Browsing by Issue Date, starting with "2019"
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- Peri-Prosthetic Bone Cysts After Total Ankle Replacement. A Systematic Review and Meta-AnalysisPublication . Arcângelo, J; Guerra-Pinto, F; Pinto, A; Grenho, A; Navarro, A; Martin Oliva, XBackground: Periprosthetic cystic osteolysis is a well-known complication of total ankle replacement. Several theories have been proposed for its aetiology, based on individual biomechanical, radiological, histopathology and outcome studies. Methods: Studies that met predefined inclusion/exclusion criteria were analysed to identify literature describing the presence of peri-prosthetic ankle cystic osteolysis. Quantitative data from the selected articles were combined and statistically tested in order to analyse possible relations between ankle peri-prosthetic bone cysts and specific implant characteristics. Results: Twenty-one articles were elected, totalizing 2430 total ankle replacements, where 430 developed peri-prosthetic cystic osteolysis. A statistically significant association (P<.001) was found between the presence of bone cysts and non-anatomic implant configuration, hydroxyapatite-coating, mobile-bearing and non tibial-stemmed implants. No significant association existed between the type of constraining and the presence of cysts (P>.05). Conclusions: Non-anatomic, mobile-bearing, hydroxyapatite-coated and non tibial-stemmed total ankle replacements are positively associated with more periprosthetic bone cysts.
- MonoMAC Syndrome Caused by a Novel GATA2 Mutation Successfully Treated by Allogeneic Hematopoietic Stem Cell TransplantationPublication . Moraes-Fontes, MF; Caramalho, Í; Hsu, AP; Holland, SM; Abecasis, M
- Recordando Costa MarquesPublication . Barros Veloso, AJ
- Immunological Reconstitution Inflammatory Syndrome and Thrombotic Microangiopathy: Severe Complications in a Child With Acquired Immunodeficiency SyndromePublication . Rocha, AP; Milheiro Silva, T; Francisco, T; Neves, C; Brites, V; Candeias, FSome patients with human immunodeficiency virus (HIV) infection deteriorate shortly after starting highly active antiretroviral therapy (HAART), the so-called immunological reconstitution inflammatory syndrome (IRIS).1 Although having a spontaneous resolution in many instances, it can be fatal.1 Worse prognosis is seen in younger children, severe immunosuppression and central nervous system IRIS, or infections with specific agents, namely, Criptococcus.2 Hemophagocytic lymphohistiocytosis (HLH) has also been described in children with HIV infection, in the context of an immunological system dysregulation.3 Thrombotic microangiopathy (TMA) became rare with the introduction of HAART, being mostly associated with advanced disease.4 HIV-associated TMA has specific clinical aspects as well as a worse prognosis than idiopathic or congenital TMA.4-9 The authors present the case of a 10-month-old boy with advanced HIV infection who developed IRIS complicated with HLH and TMA during the course of his treatment.
- Nanotechnology-Ocular Devices for Glaucoma Treatment: A Literature ReviewPublication . Cardigos, J; Ferreira, Q; Crisóstomo, S; Coelho, N; Cunha, JP; Abegão Pinto, L; Ferreira, JNanotechnology enabled the development of materials and devices with great utility in different fields of medicine. By using engineered-based nano-devices and structures, human biological systems may be controlled and repaired at a molecular scale, ultimately leading to a biological benefit. In particular, in the field of glaucoma treatment, nanotechnology may, for example, enhance drug residence time on the ocular surface and ocular bioavailability, as well as improve surgical success by both optimizing postoperative scarring and providing a wider safety window. Further studies are still needed to entirely explain the pharmacodynamics of nanotechnology-based therapeutic approaches and prove their biological consequences in human eyes. This review aims to summarize the literature concerning the advances in nanotechnology, specifically regarding ocular devices applied to the treatment of glaucoma.
- The Impact of Extent of Liver Resection Among Patients with Neuroendocrine Liver Metastasis: an International Multi-institutional StudyPublication . Sham, J; Ejaz, A; Gage, M; Bagante, F; Reames, B; Maithel, S; Poultsides, G; Bauer, T; Fields, R; Weiss, M; Pinto Marques, H; Aldrighetti, L; Pawlik, T; He, JBackground: Liver resection in patients with neuroendocrine liver metastasis (NELM) provides a survival benefit, yet the optimal extent of resection remains unknown. We sought to examine outcomes of patients undergoing non-anatomic (NAR) versus anatomic liver resection (AR) for NELM using a large international cohort of patients. Methods: Two hundred and fifty-eight patients who underwent curative intent liver resection from January 1990 to December 2016 were identified from eight institutions. Patients were excluded if they underwent concurrent ablation, had extrahepatic disease, underwent a debulking operation, or had mixed anatomic and non-anatomic resections. Overall (OS) and recurrence-free (RFS) survival were compared among patients based on the extent of liver resection (AR vs. NAR). Results: Most primary tumors were located in the pancreas (n = 117, 45.4%) or the small intestine (n = 65, 25.2%). Liver resection consisted of NAR (n = 126, 48.8%) or AR (n = 132, 51.2%) resection. The overwhelming majority of patients who underwent NAR had an estimated liver involvement of < 50% (NAR 109, 97.3% vs. AR n = 82, 65.6%; P < 0.001). Patients who underwent NAR also had higher rates of primary tumor lymph node metastasis (NAR n = 79, 71.2% vs. AR n = 37, 33.6%; P < 0.001) and microscopically positive margins (R1) (NAR n = 29, 25.7% vs. AR n = 16, 12.5%; P = 0.009). After a median follow-up of 47.7 months, 48 (18.6%) patients died and 37.0% (n = 95) had evidence of disease recurrence. Patients who underwent AR had both longer median OS (not reached) and RFS (not reached) versus patients who underwent NAR (median OS 138.3 months; median RFS 31.3 months) (both P < 0.01). After controlling for patient and disease-related factors, extent of liver resection was independently associated with an increased risk of recurrence (HR 2.39, 95% CI 1.04-5.48; P = 0.04) but not death (HR 1.92, 95% CI 0.40-9.28; P = 0.42). Conclusion: NAR was independently associated with a higher incidence of recurrence versus patients who undergo a formal anatomic hepatectomy among patients with NELM.
- Peptide Receptor Radionuclide Therapy with 177 Lu-DOTA-TATE As a Promising Treatment of Malignant Insulinoma: a Series of Case Reports and Literature ReviewPublication . Magalhães, D; Sampaio, I; Ferreira, G; Bogalho, P; Martins-Branco, D; Santos, R; Duarte, HIntroduction: Insulinomas are a rare type of pancreatic neuroendocrine tumours characterized by insulin hypersecretion. They are considered malignant when metastases are present. Traditional therapies often promote only temporarily symptomatic relief and may be associated with severe adverse effects. There is scarce experience in treating malignant insulinomas with peptide receptors radionuclide therapy (PRRNT). Patients and methods: We describe PRRNT results in four patients with inoperable malignant insulinomas with poorly controllable hypoglycaemia. All patients received therapy with 177Lu-DOTA-TATE after conventional therapies failed in controlling disease progression and symptoms. The activity administered per cycle was 4.8-7.4 GBq. The interval between cycles was 10-16 weeks. Haematology, liver and kidney function tests were performed before treatment initiation and 5 and 10 weeks after each cycle. Results: Patient 1 presented significant clinical benefit for 13 months after PRRNT, with imaging improvement. Patient 2 obtained reduction of the number and severity of hypoglycaemic episodes during 15 months after therapy. Patient 3 is asymptomatic since PRRNT first cycle performed 23 months ago and revealed significant imaging improvement. Patient 4 had resolution of hypoglycaemia only 3 days after PRRNT first cycle and today, 16 months after therapy, the disease seems to be in remission and the patient maintains euglycaemic state. PRRNT was well tolerated, with only hematologic grade 2 toxicity in patient 1 and mild kidney toxicity in patient 3. Conclusions: After the start of 177Lu-DOTA-TATE all patients achieved hypoglycaemia symptomatic control and had evident improvement of their quality of life. Three patients showed imagiological improvement suggesting reduced tumour load.
- Venous and Arterial TNF-R1 Predicts Outcome and Complications in Acute Subarachnoid HemorrhagePublication . Fragata, I; Bustamante, A; Penalba, A; Ferreira, P; Paiva Nunes, A; Canhão, P; Montaner, JBackground: There is increasing evidence for the role of inflammation in clinical outcome after subarachnoid hemorrhage (SAH). Specifically, the TNF-alfa(α) pathway seems to be relevant after SAH. Although the TNF-α main receptor, TNF-R1 is associated with aneurysm growth and rupture, its relation to prognosis is unknown. We sought to compare TNF-R1 levels in peripheral venous blood and arterial blood closer to the ruptured aneurysm to study the association of TNF-R1 blood levels with poor prognosis (modified Rankin Scale > 2 at discharge, 3 and 6 months) and complications (hydrocephalus or delayed cerebral ischemia/DCI) following SAH. Methods: We included consecutive SAH patients admitted in the first 72 h of symptoms. Blood samples were simultaneously collected from a peripheral vein and from the main parent artery of the aneurysm. Levels of TNF-R1 were measured using enzyme-linked immunosorbent assays. Results: We analyzed 58 patients. Arterial and venous levels of TNF-R1 were correlated (R = 0.706, p < 0.001). In multivariate regression analysis, venous TNF-R1 was an independent predictor of poor outcome at 6 months after adjusting by age and sex [odds ratio (OR) 11.63; 95% CI 2.09-64.7, p = 0.005] and after adjusting by Glasgow Coma Scale and Fisher scales (OR 8.74; 95% CI 1.45-52.7, p = 0.018). There was no association of TNF-R1 with DCI. A cut-off for arterial TNF-R1 of 1523.7 pg/mL had 75% sensitivity/66% specificity for the prediction of hydrocephalus. Conclusion: Levels of venous TNF-R1 are associated with poor outcome in SAH. A specific association was found between levels of arterial TNF-R1 and hydrocephalus. These results are consistent with the role of TNF-α pathway in SAH and need to be validated in larger cohorts.
- Self-Reported Low-Energy Fractures and Associated Risk Factors in People with Diabetes: a National Population-Based StudyPublication . Furtado, S; Rodrigues, A; Dias, S; Branco, J; Canhão, HAims: Clinical risk factors and bone mineral densitometry underestimate low-energy fracture (LEF) risk in people with diabetes. We aim to estimate the prevalence of LEF in diabetics, compare with nondiabetics; and evaluate possible predictors of LEF in people with diabetes. Methods: Cross-sectional, population-based study in Portuguese subjects over 40 years-old. Estimates computed as weighted proportions/means, considering sample design. Multivariate logistic regression models to evaluate the association of diabetes and LEF; and predictors of LEF in diabetics. Results: 7675 subjects were analysed, of which 1173 reported diabetes. Diabetics were older (mean age 66.0 ± 11.49y), more frequently reported osteoporosis and falls in the previous 12 months (32.4% vs. 22.9%). Prevalence of self-reported LEF was 16.2% (95% CI:13.68-19.13) among diabetics (vs. 13.3%, 95% CI:12.14-14.57, in nondiabetics); OR for the association diabetes and LEF:1.26, 95% CI:1.01-1.58, p = 0.045 (in women, adjusted OR:1.41, 95% CI:1.05-1.89, p = 0.02). Thirty percent of diabetics reported at least one major LEF and 70% in other sites. In diabetics, LEF was independently associated with self-reported osteoporosis and falls in the previous 12 months. Conclusion: People with diabetes reported more falls and had higher prevalence of self-reported LEF. Self-reported osteoporosis and falls were associated with LEF in diabetics. Our findings emphasize the need for fracture and falls preventive measures in diabetics.
- “Tá Pirando, Pirado, Pirou!". O Carnaval no Combate ao Estigma da Doença Mental no BrasilPublication . Costa de Sousa, M; Queiroga, LAo longo da História da Humanidade, a doença mental surge como um obstáculo entre o indivíduo e o seu próprio ambiente, tornando-o alienado da sociedade. O objetivo deste artigo é precisamente divulgar uma modalidade atual de combate ao estigma e à integração de pessoas com doença mental na sociedade, recorrendo a uma atividade culturalmente relevante, neste caso o Carnaval no Brasil. Tudo surgiu após a Reforma Psiquiátrica no Brasil, implementada em 2001. O tratamento da doença mental passou a ser preferencialmente em regime ambulatório ao invés do internamento, fomentando a integração do doente na sociedade e a desativação gradual dos manicómios. Dada a festividade carnavalesca ser vivida intensamente em todo o Brasil, em 2004 foi criado um “Coletivo Carnavalesco” designado por “Tá Pirando, Pirado, Pirou!”, organizado por trabalhadores do Instituto de Psiquiatria da Universidade Federal do Rio de Janeiro (UFRJ). O objetivo deste cortejo é acima de tudo desmistificar a ideia de que as pessoas com patologia mental são perigosas e proporcionar aos doentes um sentimento de pertença à sociedade, mostrando que o tratamento pode ser conduzido sem que haja exclusão social. A organização do coletivo é feita através de “Oficinas” que correspondem a atividades organizadas pelos trabalhadores do Instituto com os doentes e familiares, onde se prepara o desfile, desde a confeção da roupa até à aprendizagem do Samba. A adesão dos utentes, familiares e profissionais tem vindo a crescer, permitindo uma maior sensibilização da comunidade para a patologia mental e desta forma contribuir para a diminuição do estigma associado. Em 2018 foi criada pela primeira vez a ala infantil, representada pelo Centro de Atenção e Reabilitação à Infância e Mocidade (CARIM), vinculada ao Instituto de Psiquiatria da Universidade Federal do Rio de Janeiro (UFRJ) e que atende o público infanto-juvenil. Desde há quinze anos que este coletivo desfila na Avenida Pasteur do Rio de Janeiro, sendo considerado um dos maiores símbolos de inclusão da doença mental na comunidade brasileira.