RESUMEN
Liver transplantation is an excellent therapeutic option for terminal liver disease. During the last decades the results of liver transplantation have improved significantly with a patient survival rate of nearly 90 percent at one year and 80 percent at 5 years of follow-up. The main indications for liver transplantation include: end-stage liver disease associated to cirrhosis, acute liver failure, and hepatic tumors (mainly hepatocarcinoma). The absolute contraindications for a transplant are less frequent than in the past, and include: severe co-morbidity (cardiac or pulmonary), sepsis, advanced HIV disease and extra-hepatic malignancy. This document presents a Consensus of the main groups performing liver transplantation in Chile, about its indications and contraindications. It also reviews general aspects of liver transplantation, including the selection and referral of liver transplant candidates, allocation of organs and the evaluation of severity of liver disease.
Asunto(s)
Humanos , Carcinoma Hepatocelular/cirugía , Cirrosis Hepática/cirugía , Fallo Hepático Agudo/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado , Chile , Enfermedad Crónica , Selección de Donante , Accesibilidad a los Servicios de Salud , Trasplante de Hígado , Trasplante de Hígado/mortalidad , Trasplante de Hígado/patología , Selección de Paciente , Reoperación , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Listas de EsperaRESUMEN
Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is an autoinflammatory disorder associated to a mutation of the Tumor Necrosis Factor Receptor 1 (TNFR1) whose clinical presentation consists on recurrent episodes of prolonged fever, abdominal pain, myalgias, migratory cutaneous erythema, conjunctivitis or periorbitary edema. The diagnosis is confirmed by genetic analysis of the TNFR1 gene. Its main complication is amyloidosis and the treatment is based on the use of corticosteroids or anti-TNF antibodies. We report a 17 year-old male and 23 year-old female with the syndrome. Both cases had heterozygous mutations of the TNFR1 gene, C30R in the first case and T50M in the second case.