Aproximación al diagnóstico patológico de las enfermedades colestásicas / Approaches to Pathological Diagnosis of Cholestatic Diseases

Cualquier enfermedad que lleve a la alteración del flujo biliar o del metabolismo de las sales biliares se traduce en colestasis. Son múltiples las causas que pueden producirla, sea por su localización anatómica intrahepática o extrahepática, agudas o crónicas, con o sin lesión hepatocelular acompañante, o primarias o secundarias, por lo que resultan numerosas las entidades que deben ser consideradas como parte del diagnóstico diferencial de las enfermedades colestásicas y que plantean un gran reto diagnóstico tanto para el clínico, como para el patólogo (1). En el presente estudio se plantea una aproximación diagnóstica basada en patrones histológicos, haciendo énfasis en las enfermedades colestásicas crónicas del adulto, en próximos estudios se tratarán las de la población pediátrica.

Any disease that leads to impaired bile flow or impaired bile salt metabolism results in cholestasis. There are several causes of the disease related to intrahepatic or extrahepatic anatomical locations, to whether the disease is acute or chronic, to whether or not hepatocellular damage occurs, and to whether or not the condition is primary or secondary. The large number of entities that must be considered in the differential diagnosis of cholestatic diseases poses a major diagnostic challenge for both the clinician and the pathologist (1). This article establishes a diagnostic approach based on histologic patterns which emphasizes adult chronic cholestatic diseases. The next article will focus on the pediatric population.

Clinical manifestation and outcome of alagille syndrome in Korea: a study of 30 patients / 소아과

PURPOSE: The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. METHODS: We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death or last visit; mean follow-up : 5 years). RESULTS: Cholestasis occurred in 100 percent, cardiovascular abnormalities in 83.3 percent, butterfly vertebrae in 30.0 percent, posterior embryotoxon in 43.3 percent, and a characteristic facial appearance in 100 percent. At study conclusion, of these 30 patients, eight had died (26.7 percent); six related to Alagille syndrome. Five patients died of a liver disease complication. Liver transplantation was carried out in five of the 30 patients (16.7 percent) and one of these died due to hyperacute rejection. At age two, cholestasis improved in 17 of the 30 patients. Those who had severe cholestasis at 2 years of age tended to have a complication, such as liver cirrhosis or liver transplantation, or to have died. CONCLUSION: Hepatic complications account for the most mortalities in patients with Alagille syndrome. Careful and complete assessments should be made in children who have cholestasis at 2 years of age. Further investigations of more cases are required.