoinfected patients.

Donor-Specific Antibodies in Pediatric Intestinal and Multivisceral Transplantation: The Role of Liver and Human Leukocyte Antigen Mismatching.

Rejection is one of the most important drawbacks for graft and patient survival in intestinal and multivisceral transplantation. However, there is no consensus on the diagnostic criteria for humoral rejection, and the literature about the role of donor-specific antibodies (DSA) on allograft outcome and the risk factors that contribute to their development is scant with contradictory results. The present study analyzes the role of DSA exclusively in a pediatric cohort of 43 transplants. Among our patients, 11.6% showed preformed DSA, but they did not correlate with more rejection or less allograft survival. Having previous transplants was the main sensitization factor with an odds ratio (OR) = 44.85 (P = 0.001). In total, 16.3% of recipients developed de novo donor-specific antibodies (dnDSA), mostly directed against human leukocyte antigen (HLA) class II, polyspecific and complement fixing. Additionally, the presence of dnDSA had a deleterious effect on graft rejection (hazard ratio [HR] = 11.00; P = 0.01) and survival (HR = 66.52; P < 0.001) in an observational period of 5 years after transplantation. The inclusion of the liver emerged as the main protective factor against dnDSA development with an OR = 0.07 (P = 0.007). The analysis of HLA compatibility at the serological and epitope level with the computational tools HLAMatchmaker and PIRCHE revealed no association between HLA mismatching and dnDSA. In conclusion, this study performed in pediatric recipients shows the deleterious effect of dnDSA on intestinal transplantation supported by the complement-fixing activity observed. Additionally, the liver inclusion in the allografts showed to be a protective factor against dnDSA generation.

Complete splenic embolization for the treatment of refractory ascites after liver transplantation.

Refractory ascites is an uncommon complication that may develop postoperatively after liver transplantation. The diagnosis and treatment of this condition is a real challenge. We report two cases of patients who underwent a transplant due to cryptogenic cirrhosis and developed refractory ascites during the immediate postoperative period. This is a serious complication associated with decreased survival by up to one year and a reduced quality of life. After ruling out the main causes of ascites, a portal hyperflow was a potential etiology. This condition perpetuates itself with splenic circulation and brings about a reduction in the hepatic arterial flow. Therefore, if arterial blood flow to the spleen is diminished, venous return and portal circulation will be reduced and arterial blood flow will improve. Splenic artery embolization is a procedure introduced many years ago for the management of splenic artery steal syndrome and small-for-size living donor liver transplantation. This procedure is performed in order to reduce portal hyperflow and consequently, ascites. In conclusion, splenic artery embolization is a therapeutic option for the treatment of refractory ascites after liver transplantation.

Tumor recurrence after liver transplantation for diffuse biliary papillomatosis in the absence of invasive carcinoma.

Intraductal papillary neoplasm of the bile duct (IPNB) or biliary papillomatosis (BP) is a premalignant entity with high risk of malignant transformation. When the disease extends widely from the intrahepatic to the extrahepatic biliary tree, liver transplantation (LT) is the only option available. We present the case of a 43-year-old male who was admitted in our hospital with an acute cholangitis. He was diagnosed of diffuse biliary and pancreatic papillomatosis. Firstly, we performed a cephalic pancreaticoduodenectomy, then we completed a total pancreatectomy, and finally, after confirming the absence of foci of carcinoma infiltration or lymph nodes involvement, a LT was performed. Foci of carcinoma infiltration or lymph nodes involvement in the liver were not found. After a two-year follow-up the patient developed liver recurrence and the biopsy showed a biliary adenocarcinoma. In 2010, Vibert et al. published a series of three cases concluding that in the absence of invasive carcinoma and positive lymph nodes, LT can be performed with success. The present case is the first to describe recurrence of the disease after LT in the absence of invasive carcinoma and positive lymph nodes in the literature. When the disease affects widely the entire biliary duct, small micro-invasive foci may not be detected. Nevertheless, although we know that it is a recurrent entity, the pathogenesis is unknown, and we do not know if it is possible that papillomatosis recurs over the new liver.

Liver-kidney simultaneous transplantation in adult patients with primary hyperoxaluria. Experience at Hospital Universitario 12 de Octubre.

Primary hyperoxaluria (PH) is a metabolic liver disease with an autosomal recessive inheritance that results in oxalate overproduction that cannot be metabolized by the liver. Urinary excretion of oxalate results in lithiasis and nephrocalcinosis leading to a progressive loss of renal function that often requires renal replacement therapy despite medical treatment. Type 1 PH is the most common form and is due to a deficiency in the alanine-glycolate aminotransferase enzyme found in hepatic peroxisomes. Therefore, a liver-kidney simultaneous transplant (LKST) is the definitive treatment for end-stage renal disease (ESRD) patients. However, some studies suggest that the morbidity and mortality rates are greater when this procedure is performed instead of only a kidney transplant (IKT). Herein, we report five patients with PH and a mean glomerular filtration rate of 20.2 ± 1.3 ml/min/1.73 m2 who received a LKST between 1999 and 2015 at the Hospital Universitario 12 de Octubre. Recurrence and liver or kidney graft loss was not observed during the postoperative period and only one case of late acute rejection without graft loss was diagnosed. The recipient survival rate was 100% with a median follow up of 84 months. As LKST is a curative and safe procedure with a low mortality and high survival rate, it must be considered as the treatment of choice in adults with HP and ESRD.

5-gene differential expression predicts stability of human intestinal allografts.

In intestinal allografts, endoscopy and histology detect the injury once changes in the bowel wall architecture have occurred. We aimed to identify a molecular signature that could predict early deterioration, within histologically indistinguishable biopsies with "minimal changes" (MC) pathology. Sixty biopsies from 12 adult recipients were longitudinally taken during 8years post-transplant. They were classified as either stable (STA) or non-stable (NSTA) according to the prospectively recorded number, frequency and severity of rejection events of the allograft. In a discovery set of MC samples analyzed by RNA-Seq, 816 genes were differentially expressed in STA vs NSTA biopsies. A group of 5 genes (ADH1C, SLC39A4, CYP4F2, OPTN and PDZK1) correctly classified all NSTA biopsies in the discovery set and all STA biopsies from an independent set. These results were validated by qPCR in a new group of MC biopsies. Based on a logistic regression model, a cutoff of 0.28 predicted the probability of being a NSTA biopsy with 85% sensitivity and 69% specificity. In conclusion, by analyzing MC samples early after transplantation, the expression of a 5-gene set may predict the evolution of the bowel allograft. This prognostic biomarker may be of help to personalize care of the intestinal transplant recipient.

Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment. / Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico.

INTRODUCTION: Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors. METHODS: A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014. RESULTS: Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer. CONCLUSIONS: Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery.

Long-term results using old liver grafts for transplantation: sexagenerian versus liver donors older than 70 years.

BACKGROUND: The most practical measure to augment the available number of liver grafts and thus reduce waiting list mortality is to increase the donor age limit. We hypothesized that with careful selection of old liver donors without age limit it should be possible to obtain good patient and graft survival. METHODS: The present study comprises 351 adults who underwent liver transplantation. They were divided into three groups according to the age of the liver donors: group 1: 226 recipients of donors <60 years; group 2: 75 recipients of donors between 60 and 70 years; and group 3: 50 recipients of donors >70 years. A comparative study among the groups was performed. RESULTS: Patient survival rates at 1, 3, and 5 years were, respectively, 81.0, 76.1, and 71.1 % in group 1; 83.8, 74, and 72.2 % in group 2; and 76, 70.0, and 62.9 % in group 3 (P = NS). Graft survival at 1, 3, and 5 years was, respectively, 74.8, 69.0, and 64.1 % in group 1; 82.7, 71.4, and 69.6 % in group 2; and 71.4, 64.8, and 58.3 % in group 3 (P = NS). We analyzed the use of older grafts in recipients with HCV cirrhosis and did not find significant differences in patient and graft survival at 1, 3, and 5 years. In multivariate analysis increased donor body mass index and decreased recipient albumin were associated with lower patient and graft survival. CONCLUSIONS: Because patient and graft survival rates are not affected by donor age, well-selected older donor livers can be safely used if they show good function and preharvesting conditions.

Post-transplant biliary complications using liver grafts from deceased donors older than 70 years: Retrospective case-control study.

BACKGROUND: The shortage of liver grafts and subsequent waitlist mortality led us to expand the donor pool using liver grafts from older donors. AIM: To determine the incidence, outcomes, and risk factors for biliary complications (BC) in liver transplantation (LT) using liver grafts from donors aged > 70 years. METHODS: Between January 1994 and December 31, 2019, 297 LTs were performed using donors older than 70 years. After excluding 47 LT for several reasons, we divided 250 LTs into two groups, namely post-LT BC (n = 21) and without BC (n = 229). This retrospective case-control study compared both groups. RESULTS: Choledocho-choledochostomy without T-tube was the most frequent technique (76.2% in the BC group vs 92.6% in the non-BC group). Twenty-one patients (8.4%) developed BC (13 anastomotic strictures, 7 biliary leakages, and 1 non-anastomotic biliary stricture). Nine patients underwent percutaneous balloon dilation and nine required a Roux-en-Y hepaticojejunostomy because of dilation failure. The incidence of post-LT complications (graft dysfunction, rejection, renal failure, and non-BC reoperations) was similar in both groups. There were no significant differences in the patient and graft survival between the groups. Moreover, only three deaths were attributed to BC. While female donors were protective factors for BC, donor cardiac arrest was a risk factor. CONCLUSION: The incidence of BC was relatively low on using liver grafts > 70 years. It could be managed in most cases by percutaneous dilation or Roux-en-Y hepaticojejunostomy, without significant differences in the patient or graft survival between the groups.

Cystic liver neoplams: A single centre experience and literature review. / Neoplasias quísticas hepáticas: experiencia en un único centro y revisión de la literatura.

INTRODUCTION: The hepatic cystic tumour is a very rare neoplasm, representing about 5% of all cystic liver neoplasms. The preoperative diagnosis is difficult and can lead to confusion. The aim of this study is to analyze a number of cases operated at our centre with an histologic diagnosis of liver cystic neoplasms and also to describe the sintomathology, diagnosis and management as per the recent classification. METHODS: A retrospective analysis was performed including all the cystic liver neoplasms operated between January 2000 and December 2019. The study was performed based on the pre-existing pathology archives. The 2010 previous cases were reclassified following the new 2010 OMS classification. RESULTS: The study sample was of 10 patients, identifying 6 of them as mucinous cystic liver neoplasms, and the other 4 as intraductal papillary biliary neoplasms. The majority of the patients were women (8/10) and the median age was 47 years. Regarding the treatment, 3 hepatectomy and 7 enucleations were performed. Frozen section intraoperatively was not required in any case. In one case, variable cellular atypia with areas of adenocarcinoma was observed, and the patient received neoadyuvant chemotherapy with taxol and carboplatin. In all cases the resection margins were negative. CONCLUSION: Cystic liver neoplasms are infrequent tumours with a difficult differential diagnosis. Therefore, with a high radiological suspicious, the treatment should be a complete resection to avoid recurrences and malignancies.

Analyzing predictors of graft survival in patients undergoing liver transplantation with donors aged 70 years and over.

AIM: To increase the number of available grafts. METHODS: This is a single-center comparative analysis performed between April 1986 and May 2016. Two hundred and twelve liver transplantation (LT) were performed with donors ≥ 70 years old (study group). Then, we selected the first cases that were performed with donors < 70 years old immediately after the ones that were performed with donors ≥ 70 years old (control group). RESULTS: Graft and patient survivals were similar between both groups without increasing the risk of complications, especially primary non-function, vascular complications and biliary complications. We identified 5 risk factors as independent predictors of graft survival: recipient hepatitis C virus (HCV)-positivity [hazard ratio (HR) = 2.35; 95% confidence interval (CI): 1.55-3.56; P = 0.00]; recipient age (HR = 1.04; 95%CI: 1.02-1.06; P = 0.00); donor age X model for end-stage liver disease (D-MELD) (HR = 1.00; 95%CI: 1.00-1.00; P = 0.00); donor value of serum glutamic-pyruvic transaminase (HR = 1.00; 95%CI: 1.00-1.00; P = 0.00); and donor value of serum sodium (HR = 0.96; 95%CI: 0.94-0.99; P = 0.00). After combining D-MELD and recipient age we obtained a new scoring system that we called DR-MELD (donor age X recipient age X MELD). Graft survival significantly decreased in patients with a DR-MELD score ≥ 75000, especially in HCV patients (77% vs 63% at 5 years in HCV-negative patients, P = 0.00; and 61% vs 25% at 5 years in HCV-positive patients; P = 0.00). CONCLUSION: A DR-MELD ≥ 75000 must be avoided in order to obtain the best results in LT with donors ≥ 70 years old.

Simultaneous pancreas-kidney transplantation. Experience of the Doce de Octubre Hospital. / Trasplante de páncreas-riñón simultáneo. Experiencia del Hospital Doce de Octubre.

INTRODUCTION: Simultaneous pancreas-kidney transplantation (SPKT) constitutes the therapy of choice for diabetes type1 or type2 associated with end-stage renal disease, because is the only proven method to restore normo-glicemic control in the diabetic patient. METHODS: Retrospective and descriptive study of a series of 175 patients who underwent SPKT from March 1995 to April 2016. We analyze donor and recipient characteristics, perioperative variables and immunosuppression, post-transplant morbi-mortality, patient and graft survival, and risk factors related with patient and graft survival. RESULTS: Median age of the donors was 28years and mean age of recipients was 38.8±7.3years, being 103 males and 72 females. Enteric drainage of the exocrine pancreas was performed in 113 patients and bladder drainage in 62. Regarding post-transplant complications, the overall rate of infections was 70.3%; graft pancreatitis 26.3%; intraabdominal bleeding 17.7%; graft thrombosis 12.6%; and overall pancreas graft rejection 10.9%. The causes of mortality were mainly cardiovascular and infectious complications. Patient survival at 1, 3 and 5-year were 95.4%, 93% and 92.4%, respectively, and pancreas graft survival at 1, 3 and 5-year were 81.6%, 77.9% y 72.3%, respectively. CONCLUSIONS: In our 20-year experience of simultaneous pancreas-kidney transplantation, the morbidity rate, and 5-year patient and pancreas graft survivals were similar to those previously reported from the international pancreas transplant registries.

Complications of transarterial chemoembolization (TACE) in the treatment of liver tumors. / Complicaciones de la quimioembolización transarterial (QETA) en el tratamiento de los tumores hepáticos.

INTRODUCTION: Transarterial chemoembolization (TACE) is considered a therapeutic option. It is mostly used in hepatocellular carcinoma or liver colorectal, neuroendocrine or melanoma metastases. Although it is considered a safe procedure, TACE presents complications, such as acute cholecystitis, which is the most common. Other procedure-related complications include pulmonary embolism, hepatic abscess, bile duct injury, gastric mucosa injury and, less frequently, acute pancreatitis. The aim of this study is to review the complications following TACE for liver tumors. METHODS: We performed a retrospective study including all the TACE procedures performed in a single center during the period between January 2013 and December 2016. RESULTS: Out of the 196 patients with liver tumors who had undergone 322 TACE, 258 (80%) were male and 64 (20%) were female. Mean patient age was 66.5years. Major complications after chemoembolization included: decompensation with edema/ascites (6patients), acute cholecystitis (4), acute pancreatitis (3), liver rupture (1), liver abscess (1) and renal failure (1). Postembolization syndrome appeared in 71 (20%) patients. On multivariate analysis, it was observed that concomitant cardiovascular disease (OR: 4.5; 95%CI: 1.2-17; P=.025) is a risk factor for the development of complications. CONCLUSIONS: TACE is a safe and effective procedure for liver tumor treatment. The majority of the complications are rare and present a low incidence of mortality.

Incidence, risk factors and outcome of de novo tumors in liver transplant recipients focusing on alcoholic cirrhosis.

Orthotopic liver transplantation (OLT) is an established life-saving procedure for alcoholic cirrhotic (AC) patients, but the incidence of de novo tumors ranges between 2.6% and 15.7% and is significantly increased in comparison with patients who undergo OLT for other etiologies. Tobacco, a known carcinogen, has been reported to be between 52% and 83.3% in AC patients before OLT. Other risk factors that contribute to the development of malignancies are dose-dependent immunosuppression, advanced age, viral infections, sun exposure, and premalignant lesions (inflammatory bowel disease, Barrett's esophagus). A significantly more frequent incidence of upper aerodigestive (UAD) tract, lung, skin, and kidney-bladder tumors has been found in OLT recipients for AC in comparison with other etiologies. Liver transplant recipients who develop de novo non-skin tumors have a decreased long-term survival rate compared with controls. This significantly lower survival rate is more evident in AC recipients who develop UAD tract or lung tumors after OLT mainly because the diagnosis is usually performed at an advanced stage. All transplant candidates, especially AC patients, should be encouraged to cease smoking and alcohol consumption in the pre- and post-OLT periods, use skin protection, avoid sun exposure and over-immunosuppression, and have a yearly otopharyngolaryngeal exploration and chest computed tomography scan in order to prevent or reduce the incidence of de novo malignancies. Although still under investigation, substitution of calcineurin inhibitors for sirolimus or everolimus may reduce the incidence of de novo tumors after OLT.

[Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years]. / Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años.

BACKGROUND AND OBJECTIVE: Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. MATERIAL AND METHOD: Six patients with PAF underwent liver transplantation between April 1986 and December 2012. RESULTS: The mean age was 57.7+16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart-liver transplants sequentially were performed. Patient survival and graft was 80% at one, 3 and 5 years. CONCLUSIONS: The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage.