Topical gentamicin for the prevention of tunneled hemodialysis catheter-related infections: a randomized double-blind study.

INTRODUCTION: Infection is the second leading cause of death in dialysis patients, with catheter-related bloodstream infection being the most serious. Exit Site Infection and Tunnel Infection are also related to the catheter. OBJECTIVE: To compare the infection rates achieved with the application of either topical gentamicin or placebo to the exit-site of tunneled catheters filled with locking solution in chronic hemodialysis patients. METHODS: This randomized double-blind clinical trial compared the application of 0.1% gentamicin versus placebo to the exit site of tunneled hemodialysis catheters filled with a prophylactic locking solution. A total of 91 patients were randomly allocated to 2 groups: placebo or 0.1% gentamicin. RESULTS: Mean patient age was 60.4 (+ 15.3) years, with predominance of males (60.4%). The main cause of chronic kidney disease was diabetes (40.7%). The rates of exit site infection (placebo = 30% vs. gentamicin = 34.1%, p = 0.821), and bloodstream infection (placebo = 22% vs. gentamicin = 17.1%, p = 0.60), as well as both exit site infection and bloodstream infection incidence density per 1000 catheter-days (p = 1) did not differ between groups. The infection-free curve was also similar in both groups. CONCLUSION: The application of topical 0.1% gentamicin to the exit site of tunneled catheters filled with lock solution did not reduce infectious complications when compared to topical placebo in patients on chronic hemodialysis.

High prevalence of cardiovascular disease and risk factors among type 2 diabetes patients followed in a hospital setting in Portugal: The PICT2RE observational study.

INTRODUCTION AND OBJECTIVES: Portugal is a country with a high prevalence of type 2 diabetes (T2D) and cardiovascular disease (CVD). The prevalence of CVD and cardiovascular (CV) risk factors among T2D patients followed in hospitals in Portugal is not known. The primary objective of this study was to assess the prevalence of CVD and CV risk factors among T2D patients in a hospital setting in Portugal. The clinical management of CVD in the hospital setting was also assessed. METHODS: We performed a non-interventional, multicenter, cross-sectional study with a retrospective phase. T2D patients were consecutively invited to participate. Data were collected retrospectively. RESULTS: A total of 715 patients were included in the study. Mean age and diabetes duration were 66.6 and 17.4 years, respectively. Of these, 286 patients (40.0%) had been diagnosed with CVD, mostly ischemic heart disease (50.4%). All patients had at least one CV risk factor. CVD was significantly associated with hypertension, hypercholesterolemia, low high-density lipoprotein cholesterol, hypertriglyceridemia and smoking. During the three years prior to study inclusion, the incidence of hyperglycemic episodes and T2D complications increased among patients with established CVD, but the numbers of hospitalization episodes and specialist appointments remained stable. An improvement was observed in key cardiometabolic risk factors. CONCLUSIONS: Our study revealed a high prevalence of CVD and CV risk factors among a sample of T2D patients in a hospital setting. Patients with established CVD seem to be adequately managed but further efforts are needed at the prevention stage for better control of risk factors and morbidity.

Adrenal carcinoma as the first manifestation of a Li­Fraumeni syndrome in three paediatric patients.

Not required for clinical vignette.

RENA Study: Cross-Sectional Study to Evaluate CKD Prevalence in Portugal.

INTRODUCTION: Chronic kidney disease (CKD) is a major global public health problem associated with increased risk of cardiovascular morbidity, premature mortality, and decreased quality of life. In Portugal, the PREVADIAB study showed a prevalence of CKD stages 3-5 of 6.1%. To overcome the limitations of the PREVADIAB study, the RENA study aimed to provide an estimate of the prevalence of CKD at a national level and to characterize CKD patients. METHODS: This was a cross-sectional study including users of Primary Health Care Units aged 18 or more. After obtaining written informed consent, sociodemographic and clinical data were recorded through a structured questionnaire, anthropometric measurements were taken, and blood and urine samples were collected. All participants initially meeting the criteria for CKD were contacted at least 3 months after the initial assessment for confirmation of the analytical results. RESULTS: A total of 3,135 individuals were included, 65.4% were female, and the mean age was 56.7 ± 15.9 years. The prevalence of hypertension, dyslipidemia, and diabetes was 38, 32, and 16%, respectively, and 31% were obese. After data adjustment by gender, age group, and geographical region, the global prevalence of CKD was 20.9% (95% CI: 6.5-35.3%), with no differences between genders and a significant increase with the advance of the age groups. CONCLUSION: Our study showed a CKD prevalence above the worldwide and Europe average. Despite the study limitations, it has become clear that it is urgent to identify CKD patients earlier and to develop awareness and educational programs to prevent CKD and its associated diseases.

[Dyslipidemia Secondary to Hypothyroidism and Cholestasis]. / Dislipidemia Secundária a Hipotiroidismo e Colestase.

In about 28% of patients, dyslipidemia has a secondary cause. Many times, the treatment of these causes can lead to the total correction of dyslipidemia. We describe the case of a 50-year-old female patient with class II obesity and primary biliary cirrhosis, evaluated for mixed dyslipidemia with poor control (statins and fibrates were being administered) as well as abnormal liver tests. The investigation carried out revealed primary auto-immune hypothyroidism. After normalisation of thyroid function by treatment with levothyroxine, as well as suspending the administration of statins and fibrates, there was an improvement in the lipid profile, although hypercholesterolemia continued. During this time, the patient was diagnosed with diabetes and she re-commenced statin therapy (atorvastatin 10 mg), which resulted in a normal lipid profile being achieved. In this case, the authors set out to highlight the importance of excluding secondary causes of dyslipidemia - including hypothyroidism, and then go on to discuss particular aspects of statin therapy for liver disease.

Cerca de 28% dos doentes com dislipidemia apresentam na sua etiologia causas secundárias. A resolução de algumas destas causas pode levar à correção total da dislipidemia. Descreve-se o caso de uma doente do sexo feminino, 50 anos, com obesidade grau II e cirrose biliar primária referenciada por dislipidemia mista com mau controlo (sob estatina e fibrato) e com alterações analíticas hepáticas. No estudo efetuado constatou-se hipotiroidismo primário auto-imune. Após normalização da função tiroideia pelo tratamento com levotiroxina, além da suspensão da estatina e fibrato, verificou-se melhoria do perfil lipídico, embora mantendo hipercolesterolemia. Neste período de tempo foi-lhe diagnosticada diabetes mellitus pelo que se instituiu de novo terapêutica com estatina (atorvastatina 10 mg), com normalização do perfil lipídico. Com este caso pretende-se salientar a importância da exclusão de causas secundárias de dislipidemia, nomeadamente o hipotiroidismo, bem como discutir aspectos particulares do tratamento com estatinas na doença hepática.

Practical guide for the use of PCSK9 inhibitors in Portugal. / Guia prático para a utilização dos inibidores da PCSK9 em Portugal.

Reducing low-density lipoprotein cholesterol (LDL-C) levels is one of the most important strategies for reducing the risk of cardiovascular events. However, in clinical practice, a high proportion of patients do not achieve recommended LDL-C levels through lifestyle and lipid-lowering therapy with statins and ezetimibe. PCSK9 inhibitors (PCSK9i) are a new therapeutic option that significantly (50-60%) reduces LDL-C levels, which in clinical trials translates into an additional reduction in risk for cardiovascular events, and has a good safety profile. However, it is a high-cost therapy, and therefore its use in clinical practice should take its cost-effectiveness into account. Priority should be given to use in patients at higher cardiovascular risk and those in whom high LDL-C levels persist despite optimal lipid-lowering therapy. This consensus document aims to summarize the main data on the clinical use of PCSK9i and to make recommendations for Portugal on the profile of patients who may benefit most from this therapy.

Malignant paraganglioma and somatotropinoma in a patient with germline SDHB mutation-genetic and clinical features.

BACKGROUND: Pituitary adenomas and paragangliomas/pheocromocytomas are rare endocrine tumours, which can be sporadic or familial. During many years their coexistence in the same individual was considered a coincidental finding. However, an association between these two entities was recently demonstrated, with the possible involvement of SDHx genes. CASE REPORT: We describe a 57-year-old female patient, who was under surveillance since 1997 for a malignant paraganglioma with vertebral bone metastasis, and harboured a germline frameshift mutation in exon 6 of SDHB gene [c.587-591DelC]. Seventeen years later, she was diagnosed with acromegaly and underwent transesphenoidal endoscopic resection of a somatotropinoma. Three months after surgery she started treatment with lanreotide for residual disease. Despite initial good response, she developed resistance to first generation of somatostatin analogues and treatment had to be switched to pegvisomant. In the immunohistochemical staining, the pituitary adenoma was positive for SDHA expression, while SDHB showed an heterogeneous staining pattern, with areas markedly positive and others with positive and negative cells. CONCLUSIONS: Our findings provide useful data for understanding the link between paragangliomas/pheocromocytomas and somatotropinomas. While we confirm the well-established link between SDHB mutations and paragangliomas/pheocromocytomas, particularly with malignant paragangliomas, the preservation-at least partially-of SDHB expression in the somatotropinoma tissue does not allow drawing definite conclusions about the involvement of the SDHB mutation in pituitary adenoma.

The Genetics of Papillary Microcarcinomas of the Thyroid: Diagnostic and Prognostic Implications.

Papillary microcarcinoma of the thyroid (mPTC) is defined by the WHO as a papillary thy-roid cancer measuring 10mm or less in diameter and it is nowadays a topic of intense debate among the members of the medical community due to its apparent "epidemic" rise. Although these tumors follow almost always an indolent clinical course and carry an excellent prognosis, it is known that a small sub-set may display a potentially aggressive behavior. Nevertheless, we still lack an accurate way of predict-ing those which will cause significant disease. In an attempt to address this problem, a number of clini-co-pathologic features have been studied as poor prognostic markers in mPTC, and their association with known genetic alterations in thyroid cancer has been evaluated. Herein we review the present knowledge concerning mPTC's genetic profile, namely the prevalence of BRAF (V600E), RAS and TERT promoter mutations and RET/PTC and PAX8-PPARG rearrangements and report the results of the evaluation in the putative prognostic value of these genetic alterations in mPTC.

Graves' disease in a mediastinal mass presenting after total thyroidectomy for nontoxic multinodular goiter: a case report.

BACKGROUND: Thyrotoxicosis after total thyroidectomy is mostly iatrogenic. Rarely, a hyperfunctional thyroid remnant or ectopic tissue may be the cause. There are few cases of Graves' disease arising from thyroid tissue located in the mediastinum and none in which Graves' disease was diagnosed only after surgery. We report the case of a patient with Graves's disease in a mediastinal thyroid mass presenting 7 years after total thyroidectomy for nontoxic goiter. CASE PRESENTATION: A 67-year-old Caucasian woman presented with palpitations, fatigue and weight loss. She had a history of total thyroidectomy for nontoxic multinodular goiter at the age of 60 without any signs of malignancy on microscopic examination. She had been medicated with levothyroxine 100 µg/day since the surgery without follow-up. She was tachycardic, had no cervical mass or eye involvement. Her thyroid-stimulating hormone levels were suppressed (0.000 µU/mL) and her free thyroxine (3.22 ng/dL) and free triiodothyronine (8.46 pg/mL) levels increased. Neither mediastinal enlargement nor trachea deviation was found on chest roentgenogram. Levothyroxine treatment was stopped but our patient showed no improvement on free thyroxine or free triiodothyronine 10 days later. Thyroglobulin was increased to 294 mg/mL. A cervical ultrasound scan revealed no thyroid remnant. Her anti-thyroid-stimulating hormone receptor antibodies were high (19.7 U/L). Corporal scintigraphy demonstrated increased intrathoracic radioiodine uptake. A computed tomography scan confirmed a 60 × 40 mm mediastinal mass. Methimazole 10 mg/day was started. Three months later, her thyroid function was normal and she underwent surgical resection. Microscopic examination showed thyroid tissue with no signs of malignancy. CONCLUSIONS: Although thyrotoxicosis after total thyroidectomy is mostly due to excessive supplementation, true hyperthyroidism may rarely be the cause, which should be kept in mind. The presence of thyroid tissue after total thyroidectomy in our patient may correspond to a remnant or ectopic thyroid tissue that became hyperfunctional in the presence of anti- thyroid-stimulating hormone receptor antibodies.

InMS: Chronic insomnia disorder in multiple sclerosis - a Portuguese multicentre study on prevalence, subtypes, associated factors and impact on quality of life.

BACKGROUND: Sleep may be disrupted in Multiple Sclerosis (MS), but the prevalence of chronic insomnia disorder (CID) using standard diagnostic criteria is unknown. OBJECTIVES: To determine the prevalence of CID in an MS population, the frequency of CID subtypes, associated factors and impact on quality of life (QoL). METHODS: Multicentre, hospital-based cross-sectional study. An adapted version of the Brief Insomnia Questionnaire was applied to a consecutively recruited MS population. The influence of demographic, MS-related features, fatigue, medical and psychiatric comorbidities, nocturnal symptoms, other sleep disorders, dysfunctional beliefs about sleep in CID was evaluated. The relation between CID and QoL was analysed. RESULTS: Of 206 MS patients, 22.3% fulfilled criteria for CID, with initial insomnia in 11.7%, maintenance insomnia in 11.2% and terminal insomnia in 10.2% of patients. CID was more frequent in female patients, those with nocturnal symptoms, medical comorbidities, higher levels of anxiety, depression and fatigue. Multivariable analysis identified female sex, medical comorbidities, anxiety and fatigue as independent factors for CID. CID patients had a significantly lower self-reported QoL. CONCLUSIONS: CID is prevalent in MS patients and associated with psychiatric and medical comorbidities, as well as fatigue. It has a negative impact on QoL.

Metabolic control and therapeutic profile of patients with diabetes in Portuguese primary care (TEDDI CP).

AIM: To evaluate the metabolic control rate and to characterize the therapeutic profile of patients with Type 2 diabetes mellitus (DM2) from Portuguese primary care of National Health Service. METHODS: Cross-sectional multicentre study conducted in Portuguese primary health care units between July 2011 and May 2012. A national representative sample of 1528 DM2 patients was selected from 51 units, stratified by region. Socio-demographic, anthropometric, lifestyle, cardiometabolic risk factors, disease status, HbA1c levels and therapeutic information were collected. RESULTS: Patients' mean age was 65±10.7 years (50.4% males) and median duration of disease was 7 years: range (0-45 years). Almost 8% were smokers, 80.3% had hypertension, 61.6% hypercholesterolemia and almost 15% cardiovascular disease. Patients' health condition was classified with a score of 4 or 5 (excellent) for 60.6%. Median HbA1c was 6.6% (min-max: 4.2%-13.4%), 64.8% of the patients had HbA1c<7.0% and 49.2% HbA1c≤6.5%. Oral antidiabetics were used in 94.4% of the patients, antihypertensives in 80.6%, antidyslipidemics in 72.0%, antiplatelet agents in 50.6% and insulin in 8.3%. CONCLUSIONS: Metabolic control rate was good according to current guidelines. However, patients with higher HbA1c levels had longer time since diagnosis, worse current health condition, hypertriglyceridemia and were insulin-treated.

Salmonella: sorotipos identificados na regiäo de Säo José do Rio Preto/SP, no período de 1990-1999 / Salmonella: serotypes identified in the Säo José do Rio Preto area, SP, during the period of 1990-1999

No período de janeiro de 1990 a dezembro de 1999, no Instituto Adolfo Lutz - Lab. I de Säo José do Rio Preto-SP, foram isoladas 324 cepas de salmonella, sendo 155 de infecçöes humanas e 169 de alimentos destinados ao consumo humano. Dos 17 diferentes sorotipos identificados a partir de infecçöes humanas, S. enterica subsp. enterica sorotipo enteritidis (S. enteritidis) foi o predominante 75,0(por cento). O material fecal representou a principal fonte de isolamento das cepas, principalmente pela ocorrência dos vários surtos de doenças veiculadas por alimentos (DVA). A partir dos alimentos, 26,6(por cento) deles envolvidos em surtos de DVA, foram identificados 30 diferentes sorotipos, também com a predominância de S. enteritidis 56,2(por cento). Os alimentos apresentando maior número de isolamentos de cepas de Salmonella foram a carne e seus derivados 59,7(por cento), seguidos por ovos e seus derivados 16,0(por cento), nos quais, S. enteritidis foi o sorotipo mais prevalente 57,4(por cento) e 81,5(por cento) respectivamente. Estes resultados demonstram que S. enteritidis permanece o sorotipo mais frequentemente isolado, tanto em infecçöes humanas quanto em alimento. Os alimentos mais comumente implicados em DVA ainda säo as carnes de aves e ovos, bem como os seus derivados, quando consumidos crus ou mal cozidos. (AU)

From January 1990 to December 1999, 324 Salmonella strains were isolated at InstitutoAdolfo Lutz ­ São José do Rio Preto­SP Lab. I, 155 from human infections and 169 from food for humanconsumption. S. enterica subsp. Enterica serotype enteritidis (S. enteritidis) was the most prevalentserotype (75.0%) among the 17 different ones identified in human infections. Fecal material representedthe main human source of the isolated strains, due to several foodborne outbreaks occurring in the periodunder study. Thirty different serotypes were identified in food, S. enteritidis being the prevailing one.About 26.6% of the analyzed foods were linked to foodborne outbreaks. Foods presenting the highest rateof isolated strains were meat and its products (59.7%), followed by eggs and egg products (16.0%). Theseresults show that S. enteritidis still remains as the most common serotype isolated from human sourcesand foods. Foods frequently implicated in foodborne outbreaks are chicken meat and eggs, as well as theirproducts, particularly raw and undercooked ones. (AU)

Surtos de enfermidades transmitidas por alimentos causados por Salmonella Enteritidis / Food borne disease outbreaks caused by Salmonella Enteritidis

Säo descritos surtos de salmonelose notificados no período de julho de 1993 a junho de 1997 na regiäo Noroeste do Estado de Säo Paulo, Brasil, tendo em vista os vários surtos de veiculaçäo alimentar ocasionados por Salmonella nessa regiäo. Foram obtidos 19 inquéritos epidemiológicos para análise de dados, 87 amostras de fezes e 38 amostras de alimentos, incluindo 12 de ovos para análise microbiológica. Cepas de Salmonella foram submetidas a sorotipagem, fagotipagem e teste de sensibilidade a 13 agentes antimicrobianos. Foram acometidas 906 pessoas com 295 hospitalizaçöes. Cepas de Salmonella Enteritidis Fagotipo 4 foram isoladas de 80,5 por cento das coproculturas, de todas amostrasd de alimentos e de 41,7 por cento dos ovos. Em 22 (95,7 por cento) surtos a salmonela foi veiculada por alimentos contendo ovos crus ou semicrus. Os testes de sensibilidade a antimicrobianos revelaram sensibilidade à maioria das cepas. Considerando os resultados obtidos, torna-se necessária a implantaçäo e intensificaçäo de medidas de controle na produçäo e armazenamento dos ovos, além da orientaçäo à populaçäo quanto aos risco no consumo inadequado desse alimento

Avaliaçao da eficácia terapêutica e da tolerância da beta-ciclodextrina piroxicam na forma líquida(gotas).Estudo multicêntrico. / Efficacy and tolerability evaluation of beta-ciclodextrin piroxicam(suppled as pediatric drops)in upper respiratory tract pathologies. Multicenter study

Trata-se de um estudo multicêntrico aberto e nao comparativo,visando avaliar a eficácia terapêutica e a tolerância do produto antiinflamatorio contendo o complexo beta-ciclodextrina piroxicam,na forma liquida(gotas),no tratamento das patologias das vias aèreas superiores e tambem em outras patologias que requeiram uma atividade antiinflamatoria,antipirética e ou analgésica.O estudo foi desenvolvido em seis centros de pesquisas por 140 mèdicos distribuidos por todo o Brasil,envolvendo um total de 642 pacientes e o esquema posologico usado foi de uma gota para cada dois quilos de peso corpóreo ao dia e em dose única.A eficácia clinica foi avaliada pelos médicos atraves da intensidade dos sinais e sintomas presentes nas patologias na visita inicial,no primeiro retorno e na visita final.A avaliaçao global da eficácia clinica obteve em 96,1 por cento dos casos(617 paciente) resultados considerados pelos medicos participantes como excelente e bom.A tolerabilidade global evidenciou resultados excelentes e bons 96,6 por cento dos casos(620 pacientes)sendo que houve necessidade de interromper o tratamento em apenas 1,4 por cento dos casos(nove pacientes)devido a nao aderencia ao tratamento e a reaçoes adversas.Estes resultados permitem concluir que o complexo beta-ciclodextrina piroxicam na forma liquida,apresenta aK

Estudo multicentrico: avaliaçao da eficacia terapeutica e tolerancia de uma nova formulaçao para uso topico. / A multicenter study: evaluation of therapeutic efficacy and tolerability of a new formulation for topical application

Foi realizado um estudo multicentrico aberto,nao comparativo,visando avaliar a eficacia terapeutica e a tolerancia de uma nova formulacao de Novacort no tratamento topico de dermatoses.Neste estudo foi avaliada a nova formulacao de Novacort,constituida pela associacao de um corticoide mais potente-o dipropionato de betametasona-um antibiotico de uso topico-o sulfato de neomicina-e um antifungico de amplo espectro-o cetoconazole.O estudo foi realizado em 20 estados do Brasil, abrangendo um total de 2.451 pacientes.O esquema posologico usado foi de uma aplicacao ao dia de uma fina camada do creme sobre a area afetada; podendo em alguns casos ser necessaria a aplicacao duas vezes ao dia.A avaliacao final da eficacia terapeutica global evidenciou 97,1 por cento de resultados excelentes e bons.A tolerabilidade foi considerada excelente, sendo que foram relatados efeitos adversos em apenas 0,6 por cento dos casos estudados; em apenas 0,21 por cento dos casos (cinco pacientes

Estudo multicentrico da famotidina em patologias digestivas altas / Famotidine multicenter study

Um estudo multicentrico realizado em 20 estados do Brasil foi conduzido para determinar a eficacia terapeutica e a tolerancia da famotidina, um antagonista do receptor H2, no tratamento da ulcera duodenal gastrica, da esofagite e da dispepsia nao ulcerosa. 1321 pacientes receberam uma dose oral unica de 40 mg de famotidina ao deitar, durante quatro semanas.A eficacia e a seguranca da famotidina no tratamento das patologias foram avaliadas atraves da melhora da sintomatologia (dor, pirose, vomitos) e por endoscopia pre e pos-tratamento em todos os pacientes.A endoscopia mostrou a cicatrizacao completa das ulceras em 96 por cento dos casos, regressao completa da esofagite em 92 por cento dos casos e observou-se cura completa da sintomatologia da dispepsia nao ulcerosa em 72 por cento dos casos.A tolerabilidade global foi considerada excelente na opiniao de 93 por cento dos medicos e pacientes.Foram registrados reacoes adversas em 5,53 por cento dos casos, sendo que 86,25 por cento destes foram considerados como de intensidade leve e moderada.Em face destes resultados conclui-se que a famotidina e eficaz e segura no tratamento das patologias digestivas altas.