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.

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.

Intestinal obstruction secondary to Crohn-like disease in common variable immu-nodeficiency.

A 32-year-old male patient with common variable immunodeficiency (CVI), under treatment with human immunoglobulin G, experienced recurrent episodes of intestinal pseudo-obstruction that were medically treated. Imaging tests showed a non-obstructive ileal stenosis and biopsies performed during ileocolonoscopy revealed nod-ular lymphoid hyperplasia.

Liver transplant in a receptor with ABO incompatibility: a viable option.

We present the case of a 41-year-old male with type A blood group and a diagnosis of fulminant liver failure of an unknown cause, who was admitted to the Intensive Care Unit (ICU) with severe coagulopathy (INR 6), hyperammonemia (250 umol/l) and grade IV encephalopathy (four King's criteria and both Clichy's criteria). He also had GCS 8/15, despite TDE-MARS, dialysis and ventilator support via endotracheal tube due to encephalopathy. He was registered as status 0 for liver transplant. In the absence of a compatible donor, the patient received an ABO incompatible liver transplant (LT), group O, using the piggyback technique, without any complications. He required transfusion of five units of plasma but did not require a blood transfusion.

Fístulo-yeyunostomía como alternativa de tratamiento en la reparación de una fístula biliar compleja / Fistulo-jejunostomy as an alternative treatment in the repair of a complex biliary leak

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Uso de la octreótida en ascitis refractaria después de un trasplante hepático / Using octreotide for refractory ascites after liver transplantation

La ascitis refractaria es una patología que se asocia con una disminución de la supervivencia del paciente y un empeoramiento de su calidad de vida. Una de las causas de la misma después de un trasplante hepático es el hiperaflujo portal. Presentamos el caso de una paciente con ascitis refractaria postrasplante hepático a la cual se le realiza como tratamiento una embolización esplénica. La ascitis persiste a pesar de la embolización por repermeabilización del bazo por los vasos gástricos cortos, siendo técnicamente imposible una nueva embolización. Se decide iniciar tratamiento con octreótida, un octapéptido análogo de la somatostina, con base en su fisiopatología al producir vasoconstricción esplácnica, lo que reduce el flujo y la presión venosa portal. A los cuatro meses del inicio del tratamiento con octreótida, la paciente presenta una buena situación clínica y sin ascitis

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

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.

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

INTRODUCCIÓN: La quimioembolización transarterial (QETA) es considerada una opción terapéutica utilizada en el tratamiento del carcinoma hepatocelular y de las metástasis hepáticas secundarias del carcinoma colorrectal, tumores neuroendocrinos y melanoma ocular. Aunque es un procedimiento seguro, no está exento de complicaciones, siendo la más frecuente la colecistitis aguda. Otras complicaciones descritas son el tromboembolismo pulmonar, el absceso hepático, lesiones de la mucosa gastrointestinal, lesiones de la vía biliar, etc. El objetivo principal del estudio es revisar y describir las complicaciones derivadas de la QETA en el tratamiento de los tumores hepáticos. MÉTODOS: Se ha realizado un análisis retrospectivo de todas las QETA practicadas en nuestro centro entre enero de 2013 y diciembre de 2016. En dicho periodo se realizaron 322 QETA en 196 pacientes. RESULTADOS: Del total de procedimientos, 258 (80%) fueron realizados en hombres y 64 (20%) en mujeres. Además, la edad media de los pacientes fue de 66,5años. Las complicaciones mayores derivadas de la QETA fueron descompensación edemo-ascítica (6 casos), colecistitis aguda (4), pancreatitis aguda (3), rotura hepática (1), absceso hepático (1) y deterioro de la función renal (1). Además, el síndrome postembolización se objetivó en 71 (22%) casos. En el análisis multivariante se observó que el antecedente cardiovascular (OR: 4,5; IC95%: 1,2-17; p = 0,025) es un factor de riesgo para el desarrollo de complicaciones post-QETA. CONCLUSIONES: Las complicaciones derivadas de la QETA son poco frecuentes y con una baja incidencia de mortalidad

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 (6 patients), 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

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.

Trasplante hepatorrenal simultáneo en pacientes adultos con hiperoxaluria primaria. Experiencia del Hospital Universitario 12 de Octubre / Liver-kidney simultaneous transplantation in adult patients with primary hyperoxaluria. Experience at Hospital Universitario 12 de Octubre

La hiperoxaluria primaria es un trastorno del metabolismo autosómico recesivo que origina una hiperproducción hepática de oxalato, que no puede ser metabolizado por el hígado y se elimina por vía renal formando litiasis, nefrocalcinosis y ocasionando un deterioro progresivo y precoz de la función renal que, generalmente, a pesar del tratamiento médico precisará de una terapia renal sustitutiva. La hiperoxaluria primaria (HOP) tipo 1 es el trastorno más frecuente, se debe a un déficit de la enzima alanina-glicolato aminotransferasa que se encuentra en los peroxisomas hepáticos. Por tanto, el trasplante hepatorrenal simultáneo (THRS) es el tratamiento definitivo para los pacientes con enfermedad renal crónica terminal. Sin embargo, algunos resultados sugieren que la morbimortalidad es mayor al realizar este procedimiento frente al trasplante renal aislado. Se presentan cinco pacientes adultos con hiperoxaluria primaria y un filtrado glomerular medio de 20,2 ± 1,3 ml/min/1,73 m2 a los que se les realizó un THRS entre 1999 y 2015 en el Hospital Universitario 12 de Octubre. No se observó recurrencia de la enfermedad ni pérdida del injerto hepático o renal durante el postoperatorio y únicamente un episodio de rechazo agudo tardío sin pérdida del injerto renal. La supervivencia de los receptores fue del 100% con una mediana de seguimiento de 84 meses. Debido a que el THRS permite la curación de la enfermedad y constituye una técnica segura, con una baja morbimortalidad y elevada supervivencia, debe considerarse como el tratamiento de elección en la hiperoxaluria primaria con enfermedad renal terminal (AU)

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 (AU)

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

Introducción: El trasplante de páncreas-riñón simultáneo constituye el tratamiento de elección en la diabetes tipo 1 o tipo 2 con fallo renal terminal o preterminal (diálisis o prediálisis), por ser la única terapia que consigue el estado euglucémico (insulino-independiente) en el paciente diabético. Métodos: Estudio retrospectivo y descriptivo de una serie de 175 pacientes trasplantados de páncreas-riñón simultáneo entre marzo de 1995 y abril de 2016. Se analizan las características de los donantes y receptores, variables perioperatorias e inmunosupresión, morbimortalidad postrasplante, supervivencia del paciente e injerto y factores de riesgo de supervivencia del paciente e injerto. Resultados: La mediana de edad de los donantes fue de 28 años y la media de los receptores, de 38,8 ± 7,3años, siendo 103 hombres y 72 mujeres. La derivación duodeno-entérica se realizó en 113 casos y la duodeno-vesical, en 62. Las tasas de complicaciones postrasplante fueron las siguientes: infección global (70,3%), pancreatitis del injerto (26,3%), hemorragia intraabdominal (17,7%), trombosis del injerto (12,6%) y rechazo pancreático global (10,9%). Las causas de mortalidad fueron fundamentalmente cardiovasculares e infecciosas. La supervivencia del paciente a 1, 3 y 5 años fue del 95,4, del 93 y del 92,4%, respectivamente, mientras que la del injerto correspondió al 81,6, al 77,9 y al 72,3%, respectivamente, durante el mismo periodo. Conclusiones: En nuestra experiencia de 20 años de trasplante pancreático-renal simultáneo las tasas de morbilidad y supervivencia del paciente y del injerto a 5 años son similares a las referidas en los registros internacionales de trasplante pancreático (AU)

Introduction: Simultaneous pancreas-kidney transplantation (SPKT) constitutes the therapy of choice for diabetes type 1 or type 2 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 28 years and mean age of recipients was 38.8 ± 7.3 years, 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 (AU)

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.

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.

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.

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.

Trasplante hepático y cardiaco simultáneo en pacientes con polineuropatía amiloidótica familiar / Simultaneous liver and heart transplantation in patients with familial amyloid polyneuropathy

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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.

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

Fundamento y objetivo: La polineuropatía amiloidótica familiar (PAF) constituye el tipo más prevalente de amiloidosis sistémica hereditaria. Es una enfermedad autosómica dominante que se caracteriza por el depósito de una variante anómala de la transtiretina. Tiene una distribución mundial, con zonas endémicas localizadas en Portugal, Suecia y Japón. En España hay un foco endémico, localizado en Mallorca. El trasplante hepático es la única opción curativa para los pacientes con PAF. El objetivo de este estudio fue describir las características clínicas y demográficas de los pacientes trasplantados con diagnóstico de PAF. Material y método: Se evaluaron 6 pacientes trasplantados por PAF entre abril de 1986 y diciembre de 2012. Resultados: La edad media fue de 57,7 + 16 años, los pacientes de origen español eran mayores de 60 años. Todos los pacientes presentaban síntomas progresivos en forma de polineuropatía mixta. En 2 pacientes se realizó un doble trasplante hepatocardiaco secuencial, efectuándose en primer lugar el trasplante hepático. La supervivencia del paciente y del injerto fue del 80% a los uno, 3 y 5 años. Conclusiones: El único tratamiento etiológico eficaz para la PAF es el trasplante hepático. Una detección temprana es la clave para el tratamiento y el control, evitándose el daño orgánico irreversible (AU)

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 (AU)