RESUMO
Cushing's syndrome results from prolonged exposure to high levels of glucocorticoid hormones. ACTH-dependent Cushing's syndrome accounts for about 85% of endogenous causes. Autonomous pituitary ACTH secretion, called Cushing's disease, is responsible for 80% of these causes, whereas ectopic ACTH secretion is responsible for 20% of them. Appropriate therapy of patients with Cushing's syndrome depends on accurate diagnosis and classification of the disorder. In addition to the history and clinical evaluation, the laboratory evaluation of a patient with Cushingoid appearance is necessary to establish the diagnosis and determine the cause of hypercortisolism. A major problem in the differential diagnosis of ACTH-dependent Cushing's syndrome is distinguishing Cushing's disease from the ectopic ACTH syndrome. Both entities can have similar clinical and laboratory features. In addition, both pituitary microadenoma and ectopic ACTH-secreting tumors may be radiologically occult. Bilateral inferior petrosal sinus and peripheral vein catheterization with simultaneous collection of samples for measurement of ACTH is one of the most specific tests available to localize the source of ACTH production. We report two cases of Cushing's syndrome diagnosed by inferior petrosal sinus sampling associated with normal or low ACTH levels on routine basal hormone assay.
Assuntos
Humanos , Síndrome de ACTH Ectópico , Hormônio Adrenocorticotrópico , Cateterismo , Catéteres , Classificação , Síndrome de Cushing , Diagnóstico , Diagnóstico Diferencial , Amostragem do Seio Petroso , Plasma , VeiasRESUMO
Congenital hepatic fibrosis (CHF) is a rare developemental abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all, cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The pateint also had cystic dilation of subarchnoid space in the basal cistern and posterior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. He had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures.
Assuntos
Humanos , Adulto Jovem , Ductos Biliares , Biópsia , Encéfalo , Diagnóstico , Varizes Esofágicas e Gástricas , Fibrose , Hematemese , Hipertensão Portal , Rim , Ligadura , Fígado , Imageamento por Ressonância Magnética , Agulhas , Doenças Renais Policísticas , Rim Policístico Autossômico Recessivo , Esplenomegalia , Derivação Esplenorrenal Cirúrgica , Tomografia Computadorizada por Raios X , VarizesRESUMO
Myelodysplastic syndrome (MDS) is a hematologic disorder characterized by peripheral cytopenia and histologic feature of hematologic dysplasia. MDS has rarely been reported in association with Behcet's disease. We describe a patient with MDS associated Behcet's disease and a review of the literature.