Résumé
La colestasis, tiene incidencia de 1:60-375 ictéricos a las 2 semanas de edad, es potencialmente grave, presentan riesgos inmediatos como coagulopatías por hemorragia severa ante el déficit de la absorción de la vitamina K, y con su diagnóstico precoz, se identifican patologías que tienen tratamiento, incluso, trasplante hepático. Como consecuencia de la colestasis, hay retención de sales biliares, daño celular hepático, y descenso de la bilis a nivel intestinal, que ocasionan mala digestión de grasas y proteínas, con defectos en las vitaminas liposolubles. Existen hepatopatías primariassecundarias a una serie de entidades genéticas y metabólicas, y colestasis secundarias a otros problemas, que en el adulto generan enfermedad hepática. Es necesario establecer protocolos deidentificación del niño con ictericia.
Cholestasis has 1:60-375 incidence of jaundice at 2 weeks of age, are potentially serious, immediateand present risk of severe bleeding coagulopathy to the shortfall in the absorption of vitamin K, andearly diagnosis, identify diseases that are treatable, even liver transplantation.As a result of cholestasis, there is retention of bile salts, liver cell damage and decrease of bile in the intestine, causing poor digestion of fats and protein, with defects in soluble vitamins. There are primaryliver disease secondary to a variety of metabolic and genetic entities, and cholestasis secondary toother problems, generated in the adult liver disease. It is necessary to establish protocols for the identification of children with jaundice.
Sujets)
Enfant , Cholestase/classification , Cholestase/complications , Cholestase/diagnostic , Cholestase/épidémiologie , Cholestase/physiopathologie , Cholestase/anatomopathologie , Cholestase/sang , Conduits biliaires/traumatismes , Conduits biliaires/métabolisme , Conduits biliaires/anatomopathologie , Bile , Syndrome d'Alagille/classification , Syndrome d'Alagille/génétique , Syndrome d'Alagille/anatomopathologie , Vitamine KRésumé
Granulocytic sarcoma is a rare extramedullary tumor composed of myeloid progenitor cells. Primary involvement of the biliary tract without evidence of leukemia is exceedingly rare. Here, we report an isolated biliary granulocytic sarcoma in a 30-yr-old man who presented with jaundice, fever, and chill without any evidence of leukemia. However, five months after the diagnosis, he developed acute myelogenous leukemia with multilineage dysplasia and chromosomal abnormality. A rare possibility of biliary granulocytic sarcoma should be considered as a differential diagnosis in patients with obstructive jaundice. A histologic evaluation by aggressive diagnostic intervention is important and may improve prognosis.