Browsing by Author "Silva, R"
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- Atualização e protocolo de atuação na Drepanocitose no AdultoPublication . Major, M; Porteiro, B; Costa, M; Martins, J; Silva, R
- 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.
- Crioglobulinemia: a ponta do icebergPublication . Silva, R
- Massive Osteolytic Lesion of the Femur after Total Knee Arthroplasty.Publication . Cardoso, A; Amaro, P; Gamelas, P; Carvalho, E; Silva, R; Oliveira, MH; Jesus, MVarious failure mechanisms have been identified in total knee arthroplasty (TKA). We hereby present one case of failure, which stands out because of its rapid and destructive progression. We report the case of a 72-year-old Caucasian female patient who developed a large bone osteolytic lesion of the femur after TKA. The patient presented to our hospital 7 years after the initial surgery, complaining of persistent knee pain. The lesion affected the distal half of the femur and, after a diagnostic workup, required a resection of 20 cm and reconstruction with a tumor prosthesis. Subsequent pathological analysis revealed a reaction to cement and prosthesis components. Periprosthetic osteolysis continues to be a major problem, and a reaction to cement and prosthesis components can be an elusive cause of TKA failure.
- Overactive bladder symptom bother and health-related quality of life in patients with systemic lupus erythematosus and primary Sjögren syndromePublication . Silva, R; Romão, V; Neves, M; Garcia, R; Oliveira, S; Brites, J, et al.The objective of this paper is to assess overactive bladder (OAB) symptom bother (SB) and health-related quality of life (HRQL) among patients with systemic lupus erythematosus (SLE) and primary Sjögren syndrome (pSS). METHODS: We recruited adult SLE and pSS patients and two groups of age- and sex-matched controls. We applied the OAB questionnaire-short form (OABq-SF) to all participants to assess SB and HRQL and collected clinical information relevant for OAB. We compared the OABq-SF scores for SB and HRQL between patients and controls using univariate and multivariate linear regression analysis. RESULTS: We recruited 95 rheumatic patients (68 SLE, 27 pSS) and 231 controls. Compared to controls SLE patients showed higher OABq-SF SB scores (22.6 ± 20.4 vs 14.7 ± 17.0, p = 0.004) and lower HRQL scores (89.8 ± 15.8 vs 93.8 ± 11.4, p = 0.044). On multivariate analysis SLE was significantly associated with a higher SB score (ß-coefficient 7.13, p = 0.008) and tended to be associated with worse HRQL values (ß-coefficient -3.53, p = 0.055). Patients with pSS had numerically higher mean SB scores (22.8 ± 22.5 vs 16.2 ± 18.0, respectively, p = 0.107) and lower HRQL scores (91.0 ± 10.7 vs 93.2 ± 11.6, respectively, p = 0.369), although these differences were not statistically significant. Diagnosis of pSS was not significantly associated with SB or HRQL scores on univariate or multivariate analysis. CONCLUSIONS: Patients with SLE have significantly worse OAB-SB and poorer HRQL compared to controls. A similar trend was seen for pSS patients, especially for SB. These findings suggest that clinically subtle OAB symptoms may be present in rheumatic patients for whom, later on, bladder pain syndrome may occur.
