Partial Implant Rehabilitations in the Posterior Regions of the Jaws Supported by Short Dental Implants (7.0 mm): A 7-Year Clinical and 5-Year Radiographical Prospective Study.

Background: Short implants have been used in the restoration of edentulous jaws in the past several years. However, some studies have suggested that short implants are less successful than standard implants. The aim of this study is to investigate the outcome of short implants placed in the posterior maxilla or mandible following one-stage or immediate-function protocols with a follow-up of 7 years (clinically) and 5 years (radiographically). Methods: This study included 127 patients rehabilitated with 217 implants measuring 7 mm and supporting 157 fixed prostheses in the posterior segments of both jaws. Final abutments were delivered at the surgery stage and were loaded after 4 months in 116 patients (199 implants). The primary outcome measure was implant survival measured through life tables. Secondary outcome measures were marginal bone loss and the incidence of biological and mechanical complications at the patient level and implant level (evaluated through descriptive statistics). Results: Twenty-four patients (18.9%) with 45 implants (20.7%) were lost to the follow-up. In total, 32 implants failed (14.8%) in 22 patients (17.3%), resulting in a cumulative survival rate at 7 years of 81.2% for 7 mm implants in the rehabilitation of the posterior regions of the maxilla and mandible. The average (standard deviation) marginal bone loss was 1.47 mm (0.99 mm) at 5 years. The incidence rate of biological complications was 12.6% and 10.6% at the patient and implant levels, respectively. The incidence rate of mechanical complications was 21.3% for patients and 16.1% for implants. A higher failure rate was registered in smokers and in implant arrangements with a sequence of three fixtures in proximity. Conclusions: Within the limitations of this study, it can be concluded that the placement of 7 mm long implants for the partial implant-supported rehabilitation of atrophic posterior jaws is possible in the long term, judging by the survival rate and stable average marginal bone loss. Nevertheless, strict case selection should be performed, especially in smokers and with implant arrangements that provide a minimum of one unit in inter-implant distance.

Measuring the value of solidarity: The abem financial assistance program for out-of-pocket payments on pharmacy medicines in Portugal.

OBJECTIVE: Out-of-pocket payments for prescribed medicines are still comparatively high in Portugal. The abem program was launched in Portugal in May 2016 to aid vulnerable groups by completely covering out-of-pocket costs of prescribed medicines in community pharmacies. This study assesses the impact of the program on poverty and catastrophic health expenditures. METHODS: A longitudinal study was carried out with the analysis of several program databases (from the beginning of the program in May 2016 to September 2018) covering the cohorts of beneficiaries, daily data on medicines dispensed, social referencing entities, and solidarity pharmacies. The study provides estimates of standard poverty measures (intensity and severity) as well as the incidence of catastrophic health expenditures. RESULTS: More than 6000 beneficiaries were supported (56.8% female, 34.7% aged 65 or over), encompassing 127,510 medicines (mainly nervous system and cardiovascular system) with an average 26.9% co-payment (payments totalling €1.5 million). The program achieved substantial reductions in poverty (3.4% in intensity, 5.6% in severity), and eliminated cases with catastrophic health expenditures in medicines that would have affected 7.5% of the beneficiaries. CONCLUSIONS: Findings confirm a continuous increase in the number of beneficiaries, enabling access to medicines especially for the vulnerable elderly, and a sizable impact on eliminating out-of-pocket payments for medicines in the target population.

Atherosclerosis in the primary health care setting: A real-word data study.

INTRODUCTION AND OBJECTIVES: To characterize patients with atherosclerosis, a disease with a high socioeconomic impact, in the Lisbon and Tagus Valley Health Region. METHODS: A cross-sectional observational study was carried out through the Lisbon and Tagus Valley Regional Health Administration primary health care database, extracting data on the clinical and demographic characteristics and resource use of adult primary health care users with atherosclerosis during 2016. Different criteria were used to define atherosclerosis (presence of clinical manifestations, atherothrombotic risk factors and/or consumption of drugs related to atherosclerosis). Comparisons between different subpopulations were performed using parametric tests. RESULTS: A total of 318 692 users were identified, most of whom (n=224 845 users; 71%) had no recorded clinical manifestations. The subpopulation with clinical manifestations were older (72.0±11.5 vs. 71.3±11.0 years), with a higher proportion of men (58.0% vs. 45.9%), recorded hypertension (78.3% vs. 73.5%) and dyslipidemia (55.8% vs. 53.5%), and a lower proportion of recorded obesity (18.2% vs. 20.8%), compared to those without clinical manifestations (p<0.001). Mean blood pressure, LDL-C and glycated hemoglobin values were lower in the subpopulation with manifestations (142/74 vs. 146/76 mmHg, 101 vs. 108 mg/dl, and 6.80 vs. 6.84%, respectively; p<0.001). Each user with atherosclerosis attended 4.1±2.9 face-to-face medical consultations and underwent 8.6±10.0 laboratory test panels, with differences in subpopulations with and without clinical manifestations (4.4±3.2 vs. 4.0±2.8 and 8.3±10.3 vs. 8.7±9.8, respectively; p<0.001). CONCLUSIONS: About one in three adult primary health care users with atherosclerosis have clinical manifestations. The results suggest that control of cardiovascular risk factors is suboptimal in patients with atherosclerosis.

Treatment-resistant depression and major depression with suicide risk-The cost of illness and burden of disease.

Introduction: Treatment-Resistant Depression (TRD) and Major Depression with Suicide Risk (MDSR) are types of depression with relevant effects on the health of the population and a potentially significant economic impact. This study estimates the burden of disease and the costs of illness attributed to Treatment-Resistant Depression and Major Depression with Suicide Risk in Portugal. Methods: The disease burden for adults was quantified in 2017 using the Disability-Adjusted Life Years (DALYs) lost. Direct costs related to the health care system and indirect costs were estimated for 2017, with indirect costs resulting from the reduction in productivity. Estimates were based on multiple sources of information, including the National Epidemiological Study on Mental Health, the Hospital Morbidity Database, data from the Portuguese National Statistics Institute on population and causes of death, official data on wages, statistics on the pharmaceutical market, and qualified opinions of experts. Results: The estimated prevalence of TRD, MDSR, and both types of depression combined was 79.4 thousand, 52.5 thousand, and 11.3 thousand patients, respectively. The disease burden (DALY) due to the disability generated by TRD alone, MDSR alone, and the joint prevalence was 25.2 thousand, 21 thousand, and 4.5 thousand, respectively, totaling 50.7 thousand DALYs. The disease burden due to premature death by suicide was 15.6 thousand DALYs. The estimated total disease burden was 66.3 thousand DALYs. In 2017, the annual direct costs with TRD and MDSR were estimated at € 30.8 million, with the most important components being medical appointments and medication. The estimated indirect costs were much higher than the direct costs. Adding work productivity losses due to reduced employment, absenteeism, presenteeism, and premature death, a total cost of € 1.1 billion was obtained. Conclusions: Although TRD and MDSR represent relatively small direct costs for the health system, they have a relevant disease burden and extremely substantial productivity costs for the Portuguese economy and society, making TRD and MDSR priority areas for achieving health gains.

Marginal Bone Loss and Pink Esthetic Evaluation of Narrow-Diameter Dental Implants for Single Crowns: 1-Year Prospective Clinical Study.

PURPOSE: The aims of this study were: (1) to quantify the marginal bone loss (MBL) of 3.3-mm narrow-diameter, bonelevel, titanium-zirconia (Ti-Zr) implants with two different surfaces in single restorations after a 1-year follow-up; (2) to analyze the combinations of different variables that may influence MBL; and (3) to record the Pink Esthetic Score (PES) value and its correlation with MBL. MATERIALS AND METHODS: This is a prospective longitudinal clinical study with a 1-year follow-up after crown placement. Two different implant surfaces (sandblasted acid-etched and modified sandblasted acid-etched) were used. All bone-level and bone level-tapered implants had a diameter of 3.3 mm. Different healing and prosthetic abutments were used. Clinical, radiographic, and photographic records were taken 6 months and 1 year after placement of the restorations, and the survival rate, MBL, PES, clinical parameters, and biologic and/or mechanical complications were assessed. The correlations between the variables and MBL were verified. RESULTS: A total of 30 narrowdiameter implants were placed in 30 patients; 18 implants had a sandblasted acid-etched surface, and 12 implants had a modified sandblasted acid-etched surface. The measured MBL at 1 year after implant function had a mean value of -0.36 mm, ranging from 0 mm to -1.77 mm. There was no implant loss. A statistically significant relationship was observed between implant shape (design and length), implant placement level, healing abutment, prosthetic abutment size, gingival thickness, and MBL. The mean PES values recorded at the beginning and end of the study were 7.58 and 11.37, respectively. CONCLUSION: Narrow-diameter implants showed reduced MBL values, with the surrounding tissues remaining stable after 1 year of follow-up. The MBL did not show different values on two implant surfaces. MBL does not seem to influence esthetic outcome.

Atherosclerosis: The cost of illness in Portugal.

INTRODUCTION AND OBJECTIVES: Cardiovascular disease is the leading cause of death in Portugal and atherosclerosis is the most common underlying pathophysiological process. The aim of this study was to quantify the economic impact of atherosclerosis in Portugal by estimating disease-related costs. METHODS: Costs were estimated based on a prevalence approach and following a societal perspective. Three national epidemiological sources were used to estimate the prevalence of the main clinical manifestations of atherosclerosis. The annual costs of atherosclerosis included both direct costs (resource consumption) and indirect costs (impact on population productivity). These costs were estimated for 2016, based on data from the Hospital Morbidity Database, the health care database (SIARS) of the Regional Health Administration of Lisbon and Tagus Valley including real-world data from primary care, the 2014 National Health Interview Survey, and expert opinion. RESULTS: The total cost of atherosclerosis in 2016 reached 1.9 billion euros (58% and 42% of which was direct and indirect costs, respectively). Most of the direct costs were associated with primary care (55%), followed by hospital outpatient care (27%) and hospitalizations (18%). Indirect costs were mainly driven by early exit from the labor force (91%). CONCLUSIONS: Atherosclerosis has a major economic impact, being responsible for health expenditure equivalent to 1% of Portuguese gross domestic product and 11% of current health expenditure in 2016.

The burden of atherosclerosis in Portugal.

AIMS: This article sought to estimate the burden of disease attributable to atherosclerosis in mainland Portugal in 2016. METHODS AND RESULTS: The burden of atherosclerosis was measured in disability-adjusted life years following the latest 2010 Global Burden of Disease (GBD) methodology. Disability-adjusted life years were estimated as the sum of years of life lost (YLL) with years lived with disability (YLD). The following clinical manifestations of atherosclerosis were included: ischaemic heart disease (IHD) (including acute myocardial infarction, stable angina, and ischaemic heart failure), ischaemic cerebrovascular disease (ICVD), and peripheral arterial disease (PAD). Years of life lost were estimated based on all-cause mortality data for the Portuguese population and mortality due to IHD, ICVD, and PAD for the year 2016 sourced from national statistics. Standard life expectancy was sourced from the GBD study. Years lived with disability corresponded to the product of the number of prevalent cases by an average disability weight for all possible combinations of disease. Prevalence data for the different clinical manifestations of atherosclerosis were sourced from epidemiological studies. Disability weights were sourced from the published literature. In 2016, 15 123 deaths were attributable to atherosclerosis, which corresponded to 14.3% of overall mortality in mainland Portugal. Disability-adjusted life years totalled 260 943, 75% due to premature death (196 438 YLL) and 25% due to disability (64 505 YLD). CONCLUSION: Atherosclerosis entails a high disease burden to society. A large part of this burden would be avoidable if evidence-based effective and cost-effective interventions targeting known risk factors, from prevention to treatment, were implemented.

Indirect costs of myocardial infarction in Portugal.

INTRODUCTION: Cardiovascular disease, and particularly myocardial infarction (MI), carries a significant economic burden, through productivity losses (indirect costs) associated with temporary absence from work, that has not yet been adequately studied in Portugal. Our objective was to quantify the indirect costs of MI in the first year after admission. METHODS: Consecutive patients admitted to a single center aged <66 years who survived to discharge during a one-year period were included. Employment status on admission was assessed and for every employed patient, their monthly wage was estimated from market wage rates taken from the Ministry of Labor database according to gender and age. The duration of temporary absence from work was assessed in follow-up contacts for up to one year. Indirect costs were calculated in this sample and the results were applied to the number of MIs in Portugal during 2016 and separately to ST-elevation MI (STEMI) and non-ST-elevation acute coronary syndrome. RESULTS: A total of 219 patients were included, of whom 66.2% were working. The mean monthly labor cost was 1802 euros. A total cost of 760 521.55 euros was obtained. At national level there were 4133 patients aged <66 years admitted with acute MI who survived to discharge. Costs were higher in STEMI patients and the total indirect cost was estimated at 10.12 million euros. CONCLUSIONS: In Portugal, the costs to society of disability-generated productivity losses exceed ten million euros in the first year after MI. Strategies to promote an earlier return to work are needed to lower these costs.

Costs and consequences of the Portuguese needle-exchange program in community pharmacies.

BACKGROUND: Needle-exchange programs (NEPs) reduce infections in people who inject drugs. This study assesses the impact community pharmacies have had in the Needle-Exchange Program in Portugal since 2015. METHODS: Health gains were measured by the number of human immunodeficiency virus (HIV) and hepatitis C virus (HCV) infections averted, which were estimated, in each scenario, based on a standard model in the literature, calibrated to national data. The costs per infection were taken from national literature; costs of manufacturing, logistics and incineration of injection materials were also considered. The results were presented as net costs (i.e., incremental costs of the program with community pharmacies less the costs of additional infections avoided). RESULTS: Considering a 5-year horizon, the Needle Exchange Program with community pharmacies would account for a 6.8% (n = 25) and a 6.5% reduction (n = 22) of HCV and HIV infections, respectively. The present value of net savings generated by the participation of community pharmacies in the program was estimated at €2,073,347. The average discounted net benefit per syringe exchanged is €3.01, already taking into account a payment to community pharmacies per needle exchanged. INTERPRETATION: We estimate that the participation of community pharmacies in the Needle Exchange Program will lead to a reduction of HIV and HCV infections and will generate over €2 million in savings for the health system. CONCLUSIONS: The intervention is estimated to generate better health outcomes at lower costs, contributing to improving the efficiency of the public health system in Portugal.

Current costs of heart failure in Portugal and expected increases due to population aging. / Os custos da insuficiência cardíaca em Portugal e a sua evolução previsível com o envelhecimento da população.

INTRODUCTION AND OBJECTIVES: Heart failure (HF) is a growing public health problem. This study estimates the current and future costs of HF in mainland Portugal. METHODS: Costs were estimated based on prevalence and from a societal perspective. The annual costs of HF included direct costs (resource consumption) and indirect costs (productivity losses). Estimates were mostly based on data from the Diagnosis-Related Groups database, real-world data from primary care, and the opinions of an expert panel. Costs were estimated for 2014 and, taking population aging into account, changes were forecast up to 2036. RESULTS: Direct costs in 2014 were €299 million (39% for hospitalizations, 24% for medicines, 17% for exams and tests, 16% for consultations, and the rest for other needs, including emergencies and long-term care). Indirect costs were €106 million (16% for absenteeism and 84% for reduced employment). Between 2014 and 2036, due to demographic dynamics, total costs will increase from €405 to €503 million. Per capita costs are estimated to rise by 34%, which is higher than the increase in total costs (+24%), due to the expected reduction in the resident population. CONCLUSIONS: HF currently has a significant economic impact, representing around 2.6% of total public health expenditure, and this is expected to increase in the future. This should be taken into account by health policy makers, alerting them to the need for resource management in order to mitigate the impact of this disease.

Cost-effectiveness of sacubitril/valsartan for the treatment of patients with heart failure with reduced ejection fraction in Portugal.

Objectives: This study assesses the cost-effectiveness of sacubitril/valsartan versus enalapril in patients with symptomatic heart failure with reduced ejection fraction (HFrEF).Methods: We used a previously developed Markov model calibrated with patient-level data from the PARADIGM-HF trial, adapted to the Portuguese setting. The model considers two health states (alive or dead) and uses regression analyzes to estimate hospitalizations and deaths over time. A panel of experts estimated resource consumption in the outpatient setting. To estimate resource consumption with hospitalizations, the National Health Service Diagnosis Related Groups database was used. Unit costs were based on national legislation, and on the Infomed database. The model considers a societal perspective, a time horizon of 30-years, and a 5% annual discount rate. Sensitivity analyses assessed the robustness of results.Results: Sacubitril/valsartan increases life expectancy by 0.5 life-years, corresponding to 0.4 incremental quality adjusted life-years (QALY) versus enalapril. The estimated incremental cost-effectiveness ratio (ICER) is 22,702€/QALY. Sensitivity analysis shows that results are robust, but sensitive to the parameter estimates of the cardiovascular survival curve.Conclusion: Sacubitril/valsartan is a cost-effective therapeutic option in the treatment of Portuguese patients with HFrEF and translate into significant health gains and increased life expectancy versus the current standard of care.

Cost-effectiveness of the 13-valent pneumococcal conjugate vaccine in adults in Portugal versus "no vaccination" and versus vaccination with the 23-valent pneumococcal polysaccharide vaccine.

The burden of pneumococcal disease in adults is substantial from a social and economic point of view. This study assessed the cost-effectiveness of the 13-valent pneumococcal conjugate vaccine (PCV13) for the prevention of invasive pneumococcal disease and pneumococcal pneumonia in adults versus "no vaccination" and versus vaccination with the 23-valent pneumococcal polysaccharide vaccine (PPSV23). A Markov model was used to simulate three strategies: no vaccination, complete vaccination with PPSV23 and complete vaccination with PCV13. The comparison between strategies allowed the estimation of clinical and economic outcomes including incremental cost-effectiveness ratios (ICER) and incremental cost-utility ratios (ICUR). The model took into account the distributions of age, risk profile, vaccination status, type of immunization and time since vaccination in the population. A societal perspective was adopted and a lifetime horizon was considered. Different sources of data and assumptions were used to calibrate PPSV23 and PCV13 effectiveness. Inpatient costs were based on the 2013 diagnosis-related group (DRG) database for National Health Service (NHS) hospitals and expert opinion; NHS official tariffs were the main source for unitary costs. PCV13 shows ICURs of €17,746/QALY and €13,146/QALY versus "no vaccination" and vaccination with PPSV23, respectively. Results proved to be robust in univariate sensitivity analyses, where all ratios were below a €20,000 threshold, with the exception of the scenario with PCV13 effectiveness halved. In a probabilistic sensitivity analysis, 94% of simulations showed cost-effectiveness ratios lower than €20,000/QALY, in both strategies. It was found that PCV13 is a cost-effective strategy to prevent pneumococcal disease in adults in Portugal.

The current and future burden of heart failure in Portugal.

AIMS: Heart failure (HF) is a clinical syndrome with significant social and economic burden. We aimed to estimate the burden of HF in mainland Portugal over a 22-year time horizon, between 2014 and 2036. METHODS AND RESULTS: Heart failure burden was measured in disability-adjusted life years (DALYs), resulting from the sum of years of life lost (YLL) due to premature death and years lost due to disability (YLD). YLL were estimated based on the Portuguese mortality rates reported by the European Detailed Mortality Database. For YLD, disease duration and the overall incidence were estimated using an epidemiological model developed by the World Health Organization (DISMOD II). Disability weights were retrieved from published literature. The impact of ageing was estimated with a shift-share analysis using official demographic projections. In 2014, 4688 deaths were attributed to HF, corresponding to 4.7% of the total deaths in mainland Portugal. DALYs totalled 21 162, 53.9% due to premature death (YLL: 11 398) and 46.1% due to disability (YLD: 9765). Considering only population ageing over a 22-year horizon, the deaths and burden of HF are expected to increase by 73.0% and 27.9%, respectively, reaching 8112 deaths and 27 059 DALYs lost due to HF in 2036. DALY's growth is mainly driven by the increase of YLL, whose contribution to overall burden will increase to 62.0%. CONCLUSIONS: Heart failure is an emerging and growing health problem where significant health gains may be obtained. The projected significant increase of HF burden highlights the need to set HF as a priority for healthcare system.

Allergic contact dermatitis caused by dexpanthenol-Probably a frequent allergen.

BACKGROUND: Panthenol (synonym: dexpanthenol), the alcohol analogue of panthothenic acid, is frequently included in moisturizers, wound-healing agents, and other cosmetics, and has been shown to be responsible for allergic contact dermatitis (ACD). OBJECTIVES: To evaluate the frequency of ACD caused by dexpanthenol, and to characterize reactive patients. METHODS: We retrospectively reviewed the files of patients patch tested between 2009 and 2017 in the Department of Dermatology of the Coimbra's University Hospital and describe patients who reacted to dexpanthenol 5% pet., tested initially in a cosmetic/vehicle series and in the last 3 years in consecutive patients. RESULTS: Among 2171 patients, 26 (1.2%) had positive patch test reactions to dexpanthenol, mostly patients tested for chronic eczema (88.5%, n = 23), either widespread (5), or localized to the hands (5), face (4), or legs (7). Relevance could be traced in 20 patients (76.9%), related to the use of Bepanthene cream (15), moisturizers (3), topical medications (1), and a shampoo (1). Twenty-five of 26 patients (96.2%) reacted to several other allergens, mostly ingredients of cosmetic or pharmaceutical products. CONCLUSIONS: Although ACD caused by dexpanthenol is considered to be rare, it may be frequently overlooked. As we found a relatively high frequency of relevant cases, in agreement with a previous study, the inclusion of dexpanthenol in patch test series, at least in cosmetic and topical drug series, is encouraged.

The impact of osteoarthritis on early exit from work: results from a population-based study.

BACKGROUND: Osteoarthritis (OA) is a leading cause of pain and disability, which may be a source of productivity losses. The objectives of this study were to describe the impact of OA, namely through pain and physical disability, on early exit from work and to calculate its economic burden. METHODS: We analysed data from the national, cross-sectional, population-based EpiReumaPt study (Sep2011-Dec2013) in which 10,661 individuals were randomly surveyed in order to capture all cases of rheumatic diseases. We used all participants aged 50-64, near the official retirement age, who were clinically validated by experienced rheumatologists (n = 1286), including OA cases. A national database was used to calculate productivity values by gender, age and region, using the human capital approach. The impact of OA on the likelihood of early exit from work and the population attributable fractions used to calculate due economic burden (indirect costs) were obtained at the individual level by logistic regression. All results were based on weighted data. RESULTS: Almost one third of the Portuguese population aged 50-64 had OA (29.7%; men: 16.2% and women: 43.5%) and more than half were out of paid work (51.8%). Only knee OA is associated with early exit from work (OR: 2.25; 95%CI: 1.42-3.59; p = 0.001), whereas other OA locations did not reach any statistical difference. Furthermore, we observed an association between self-reported longstanding musculoskeletal pain (OR: 1.55; 95%CI: 1.07-2.23; p = 0.02) and pain interference (OR: 1.35; 95%CI: 1.13-1.62; p = 0.001) with early exit from work. We also detected a clear relationship between levels of disability, measured by the Health Assessment Questionnaire (HAQ), and the probability of work withdrawal. The estimated annual cost of early exit from work attributable to OA was €656 million (€384 per capita; €1294 per OA patient and €2095 per OA patient out-of-work). CONCLUSIONS: In this study, we observed an association between OA and early exit from work, largely dependent on pain and disability. This relationship translates into a meaningful economic burden amounting to approximately 0.4% of the national Gross Domestic Product (GDP). The high prevalence and the impact of this disabling chronic disease highlight the need to prioritize policies targeting early exit from work in OA.

Tunneled island flaps in facial defects reconstruction.

The reconstruction of facial surgical defects is usually challenging for the dermatologic surgeon. Three different cases of facial defects in which tunneled island flaps were used are reported. In 2 cases, wide defects involving the nasal dorsum and ala were repaired using a nasolabial island flap tunneled through the lateral side of the nose. A tunneled island glabellar flap was used for medial canthus reconstruction in the third case. Despite complex pedicle dissection and frequent trapdoor deformation, tunneled island flaps allow reconstruction of wide defects in a single-staged procedure, camouflaging the scar of the donor area in boundaries of cosmetic units and preserving the facial central symmetry.

[Gorlin-Goltz Syndrome: Diagnosis and Treatment Options]. / Síndrome de Gorlin-Goltz: Diagnóstico e Hipóteses de Tratamento.

The Gorlin-Goltz syndrome is a rare autosomal dominant hereditary condition, with complete penetrance and variable expressivity. Characterized by the appearance of multiple basaliomas, and often the development of keratocyst, it can also express itself by the presence of palmar/plantar depressions, calcification of brain sickle, and skeletal birth defects, although less frequently. This article presents two cases involving direct relatives, referred after the identification of several basaliomas and jaw cysts. After establishing the diagnosis, given the identification of three major criteria, the treatment consisted in the excision of the lesions followed, in one case, with vismodegib treatment resulting in complete remission. Gorlin-Goltz syndrome is therefore a multidisciplinary challenge, whose variable morbidity and high risk of recurrence make treatment and surveillance critical. However, new molecular targeted therapies have brought a new hope in treating these patients.

A síndrome de Gorlin-Goltz é uma patologia hereditária, rara, autossómica dominante de penetrância completa, com expressividade variável. Caracterizada pelo aparecimento de múltiplos basaliomas, cursa, frequentemente, com o desenvolvimento de queratoquistos. Depressões palmares/plantares, calcificações da foice cerebral, anomalias esqueléticas congénitas, constituem, igualmente, critérios de diagnóstico, embora menos frequentes. Este artigo expõe dois casos clínicos, envolvendo familiares diretos, referenciados após identificação de vários basaliomas e quistos maxilares. Estabelecido o diagnóstico, perante a identificação de três critérios major, o tratamento consistiu na excisão das lesões, seguida, num dos casos, da terapêutica com vismodegib, com remissão completa. A síndrome de Gorlin-Goltz é, assim, um desafio multidisciplinar, cuja morbilidade variável e o elevado risco de recorrência tornam fundamental o tratamento e vigilância. Contudo, o aparecimento de novas terapêuticas moleculares dirigidas trazem uma nova esperança no tratamento destes doentes.

Interventions aiming to reduce early retirement due to rheumatic diseases.

INTRODUCTION: Aging of the population and early retirement translates into productivity losses to society. Persistence of working life is crucial to counteract this sustainability issue faced by western countries. Musculoskeletal and rheumatic diseases (RD) may cause work disability and early exit from work, including early retirement. The objective of this article is to review the current knowledge about interventions aiming to reduce early retirement due to RD. METHODS: We searched PubMed and The Cochrane Library for studies either in English or Portuguese between January 2000 and June 2016 that evaluated the impact of interventions targeting early retirement in RD patients still at work. We also searched for grey literature from Portuguese institutional repositories. RESULTS: We identified several published studies testing pharmacologic and non-pharmacologic vocational rehabilitation interventions. None was specifically identified for Portugal. The general low quality of the literature and its inconsistency makes it unfeasible to draw definitive conclusions. However, some broad recommendations might be outlined. An effective intervention must: 1) act upon different levels (e.g. RD patient, workplace), involving several stakeholders (e.g. rheumatologists, occupational physicians, employers); 2) prioritize the right patients (e.g. more disabling RD); and 3) consider the patients' role, for instance by including an element of patient education and support. Despite the lack of good quality evidence on this field, there seems to be a growing interest in the international scientific community with several ongoing studies promoting such interventions. This promising data will be very useful to set up effective policies. CONCLUSIONS: This article summarizes the current knowledge about the impact of interventions to avoid or mitigate early retirement in RD patients. It highlights the demand for further research and it also contributes to aware decision-makers about the relevance of this topic, particularly in Portugal.