Publications by authors named "Abilio Reis"

21 Publications

  • Page 1 of 1

Incidence of symptomatic venous thromboembolism following hospitalization for coronavirus disease 2019: Prospective results from a multi-center study.

Thromb Res 2021 02 11;198:135-138. Epub 2020 Dec 11.

Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.

Background: Thrombosis and pulmonary embolism appear to be major causes of mortality in hospitalized coronavirus disease 2019 (COVID-19) patients. However, few studies have focused on the incidence of venous thromboembolism (VTE) after hospitalization for COVID-19.

Methods: In this multi-center study, we followed 1529 COVID-19 patients for at least 45 days after hospital discharge, who underwent routine telephone follow-up. In case of signs or symptoms of pulmonary embolism (PE) or deep vein thrombosis (DVT), they were invited for an in-hospital visit with a pulmonologist. The primary outcome was symptomatic VTE within 45 days of hospital discharge.

Results: Of 1529 COVID-19 patients discharged from hospital, a total of 228 (14.9%) reported potential signs or symptoms of PE or DVT and were seen for an in-hospital visit. Of these, 13 and 12 received Doppler ultrasounds or pulmonary CT angiography, respectively, of whom only one patient was diagnosed with symptomatic PE. Of 51 (3.3%) patients who died after discharge, two deaths were attributed to VTE corresponding to a 45-day cumulative rate of symptomatic VTE of 0.2% (95%CI 0.1%-0.6%; n = 3). There was no evidence of acute respiratory distress syndrome (ARDS) in these patients. Other deaths after hospital discharge included myocardial infarction (n = 13), heart failure (n = 9), and stroke (n = 9).

Conclusions: We did not observe a high rate of symptomatic VTE in COVID-19 patients after hospital discharge. Routine extended thromboprophylaxis after hospitalization for COVID-19 may not have a net clinical benefit. Randomized trials may be warranted.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.thromres.2020.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7836837PMC
February 2021

Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial.

Eur Heart J 2020 Nov 24. Epub 2020 Nov 24.

Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK.

Aims: This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

Methods And Results: For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17-33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18-51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2  P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group.

Conclusion: This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/eurheartj/ehaa696DOI Listing
November 2020

Severe Pulmonary Hypertension Management Across Europe (PHAROS): an ERS Clinical Research Collaboration.

Eur Respir J 2020 05 7;55(5). Epub 2020 May 7.

Clinical Dept of Respiratory Diseases, Pulmonary Hypertension Center, UZ Leuven, Leuven, Belgium.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1183/13993003.01047-2020DOI Listing
May 2020

Pulmonary arterial hypertension registries: past, present and into the future.

Eur Respir Rev 2019 12 18;28(154). Epub 2019 Dec 18.

Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1183/16000617.0128-2019DOI Listing
December 2019

Disability and its clinical correlates in pulmonary hypertension measured through the World Health Organization Disability Assessment Schedule 2.0: a prospective, observational study.

J Bras Pneumol 2019 May 30;45(4):e20170355. Epub 2019 May 30.

. Institute of Electronics and Telematics Engineering of Aveiro, University of Aveiro, Aveiro, Portugal.

Objective: To characterise the degree of disability in pulmonary hypertension (PH) patients based on the World Health Organisation Disability Assessment Schedule 2.0 (WHODAS 2.0).

Method: A prospective and observational study of patients with documented PH (N = 46). Patients completed the WHODAS 2.0 questionnaire during a scheduled routine clinical visit, and their demographic and clinical characteristics were retrieved from electronic medical records (EMR). In subsequent visits, selected clinical variables were registered to assess disease progression.

Results: WHODAS 2.0 scores were indicative of mild to moderate disability for the domains of mobility (22.0 ± 23.2), life activities (23.7 ± 25.5), and participation in society (17.2 ± 15.9), as well as total WHODAS 2.0 score (15.3 ± 15.2). For the domains of cognition (9.1 ± 14.1), self-care (8.3 ± 14.4), and interpersonal relationships (11.7 ± 15.7), scores were lower. Disability scores were, generally, proportional to the PH severity. The main baseline correlates of disability were World Health Organisation (WHO) functional class, fatigue, dyspnoea, 6-minute walking distance (6MWD), and N-terminal pro b-type natriuretic peptide (NTproBNP). Baseline WHODAS 2.0 scores showed significant associations with disease progression. However, this effect was not transversal to all domains, with only a few domains significantly associated with disease progression variables.

Conclusions: This PH population shows mild disability, with higher degree of disability in the domains of mobility and life activities. This study is the first one to assess disability in PH using WHODAS 2.0. Further studies should apply this scale to larger PH populations with suitable representations of more severe PH forms.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1590/1806-3713/e20170355DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6733722PMC
May 2019

ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension.

Eur Respir J 2019 02 28;53(2). Epub 2019 Feb 28.

Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, UK.

Objectives of this European Respiratory Society task force were to summarise current studies, to develop strategies for future research and to increase availability and awareness of exercise training for pulmonary hypertension (PH) patients.An evidence-based approach with clinical expertise of the task force members, based on both literature search and face-to-face meetings was conducted. The statement summarises current knowledge and open questions regarding clinical effects of exercise training in PH, training modalities, implementation strategies and pathophysiological mechanisms.In studies (784 PH patients in total, including six randomised controlled trials, three controlled trials, 10 prospective cohort studies and four meta-analyses), exercise training has been shown to improve exercise capacity, muscular function, quality of life and possibly right ventricular function and pulmonary haemodynamics. Nevertheless, further studies are needed to confirm these data, to investigate the impact on risk profiles and to identify the most advantageous training methodology and underlying pathophysiological mechanisms.As exercise training appears to be effective, cost-efficient and safe, but is scarcely reimbursed, support from healthcare institutions, commissioners of healthcare and research funding institutions is greatly needed. There is a strong need to establish specialised rehabilitation programmes for PH patients to enhance patient access to this treatment intervention.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1183/13993003.00332-2018DOI Listing
February 2019

Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal.

Rev Port Cardiol 2018 09 23;37(9):749-757. Epub 2018 Aug 23.

Unidade de Doença Vascular Pulmonar, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal.

Objectives: This study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center.

Methods: We studied PAH and CTEPH patients diagnosed between January 2005 and December 2016. Cumulative survival was estimated using the Kaplan-Meier method. Survival trends were compared over two periods (2005-2010 vs. 2011-2016).

Results: Of the 142 studied PH patients (age 54±18 years; 31% male), 47 had CTEPH and 95 had group 1 PH. Most patients with CTEPH and idiopathic/heritable PAH (I/HPAH) were in NYHA III-IV at diagnosis (64% and 57%, respectively). At the time of death, 31% of patients with connective tissue disease (CTD)-associated PAH (CTD-PAH) and all I/HPAH patients were on double or triple combination therapy. No patient underwent lung transplantation. Pulmonary endarterectomy or angioplasty were performed in 36% of CTEPH patients. Age at diagnosis tended to increase over time in CTD-PAH (53±15 vs. 63±15 years; p=0.13) and I/HPAH (39±15 vs. 51±19 years; p=0.10). The five-year survival estimates for I/HPAH, CTD-PAH and CTEPH patients were 80%, 52%, and 81%, respectively. Over time, CTD-PAH and CTEPH showed better five-year survival (33 vs. 67% and 77 vs. 84%), but I/HPAH did not (84 vs. 75%).

Conclusions: Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.repc.2018.02.009DOI Listing
September 2018

Health-Related Quality of Life in Pulmonary Hypertension and Its Clinical Correlates: A Cross-Sectional Study.

Biomed Res Int 2018 19;2018:3924517. Epub 2018 Mar 19.

MedInUP, Department of Pharmacology and Therapeutics, Faculty of Medicine, University of Porto, Porto, Portugal.

Background: Health-related quality of life (HRQoL) impairment is common in pulmonary hypertension (PH), but its clinical predictors are not well established. This study aims to characterize the HRQoL of patients with pulmonary arterial hypertension (PAH) and other precapillary forms of PH (pcPH) and to explore its clinical correlates.

Materials And Methods: A cross-sectional, observational study of patients with documented PAH and other forms of pcPH. Patients completed two patient-reported outcome measures (PROM): Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and Nottingham Health Profile (NHP). Clinical characteristics were retrieved from electronic medical records.

Results: Mean CAMPHOR and NHP scores for the study population were indicative of a moderate HRQoL impairment. Patients in World Health Organisation Functional Classes (WHO FC) III/IV showed significantly worse HRQoL. The main clinical correlates of HRQoL were WHO FC, 6-minute walking distance (6MWD), and Borg dyspnoea index. Overall quality of life (QoL), assessed through CAMPHOR's QoL domain, showed patterns comparable to HRQoL measured by both instruments.

Conclusions: HRQoL, measured by two different PROMs, is impaired in Portuguese patients with PAH and other forms of pcPH, particularly in patients with increased disease severity. WHO FC, 6MWD, and Borg dyspnoea index are highly correlated with HRQoL and QoL.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/3924517DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5884279PMC
September 2018

MicroRNA-424(322) as a new marker of disease progression in pulmonary arterial hypertension and its role in right ventricular hypertrophy by targeting SMURF1.

Cardiovasc Res 2018 01;114(1):53-64

CNC.IBILI, University of Coimbra, Coimbra, Portugal.

Aims: MicroRNAs (miRNAs) have been implicated in the pathogenesis of pulmonary hypertension (PH), a multifactorial and progressive condition associated with an increased afterload of the right ventricle leading to heart failure and death. The main aim of this study was to correlate the levels of miR-424(322) with the severity and prognosis of PH and with right ventricle hypertrophy progression. Additionally, we intended to evaluate the mechanisms and signalling pathways whereby miR-424(322) secreted by pulmonary arterial endothelial cells (PAECs) impacts cardiomyocytes.

Methods And Results: Using quantitative real-time PCR, we showed that the levels of circulating miR-424(322) are higher in PH patients when compared with healthy subjects. Moreover, we found that miR-424(322) levels correlated with more severe symptoms and haemodynamics. In the subgroup of Eisenmenger syndrome patients, miR-424(322) displayed independent prognostic value. Furthermore, we demonstrated that miR-424(322) targets SMURF1, through which it sustains bone morphogenetic protein receptor 2 signalling. Moreover, we showed that hypoxia induces the secretion of miR-424(322) by PAECs, which after being taken up by cardiomyocytes leads to down-regulation of SMURF1. In the monocrotaline rat model of PH, we found an association between circulating miR-424(322) levels and the stage of right ventricle hypertrophy, as well as an inverse correlation between miR-424(322) and SMURF1 levels in the hypertrophied right ventricle.

Conclusions: This study shows that miR-424(322) has diagnostic and prognostic value in PH patients, correlating with markers of disease severity. Additionally, miR-424(322) can target proteins with a direct effect on heart function, suggesting that this miRNA can act as a messenger linking pulmonary vascular disease and right ventricle hypertrophy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/cvr/cvx187DOI Listing
January 2018

Portuguese validation of the Cambridge pulmonary hypertension outcome review (CAMPHOR) questionnaire.

Health Qual Life Outcomes 2016 Jul 26;14(1):110. Epub 2016 Jul 26.

Department of Health Sciences, University of Aveiro, Aveiro, Portugal.

Background: Patients with pulmonary arterial hypertension (PAH) and other forms of precapillary pulmonary hypertension (PH) have impaired quality of life (QoL). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is a PH-specific patient-reported outcome measure that assesses symptoms, activity limitations and QoL. It was originally developed in UK-English. The main objective of this study was to create an adaptation of the CAMPHOR suitable for a Portuguese-speaking population.

Methods: A multi-step approach was followed: bilingual and lay panel translation; cognitive debriefing interviews; and psychometric testing in repeated postal surveys (2 weeks apart) including assessment of internal consistency, reproducibility and validity. The Nottingham Health Profile (NHP) questionnaire was used as a comparator instrument to test convergent validity.

Results: The CAMPHOR was translated without difficulty by the two panels. Cognitive debriefing interviews showed the questionnaire was easily understood and considered relevant to patients' experience with their illness. Psychometric evaluation was performed with 50 PAH patients (47 ± 14 years, 37 women). Cronbach's alpha coefficients showed good internal consistency for the three CAMPHOR scales [Symptoms = 0.95; Activities = 0.93 and QoL = 0.94]. Test-retest coefficients showed that all scales had excellent reliability (Symptoms = 0.94; Activities = 0.89 and QoL = 0.93), indicating low levels of random measurement error. The CAMPHOR correlated as expected with the NHP. The magnitude of correlations followed a similar pattern to those in the original development study. The CAMPHOR also exhibited evidence of known group validity in its ability to distinguish between self-reported severity and general health groups.

Conclusions: A valid and reliable version of the CAMPHOR questionnaire for the European Portuguese-speaking population was developed and is recommended for use.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12955-016-0513-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4962538PMC
July 2016

Pulmonary hypertension in Portugal: first data from a nationwide registry.

Biomed Res Int 2013 21;2013:489574. Epub 2013 Oct 21.

Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal ; Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal.

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical.

Methods: We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH).

Results: Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min(-1)·m(-2)); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015).

Conclusions: We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2013/489574DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3818811PMC
June 2014

Adiponectin levels are elevated in patients with pulmonary arterial hypertension.

Clin Cardiol 2014 Jan 1;37(1):21-5. Epub 2013 Oct 1.

Department of Physiology and Cardiothoracic Surgery, Faculty of Medicine, University of Porto, Porto, Portugal; Cardiology Department, Hospital Santo António, Centro Hospitalar do Porto, Porto, Portugal.

Background: In addition to insulin-sensitizing effects, adiponectin influences several mechanisms involved in pulmonary arterial hypertension (PAH) pathobiology. Insulin resistance has been associated with PAH, and elevated adiponectin levels have been described in left heart failure (HF) as a response to the increased metabolic stress. No studies have been performed in right HF or PAH patients.

Hypothesis: Compared to healthy controls, PAH patients have a different plasma adipocytokine profile, higher insulin resistance, and higher inflammatory systemic activation.

Methods: A case-control study was conducted in PAH patients individually matched for sex, age, and body mass index. We characterized the clinical features, functional status (6-minute walking test), and hemodynamic profile of cases (n=25). We measured insulin resistance (homeostasis model assessment and high-density lipoprotein/triglycerides ratio), inflammatory systemic activation (high-sensitivity C-reactive protein), and plasma adipocytokine profile (adiponectin, leptin, visfatin, and resistin) in cases and controls.

Results: PAH patients had significantly higher adiponectin levels than controls (12.4±6.9 vs 8.1±4.5 µg/mL; P<0.05) and higher high-sensitivity C-reactive protein (2.96±3.2 vs 1.08±1.1; P<0.05). No statistically significant differences were found in plasma levels of leptin, visfatin, and resistin between groups.

Conclusions: Adiponectin levels are increased in PAH patients compared to controls. Further studies are needed to study the potential role of adiponectin as a PAH biomarker.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/clc.22210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6649442PMC
January 2014

Venous thromboembolism risk factors and practices of prophylaxis: ENDORSE study results in Portugal.

Acta Med Port 2011 Nov-Dec;24(6):951-60. Epub 2012 Feb 20.

Internal Medicine Service, Hospital Garcia de Orta, Almada, Portugal.

Background: Venous thromboembolism (VTE) risk assessment is a cornerstone for the achievement of best practices and outcomes. Epidemiologic data and practices related to venous thromboprophylaxis as considered by the global ENDORSE study, (Epidemiologic International Day for the Evaluation of Patients at Risk for Venous Thromboembolism in the Acute Hospital Care Setting), enrolled 68,183 patients from 32 countries, in which Portugal. Within ENDORSE, data from all participant countries analyzed to determine their risk of VTE and to evaluate the suitability of prophylaxis.

Methods: European patients were enrolled from randomly selected hospitals in Portugal (European Hospital Register), according to ENDORSE study inclusion/exclusion criteria. The Seventh ACCP evidence-based consensus guidelines were employed to evaluate VTE risk and prophylaxis use.

Results: From a total of 3,145 beds assessed, 2,183 were considered eligible and 1,632 met all criteria. Of these, 860 (52.7%; 95% CI 50.3-55.1) were at risk of VTE: 525 surgical patients (68.9%; 95% CI 65.5-72.1) and 335 medical patients (38.5%; 95% CI 35.3-41.2). The rate of prophylaxis according to ACCP guidelines in overall patients at risk was 58.5% (503 patients). The prophylaxis rate for VTE was 59% (310 patients) in surgical patients and 57.6% (n=193) in medical patients. 39.7% of surgical patients and 39.4 % of medical patients who did not meet the criteria for prophylaxis were also on prophylaxis with an anticoagulant, which was considered to be inappropriate.

Conclusions: More than a half of these hospitalized patients in Portugal were deemed at risk of VTE and less than two-thirds of them received appropriate prophylaxis. New strategies are required for implementation of venous thromboprophylaxis in Portuguese hospitals.
View Article and Find Full Text PDF

Download full-text PDF

Source
November 2012

[Prevention and treatment of venous thromboembolism: the place of new oral anticoagulants].

Authors:
Abílio Reis

Rev Port Cardiol 2012 Apr;31 Suppl 1:45-50

Serviço de Medicina Interna, Consulta de Doença Vascular Pulmonar, Hospital de Santo António, Centro Hospitalar do Porto, EPE, Porto, Portugal.

Venous thromboembolism (VTE) is still an important problem of Public Health, due to its impact in terms of morbidity, mortality, resource allocation and associated costs. In the prevention and treatment of VTE, pharmacological therapy is well defined and efficacious but has some inconveniences that leave space for improvement. Several new oral anticoagulants are being developed and tested for the prevention and treatment of VTE. The better studied are the selective Factor Xa inhibitors apixaban, rivaroxaban and edoxaban, and the thrombin antagonist dabigatran. They all are orally administrated, don't have important interactions with food or other drugs, have a convenient fixed-dose regimen and a predictable action, and dispense routine monitoring of their anticoagulant effect. The major part of them has phase III studies concluded and published. Some of them are already approved by de European Medicines Agency (EMA) and the Food and Drug Administration (FDA) and recommended by the international guidelines. Rivaroxaban is approved by the EMA for the treatment of deep venous thrombosis (DVT) and for the prevention of recurrences of DVT and pulmonary embolism. In this article the available evidences are reviewed, the place of the new oral anticoagulants is discussed and future perspectives regarding the prevention and treatment of VTE are outlined.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/S0870-2551(12)70039-2DOI Listing
April 2012

Posterior fixation of the upper cervical spine: contemporary techniques.

J Am Acad Orthop Surg 2011 Feb;19(2):63-71

Department of Sports Medicine and Orthopaedic Surgery, University of Washington Medical Center, Seattle, WA, USA.

Instrumentation in the upper cervical spine has changed considerably in the past two decades. Previous stand-alone wiring techniques have been made largely obsolete with the development of occipital segmental plating, transarticular screws, and C1 lateral mass screws, as well as a myriad of C2 fixation options, including pedicle, pars, and translaminar screws. Polyaxial screws and segmental fixation are more user-friendly than stand-alone wiring and provide a stronger construct. Awareness of the risks and benefits associated with the use of modern instrumentation and thorough familiarity with the anatomy of the upper cervical spine are essential to avoid complications and optimize outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5435/00124635-201102000-00001DOI Listing
February 2011

Diagnosis and treatment of craniocervical dissociation in 48 consecutive survivors.

Evid Based Spine Care J 2010 Aug;1(2):69-70

Department of Orthopaedics and Sports Medicine, Harborview Medical Center, Seattle, Washington, USA.

Study Type:  Case series Introduction:  Craniocervical dissociation (CCD) is an uncommon and frequently fatal injury with few reports in the literature of survivors. Advances in automobile safety and improved emergency medical services have resulted in increased survival. Timely diagnosis and treatment are imperative for optimal outcome. Regrettably, the presence of multiple life threatening injuries, low clinical suspicion, and lack of familiarity with the upper cervical radiographic anatomy frequently lead to missed or delayed diagnosis.

Objective:  This paper represents the largest series of surgically treated CCD survivors. The goal of this study is to determine if any improvements have been made in the timely diagnosis of CCD while performing a complete patient evaluation.

Methods:  Following institutional review board approval, a search of the Harborview Medical Center (HMC) trauma registry was conducted for all surgically treated CCD patients between 1996 and 2008. Forty-eight consecutive cases were identified. A retrospective review of the radiological and clinical results with emphasis on timing of diagnosis, modality used for diagnosis (Figures 1 and 2), clinical effect of delayed diagnosis, potential clinical or imaging warning signs, and response to treatment was performed. Thirty-one patients treated from 2003 to 2008 were compared to 17 patients that were treated from 1996 to 2002 and reported previously.1 Figure 1 Initial lateral C-spine radiograph obtained as part of the initial ATLS survey demonstrating an occiput C1 distractive injury.Figure 2 Sagittal C-spine CT scan obtained as part of the initial ATLS survey demonstrating an occiput C2 distractive injury. All patients sustained high-energy injuries and were evaluated according to standard Advanced Trauma Life Support (ATLS) protocols. Once CCD was identified or suspected, provisional stabilization was applied and MRI evaluation performed (Figure 3). Definitive surgical management with rigid posterior instrumentation and fusion was performed as soon as physiologically possible (Figures 4 and 5). Figure 3a-b Preoperative coronal T2 MRI sequences demonstrating increased signal intensity on the occiput-C1 and C1-2 joints.Figure 4 Postoperative lateral C-spine x-ray showing rigid posterior instrumented fusion from occiput to C2.Figure 5 Postoperative sagittal C-spine x-ray showing rigid posterior instrumented fusion from occiput to C2.

Results:  Craniocervical dissociation was identified on initial cervical spine imaging in 26 patients (84%). The remaining five patients (16%) were diagnosed by cervical spine MRI. Twenty-three patients (74.2%) were diagnosed within 24 hours of presentation, four (22.6%) were diagnosed between 24 and 48 hours, and one (3.2%) experienced a delay of greater than 48 hours (Table 1). In comparison, four (24%) of the previously treated 17 patients were diagnosed on initial cervical spine imaging. Four patients (24%) were diagnosed within 24 hours of presentation, nine (52%) were diagnosed between 24 and 48 hours, and four (24%) experienced a delay of greater than 48 hours. There were no cases of craniocervical pseudarthrosis or hardware failure during a mean nine-month follow-up period. Four patients expired during their hospital course. The mean American Spinal Injury Association (ASIA) motor score of 47 improved to 60, and the number of patients with useful motor function (ASIA Grade D or E) increased from eight (26%) preoperatively to 17 (55%) postoperatively.

Conclusions:  Improvements have been made in time to diagnosis of CCD in recent years. Increased awareness and the routine use of CT scan as part of the initial ATLS evaluation account for this progress. Expedited diagnosis has decreased preoperative neurological deterioration. However, differences in length of follow-up between the two groups preclude conclusions about its effect on long-term neurological outcome. [Table: see text].
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0028-1100920DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3623094PMC
August 2010

Guidelines for the management of pulmonary hypertension patients.

Rev Port Pneumol 2010 Jul;16 Suppl 4:S7-S85

Grupo de Estudos de Hipertensão Pulmonar da Sociedade Portuguesa de Cardiologia/Pulmonary Hypertension Study Group of the Portuguese Society of Cardiology.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/S0873-2159(15)30103-3DOI Listing
July 2010

Editorial.

Rev Port Pneumol 2010 Jul;16 Suppl 4:S5-6

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/S0873-2159(15)30102-1DOI Listing
July 2010

[Guidelines for the management of pulmonary hypertension patients].

Rev Port Cardiol 2010 Feb;29(2):253-89

Núcleo de Estudos de Doença Vascular Pulmonar da Sociedade Portuguesa de Medicina Interna, Lisboa, Portugal.

Introduction: Pulmonary hypertension (PH) was until recently an obscure pathology which frequently went unidentified as it lacked a precise diagnostic strategy. Recent years have seen advances in the knowledge of the pathogenesis and mechanisms of vascular lesion of PH. This has led to the scientific community's growing interest in this area, an interest manifested in appreciable progress in the pathology's clinical characterisation, diagnostic strategies and the development of effective drugs. All of this together has been fundamental in changing the previously unfavourable prognosis of this disease. This evolution implies the need to rationalise the use of available resources through organisation of healthcare services, defining the role of each level of care, and developing norms for good clinical management practices in keeping with best medical practice guidelines. These twin aspects have attracted the interest of the scientific community, as shown by the wealth of literature, and have led healthcare authorities to introduce regulatory mechanisms. In order to improve clinical practice, the Pulmonary Vascular Disease Study Group (NEDVP) of the Portuguese Society of Internal Medicine (SPMI), the Pulmonary Hypertension Study Group (GEHTP) of the Portuguese Society of Cardiology and the boards of the Portuguese Societies of Pulmonology and Paediatric Cardiology created an interdisciplinary working group. The group's remit was to draft this document, "Guidelines for the management of pulmonary hypertension patients", based on a review of the literature and the authors' clinical expertise. These guidelines aim to present all the relevant evidence on the diagnostic and treatment strategy of PH and the definition of requirements for referral centres. The organisation of care is fundamental for an appropriate and rational use of the available resources and for the better care of the patient.
View Article and Find Full Text PDF

Download full-text PDF

Source
February 2010

Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation.

Am J Respir Crit Care Med 2010 Apr 7;181(8):851-61. Epub 2010 Jan 7.

Université Paris-Sud, Faculté de médecine, Kremlin Bicêtre, 92140 Clamart, France.

Rationale: Activin A receptor type II-like kinase-1 (ACVRL1, also known as ALK1) mutation is a cause of hereditary hemorrhagic telangiectasia (HHT) and/or heritable pulmonary arterial hypertension (PAH).

Objectives: To describe the characteristics of patients with PAH carrying an ACVRL1 mutation.

Methods: We reviewed clinical, functional, and hemodynamic characteristics of 32 patients with PAH carrying an ACVRL1 mutation, corresponding to 9 patients from the French PAH Network and 23 from literature analysis. These cases were compared with 370 patients from the French PAH Network (93 with a bone morphogenetic protein receptor type 2 [BMPR2] mutation and 277 considered as idiopathic cases without identified mutation). Distribution of mutations in the ACVRL1 gene in patients with PAH was compared with the HHT Mutation Database.

Measurements And Main Results: At diagnosis, ACVRL1 mutation carriers were significantly younger (21.8 +/- 16.7 yr) than BMPR2 mutation carriers and noncarriers (35.7 +/- 14.9 and 47.6 +/- 16.3 yr, respectively; P < 0.0001). In seven of the nine patients from the French PAH Network, PAH diagnosis preceded manifestations of HHT. ACVRL1 mutation carriers had better hemodynamic status at diagnosis, but none responded to acute vasodilator challenge and they had shorter survival when compared with other patients with PAH despite similar use of specific therapies. ACVRL1 mutations in exon 10 were more frequently observed in patients with PAH, as compared with what was observed in the HHT Mutation Database (33.3 vs. 5%; P < 0.0001).

Conclusions: ACVRL1 mutation carriers were characterized by a younger age at PAH diagnosis. Despite less severe initial hemodynamics and similar management, these patients had worse prognosis compared with other patients with PAH, suggesting more rapid disease progression.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1164/rccm.200908-1284OCDOI Listing
April 2010

Pulmonary alveolar proteinosis: a rare pulmonary toxicity of sirolimus.

Transpl Int 2007 Mar;20(3):291-6

Nephrology Department, Hospital Geral de Santo António, Porto, Portugal.

The aim of our paper is to describe an unusual pulmonary toxicity of sirolimus (SRL) in a kidney transplant recipient. We present a 34-year-old woman with a second renal transplantation, complicated with steroid-resistant acute rejection and chronic allograft dysfunction. Two years after initiating SRL, she presented complaints of progressive dyspnoea, nonproductive cough, chest pain and low-grade fever of 1 month duration. She had chronic allograft nephropathy and slight elevation of lactic dehydrogenase levels. After exclusion of common reasons of this condition, a computed tomography (CT) of the thorax and bronchoscopy was performed, revealing ground-glass opacification with polygonal shapes on CT and an opaque appearance with numerous macrophages on bronchoalveolar lavage. The alveolar macrophages stained positive by Periodic acid-Schiff. Diagnosis of pulmonary alveolar proteinosis (PAP) was made and drug-induced toxicity was suspected. SRL was withdrawn with marked improvement in the patients' clinical and radiological status. PAP resolved within 3 months without further therapy. PAP is a very rare complication of SRL therapy with only a few cases described. Withdrawal of SRL with conversion to another immunosuppressant seems to be an appropriate procedure in this condition.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1432-2277.2006.00408.xDOI Listing
March 2007