São João da Madeira, Portugal
São João da Madeira, Portugal

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Teixeira S.,Centro Hospitalar Of Sao Joao
Journal of Craniofacial Surgery | Year: 2017

BACKGROUND:: Pharyngocutaneous fistula is a common complication of laryngopharyngeal surgery and is associated with increased morbidity and mortality. Beyond the classical management, negative-pressure wound therapy (NPWT) can be an alternative and effective treatment. CLINICAL REPORT:: Two patients with pT3N0M0 squamous cell carcinoma of pyriform sinus were subjected to total laryngectomy and pharyngoesophageal reconstruction of a circular (patient 1) and an anterior wall defect (patient 2) with radial forearm free flap and pectoralis major muscle flap, respectively. Both developed a pharyngocutaneous fistula and NPWT was used.A significant decrease of the fistula aperture and exudate was observed after 22 and 21 days of NPWT in patients 1 and 2, respectively. After that standard wound care was instituted and closure of the fistulae was accomplished in 5 and 7 days, respectively. CONCLUSION:: Negative-pressure wound therapy can be an effective treatment for pharyngocutaneous fistula closure, either in the setting of fistulae that persist besides multiple surgical revisions using muscle flaps or as a first-line therapy when fistulae develops. © 2017 by Mutaz B. Habal, MD.


Ferreira J.P.,Abel Salazar Biomedical Sciences Institute | Santos M.,Abel Salazar Biomedical Sciences Institute | Almeida S.,University of Lisbon | Marques I.,Abel Salazar Biomedical Sciences Institute | And 2 more authors.
European Journal of Internal Medicine | Year: 2014

Background/objectives: Mineralocorticoid receptor antagonist (MRA) use in acutely decompensated chronic heart failure (ADCHF) may improve congestion through diuretic effect and prevent neurohormonal activation. We aimed to evaluate the clinical effect and safety of spironolactone in ADCHF. Methods: Prospective, experimental, single-center, and single-blinded trial. Patients were treated with: standard ADCHF therapy or oral spironolactone 50-100 mg/d plus standard ADCHF therapy. Results: During a 1 year period, 100 patients were enrolled, 50 included in the treatment group. Mean (SD) spironolactone dose (mg) at day 1 was 94.5 ± 23.3 and at day 3 was 62.7 ± 24.3. Worsening renal function (increase in pCr ≥ 0.3 mg/dL from day 1 to day 3) was more likely to occur in control group (20% vs. 4%; p = 0.038), serum potassium did not differ between groups, and plasma NTproBNP had a significant decrease in spironolactone group at day 3 (median [IQR], 2488 [4579] vs. 1555 [1832]; p = 0.05). Furthermore, a greater proportion of patients in the treatment group were free of congestion at day 3: less edema, rales, jugular venous pressure (JVP) and orthopnea (all, p < 0.05). In addition, a significantly higher proportion of patients were on oral furosemide at day 3 (44% vs. 82%; p < 0.001). Conclusions: Our study supports the safety of high dose spironolactone in ADCHF and suggests a positive impact in the resolution of congestion. The important findings of our pilot study need to be confirmed in larger trials. © 2013 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.


Da Corte F.C.,Centro Hospitalar Of Sao Joao | Neves N.,University of Porto
European Journal of Orthopaedic Surgery and Traumatology | Year: 2014

Rheumatoid arthritis (RA) is the most common inflammatory disease of the cervical spine (CS). After hands and feet, CS is the most commonly involved segment, being present in more than half of the patients with RA. Especially in the CS, RA may cause degeneration of ligaments, leading to laxity, instability and subluxation of the vertebral bodies. This is often asymptomatic or symptoms are erroneously attributed to peripheral manifestations. Otherwise, this may cause compression of spinal cord (SC) and medulla oblongata leading to severe neurologic deficits and even sudden death. Owing to its potentially debilitating and life-threatening sequelae, inevitable progression once neurologic deficits occur and the poor medical condition of afflicted patients, CS involvement remains a priority in the diagnosis and its treatment will remain a challenge. The surgical approach aims a solid fixation of the upper cervical spine, giving stability, preventing neurologic deterioration and injury to the SC, leading to improved neurologic function, vascular integrity and maintenance of sagittal balance. The recent advances in surgical techniques, complete understanding of the anatomy and precise preoperative evaluation led to safer and more effective procedures that have decreased complication rates. Based on the fact that when a patient becomes myelopathic the rate of long-term mortality increases and the chance of neurologic recovery decreases, many authors agree that early surgical intervention, before the onset of neurologic deficits, gives a more satisfactory outcome. However, the timing when a prophylactic stabilization should occur is poorly defined, and so, patients with radiographic instability but without evidence of neurologic deficit are still the most difficult to manage. © 2013 Springer-Verlag France.


Oliveira C.,University of Porto | Pinheiro H.,University of Porto | Figueiredo J.,University of Porto | Seruca R.,University of Porto | And 2 more authors.
Progress in Molecular Biology and Translational Science | Year: 2013

The only gastric cancer (GC) syndrome with a proven inherited defect is designated as hereditary diffuse gastric cancer (HDGC) and is caused by germline E-cadherin/CDH1 alterations. Other E-cadherin-associated hereditary disorders have been identified, encompassing HDGC families with or without cleft-lip/palate involvement, isolated early-onset diffuse GCs, and lobular breast cancer families without GC. To date, 141 probands harboring more than 100 different germline CDH1 alterations, mainly point mutations and large deletions, have been described in these different settings. A third of all HDGC families described so far carry recurrent CDH1 alterations. Full screening of CDH1 is recommended in patients fulfilling the HDGC criteria and total prophylactic gastrectomy is the only reliable intervention for carriers of pathogenic alterations. In this chapter, we discuss CDH1-associated syndromes, frequency and type of CDH1 germline alterations, clinical criteria, and guidelines for genetic counseling, molecular pathology, and available animal/cell line models of the disease. © 2013 Elsevier Inc.


Carneiro F.,University of Porto | Carneiro F.,Centro Hospitalar Of Sao Joao
Pathologe | Year: 2012

In 1998, Guilford et al. identified the hereditary diffuse gastric cancer (HDGC) syndrome, caused by germline alterations at the CDH1 (E-cadherin) gene. To date, 141 probands harboring more than 100 different germline CDH1 alterations, mainly point mutations and large deletions, have been described. In mutation-positive individuals prophylactic total gastrectomy is recommended. The systematic histological study of prophylactic gastrectomies shows intramucosal signet-ring cell carcinoma and pre-invasive lesions including in situ signet ring carcinoma with pagetoid spread of signet ring cells. In 2011, a new hereditary gastric cancer syndrome was identified: gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). GAPPS is a unique gastric polyposis syndrome with a significant risk of gastric adenocarcinoma, characterized by the autosomal dominant transmission of fundic gland polyposis, with areas of dysplasia or intestinal-type GC, restricted to the proximal stomach, with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes. © 2012 Springer-Verlag Berlin Heidelberg.


Linhares P.,Centro Hospitalar Of Sao Joao | Carvalho B.,Centro Hospitalar Of Sao Joao | Vaz R.,Centro Hospitalar Of Sao Joao
Acta Neurochirurgica | Year: 2013

Background Infection constitutes a serious adverse event in deep brain stimulation (DBS) surgery, being responsible for difficult therapeutic decisions that may ultimately involve the removal of implanted material. Some cases begin with skin erosion and wound dehiscence of the retroauricular incision, which is one of the most fragile points. Several techniques of rotation flaps and skin reconstruction, as well as prolonged antibiotic regimens, have been proposed as therapeutic options. To prevent the onset of this complication, the authors propose a one-step tunneling technique of DBS extensions, avoiding the opening of the retroauricular space. Methods We describe a surgical technique of a one-step tunneling of DBS extensions in 20 patients submitted to subthalamic DBS for Parkinson's disease, avoiding the opening of the retroauricular space. Results After implantation of the extensions using this technique, we had no erosions of the retroauricular skin, with a consequent reduction in the number of infections. Conclusions The authors describe an easy surgical technique that allows reduction of wound and erosion complications, with great benefits for DBS patients. © Springer-Verlag Wien 2013.


Vieira T.,Centro Hospitalar Of Sao Joao | de Oliveira J.F.,Centro Hospitalar Of Sao Joao | da Graca Castel-Branco M.,Centro Hospitalar Of Sao Joao
Allergologia et Immunopathologia | Year: 2014

Background: The impact of severe asthma on patients' quality of life (QoL) has been previously demonstrated, as well the difficulties in controlling the disease. We aimed to evaluate the effect of omalizumab on QoL and asthma control, and its safety and tolerability in real-life conditions in Portugal. Methods: Prospective and open-label study in 15 adult patients with uncontrolled severe persistent allergic asthma on omalizumab treatment ≥16 weeks (w). The short (at 16. w) and long-term (at 1 and 2 years) (y) effects of omalizumab were assessed through the Asthma Life Questionnaire (ALQ) and the Asthma Control Test (ACT). Other secondary outcomes were evaluated. Results: A significant reduction in ALQ total score (at 16. w, p= 0.002; at 1. y, p= 0.033 and at 2. y, p= 0.024), as well as in the 'non-scheduled medical visits' and the 'medication use' domains in both the short and long terms was observed. Regarding ACT, we verified a significant improvement in total score (at 16. w, p= 0.004; at 1. y, p= 0.004 and at 2. y, p= 0.008) and in almost all of the five individual questions. Asthma exacerbations and unscheduled health care visits were significantly decreased. There was a significant rise in lung function and a decrease in daily inhaled steroids dose. The most frequent adverse effects were headaches and nausea. Conclusions: Omalizumab promoted a global benefit on QoL and asthma control outcomes. It also yielded a reduction in asthma exacerbations and unscheduled health care visits, a steroid-sparing effect, and an improvement in lung function. The drug was found to be generally safe and well-tolerated. © 2012 SEICAP.


Vieira-Baptista P.,Centro Hospitalar Of Sao Joao
Journal of Lower Genital Tract Disease | Year: 2016

OBJECTIVES: The authors express their concern about the impact that the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition “genito-pelvic pain/penetration disorder” may have on vulvodynia, namely, its nondiagnosis or assumption that it is a merely psychological issue. METHODS: A review of the concepts of “genito-pelvic pain/penetration disorder,” “vaginismus,” “dyspareunia,” and “vulvodynia” was performed to understand if the suspicion that the more recent and broader DSM concept can have a negative impact on vulvodynia patients. RESULTS: The definition and referred associated conditions of the “genito-pelvic pain/penetration disorder” overlap those of provoked vestibulodynia, a form of vulvodynia characterized by mechanical allodynia localized to the vulvar vestibule. Despite the footnote that the diagnosis of the former is made after the exclusion of a better explanation for the complaints, the authors believe that a significant number of vulvodynia patients will be included in the “genito-pelvic pain/penetration disorder,” thus risking an inappropriate therapeutic approach. “Vaginismus” and “dyspareunia” may occur together, but it is not always the case; assuming otherwise may have a negative impact in the treatment of these women. CONCLUSIONS: The “genito-pelvic pain/penetration disorder” diagnosis may help the clinical approach of women with dyspareunia and/or an increased pelvic muscle tonus. However, it may have a significant negative impact in the approach and treatment of affected women: treatment cannot be “one size fits all.” A significant number of women with this diagnosis will have vulvodynia, and exclusively psychological/psychiatric approach would be suboptimal for the condition. Copyright © 2016 by the American Society for Colposcopy and Cervical Pathology


Massano J.,Centro Hospitalar Of Sao Joao | Massano J.,University of Porto | Garrett C.,Centro Hospitalar Of Sao Joao | Garrett C.,University of Porto
Frontiers in Neurology | Year: 2012

Parkinson's disease is a common and often debilitating disorder, with a growing prevalence accompanying global population aging. Current drug therapy is not satisfactory enough for many patients, especially after a few years of symptom progression. This is mainly due to the motor complications that frequently emerge as disease progresses. Deep brain stimulation (DBS) is a useful therapeutic option in carefully selected patients that significantly improves motor symptoms, functional status, and quality of life. However, cognitive impairment may limit patient selection for DBS, as patients need to have sufficient mental capabilities in order to understand the procedure, as well as its benefits and limitations, and cooperate with the medical team throughout the process of selection, surgery, and postsurgical follow-up. On the other hand it has been observed that certain aspects of cognitive performance may decline after DBS, namely when the therapeutic target is the widely used subthalamic nucleus. These are important pieces of information for patients, their families, and health care professionals. This manuscript reviews these aspects and their clinical implications. © 2012 Massano and Garrett.


Meireles J.,Centro Hospitalar Of Sao Joao | Meireles J.,University of Porto | Massano J.,Centro Hospitalar Of Sao Joao | Massano J.,University of Porto
Frontiers in Neurology | Year: 2012

Parkinson's disease (PD) is a common, disabling, neurodegenerative disorder. In addition to classical motor symptoms, non-motor features are now widely accepted as part of the clinical picture, and cognitive decline is a very important aspect of the disease, as it brings an additional significant burden for the patient and caregivers. The diagnosis of cognitive decline in PD, namely mild cognitive impairment (MCI) and dementia, can be extremely challenging, remaining largely based on clinical and cognitive assessments. Diagnostic criteria and methods for PD dementia and MCI have been recently issued by expert work groups. This manuscript has synthesized relevant data in order to obtain a pragmatic and updated review regarding cognitive decline in PD, from milder stages to dementia. This text will summarize clinical features, diagnostic methodology, and therapeutic issues of clinical decline in PD. Relevant clinical genetic issues, including recent advances, will also be approached. © 2012 Meireles and Massano.

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