RESUMEN
A 13-year-old girl presented with features of intestinal obstruction. At surgery, the terminal 25 cm of ileum, which was resected along with the right colon, showed plexiform neurofibromatosis of the serosa and mesentery, hyperplastic submucosal and myenteric nerve plexuses and proliferation of neural tissue in the lamina propria, which manifested as diffuse polyposis of the ileal mucosa. The patient had a single inconspicuous external neurofibroma and a few café-au-lait spots on the back.
Asunto(s)
Adolescente , Manchas Café con Leche/patología , Colectomía , Colon/patología , Femenino , Humanos , Íleon/patología , Obstrucción Intestinal/etiología , Poliposis Intestinal/etiología , Laparotomía , Neurofibromatosis/complicacionesRESUMEN
Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.
Asunto(s)
Síndrome de Alagille/diagnóstico , Preescolar , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , India , Pruebas de Función Hepática , Medición de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
Patients presenting with acute disseminated intravascular coagulation (DIC) as the first symptom of malignancy are rare. A 68-year-old man presented with DIC. On evaluation, he was found to have adenocarcinoma of the stomach. Resection of the growth controlled the DIC for a few days, after which the patient developed altered coagulation parameters and sepsis, and succumbed.