Browsing by Issue Date, starting with "2020-03"
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- Leigh Syndrome with Atypical Cerebellum Imaging FeaturesPublication . Veiga, M; Marecos, C; Vieira, JP; Conceição, C
- Ocular Involvement in Systemic Lupus Erythematosus Patients: a Paradigm Shift Based on the Experience of a Tertiary Referral CenterPublication . Dias-Santos, A; Ferreira, J; Pinheiro, S; Cunha, JP; Alves, M; Papoila, AL; Moraes-Fontes, MF; Proença, RObjective: To evaluate ocular involvement in a cohort of systemic lupus erythematosus (SLE) patients of a tertiary referral center and to compare the results with the existing literature. Methods: Patients underwent a complete ophthalmological evaluation, including visual acuity, slit-lamp examination, fluorescein staining, Schirmer-I test, Goldmann applanation tonometry, fundoscopy, 10-2 automated threshold visual fields, fundus autofluorescence and spectral-domain optical coherence tomography to screen for hydroxychloroquine (HCQ) macular toxicity. Results: A total of 161 patients (16 men and 145 women) were enrolled in this study. The mean age was 47.6 years and the mean disease duration was 11.5 years. Fifty patients (31.1%) had at least one ocular manifestation of SLE. The most frequent manifestation was dry eye syndrome (12.4%), immediately followed by cataracts (11.2%) and HCQ macular toxicity (11.2%). Among patients with HCQ maculopathy, two presented with an atypical spectral-domain optical coherence tomography pattern. Five patients (3.1%) presented with glaucoma, two patients (1.2%) presented with SLE retinopathy while only one presented with lupus choroidopathy (0.6%). Conclusions: Compared with previous studies, we conclude there has been a significant reduction in disease-related ocular complications, particularly those associated with poor systemic disease control. On the other hand, drug and age-related complications are assuming a prominent role in the ophthalmic care of these patients.
- Sentinel Node Total Tumour Load As a Predictive Factor for Non-Sentinel Node Status in Early Breast Cancer Patients – The porttle studyPublication . Fougo, JL; Amendoeira, I; Brito, MJ; Correia, AP; Gonçalves, A; Honavar, M; Machado, A; Magalhães, A; Marta, S; Nogueira, M; Peleteiro, B; Pontes, POSNA is a molecular assay for the detection of sentinel node metastasis. TTL emerged as a concept that seems to accurately predict the status of the NSN. Authors tried to confirm this motion. This is a retrospective and multicentric study that analyzed 2164 patients, 579 of whom had positive SN and completion AD. Logistic regression models were performed in order to identify a suitable cutoff to identify patients who benefit from AD. Univariate and multivariate regression analysis showed a relationship between TTL>30000 and the presence of NSN metastasis (OR 2.84, CI 1.99-4.08, p < 0.001). Logistic regression indicated that the cutoff of 30000 copies/μL better discriminates patients with NSN positivity and allows wide use of these criteria. This cutoff value may safely assist clinicians and patients to decide to proceed or not with an AD.
- Utilizing Machine Learning for Pre- and Postoperative Assessment of Patients Undergoing Resection for BCLC-0, A and B Hepatocellular Carcinoma: Implications for Resection Beyond the BCLC GuidelinesPublication . Tsilimigras, D; Mehta, R; Moris, D; Sahara, K; Bagante, F; Paredes, A; Farooq, A; Ratti, F; Pinto Marques, H; Silva, S; Soubrane, O; Lam, V; Poultsides, G; Popescu, I; Grigorie, R; Alexandrescu, S; Martel, G; Workneh, A; Guglielmi, A; Hugh, T; Aldrighetti, L; Endo, I; Pawlik, TBackground: There is an ongoing debate about expanding the resection criteria for hepatocellular carcinoma (HCC) beyond the Barcelona Clinic Liver Cancer (BCLC) guidelines. We sought to determine the factors that held the most prognostic weight in the pre- and postoperative setting for each BCLC stage by applying a machine learning method. Methods: Patients who underwent resection for BCLC-0, A and B HCC between 2000 and 2017 were identified from an international multi-institutional database. A Classification and Regression Tree (CART) model was used to generate homogeneous groups of patients relative to overall survival (OS) based on pre- and postoperative factors. Results: Among 976 patients, 63 (6.5%) had BCLC-0, 745 (76.3%) had BCLC-A, and 168 (17.2%) had BCLC-B HCC. Five-year OS among BCLC-0/A and BCLC-B patients was 64.2% versus 50.2%, respectively (p = 0.011). The preoperative CART model selected α-fetoprotein (AFP) and Charlson comorbidity score (CCS) as the first and second most important preoperative factors of OS among BCLC-0/A patients, whereas radiologic tumor burden score (TBS) was the best predictor of OS among BCLC-B patients. The postoperative CART model revealed lymphovascular invasion as the best postoperative predictor of OS among BCLC-0/A patients, whereas TBS remained the best predictor of long-term outcomes among BCLC-B patients in the postoperative setting. On multivariable analysis, pathologic TBS independently predicted worse OS among BCLC-0/A (hazard ratio [HR] 1.04, 95% confidence interval [CI] 1.02-1.07) and BCLC-B patients (HR 1.13, 95% CI 1.06-1.19) undergoing resection. Conclusion: Prognostic stratification of patients undergoing resection for HCC within and beyond the BCLC resection criteria should include assessment of AFP and comorbidities for BCLC-0/A patients, as well as tumor burden for BCLC-B patients.
- Acute-on-Chronic Liver Failure Syndrome - Clinical Results from an Intensive Care Unit in a Liver Transplant CenterPublication . Pereira, R; Bagulho, L; Sousa Cardoso, FObjective: To characterize a cohort of acute-on-chronic liver failure patients in Intensive Care and to analyze the all-cause 28-day mortality risk factors assessed at ICU admission and day 3. Methods: This was a retrospective cohort study of consecutive patients admitted to the intensive care unit between March 2013 and December 2016. Results: Seventy-one patients were included. The median age was 59 (51 - 64) years, and 81.7% of patients were male. Alcohol consumption alone (53.5%) was the most frequent etiology of cirrhosis and infection (53.5%) was the most common acute-on-chronic liver failure precipitating event. At intensive care unit admission, the clinical severity scores were APACHE II 21 (16 - 23), CLIF-SOFA 13 (11 - 15), Child-Pugh 12 (10 - 13) and MELD 27 (20 - 32). The acute-on-chronic liver failure scores were no-acute-on-chronic liver failure: 11.3%; one: 14.1%; two: 28.2% and three: 46.5%; and the number of organ failures was one: 4.2%; two: 42.3%; three: 32.4%; four: 16.9%; and five: 4.2%. Liver transplantation was performed in 15.5% of patients. The twenty-eight-day mortality rate was 56.3%, and the in-ICU mortality rate was 49.3%. Organ failure at intensive care unit admission (p = 0.02; OR 2.1; 95%CI 1.2 - 3.9), lactate concentration on day 3 (p = 0.02; OR 6.3; 95%CI 1.4 - 28.6) and the international normalized ratio on day 3 (p = 0.03; OR 10.2; 95%CI 1.3 - 82.8) were independent risk factors. Conclusion: Acute-on-chronic liver failure patients presented with high clinical severity and mortality rates. The number of organ failures at intensive care unit admission and the lactate and international normalized ratio on day 3 were independent risk factors for 28-day mortality. We consider intensive care essential for acute-on-chronic liver failure patients and timely liver transplant was vital for selected patients.
- Catatonia in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis Misdiagnosed as SchizophreniaPublication . Ponte, A; Brito, A; Nóbrega, C; Pinheiro, S; Gama Marques, JAnti-N-Mmethyl-D-aspartate receptor encephalitis is an autoimmune disease of the central nervous system with prominent neurologic and psychiatric features. Symptoms appear progressively and sometimes with an exclusively psychiatric initial presentation. The patient's evaluation should be meticulous, and we should use all the diagnostic tests required for the exclusion of entities that can mimic this disease. We report the diagnostic investigation of a case of anti-N-methyl-D-aspartate receptor encephalitis in a patient with a previous diagnosis of schizophrenia with poor response to antipsychotics. The aim of this case report is to highlight the importance of close surveillance for neuropsychiatric symptoms, especially catatonia, and to recognize autoimmune encephalitis in the differential diagnosis of psychotic disorders with neurological symptoms and resistance or intolerance to antipsychotics. A prompt diagnosis will contribute to a faster onset of therapy and an overall improvement in prognosis.
- Quality of Life in Subjects with Upper- and Lower-Limb Spasticity Treated with IncobotulinumtoxinAPublication . Fheodoroff, K; Rekand, T; Medeiros, L; Koßmehl, P; Wissel, J; Bensmail, D; Scheschonka, A; Flatau-Baqué, B; Simon, O; Dressler, D; Simpson, DMBackground: We evaluated quality of life among subjects with upper- and lower-limb spasticity who received escalating doses of incobotulinumtoxinA (total body doses up to 800 U) in the prospective, single-arm, dose-titration TOWER study. Methods: In this exploratory trial, subjects (N = 155; 18-80 years of age) with upper- and lower-limb spasticity due to cerebral causes who were deemed to require total body doses of up to 800 U incobotulinumtoxinA received three consecutive injection cycles of incobotulinumtoxinA (400, 600, and up to 800 U), each with 12 to 16 weeks' follow-up. QoL was assessed using the EuroQol 5-dimensions questionnaire, three-level (EQ-5D), before and 4 weeks post-injection in each injection cycle and at the end of injection cycle 3. Results: The mean EQ-5D visual analog scale scores of 155 participants continuously improved from study baseline to 4 weeks post-injection in all injection cycles (mean [standard deviation] change 6.7 [14.1], 9.6 [16.3], and 8.6 [17.0] for injection cycles 1, 2, and 3, respectively; p < 0.0001 for all, paired sample t-test). In general, among those with a change in the EQ-5D rating of their condition, the proportion of subjects with 'improvement' was greater than that with 'worsening' for individual EQ-5D dimensions across all injection cycles. At the end of injection cycle 3, the proportion of subjects rating their condition as 'normal' increased from study baseline for all dimensions, and there was a ≥ 46% reduction in the proportion of subjects with a rating of 'severe impairment'. Conclusion: These preliminary results suggest that escalating incobotulinumtoxinA doses up to 800 U are associated with improvement in quality of life ratings in subjects with multifocal upper- and lower-limb spasticity, and form a basis for future comparator studies.