RESUMO
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.
Assuntos
Adolescente , Manchas Café com Leite/patologia , Colectomia , Colo/patologia , Feminino , Humanos , Íleo/patologia , Obstrução Intestinal/etiologia , Polipose Intestinal/etiologia , Laparotomia , Neurofibromatoses/complicaçõesRESUMO
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.
Assuntos
Síndrome de Alagille/diagnóstico , Pré-Escolar , Progressão da Doença , Feminino , Seguimentos , Humanos , Índia , Testes de Função Hepática , Medição de Risco , Índice de Gravidade de DoençaRESUMO
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.