Browsing by Author "Mesquita, R"
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- New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case ReportsPublication . Mesquita, R; Sousa, M; Trinidad, C; Pinto, E; Badiola, IPulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm of vascular origin. There are three different major imaging patterns identified in thoracic manifestation of epithelioid hemangioendothelioma: (1) multiple pulmonary nodules; (2) multiple pulmonary reticulonodular opacities; and (3) diffuse infiltrative pleural thickening. Radiographically, presence of bilateral multiple nodules is the most common pattern of presentation. The diagnosis is made on the basis of histopathological findings and confirmed by positive immunohistochemistry staining. Although the prognostic factors for PEH have not yet been well established, a better prognosis is usually associated with the multinodular pattern. We report two different imagological presentations of this rare disease, based on two institutional experiences, along with a review of the relevant literature.
- Severe Intestinal Bleeding in a Woman with Glanzmann ThrombastheniaPublication . Mesquita, R; Santos, I; Monteiro, HGlanzmann thrombasthenia (GT) is a rare inherited disorder associated with abnormal platelet function. Non-surgical bleeding is common and can be life-threatening. A 70-year-old woman with GT presented with a 3 week history of melena and asthenia. She had hemoglobin of 3.8 g/dL. Both upper and lower endoscopies were inconclusive. Video capsule endoscopy showed multiple angioectasias and erosions in the distal ileum. Following several days of recurring intestinal hemorrhage, only treatment with recombinant activated factor VII proved to be effective, enabling patient stabilization with no further bleeding. Control and prevention of bleeding among these patients is imperative and remains challenging. Learning points: Glanzmann thrombasthenia is a rare inherited bleeding disorder of platelet function caused by a quantitative or qualitative defect of the platelet membrane glycoprotein (GP) IIb/IIIa (integrin αIIbβ3) complex. Bleeding is a concern in patients with GT due to its potential risk of severity and uncontrollability. It also greatly impacts on the quality of life of the patient.Recombinant FVIIa has been reported to be a safe and effective hemostatic agent when bleeding is not responsive to local management, antifibrinolytics or platelet transfusion.