Résumé
Gardners syndrome is a single gene disorder with variable manifestations associated with the adenomatous polyposis coli (APC) gene; including gastrointestinal polyposis and osteomas associated with a variety of benign soft tissue tumors and other extraintestinal manifestations. Infrequently associated tumors include papillary carcinoma of the thyroid, biliary neoplasia and adrenal neoplasia, both benign and malignant. It is classified as a variant of classic familial adenomatous polyposis (FAP) and is an autosomal dominant disease. The incidence of incidentaloma in FAP patients is known to be higher than general population. The occurrence of an adrenal carcinoma could affect a patient's prognosis with Gardners syndrome. Increased awareness of this lesion is important in patients with Gardners syndrome who have extended lifespan after prophylactic colectomy. We report a 37 year old case of Gardners syndrome associate with adrenal adenoma.
Sujets)
Adulte , Humains , Adénomes , Polypose adénomateuse colique , Tumeurs de la surrénale , Carcinome papillaire , Colectomie , Syndrome de Gardner , Incidence , Ostéome , Pronostic , Glande thyroideRésumé
Arsenic trioxide is the most prevalent natural inorganic form of arsenic, which is a widely used heavy metal and is ubiquitously distributed in the environment. A 71-year-old man ingested about 10 g of arsenic trioxide powder in an attempted suicide. He developed severe vomiting, watery diarrhea and abdominal pain, and he presented to the emergency room 15 hours after the ingestion. On admission he was slightly drowsy, but shocked. He showed metabolic acidosis, acute renal failure and abnormal electrocardiogram suspecting myocardial infarction. The chest and abdominal X-rays revealed radiopaque materials in the stomach and small bowel. The stomach was irrigated via a nasogastric tube and activated charcoal was given to bind unabsorbed arsenic. The whole bowel irrigation was tried to remove the remained arsenic in the gastrointestinal tract and BAL (British anti-lewisite) was repeatedly administrated. Despite of intensive supporting management with supplemental oxygen, aggressive volume expansion, correction of metabolic disturbances and administration of cardiotonic agents, he developed progressive hypotension and died 42 hours after the ingestion. Random urine arsenic concentration, which was collected at the time of admission, but confirmed after death, was 3,564 microgram/L.
Sujets)
Sujet âgé , Humains , Douleur abdominale , Acidose , Atteinte rénale aigüe , Intoxication par l'arsenic , Arsenic , Cardiotoniques , Charbon de bois , Diarrhée , Consommation alimentaire , Électrocardiographie , Service hospitalier d'urgences , Tube digestif , Hypotension artérielle , Infarctus du myocarde , Oxygène , Intoxication , Choc , Estomac , Tentative de suicide , Thorax , VomissementRésumé
Idiopathic hyperammonemia is a rare and serious complication of intensive cytoreductive chemotherapy for hematologic malignancies as well as after autologus or allogeneic bone marrow transplantation (BMT). A 42-year-old woman with Philadelphia chromosome positive acute lymphoblastic leukemia was undertaken unrelated BMT. Fourteen days later, the patient developed hallucination, mental confusion, lethargy, incoordination and stupor. Laboratory tests revealed normal serum aminotransferases. The serum ammonia level is moderately increased. Parenteral alimentation was discontinued to lower protein intake and treatment with lactulose, metronidazole, carnitine, and flumazenil was started. Also, hemodialysis was performed. Despite of these appropriate therapies, she died of idiopathic hyperammonenia at twenty-two days after allogeneic BMT.
Sujets)
Adulte , Femelle , Humains , Ammoniac , Ataxie , Transplantation de moelle osseuse , Moelle osseuse , Carnitine , Traitement médicamenteux , Flumazénil , Hallucinations , Tumeurs hématologiques , Hyperammoniémie , Lactulose , Léthargie , Métronidazole , Chromosome Philadelphie , Leucémie-lymphome lymphoblastique à précurseurs B et T , Dialyse rénale , État de stupeur , TransaminasesRésumé
Eosinophilic cholecystitis is a rare condition of cholecystitis which was first described by the Albot in 1949. We describe a case of eosinophilic cholecystitis associated with Clonorchis sinensis. A 42-year-old woman presented with a classic history of acute cholecystitis and a peripheral eosinophilia of 56% without an allergic history. An abdominal CT scan showed thickened, edematous gallbladder wall but no cholelithiasis, and an cholecystectomy was performed. Pathological examination revealed diffuse infiltration of eosinophils throughout all gallbladder wall layers and marked eosinophilic infiltration of the liver. Skin test for clonorchis sinensis was strong positive and serologic test for Clonorchis sinensis antibody was high titer. The serum alkaline phosphatase, SGOT, SGPT and eosinophil count normalized within a week after praziquantel medication.
Sujets)
Adulte , Femelle , Humains , Alanine transaminase , Phosphatase alcaline , Aspartate aminotransferases , Cholécystectomie , Cholécystite , Cholécystite aigüe , Lithiase biliaire , Clonorchiase , Clonorchis sinensis , Éosinophilie , Granulocytes éosinophiles , Vésicule biliaire , Hépatite , Foie , Praziquantel , Tests sérologiques , Tests cutanés , TomodensitométrieRésumé
Intramedullary spinal cord metastasis (ISCM) has rarely been reported in patients wit carcinomas. In about half the ISCM reported the primary origins are lung cancer, with small cell lung cancer responsible for almost all reported cases. Thus, ISCM from small cell lung cancer is relatively well documented, but ISCM from nonsmall cell lung cancer is rarely diagnosed prior to the patients' demise, so very little data about such patients is available. Spine MRI is the most sensitive technique for diagnosing ISCM. ISCM are now being encountered with increasing frequency due to the increasing survival rates of lung cancer patients, and the development of new imaging technique. We reported a case of an ISCM from non-small cell lung cancer with a brief review of the literature.
Sujets)
Humains , Carcinome pulmonaire non à petites cellules , Tumeurs du poumon , Imagerie par résonance magnétique , Métastase tumorale , Carcinome pulmonaire à petites cellules , Moelle spinale , Rachis , Taux de survieRésumé
Intramedullary spinal cord metastasis (ISCM) has rarely been reported in patients wit carcinomas. In about half the ISCM reported the primary origins are lung cancer, with small cell lung cancer responsible for almost all reported cases. Thus, ISCM from small cell lung cancer is relatively well documented, but ISCM from nonsmall cell lung cancer is rarely diagnosed prior to the patients' demise, so very little data about such patients is available. Spine MRI is the most sensitive technique for diagnosing ISCM. ISCM are now being encountered with increasing frequency due to the increasing survival rates of lung cancer patients, and the development of new imaging technique. We reported a case of an ISCM from non-small cell lung cancer with a brief review of the literature.