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Browsing MED - Artigos publicados em revistas não indexadas by Author "Aldomiro, F"
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- Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential DiagnosisPublication . Serra, JT; Silva, R; Aldomiro, F; Duarte, FPLangerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. A 69-year-old woman with immune thrombocytopenic purpura underwent surgery for the removal of giant skin haemangiomas. During post-operative care, intravascular disseminated coagulopathy developed. After weeks of corticosteroids and immunosuppressive therapy with no clinical improvement, pulmonary tuberculosis was diagnosed and appropriate treatment initiated. Despite all the efforts, the patient's clinical condition kept worsening and she eventually died. An autopsy revealed bone marrow Langerhans cell histiocytosis. In this case, the patient's autoimmune background together with tuberculosis and intravascular disseminated coagulopathy masked the presentation and made the diagnosis of a rapidly progressive fatal disease very difficult.
- Encefalopatia de hashimoto: uma causa de demência reversívelPublication . Oliveira, I; Urzal, J; Correia, I; Aldomiro, FA Encefalopatia de Hashimoto (EH) é caracterizada pelo desenvolvimento de sintomas neuropsiquiátricos, tipicamente com alteração cognitiva de instalação rápida sendo por isso diagnóstico diferencial de demências rapidamente progressivas (DRP). Reportamos o caso de uma mulher de 72 anos que recorre por confusão mental, desorientação temporo-espacial e tremor simétrico dos membros superiores, associado a hipertonia e hiperreflexia. Neste contexto postulou-se a hipótese de DRP. A tomografia computorizada e a ressonância magnética crânio-encefálica mostraram alterações inespecíficas. O eletroencefalograma mostrou lentificação difusa ligeira. A avaliação do líquido cefalorraquidiano (LCR) revelou hiperproteinorráquia. Foram excluídas causas infeciosas, metabólicas, medicamentosas, neoplásicas e paraneoplásicas. Identificou-se um hipotiroidismo com aumento sérico dos anticorpos anti-tiroglobulina 3000 UI/mL e anti-peroxidase 760 UI/mL. Foi assumida a hipótese de EH e foi iniciada corticoterapia sistémica, com regressão sintomática. Discutimos a importância do diagnóstico diferencial das demências de aparecimento recente, nomeadamente em idosos, por existirem múltiplas causas reversíveis quando identificadas e tratadas atempadamente.
- Nódulo de irmã Mary-Joseph – a primazia da clínicaPublication . Pedro, B; Sousa, R; Patrícia, T; Aldomiro, FO nódulo da Irmã Mary Joseph (NIMJ) trata-se de um nódulo palpável na região peri-umbilical, com origem em tumores metastáticos avançados intra-abdominais ou intra-pélvicos. O nódulo da Irmã Mary Joseph pode ser o primeiro sinal de neoplasia e indica doença avançada, bem como, mau prognóstico. Apresentamos o caso de um homem de 61 anos de idade, com um nódulo umbilical à admissão, cujo estudo adicional revelou um tumor do pâncreas.
- Pituitary Apoplexy May Be Mistaken for Temporal ArteritisPublication . Pedro, B; Patrícia, T; Aldomiro, FPituitary apoplexy is a rare endocrine emergency, characterized by a sudden increase in pituitary gland volume secondary to acute ischaemic infarction or haemorrhage of the pituitary gland, usually in the presence of a pituitary adenoma. We present the case of a 79-year-old man admitted for new-onset, bi-temporal and severe headache, associated with photophobia and vomiting, whose additional study revealed pituitary apoplexy. This case highlights the need for high clinical suspicion of this rare entity in order to reduce the associated mortality.
- Sarcoidosis and Multiple Myeloma: A Case Report and Literature ReviewPublication . Serra, J; Martinho, A; Duarte, F; Aldomiro, FThe existence of a sarcoidosis-lymphoma syndrome has been previously proposed since the relation between sarcoidosis and an increased risk of lymphoproliferative disorders is well established. Multiple myeloma is a malignant multifocal proliferation of clonal plasma cells within the bone marrow, and its association with sarcoidosis has been rarely described. We present a concurrent diagnosis of sarcoidosis and multiple myeloma and make a brief analysis of the reported cases in the literature. A 65-year-old woman underwent surgery for the excision of a wrist mass that presented 3 years before. Histological analysis showed sarcoid-type epithelioid granulomas without necrosis, establishing soft tissue sarcoidosis. Further evaluation revealed marked interstitial lung parenchyma lesions and large intrathoracic adenopathies. Bronchofibroscopy with transbronchial biopsy confirmed lung sarcoidosis. In addition, blood analysis showed monoclonal IgG kappa gammopathy. A bone marrow biopsy confirmed hypercellularity with 60% plasma cells and plasmocyte infiltration. Thus, the diagnosis of systemic sarcoidosis and multiple myeloma was established simultaneously. In a brief review of the literature, we identified 33 reports of cases with both sarcoidosis and multiple myeloma. We point out the importance of a high level of suspicion for the association of sarcoidosis with malignant haematological diseases such as multiple myeloma.
- Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester DiseasePublication . Martins, N; Achega, M; Rodrigues, A; Aldomiro, FA 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain injuries. Patient was admitted with acute ischemic stroke of right hemisphere. There was full recovery within 24 hours and etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous 2 years. Water deprivation test followed by the administration of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal. Full body CT scan showed arterial wall thickening suggestive of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.