RESUMEN: Este estudio tuvo como objetivo evaluar los aspectos morfológicos craneofaciales de los pacientes con síndrome de Sheehan (SHS). Se realizó un estudio observacional con 19 mujeres diagnosticadas con SHS y 19 controles asociados por edad y sexo. Se obtuvieron radiografías cefalométricas laterales y se analizaron 30 medidas lineales y angulares mediante el software Radiocef Studio 2. La edad media de los pacientes fue de 65,47 ± 10,19 años. Los principales hallazgos fueron proposición maxilar (52,63 %) y mandíbula (52,63 %) con respecto a la base del cráneo, prognatismo mandibular en 73,68 %, patrón de crecimiento profundo en 42,1 %, aumento del plano mandibular en 36,84 % y reducción de la altura facial anterior. El grupo SHS mostró diferencias estadísticamente significativas en SNB (p=0,026), N-Me (p=0,006), longitud del paladar blando (p=0,011) y Ena-Me (p<0,001) en comparación con los controles. El análisis de la puntuación de la desviación estándar reveló valores alterados en relación con las longitudes maxilares y mandibulares totales. El SHS mostró una morfología craneofacial alterada, caracterizada por prognatismo maxilomandibular, tipo braquifacial, aumento del plano mandibular y reducción de la longitud del velo del paladar. Este estudio informa hallazgos novedosos en SHS.

ABSTRACT: This study aimed to assess the craniofacial morphologic aspects of Sheehan’s syndrome (SHS) patients.An observational study was performed with 19 women diagnosed with SHS and 19 controls matched by age and sex. Lateral cephalometric radiographs were obtained, and 30 linear and angular measurements were analyzed using the Radiocef Studio 2 software. The mean age of patients was 65.47 ± 10.19 years. The main findings were propositioned maxilla (52.63 %) and mandible (52.63 %) relative to the cranial base, mandibular prognathism in 73.68 %, deep growth pattern in 42.1 %, increased mandibular plane in 36.84 %, and reduction in anterior facial height. The SHS group showed statistically significant differences in SNB (p=0.026), N-Me (p=0.006), soft palate length (p=0.011), and Ena-Me (p<0.001) in comparison with controls. The standard deviation score analysis revealed altered values in relation to total maxillary and mandibular lengths. SHS showed altered craniofacial morphology, characterized by maxillo- mandibular prognathism, brachyfacial type, increased mandibular plane, and reduction in soft palate length. This study reports novel findings in SHS.

ABSTRACT BACKGROUND: Neuroendocrine tumors are rare neoplasms of uncertain biological behavior. The liver is one of the most common sites of metastases, occurring in 50% of patients with metastatic disease. AIMS: To analyze a clinical series in liver transplant of patients with neuroendocrine tumors metastases. METHODS: A retrospective descriptive study, based on the review of medical records of patients undergoing liver transplants due to neuroendocrine tumor metastases in a single center in northeast Brazil, over a period of 20 years (January 2001 to December 2021). RESULTS: During the analyzed period, 2,000 liver transplants were performed, of which 11 were indicated for liver metastases caused by neuroendocrine tumors. The mean age at diagnosis was 45.09±14.36 years (26–66 years) and 72.7% of cases were females. The most common primary tumor site was in the gastrointestinal tract in 64% of cases. Even after detailed investigation, three patients had no primary tumor site identified (27%). Overall survival after transplantation at one month was 90%, at one year was 70%, and five year, 45.4%. Disease-free survival rate was 72.7% at one year and 36.3% at five years. CONCLUSIONS: Liver transplantation is a treatment modality with good overall survival and disease-free survival results in selected patients with unresectable liver metastases from neuroendocrine tumors. However, a rigorous selection of patients is necessary to obtain better results and the ideal time for transplant indication is still a controversial topic in the literature.

RESUMO RACIONAL: Os tumores neuroendócrinos são neoplasias raras de comportamento biológico incerto. O fígado é um local comum de metástase, ocorrendo em 50% dos pacientes com doença metastática. OBJETIVOS: Analisar casuística de transplante hepático por metástases de tumores neuroendócrinos. MÉTODOS: Estudo descritivo retrospectivo com revisão de prontuários de pacientes submetidos a transplante hepático por metástases de tumores neuroendócrinos em um único centro no Nordeste do Brasil durante 20 anos (janeiro de 2001 a dezembro de 2021). RESULTADOS: Durante o período analisado, foram realizados 2.000 transplantes hepático, sendo 11 indicados por metástases hepáticas de tumores neuroendócrinos. A média de idade ao diagnóstico foi de 45,09±14,36 anos (26–66 anos) e 72,7% dos casos eram do sexo feminino. O local do tumor primário mais comum foi o trato gastrointestinal (64% dos casos). Após detalhada investigação, três pacientes não tiveram o local do tumor primário identificado (27%). A sobrevida global um mês e após um ano do transplante foi de 90 e 70%, respectivamente. A sobrevida após 5 anos foi de 45,4%. A taxa de sobrevida livre de doença foi de 72,7% no primeiro ano e 36,3% em cinco anos. CONCLUSÕES: O transplante hepático é uma modalidade de tratamento com bons resultados de sobrevida global e sobrevida livre de doença, em pacientes selecionados com metástases hepáticas irressecáveis de tumores neuroendócrinos. No entanto, a seleção rigorosa dos pacientes é necessária para obter melhores resultados e o momento ideal para a indicação do transplante ainda é um tema controverso na literatura.

ABSTRACT Objective: The COVID-19 pandemic has profoundly disrupted health care worldwide. We aimed to evaluate the impact of the first COVID-19 wave on the treatment of our patients with acromegaly. Subjects and methods: A standard questionnaire was systematically applied to all patients and included questions on general health status, whether all laboratory workup had been done, common signs and symptoms of acromegaly, treatment adherence, and previous COVID-19 symptoms and diagnosis. Results: We attempted to contact 136 patients with acromegaly at regular follow-up at our institution and contacted 101 of them successfully. In all, 37% of the patients reported symptoms of acromegaly, which was more common among women. A total of 27 patients were lost to follow-up (including 19 who interrupted treatment during the pandemic) mainly for fear of becoming infected by the SARS-CoV-2. Of these, 24 resumed follow-up after our contact. Conclusions: The current COVID-19 pandemic has strongly impacted the follow-up of patients with acromegaly. Telemedicine can be an important tool to maintain regular treatment in the current or future pandemics.

Abstract Introduction: It is challenging to diagnose syncope in patients with pacemakers. Because these patients have increased morbidity and mortality risks, they require immediate attention to determine the causes in order to provide appropriate treatment. This study aimed to investigate the causes and predictive factors of syncope as well as the methods used to diagnose syncope in cardiac pacemaker patients. Methods: Patients with pacemakers implanted owing to sinus node disease or atrioventricular block were evaluated with standardized questionnaires, endocavitary electrograms, and other tests based on the suspected causes of syncope. Mann-Whitney U tests were used to analyze continuous variables and Chi-squared or Fisher’s exact tests were used for categorical variables. Logistic regression was used for multivariate analyses. Statistical significance was P<0.05. Results: The study included 95 patients with pacemakers: 47 experienced syncope in the last 12 months and 48 did not. Of the 100 documented episodes of syncope, 48.9% were vasovagal syncopes, 17% had cardiac-related causes, 10.6% had unknown causes, and 8.5% had pacemaker failure. The multivariate analysis showed that a New York Heart Association (NYHA) Functional Class II was a significant factor for developing syncope (P<0.01). Conclusion: While the most common type of syncope in pacemaker patients was neurally mediated, it is important to perform detailed evaluations in this population as the causes of syncope can be life-threatening. The best diagnostic methods were stored electrogram analysis and the tilt table test. NYHA Functional Class II patients were found to have a higher risk for syncope.

RESUMO INTRODUÇÃO A síndrome de sobrecarga de ferro engloba um grande espectro de etiologias que levam a um aumento da quantidade de ferro nos tecidos. Esse ferro excede a capacidade de proteção dos tecidos, levando a dano oxidativo e lesão tissular. Tratamento pode prevenir esse dano, levando à melhor sobrevida. METODOLOGIA A literatura dos últimos cinco anos foi revisada por meio de pesquisa na base de dados PubMed buscando identificar estratégias de tratamento. DISCUSSÃO Medidas farmacológicas e não farmacológicas estão disponíveis para o tratamento da síndrome de sobrecarga de ferro e devem ser utilizadas de acordo com a etiologia e a aceitação do paciente. A flebotomia terapêutica é base do tratamento dos pacientes com hemocromatose hereditária. Pacientes com sobrecarga transfusional ou aqueles que não toleram flebotomias devem utilizar quelantes de ferro. CONSIDERAÇÕES FINAIS Avanços no entendimento da síndrome de sobrecarga de ferro têm levado a grandes progressos na terapêutica, com promessas de abordagem de novos alvos farmacológicos. A evolução da pesquisa tem possibilitado melhor aderência com o uso de quelantes orais e com possibilidade de drogas menos tóxicas.

SUMMARY INTRODUCTION Iron overload is a broad syndrome with a large spectrum of causative etiologies that lead to iron deposition. When iron exceeds defenses, it causes oxidative damage and tissular disfunction. Treatment may prevent organ dysfunction, leading to greater life expectancy. METHODS Literature from the last five years was reviewed through the use of the PubMed database in search of treatment strategies. DISCUSSION Different pharmacological and non-pharmacological strategies are available for the treatment of iron overload and must be used according to etiology and patient compliance. Therapeutic phlebotomy is the basis for the treatment of hereditary hemochromatosis. Transfusional overload patients and those who cannot tolerate phlebotomy need iron chelators. CONCLUSION Advances in the understanding of iron overload have lead to great advances in therapies and new pharmacological targets. Research has lead to better compliance with the use of oral chelators and less toxic drugs.

Resumo: O objetivo foi estimar a prevalência do uso de adoçantes pela população adulta brasileira e características dos usuários. Análise de dados da Pesquisa Nacional de Acesso, Utilização e Promoção do Uso Racional de Medicamentos (PNAUM, 2014), um inquérito nacional de base populacional. O desfecho de interesse foi o uso autorreferido de adoçantes entre brasileiros com 20 anos ou mais. As variáveis analisadas foram sexo, idade em anos completos, região do Brasil, escolaridade em anos completos e classificação econômica segundo o Critério Classificação Econômica Brasil da Associação Brasileira de Empresas de Pesquisa (ABEP). Os indicadores das condições de saúde foram: relato de doença crônica não transmissíveis (DCNT), número de DCNT e índice de massa corporal (IMC). A prevalência do uso de adoçantes na população adulta brasileira foi de 13,4% (IC95%: 12,5-14,3), sendo maior entre as pessoas do sexo feminino e no grupo com 60 anos ou mais, nas regiões Nordeste e Sudeste, entre pessoas da classe econômica A/B e entre indivíduos obesos. As pessoas com doenças crônicas (em especial diabetes) foram as que mostraram maior prevalência de uso de adoçantes, sendo o uso maior quanto maior o número de comorbidades relatadas. A prevalência de uso de adoçantes foi de 13,4% e mostrou-se associada a características sociodemográficas e de saúde.

Abstract: The objective was to estimate the prevalence of artificial sweetener use by the adult Brazilian population and users’ characteristics. Analysis of data from the Brazilian National Survey on Access, Utilization, and Promotion of Rational Use of Medicines (PNAUM, 2014), a nationwide population-based survey. The target outcome was self-reported use of sweeteners by Brazilians 20 years and older. The independent variables were sex, age, major geographic region of Brazil, schooling in complete years, and economic status according to the Brazilian Economic Classification Criterion of the Brazilian Association of Research Companies (ABEP). The health condition indicators were: self-reported noncommunicable diseases (NCDs), number of NCDs, and body mass index (BMI). Prevalence of sweetener use in the Brazilian adult population was 13.4% (95%CI: 12.5-14.3), and it was higher in females and in persons 60 years or older, in the Northeast and Southeast, among individuals from economic classes A and B, and among obese individuals. Persons with chronic diseases (especially diabetes) showed the highest prevalence of use of sweeteners, and their use increased with the number of reported comorbidities. Prevalence of use of artificial sweeteners was 13.4% and was associated with sociodemographic and health characteristics.

Resumen: El objetivo fue estimar la prevalencia del uso de edulcorantes por parte de la población adulta brasileña y las características de los usuarios. Análisis de datos de la Encuesta Nacional de Acceso, Utilización y Promoción del Uso Racional de Medicamentos (PNAUM, 2014), una encuesta nacional de base poblacional. El resultado de interés fue el uso autoinformado de edulcorantes entre brasileños con 20 años o más. Las variables analizadas fueron: sexo, edad (años completados), región de Brasil, escolaridad (años completados), así como la clasificación económica según el Criterio Clasificación Económica Brasil de la Asociación Brasileña de Empresas de Investigación (ABEP). Los indicadores de las condiciones de salud fueron: informe de enfermedades crónicas (DCNT), número de DCNT e índice de masa corporal (IMC). La prevalencia del uso de edulcorantes en la población adulta brasileña fue de un 13,4% (IC95%: 12,5-14,3), siendo mayor entre las personas de sexo femenino y en el grupo con 60 años o más, en las regiones Nordeste y Sudeste, entre personas de clase económica A/B y entre individuos obesos. Las personas con enfermedades crónicas (en especial diabetes) fueron las que mostraron una mayor prevalencia de uso de edulcorantes, siendo el uso mayor, cuanto mayor fuera el número de comorbilidades informadas. Conclusiones: la prevalencia de uso de edulcorantes fue de un 13,4% y se mostró asociada a características sociodemográficas y de salud.

Resumo: O objetivo foi estimar a prevalência do uso de adoçantes pela população adulta brasileira e características dos usuários. Análise de dados da Pesquisa Nacional de Acesso, Utilização e Promoção do Uso Racional de Medicamentos (PNAUM, 2014), um inquérito nacional de base populacional. O desfecho de interesse foi o uso autorreferido de adoçantes entre brasileiros com 20 anos ou mais. As variáveis analisadas foram sexo, idade em anos completos, região do Brasil, escolaridade em anos completos e classificação econômica segundo o Critério Classificação Econômica Brasil da Associação Brasileira de Empresas de Pesquisa (ABEP). Os indicadores das condições de saúde foram: relato de doença crônica não transmissíveis (DCNT), número de DCNT e índice de massa corporal (IMC). A prevalência do uso de adoçantes na população adulta brasileira foi de 13,4% (IC95%: 12,5-14,3), sendo maior entre as pessoas do sexo feminino e no grupo com 60 anos ou mais, nas regiões Nordeste e Sudeste, entre pessoas da classe econômica A/B e entre indivíduos obesos. As pessoas com doenças crônicas (em especial diabetes) foram as que mostraram maior prevalência de uso de adoçantes, sendo o uso maior quanto maior o número de comorbidades relatadas. A prevalência de uso de adoçantes foi de 13,4% e mostrou-se associada a características sociodemográficas e de saúde.

Abstract: The objective was to estimate the prevalence of artificial sweetener use by the adult Brazilian population and users’ characteristics. Analysis of data from the Brazilian National Survey on Access, Utilization, and Promotion of Rational Use of Medicines (PNAUM, 2014), a nationwide population-based survey. The target outcome was self-reported use of sweeteners by Brazilians 20 years and older. The independent variables were sex, age, major geographic region of Brazil, schooling in complete years, and economic status according to the Brazilian Economic Classification Criterion of the Brazilian Association of Research Companies (ABEP). The health condition indicators were: self-reported noncommunicable diseases (NCDs), number of NCDs, and body mass index (BMI). Prevalence of sweetener use in the Brazilian adult population was 13.4% (95%CI: 12.5-14.3), and it was higher in females and in persons 60 years or older, in the Northeast and Southeast, among individuals from economic classes A and B, and among obese individuals. Persons with chronic diseases (especially diabetes) showed the highest prevalence of use of sweeteners, and their use increased with the number of reported comorbidities. Prevalence of use of artificial sweeteners was 13.4% and was associated with sociodemographic and health characteristics.

Resumen: El objetivo fue estimar la prevalencia del uso de edulcorantes por parte de la población adulta brasileña y las características de los usuarios. Análisis de datos de la Encuesta Nacional de Acceso, Utilización y Promoción del Uso Racional de Medicamentos (PNAUM, 2014), una encuesta nacional de base poblacional. El resultado de interés fue el uso autoinformado de edulcorantes entre brasileños con 20 años o más. Las variables analizadas fueron: sexo, edad (años completados), región de Brasil, escolaridad (años completados), así como la clasificación económica según el Criterio Clasificación Económica Brasil de la Asociación Brasileña de Empresas de Investigación (ABEP). Los indicadores de las condiciones de salud fueron: informe de enfermedades crónicas (DCNT), número de DCNT e índice de masa corporal (IMC). La prevalencia del uso de edulcorantes en la población adulta brasileña fue de un 13,4% (IC95%: 12,5-14,3), siendo mayor entre las personas de sexo femenino y en el grupo con 60 años o más, en las regiones Nordeste y Sudeste, entre personas de clase económica A/B y entre individuos obesos. Las personas con enfermedades crónicas (en especial diabetes) fueron las que mostraron una mayor prevalencia de uso de edulcorantes, siendo el uso mayor, cuanto mayor fuera el número de comorbilidades informadas. Conclusiones: la prevalencia de uso de edulcorantes fue de un 13,4% y se mostró asociada a características sociodemográficas y de salud.

SUMMARY Insulin autoimmune syndrome (IAS, Hirata's disease) is a rare hypoglycemic disorder characterized by spontaneous hypoglycemia associated with extremely high circulating insulin levels and positive anti-insulin antibody results. Thus far, most cases have been reported in Asian countries, notably Japan, with few cases reported in western countries. As a possible cause, it is associated with the use of drugs containing sulfhydryl radicals, such as captopril. This report refers to a 63-year-old female Brazilian patient with a history of postprandial hypoglycemia. After extensive investigation and exclusion of other causes, her hyperinsulinemic hypoglycemia was considered to have likely been induced by captopril. Most cases of IAS are self-limiting. However, dietary management, corticosteroids, plasmapheresis, and rituximab have already been used to treat patients with IAS. In our case, after discontinuation of captopril, an initial decrease in insulin autoantibody levels was observed followed by improvement in episodes of hypoglycemia. Although it is a rare disease, IAS should be considered in the differential diagnosis of endogenous hyperinsulinemic hypoglycemia. Patients with suspected IAS must be screened for autoimmunity-related drugs for insulin. Initial clinical suspicion of IAS can avoid unnecessary costs associated with imaging examinations and/or invasive surgical procedures.

Abstract INTRODUCTION Benznidazole (BNZ) is a drug available for the etiological treatment of Chagas disease. However, this drug is toxic and has a limited effectiveness on the chronic phase of this disease, often leading to poor treatment adherence. METHODS: This is a descriptive and exploratory study conducted at the Pharmaceutical Care Service for Chagas disease patients of the Federal University of Ceará. Drug-related problems (DRPs) and pharmaceutical interventions (PIs) were classified according to the Second Consensus of Granada. RESULTS: The average age of patients with Chagas disease was 62 years, with the majority residing in the Ceará countryside (86.7%), and having low education levels (63.3% with elementary school education). Regarding family income, most patients belonged to a household that earned ≤1-2 times the minimum wage per month. Approximately 73% of these patients complied with the BNZ treatment, and nearly 7% underwent therapy interruption after medical evaluation. A total of 189 DRPs were identified, of which 51.9% (n=98) were classified as potential, and 48.1% (n=91) as actual. The most frequent DRPs were related to safety (qualitative safety; n=70; 37%), necessity (non-adherence; n=52; 27.5%), and effectiveness (qualitative effectiveness/non-optimal drug selection; n=45; 23.8%). Among the 216 PIs conducted, the majority were related to patient education (n=168; 77.8%) and pharmacological strategy (n=42; 19.4%). CONCLUSIONS: This study indicates the need for pharmacotherapeutic monitoring in patients with Chagas because of the high number of therapeutic interventions, DRPs (approximately 3 DRPs/patient), BNZ adherence, and polypharmacy.

Resumo Fundamento: Pacientes submetidos à ressincronização cardíaca podem evoluir com padrões de resposta acima do esperado, com normalização dos parâmetros clínicos e ecocardiográficos. Objetivo: Analisar as características clínicas e ecocardiográficas desta população de super-respondedores, comparando-as com os demais pacientes submetidos à terapia de ressincronização cardíaca. Métodos: Estudo de coorte observacional, prospectivo, envolvendo 146 pacientes, consecutivamente submetidos a implantes de ressincronizador cardíaco. Para comparação das variáveis, foram realizados o teste exato de Fisher e o teste de Mann-Whitney. Foram considerados super-respondedores os pacientes com fração de ejeção > 50 % e classe funcional I/II (New York Heart Association) após 6 meses da terapia de ressincronização cardíaca. Resultados: A idade média foi de 64,8 ± 11,1 anos, sendo 69,8% do sexo masculino, com mediana da fração de ejeção de 29%, sendo 71,5% com bloqueio de ramo esquerdo, 12% com bloqueio de ramo direito associado a bloqueios divisionais; 16,3% com marca-passo cardíaco definitivo, 29,3% com miocardiopatia isquêmica, 59,4% com miocardiopatia dilatada e 11,2% com miocardiopatia chagásica. Foram observados 24 (16,4%) super-respondedores, sendo que 13 (8,9%) apresentaram normalização da fração de ejeção, dos diâmetros diastólicos do ventrículo esquerdo e da classe funcional. Quando comparados com os pacientes não super-respondedores, em relação às características pré-implante, os super-respondedores apresentaram-se mais no sexo feminino (58,3% vs. 22,8%; p = 0,002), maior índice de massa corporal (26,8 vs. 25,5; p = 0,013), maior fração de ejeção basal (31,0 vs. 26,9; p = 0,0003) e menores diâmetros diastólicos do ventrículo esquerdo (65,9 mm vs. 72,6 mm; p = 0,0032). Dez pacientes (41,6% dos super-respondedores) com bloqueio de ramo direito e bloqueio divisional evoluíram como super-respondedores, entretanto apenas um paciente com doença de Chagas e apenas na primeira avaliação. Conclusões: Os super-respondedores apresentaram cardiopatia de base menos avançada e sem diferenças em relação ao tipo de distúrbio de condução basal. Pacientes com bloqueio de ramo direito e bloqueio divisional, mas sem cardiopatia chagásica podem também evoluir como super-respondedores.

Abstract Background: Patients submitted to cardiac resynchronization may develop response patterns that are higher than expected, with normalization of clinical and echocardiographic parameters. Objective: To analyze the clinical and echocardiographic characteristics of this population of super-responders, comparing them with the other patients submitted to cardiac resynchronization therapy. Methods: A prospective, observational cohort study involving 146 patients consecutively submitted to cardiac resynchronization implants. Fisher's exact test and Mann-Whitney test were performed to compare the variables. Patients with ejection fraction > 50% and functional class I/II (New York Heart Association) were considered super-responders after 6 months of cardiac resynchronization therapy. Results: Mean age was 64.8 ± 11.1 years, with 69.8% of males, with a median ejection fraction of 29%, 71.5% with left bundle-branch block, 12% with right bundle-branch block associated with hemiblocks; 16.3% wearing a definitive cardiac pacemaker, 29.3% with ischemic cardiomyopathy, 59.4% with dilated cardiomyopathy, and 11.2% with Chagasic cardiomyopathy. Twenty-four (16.4%) super-responders were observed, and 13 (8.9%) showed normalization of the ejection fraction, left ventricular diastolic diameters and functional class. When compared to the non-super-responder patients, in relation to the pre-implantation characteristics, the super-responders were more often females (58.3% vs. 22.8%, p = 0.002), had higher body mass index (26.8 vs. 25.5, p = 0.013), higher baseline ejection fraction (31.0 vs. 26.9, p = 0.0003), and lower left ventricular diastolic diameters (65.9 mm vs. 72.6 mm, p = 0.0032). Ten patients (41.6% of super-responders) with right bundle-branch block and hemiblock progressed to super-responders, although there was only one patient with Chagas' disease among them, and only at the first assessment. Conclusions: Super-responders had less advanced heart disease at baseline and no differences regarding the type of conduction disorder at baseline. Patients with right bundle-branch block and hemiblock, but without Chagasic heart disease may also progress as super-responders.

Resumo Fundamento: A terapia de ressincronização cardíaca (TRC) é um tratamento recomendado pelas principais diretrizes mundiais. Entretanto, 30%-40% dos pacientes selecionados não respondem ao tratamento. Objetivo: Elaborar um modelo ecocardiográfico preditor de risco de óbito cardíaco ou transplante (Tx) após 1 ano da TRC. Método: Estudo observacional, prospectivo, com inclusão de 116 pacientes, sendo 69,8% do sexo masculino, com idade de 64,89 ± 11,18 anos, CF III (68,1%) e IV ambulatorial (31,9%), bloqueio de ramo esquerdo em 71,55%, e mediana da fração de ejeção (FE) de 29%. Avaliações foram feitas no período pré-implante e 6-12 meses após, e correlacionadas com mortalidade cardíaca/Tx no final do seguimento. Foram realizadas análises de regressão de Cox e logística, com a curva ROC e de sobrevida de Kaplan-Meier. O modelo foi validado internamente pelo “bootstrapping”. Resultados: Ocorreram 29 (25%) óbitos/Tx durante o seguimento de 34,09 ± 17,9 meses. A mortalidade cardíaca/Tx foi de 16,3 %. No modelo multivariado de Cox, as variáveis FE < 30%, disfunção diastólica grau III/IV e regurgitação mitral grau III, aferidas com 6-12 meses, relacionaram-se independentemente a aumento de mortalidade cardíaca ou Tx, com razões de risco de 3,1, 4,63 e 7,11, respectivamente. A área sob a curva ROC foi de 0,78. Conclusão: FE menor que 30%, disfunção diastólica grave e regurgitação mitral severa indicam pior prognóstico após 1 ano da TRC, devendo ser consideradas outras opções terapêuticas na presença da combinação de duas dessas variáveis.

Abstract Background: Cardiac resynchronization therapy (CRT) is the recommended treatment by leading global guidelines. However, 30%-40% of selected patients are non-responders. Objective: To develop an echocardiographic model to predict cardiac death or transplantation (Tx) 1 year after CRT. Method: Observational, prospective study, with the inclusion of 116 patients, aged 64.89 ± 11.18 years, 69.8% male, 68,1% in NYHA FC III and 31,9% in FC IV, 71.55% with left bundle-branch block, and median ejection fraction (EF) of 29%. Evaluations were made in the pre‑implantation period and 6-12 months after that, and correlated with cardiac mortality/Tx at the end of follow-up. Cox and logistic regression analyses were performed with ROC and Kaplan-Meier curves. The model was internally validated by bootstrapping. Results: There were 29 (25%) deaths/Tx during follow-up of 34.09 ± 17.9 months. Cardiac mortality/Tx was 16.3%. In the multivariate Cox model, EF < 30%, grade III/IV diastolic dysfunction and grade III mitral regurgitation at 6‑12 months were independently related to increased cardiac mortality or Tx, with hazard ratios of 3.1, 4.63 and 7.11, respectively. The area under the ROC curve was 0.78. Conclusion: EF lower than 30%, severe diastolic dysfunction and severe mitral regurgitation indicate poor prognosis 1 year after CRT. The combination of two of those variables indicate the need for other treatment options.

ResumoFundamento:A terapia de ressincronização cardíaca pode demonstrar resultados desfavoráveis em 30-40% dos casos.Objetivo:Este estudo teve por objetivo desenvolver modelos preditores para o desfecho combinado de morte cardíaca ou transplante (Tx) em diferentes estágios da terapia de ressincronização cardíaca (TRC).Métodos:Estudo prospectivo observacional de 116 pacientes com idade média de 64,8 ± 11,1 anos, dos quais 68,1% estavam em classe funcional (CF) III e 31,9% em classe IV ambulatorial. Variáveis clínicas, eletrocardiográficas e ecocardiográficas foram avaliadas com regressão de Cox e curvas de Kaplan-Meier.Resultados:O desfecho mortalidade/Tx cardíacos foi de 16,3% durante seguimento de 34,0 ± 17,9 meses. No período pré-implante, disfunção ventricular direita (DVD), fração de ejeção < 25% e uso de altas doses de diuréticos (ADD) aumentaram o risco de morte e Tx cardíacos em 3,9, 4,8 e 5,9 vezes, respectivamente. No primeiro ano após TRC, DVD, AHDD e hospitalização por insuficiência cardíaca congestiva elevaram o risco de morte (razões de risco de 3,5, 5,3 e 12,5, respectivamente). No segundo ano após TRC, DVD e CF III/IV foram fatores de risco significativos de mortalidade no modelo multivariado de Cox. As acurácias dos modelos foram 84,6% na pré-implante, 93% no primeiro ano após TRC e 90,5% no segundo ano após TRC. Os modelos foram validados por bootstrapping.Conclusão:Desenvolvemos modelos preditores de morte e Tx cardíacos em diferentes estágios de TRC com base na análise de variáveis clínicas e ecocardiográficas simples e de fácil obtenção. Os modelos mostraram boa acurácia e ajuste, foram validados internamente e são úteis para a seleção, o monitoramento e a orientação de pacientes indicados para TRC.

AbstractBackground:30-40% of cardiac resynchronization therapy cases do not achieve favorable outcomes.Objective:This study aimed to develop predictive models for the combined endpoint of cardiac death and transplantation (Tx) at different stages of cardiac resynchronization therapy (CRT).Methods:Prospective observational study of 116 patients aged 64.8 ± 11.1 years, 68.1% of whom had functional class (FC) III and 31.9% had ambulatory class IV. Clinical, electrocardiographic and echocardiographic variables were assessed by using Cox regression and Kaplan-Meier curves.Results:The cardiac mortality/Tx rate was 16.3% during the follow-up period of 34.0 ± 17.9 months. Prior to implantation, right ventricular dysfunction (RVD), ejection fraction < 25% and use of high doses of diuretics (HDD) increased the risk of cardiac death and Tx by 3.9-, 4.8-, and 5.9-fold, respectively. In the first year after CRT, RVD, HDD and hospitalization due to congestive heart failure increased the risk of death at hazard ratios of 3.5, 5.3, and 12.5, respectively. In the second year after CRT, RVD and FC III/IV were significant risk factors of mortality in the multivariate Cox model. The accuracy rates of the models were 84.6% at preimplantation, 93% in the first year after CRT, and 90.5% in the second year after CRT. The models were validated by bootstrapping.Conclusion:We developed predictive models of cardiac death and Tx at different stages of CRT based on the analysis of simple and easily obtainable clinical and echocardiographic variables. The models showed good accuracy and adjustment, were validated internally, and are useful in the selection, monitoring and counseling of patients indicated for CRT.

A toxicidade de diversos poluentes ambientais em seres humanos e demais espécies tem sido habitualmente investigada quanto aos seus efeitos teratogênicos e cancerígenos. Nas últimas décadas, muitos contaminantes têm demonstrado efeitos adversos sobre o sistema endócrino. Atualmente, cerca de onze milhões de substâncias químicas são conhecidas em todo mundo, sendo três mil delas produzidas em larga escala. Numerosos compostos químicos de uso doméstico, industrial e agrícola possuem comprovada atividade hormonal. Entre os produtos químicos com atividade estrogênica, destacam-se hormônios presentes em cosméticos, anabolizantes utilizados em rações animais, fitoestrógenos e poluentes orgânicos persistentes (POPs). Esses agentes que estão presentes nos efluentes industriais, residenciais e das estações de tratamento de água e esgoto representam uma importante fonte de contaminação ambiental. O Programa Internacional de Segurança Química (International Programme on Chemical Safety - IPCS) define como interferente endócrino substâncias ou misturas presentes no ambiente capazes de interferir nas funções do sistema endócrino, causando efeitos adversos em um organismo intacto ou na sua prole. No presente artigo, os autores apresentam uma revisão da literatura atual sobre o papel desses elementos nas doenças endócrinas e metabólicas, os prováveis mecanismos de ação envolvidos, discutindo-se perspectivas futuras em termos de investigação e estratégias para prevenção e redução dos seus possíveis danos.

The toxicity of various pollutants has been routinely investigated according to their teratogenic and carcinogenic effects. In the last few decades, however, many of such pollutants have been shown to adversely affect the endocrine system of human beings and other species. Currently, more than eleven million chemical substances are known in the world, and approximately 3,000 are produced on a large scale. Numerous chemical composites of domestic, industrial and agricultural use have been shown to influence hormonal activity. Examples of such chemical products with estrogenic activity are substances used in cosmetics, anabolizing substances for animal feeding, phytoestrogens and persistent organic pollutants (POPs). These agents are seen in residential, industrial and urban sewerage system effluents and represent an important source of environmental contamination. The International Programme on Chemical Safety (IPCS) defines as endocrine disruptors substances or mixtures seen in the environment capable of interfering with endocrine system functions resulting in adverse effects in an intact organism or its offspring. In this article the authors present a current literature review about the role of these pollutants in endocrine and metabolic diseases, probable mechanisms of action, and suggest paths of investigation and possible strategies for prevention and reduction of its possible damages.

OBJETIVOS: Tumores testiculares são uma rara condição associada à hiperplasia adrenal congênita (HAC) que decorrem da hiperplasia de restos adrenais intratesticulares (HRA), raramente ocorrendo associados a neoplasias malignas. Sua diferenciação histológica com tumores de células de Leydig é muito difícil, podendo levar a orquiectomias desnecessárias. O objetivo deste relato foi apresentar esse dilema diagnóstico em um paciente com HAC e tumores testiculares bilaterais. MÉTODOS: Relatou-se o caso de um paciente masculino, 16 anos, com diagnóstico de HAC desde os 3 anos de idade, que apresentava tumorações testiculares endurecidas, indolores e de crescimento lento, sendo encaminhado para orquiectomia bilateral. RESULTADOS: Foi decidido por tratamento conservador com prednisona, havendo significativa diminuição do volume testicular e normalização dos níveis de andrógenos. CONCLUSÃO: Este caso demonstra a importância de sempre se considerar a hipótese de HRA intratesticulares no diagnóstico diferencial dos tumores testiculares. A investigação e a conduta devem ser conduzidas de maneira cautelosa para se evitar orquiectomias desnecessárias.

OBJECTIVES: Testicular tumors are a rare condition associated with congenital adrenal hyperplasia (CAH), originated from intratesticular adrenal rest tumors, and they are rarely associated with malignant tumors. Their histological differentiation from Leydig-cell tumors is quite difficult, which would lead to inappropriate orchiectomies. Thus the objective of this report was to present this diagnostic dilemma. METHODS: Reported the case of 16-yr-old boy with previous diagnosis of CAH with bilateral testicular enlargement who was recommended to be submitted to a bilateral orchiectomy. RESULTS: Considering this findings, it was decided to treat conventionally with prednisone with significant reduction of testicular volume, and normalization of androgens levels. CONCLUSION: This case shows the importance of intratesticular adrenal rest tumors in the differential diagnosis of testicular tumors. Cautious approach during investigation and treatment are recommended to avoid inappropriate orchiectomies.