Résumé
La hipertensión porta (HTP) es el resultado del incremento de la presión dentro del sistema venoso porta. Se presenta con poca frecuencia en el paciente pediátrico pero es una de las mayores causas de morbilidad y mortalidad en el niño con enfermedad hepática. La mayoría de los pacientes con http presentan un estado hiperdinámico, lo cual aumenta el flujo venoso porta y mantiene la hipertensión. Puede ser secundaria a obstrucción a nivel prehepático, intrahepático o extrahehepático.
Portal hypertension (PH) is the result of increased pressure within the portal venous system. It occurs infrequently in the pediatric patient but it is a major cause of morbidity and mortality in children with liver disease. Most patients with PH have a hyperdynamic state, which increases venous flow and portal hypertension remains. May be secondary to obstruction at prehepatic, intrahepatic or extrahehepatic.
Sujets)
Humains , Mâle , Femelle , Enfant d'âge préscolaire , Enfant , Ascites/classification , Splénomégalie/classification , Splénomégalie/complications , Hématémèse/mortalité , Hématémèse/sang , Hypertension portale/épidémiologie , Hypertension portale/mortalité , Hypertension portale/anatomopathologie , Dégénérescence hépatolenticulaire/classification , Dégénérescence hépatolenticulaire/diagnostic , Mucoviscidose/classification , Troubles nutritionnels de l'enfant/étiologie , Troubles nutritionnels de l'enfant/génétique , Troubles nutritionnels de l'enfant/mortalité , Troubles nutritionnels de l'enfant/sangRésumé
Wilson's disease is an inherited disorder of copper metabolism. We report 16 patients (6 males) with the disease; 6 had hepatic involvement and 3 were asymptomatic. The age onset was 9 years for hepatic and 17 years for neurologic involvement. The mean delay in diagnosis was 14 months. Chronic hepatitis, cirrhosis and fulminant hepatic failure were the clinical forms of liver disease. Patients with neurologic disorders had behavioral disturbances and extrapyramidal manifestations such as dystonia and parkinsonism. Patients had a good response to penicillamine, except 3 that died of liver complications, in whom the treatment was delayed or discontinued. We conclude that this metabolic disease must be suspected in pubertal children and in adults of less than 30 years old with liver disease of unknown origin or behavioral alterations associated to an extrapyramidal syndrome