Fernandes I.C.,Dermatovenereology Clinic of the Porto Hospital Center |
Teixeira M.A.,Porto Hospital Center |
Freitas I.,Porto Hospital Center |
Alves R.,Porto Hospital Center |
Lima M.,Dermatovenereology Clinic of the Porto Hospital Center
Anais Brasileiros de Dermatologia | Year: 2014
Background: Mastocytosis is a clonal disorder characterized by the accumulation of abnormal mast cells in the skin and/or in extra-cutaneous organs. Objectives: To present all cases of mastocytosis seen in the Porto Hospital Center and evaluate the performance of World Health Organization diagnostic criteria for systemic disease. Methods: The cases of twenty-four adult patients with mastocytosis were reviewed. Their clinical and laboratorial characteristics were assessed, and the properties of the criteria used to diagnose systemic mastocyto-sis were evaluated. Results: The age of disease onset ranged from 2 to 75 years. Twenty-three patients had cutaneous involvement and 75% were referred by dermatologists. Urticaria pigmentosa was the most common manifestation of the disease. One patient with severe systemic mast cell mediator-related symptoms showed the activating V560G KIT mutation. The bone marrow was examined in 79% of patients, and mast cell immunophenotyping was performed in 67% of the participants. Systemic disease was detected in 84% of cases, and 81% of the sample had elevated serum tryptase levels. All the diagnostic criteria for systemic mastocytosis had high specificity and positive predictive value. Bone marrow biopsy had the lowest sensitivity, negative predictive value and efficiency, while the highest such values were observed for mast cell immunophenotyping. Patients were treated with regimens including antihistamines, sodium cromoglycate, alpha-interferon, hydroxyurea and phototherapy. Conclusions: Cutaneous involvement is often seen in adult mastocytosis patients, with most individuals presenting with indolent systemic disease. Although serum tryptase levels are a good indicator of mast cell burden, bone marrow biopsy should also be performed in patients with normal serum tryptase, with flow cytometry being the most adequate method to diagnose systemic disease. © 2013 by Anais Brasileiros de Dermatologia.
Coutinho M.B.,Lusiadas Hospital |
Marques C.,University of Girona |
Mendes G.J.,Porto Hospital Center |
Goncalves C.,Widex Hearing Center
The Journal of laryngology and otology | Year: 2015
OBJECTIVE: To report a case of successful bone-anchored hearing aid implantation in an adult patient with type III osteogenesis imperfecta, which is commonly regarded as a contraindication to this procedure.CASE REPORT: A 45-year-old man with type III osteogenesis imperfecta presented with mixed hearing loss. There was a mild sensorineural component in both ears, with an air-bone gap between 45 and 50 dB HL. He was implanted with a bone-anchored hearing aid. The audiological outcome was good, with no complications and good implant stability (as measured by resonance frequency analysis).CONCLUSION: To our knowledge, this is the first recorded case of bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.
PubMed | Theresien Krankenhaus Mannheim, National and Kapodistrian University of Athens, University of Rome Tor Vergata, Instituto Valenciano Of Oncologia and 26 more.
Type: Journal Article | Journal: World journal of urology | Year: 2016
To reach standardized terminology in focal therapy (FT) for prostate cancer (PCa).A four-stage modified Delphi consensus project was undertaken among a panel of international experts in the field of FT for PCa. Data on terminology in FT was collected from the panel by three rounds of online questionnaires. During a face-to-face meeting on June 21, 2015, attended by 38 experts, all data from the online rounds were reviewed and recommendations for definitions were formulated.Consensus was attained on 23 of 27 topics; Targeted FT was defined as a lesion-based treatment strategy, treating all identified significant cancer foci; FT was generically defined as an anatomy-based (zonal) treatment strategy. Treatment failure due to the ablative energy inadequately destroying treated tissue is defined as ablation failure. In targeting failure the energy is not adequately applied to the tumor spatially and selection failure occurs when a patient was wrongfully selected for FT. No definition of biochemical recurrence can be recommended based on the current data. Important definitions for outcome measures are potency (minimum IIEF-5 score of 21), incontinence (new need for pads or leakage) and deterioration in urinary function (increase in IPSS >5 points). No agreement on the best quality of life tool was established, but UCLA-EPIC and EORTC-QLQ-30 were most commonly supported by the experts. A complete overview of statements is presented in the text.Focal therapy is an emerging field of PCa therapeutics. Standardization of definitions helps to create comparable research results and facilitate clear communication in clinical practice.
Gil-Nagel A.,Hospital Ruber International |
Elger C.,University of Bonn |
Ben-Menachem E.,Sahlgren University Hospital |
Halasz P.,Experimental Medical Research Institute |
And 8 more authors.
Epilepsia | Year: 2013
Purpose: To evaluate the efficacy and safety profile of eslicarbazepine acetate (ESL) added to stable antiepileptic therapy in adults with partial-onset seizures. Methods: Data from 1,049 patients enrolled from 125 centers, in 23 countries, in three phase III double-blind, randomized, placebo-controlled studies were pooled and analyzed. Following a 2-week titration period, ESL was administered at 400 mg, 800 mg, and 1,200 mg once-daily doses for 12 weeks. Key Findings: Seizure frequency was significantly reduced with ESL 800 mg (p < 0.0001) and 1,200 mg (p < 0.0001) compared to placebo. Median relative reduction in seizure frequency was, respectively, 35% and 39% (placebo 15%) and responder rate was 36% and 44% (placebo 22%). ESL was more efficacious than placebo regardless of gender, geographic region, epilepsy duration, age at time of diagnosis, seizure type, and number and type of concomitant antiepileptic drugs (AEDs). Incidence of adverse events (AEs) and AEs leading to discontinuation were dose dependent. AEs occurred mainly during the first weeks of treatment, with no difference between groups after 6 weeks. Most common AEs (>10% patients) were dizziness, somnolence, and headache. The incidence of AEs in ESL groups compared to placebo was generally consistent among different subpopulations. Significance: Once-daily ESL 800 mg and 1,200 mg showed consistent results across all efficacy and safety end points. Results were independent of study population characteristics and type and number of concomitant AEDs. © 2012 International League Against Epilepsy.
Fraga A.,Porto Hospital Center |
Fraga A.,Abel Salazar Biomedical Sciences Institute |
Ribeiro R.,Porto Hospital Center |
Ribeiro R.,Portuguese Institute of Oncology |
And 7 more authors.
Clinical Genitourinary Cancer | Year: 2015
Angiogenesis, increased glycolysis, and cellular adaptation to hypoxic microenvironment are characteristic of solid tumors, including prostate cancer. These representative features are the cornerstone of cancer biology, which are well correlated with invasion, metastasis, and lethality, as well as likely with the success of prostate cancer treatment (eg, tumor hypoxia has been associated with resistance to chemotherapy and radiotherapy). It is well established that prostate cancer cells also metabolically depend on enhanced glucose transport and glycolysis for expansion, whereas growth is contingent with neovascularization to permit diffusion of oxygen and glucose. While hypoxia inducible factor 1 alpha (HIF-1α) remains the central player, the succeeding activated molecules and pathways track distinct branches, all positively correlated with the degree of intratumoral hypoxia. Among these, the vascular endothelial growth factor axis as well as the lysyl oxidase and carbonic anhydrase IX activities are notable in prostate cancer and merit further study. Here, we demonstrate their linkage with HIF-1α as a tentative explanatory mechanism of prostate cancer aggressiveness. Hypoxia drives a tale where HIF-1α-dependent effects lead to many influences in distinct key cancer biology features, rendering targeted therapies toward targets at the endings less efficient. The most appropriate approach will be to inhibit the upstream common driver (HIF-1α) activity. Additional translational and clinical research initiatives in prostate cancer are required to prove its usefulness. © 2015 Elsevier Inc. All rights reserved.
Martins M.V.,University of Porto |
Peterson B.D.,Chapman University |
Almeida V.,University of Porto |
Mesquita-Guimaraes J.,Porto Hospital Center |
Costa M.E.,University of Porto
Human Reproduction | Year: 2014
STUDY QUESTION: Is perceived social support from partner, family, and friends associated with increased infertility-related stress? SUMMARY ANSWER: While men's perceived support did not seem to influence their partners' stress, women's perceptions of spousal and familial support can affect the way men deal with the challenge of infertility. WHAT IS KNOWN ALREADY: Previous studies showed that low levels of social support are associated with poor psychosocial adjustment and treatment termination in women and men. Studies examining the impact of social support using the couple as unit of analysis are lacking. STUDY DESIGN, SIZE, DURATION: A cross-sectional sample of 613 Portuguese patients participated in the research, online over a 3-month period, and in a public fertility clinic over 11 months. PARTICIPANTS/ MATERIALS, SETTING, METHODS: The final sample comprised 213 married or cohabiting couples (191 from the fertility clinic) who were actively attempting to have a child, were seeking infertility treatment and had not undergone previous preimplantation genetic diagnosis. Perceived social support was assessed through the Multidimensional Scale of Perceived Social Support and infertility-related stress was assessed with the fertility problem inventory. Hypotheses were tested by applying the actor-partner interdependence model using structural equation modeling. MAIN RESULTS AND THE ROLE OF CHANCE: Couples had been living together for an average (±SD) of 6 ± 3.5 years, and attempting a pregnancy for 3.8 ± 2.6 years. Nearly half of the couples had undergone infertility treatment (41.3%). Infertility stress was found to be associated with low family support for women (β = -0.27, P =. 003), and low partner support for both men (β = -0.29, P =. 001) and women (β = -0.45, P =. 006). Both women and men's perceived friend support were not significantly related to male or female infertility stress. Men infertility stress was also associated with their partners low levels of partner (β = -0.24, P =. 049) and family support (β = -0.23, P <. 001). No significant partner effects were observed for women. Despite being related to actor effects alone (female partner and family support), the explained variance of the model in women's fertility stress was greater (R2 = 21%) than that (R2 = 15.6%) for the combined actor and partner effects in men's fertility stress (male partner support, female partner and family support). LIMITATIONS, REASONS FOR CAUTION: The study data are cross-sectional and the generalizability of results is limited by self-selection. The characteristics of non-participants in both the clinical and online samples were not available, the perception of infertility-specific supportive behaviors was not assessed and differential analyses according to infertility diagnosis were not included in this study. WIDER IMPLICATIONS OF THE FINDINGS: Our data underline the importance of partner support in alleviating the burden of infertility. Men may experience infertility indirectly through the impact that it has on their partners. Our findings reinforce the need to involve the male partner throughout the whole treatment process and for couple-based interventions when providing infertility counseling. Further prospective research should be aimed at investigating the male experience of infertility.STUDY FUNDING/COMPETING INTERESTThis research was supported by a PhD scholarship from the Portuguese Foundation for Science and Technology granted to M.M. (FCT, SFRH/BD/44232/2008). The authors have no conflicts of interest to declare. © The Author 2013.
Casanova J.,Porto Hospital Center |
Paiva C.,Porto Hospital Center |
Carvalho C.,Porto Hospital Center |
Cunha A.C.,Porto Hospital Center
Journal of Reproductive Medicine | Year: 2014
BACKGROUND: Vascular anastomoses in monochorionic placentas are the key factor in the development of several complications, including twin anemiapolycythemia sequence (TAPS). TAPS is an uncommon form of chronic fetofetal transfusion, characterized by large intertwin hemoglobin (Hb) differences in the absence of amniotic fluid discordances. CASE: We report 3 unusual cases of spontaneous TAPS. Looking to the intertwin Hb differences, each case fits in a different stage of postnatal twin anemia-polycythemia classification. Five of the 6 twins had a good outcome despite major hemodynamic changes. CONCLUSION: Spontaneous TAPS is by far more infrequent than its postlaser counterpart. Our report is somewhat uncommon due to the fact that the 3 cases were spontaneous, consecutive, and each one fit as an example of the various degrees of severity according to the described classifications of this condition. © Journal of Reproductive Medicine®, Inc.
PubMed | Porto Hospital Center
Type: Journal Article | Journal: Urology case reports | Year: 2016
Studers neobladder lithiasis is a rare but important long term complication of this orthotopic bladder substitute technique. We report a case of a 45 year-old male patient, submitted to a radical cystoprostatectomy with a Studers orthotopic neobladder 4 years before, presenting bad compliance to recommended urinary habits, increased production of mucus and high post voiding residue. CT scan and urethrocystography showed a distended pouch with 2 major sacculations with narrow communication and a stone in each sacculation. A minimally invasive endoscopic technique was successfully used in the treatment of the 2 small calculus.
PubMed | Porto Hospital Center
Type: Journal Article | Journal: Acta otorrinolaringologica espanola | Year: 2016
Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is an autosomal dominant disorder characterised by the presence of multiple arteriovenous malformations (AVMs) affecting multiple organs. Many procedures have been used for epistaxis control in patients with this disorder. The objective of this study was to report the treatment of severe HHT-related epistaxiswith the modified Youngs procedure.We describe the treatment of 4 patients with severe blood-transfusion-dependent epistaxis who underwent a modified Youngs procedure in a tertiary hospital. The nasal closure was bilateral and complete in all cases. All patients were followed for 12 months or longer.The procedure was well tolerated and complete cessation of bleeding was achieved in all the patients.Youngs technique is a safe surgical procedure, well tolerated by patients with severe epistaxis and HHT.
PubMed | University of Porto, Abel Salazar Biomedical Sciences Institute and Porto Hospital Center
Type: Journal Article | Journal: PloS one | Year: 2016
Noninvasive ventilation (NIV) is being used increasingly in patients who have a do not intubate (DNI) order. However, the impact of NIV on the clinical and health-related quality of life (HRQOL) in the emergency setting is not known, nor is its effectiveness for relieving symptoms in end-of-life care.The aim of this prospective study was to determine the outcome and HRQOL impact of regular use of NIV outcomes on patients with a DNI order who were admitted to the emergency room department (ED).Eligible for participation were DNI-status patients who receive NIV for acute or acute-on-chronic respiratory failure when admitted to the ED of a tertiary care, university-affiliated, 600-bed hospital between January 2014 and December 2014. Patients were divided into 2 groups: (1) those whose DNI order related to a decision to withhold therapy and (2) those for whom any treatment, including NIV, was provided for symptom relief only. HRQOL was evaluated only in group 1, using the 12-item Short Form Health Survey (SF-12). Long-term outcome was evaluated 90 days after hospital discharge by means of a telephone interview.During the study period 1727 patients were admitted to the ED, 243 were submitted to NIV and 70 (29%) were included in the study. Twenty-nine (41%) of the 70 enrollees received NIV for symptom relief only (group2). Active cancer [7% vs 35%, p = 0,004] and neuromuscular diseases [0% vs. 17%] were more prevalent in this group. NIV was stopped in 59% of the patients in group 2 due to lake of clinical benefit. The in-hospital mortality rate was 37% for group 1 and 86% for group 2 0,001). Among patients who were discharged from hospital, 23% of the group 1 and all patients in group 2 died within 90 days. Relative to baseline, no significant decline in HRQOL occurred in group 1 by 90 days postdischarge.The survival rate was 49% among DNI-status patients for whom NIV was used as a treatment in ED, and these patients did not experience a decline in HRQOL throughout the study. NIV did not provide significant relief of symptoms in more than half the patients who receive it for that purpose.