RESUMEN
We analyzed 31 patients with a diagnosis of primary biliary cirrhosis, 29 of them males, aged 23 to 72 years. Liver biopsy was diagnostic in all showing initial findings of the disease in 5. Echotomography and cholangiography demonstrated a patent biliary, tract. Antimithocondrial antibodies were present in 94% of patients. Alkaline phosphatase and biliary acid levels were useful for diagnosis. Pruritus was present with varying intensity in all patients, with premenstural exacerbations in 5 females who had cholestasis of pregnancy or hepatitis caused by progestin drugs before developing cirrhosis. Recurrent urinary tract infection was present in 8 patients, osteosporis in 24, Sjogren's syndrome in 24 and Crest syndrome in 4. Survival ranged from 1 to 12 years, death being caused by rupted esophageal varices in 12 patients and by liver failure in 7. Persistance of pruritus and altered liver function tests after cholestasis of pregnancy or hepatitis caused by progestins should lead to investigation of biliary cirrhosis
Asunto(s)
Adulto , Persona de Mediana Edad , Humanos , Masculino , Femenino , Cirrosis Hepática Biliar/diagnóstico , Estudios de Seguimiento , Colestasis Intrahepática/diagnósticoAsunto(s)
Adolescente , Adulto , Persona de Mediana Edad , Humanos , Masculino , Femenino , Cardiomiopatía Dilatada/complicaciones , Miocarditis/complicaciones , Biopsia , Cardiomiopatía Dilatada/patología , Incidencia , Estudios Retrospectivos , Endocardio/patología , Miocarditis/patología , Miocarditis/epidemiología , Miocardio/patologíaRESUMEN
El fibroxantosarcoma es una neoplasia maligna rara, de tejidos blandos, cuya localización topográfica más frecuente se presenta en las extremidades y en la pared torácica. La ubicación renal se ha reportado en 7 oportunidades previas a nuestro informe. Se presenta el caso clínico, el tratamiento a que fue sometido y se discute su resultado luego de 1 año de seguimiento