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Background The aim of this study was to investigate the effect of CYB2B6 (c.516G>T, rs3745274), CYP2C9 (c.1075A>C, rs1057910) and UGT1A9 (c.98T>C, rs72551330) polymorphisms on the pharmacokinetics of single-drug propofol in adult patients undergoing intravenous sedation. Methods In this prospective clinical study, a total of 124 patients undergoing anaesthesia with propofol, as a single drug, were evaluated when undergoing colonoscopy procedure. Clinical variables were obtained from the patient’s anamnesis prior to performing the anaesthetic procedure, in the moment of the patient’s loss of consciousness, during the colonoscopy exam (recorded every 5 min) and in the awakening time. Results Polymorphic genotypes for the rs3745274 and rs1057910 polymorphisms were associated with bispectral index, target-controlled infusion (TCI)/effector concentration of propofol and TCI/plasma concentration of propofol values. Based on multivariate analysis, it was observed that weight, age, surgery time, systolic blood pressure and the rs1057910 polymorphism corresponded to predictive values for the dose of propofol used. Weight (B = 4.807±0.897), age (B = 1.834±0.834) and duration of surgery (B = 8.164±1.624) corresponded to factors associated with increased propofol dose, while systolic blood pressure (B = −1.892±0.679) and the genotypes (AA vs CA) of the single nucleotide polymorphism (SNP) rs1057910 CYPP2C9 gene (B = −74.161±26.820) decreased the total dose of propofol used. Conclusion We concluded that the rs1057910 and rs3745274 polymorphisms affect the metabolism of propofol in patients exclusively submitted to this drug. Thus, the knowledge of the polymorphic genotypes of the CYPP2C9 and CYB2B6 genes may be predictive of different metabolising phenotypes, suggesting expected behaviours of BIS parameter in the anaesthetic procedure, which contributes to safer monitoring by anaesthesiologists during the clinical intervention.

Objectives: To summarize the evidence on the feasibility of maxillomandibular imaging exams-related fractal dimension (FD) in screening patients with osteoporosis. Methods: This registered systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses of Diagnostic Test Accuracy statement. High sensitivity search strategies were developed for six primary databases and grey literature. Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) items evaluated the risk of bias, and the GRADE approach assessed the evidence certainty. Results: From 1034 records initially identified through database searching, four studies were included (total sample of 747 patients [osteoporosis, 136; control group, 611]). The meta-analysis showed that the overall sensitivity and specificity of the FD were 86.17 and 72.68%, respectively. In general, all studies showed low RoB and applicability concern. The certainty of the evidence was very low to moderate. Conclusions: This systematic review showed that the jaw-related FD presented sensitivity and specificity values higher than 70%, and its sensitivity in osteoporosis screening was a better parameter than specificity.

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

Introduction: Syncopes may represent a high risk of morbidity and mortality in certain populations. Objective: To investigate the results of complementary methods used in the diagnosis of syncope in patients with pacemakers (PM). Methods: Participants from the Arrhythmias and Pacemaker Outpatient Unit of the University Hospital Walter Cantídio were recruited. Patients with uni or bicameral PM implanted in the last 10 years due to sinus node or atrioventricular block, with analytical capacity of endocavitary electrograms (EGM) were included and assessed clinically by specific questionnaires and EGM analysis. Other tests were ordered according to clinical suspicion, such as the 24h Holter, Tilt test, Carotid sinus hypersensitivity test or an electrophysiology study. Results: The Tilt test diagnosed vasovagal syncopes in almost 1/3 of the cases. The computerized analysis of the EGM presented a high diagnostic capacity, having determined the arrhythmic etiology or PM failures in 27.6% of the cases. Holter 24hs assisted in the investigation in 18.7% of the cases. Conclusion: Pacemaker tests with their stored electrograms and Tilt test were the best methods for etiological diagnosis of syncope in patients with cardiac pacemaker. Introdução: As síncopes podem representar elevado risco de morbimortalidade em determinadas populações. Objetivo: Investigar os resultados dos métodos complementares utilizados no diagnóstico das síncopes em portadores de marca-passos (MP). Metodologia: Foram recrutados participantes do ambulatório de Arritmias e Marca-passo do Hospital Universitário Walter Cantídio , sendo incluídos portadores de MP uni ou bicameral implantados nos últimos 10 anos por doença do nó sinusal ou bloqueio atrioventricular, com capacidade analítica dos eletrogramas endocavitários (EGM), sendo avaliados clinicamente por questionários específicos e análise dos EGM. Demais exames foram solicitados de acordo com a suspeita clínica, como o Holter 24h, Tilt teste, Teste de Hipersensibilidade do Seio Carotídeo ou Estudo Eletrofisiológico. Resultados: O Tilt teste diagnosticou síncopes neuromediadas em quase 1/3 dos casos. A análise computadorizada dos EGM apresentou elevada capacidade diagnóstica, tendo determinado a etiologia arrítmica ou por falhas do MP em 27,6% dos casos. O Holter 24hs auxiliou na investigação em 18,7% dos casos. Conclusão: Os testes do marca-passo com as análises de seus eletrogramas armazenados e o Tilt teste foram os melhores métodos para o diagnóstico etiológico das síncopes em portadores de MP cardíaco.

Objectives: To evaluate the epidemiology of patients with Cushing’s disease who are followed at the Hospital Universitário Walter Cantídio (HUWC) and compare our data with that of the current literature. Methods: An observational, longitudinal and retrospective study was carried out, including patients with current or previous Cushing’s Disease (DC) follow-up at the HUWC Endocrinology Service. Data analysis was performed by the SPSS program, and the significance level adopted for the statistical tests was 5% (p

Primary hyperparathyroidism (pHPT) is the third most common endocrinopathy, affecting 1-3% of postmenopausal women, with a total incidence of 21.6 cases per 100,000 people in the adult population. This study aimed to analyze the oral health and related aspects of individuals with pHPT.A cross-sectional observational study was carried out on 51 patients diagnosed with pHPT associated with multiple endocrine neoplasia type 1 (MEN-1) (G1) or sporadic pHPT (G2). The oral aspects investigated were periodontal parameters, salivary flow, presence of dental caries, number of restored or missing teeth, and presence of tori. The biochemical parameters were collected in periods close to the dental evaluation.In G1, 29 individuals (19 females) aged 40.24±13.06 years were included; in G2, 22 individuals (21 females) aged 64.09±10.01 years were included. Grade 2 mobility (Despite the greater number of younger individuals, higher tooth loss and periodontal changes were observed in G1 patients. Differences in the degree of severity of pHPTor socioeconomic status alone could not explain these findings.

Made available in DSpace on 2020-12-12T02:23:37Z (GMT). No. of bitstreams: 0 Previous issue date: 2020-04-01 Objective: To evaluate the maternal–fetal outcomes of CAB-induced pregnancies in patients with prolactinoma in a large cohort. Methods: The prevalence of tumor growth, miscarriage, preterm, low birth weight, congenital malformations and impairment in neuropsychological development in children among women treated with CAB were assessed in a Brazilian multicentre retrospective observational study, Results: We included 194 women with a mean age of 31 (17–45) years, 43.6% presenting microadenomas and 56.4% macroadenomas, at prolactinoma diagnosis. In 233 pregnancies, CAB was withdrawn in 89%, after pregnancy confirmation. Symptoms related to tumor growth occurred in 25 cases, more frequently in macroadenomas. The overall miscarriage rate was 11%, although higher in the subgroup of patients with CAB maintainance after pregnancy confirmation (38% vs. 7.5%). Amongst the live-birth deliveries, preterm occurred in 12%, low birth weight in 6% and congenital malformations in 4.3%. Neuropsychological development impairment was reported in 7% of cases. Conclusions: Our findings confirm previous results of safety in maternal and fetal outcomes in CAB-induced pregnancies; nevertheless, CAB maintenance after pregnancy confirmation was associated with higher miscarriage rate; result that must be further confirmed. Division of Endocrinology and Metabolism Hospital das Clinicas Neuroendocrine Unit University of Sao Paulo Medical School, Av. Dr. Enéas de Carvalho Aguiar, no 155, 8° andar, bloco 3 (Endocrinologia) Division of Neurosurgery Institute of Psychiatry University of Sao Paulo Medical School Federal University of Rio de Janeiro University of Sao Paulo Medical School of Ribeirao Preto Federal University of Pernambuco Federal University of Ceara Escola Paulista de Medicina Universidade Federal de São Paulo State University of Campinas Endocrine Division (SEMPR) Department of Internal Medicine Federal University of Parana Irmandade da Santa Casa de Misericórdia de São Paulo Division of Endocrinology Hospital de Clinicas de Porto Alegre (UFRGS) Institute of Medical Assistance to the State Public Hospital São Paulo State University (UNESP) Medical School Federal University of Minas Gerais Disciplina de Obstetrícia Departamento de Obstetrícia e Ginecologia Faculdade de Medicina (FMUSP) Universidade de São Paulo São Paulo State University (UNESP) Medical School

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.

The present study aimed to analyze cephalometric predictors of abnormal sella turcica (ST) in Sheehan Syndrome (SS) patients. An observational case-control study was performed with SS volunteers from the Endocrinology and Diabetology Service of the Walter Cantídio University Hospital (Brazil). The sample was composed of 32 patients allocated in two groups: case group (16 adult women diagnosed with SS) and control group (16 healthy individuals matched by sex and age). Analyzes of the linear dimensions (length, diameter, and depth) of the ST on lateral teleradiographies were made using the Radiocef Studio 2 software. The morphological pattern (anterior oblique wall, double floor contour, bridged, irregularities of the dorsal face, pyramidal) was also assessed. The subjects mean age was 65.47 ± 10.19 years. SS patients had lower mean length (p

The Pharmaceutical Care Service of the Chagas Disease Research Laboratory (LPDC), in the State of Ceará, Brazil, treats patients with Chagas disease (CD), characterized as a chronic, neglected disease that requires full patient follow-up. Objective: determine the socioeconomic and demographic profile of patients with CD treated at the LPDC. A descriptive cross-sectional study was carried out, in which 507 patients were treated from January 2007 to May 2016. The variables collected were gender, age, place of birth, schooling, family income, type of health care, occupation, living conditions and habits. The profile of Trypanosoma cruzi infected individuals consisted of 51.1% males; mean age of 50.4 years; 26.6% were small farmers; 48.7% had not finished elementary school; 36.9% had a family income of one minimum wage; 77.7% did not have private health insurance and most of them came from rural areas. Regarding habits, 66.5% were nonsmokers and 70.0% were sedentary. Additionally, an improvement was noted in housing conditions, with most patients moving from wattle and daub huts, which were their first homes, to brick houses. We conclude that the analysis of the socioeconomic and demographic profile of patients treated at the LPDC showed that most of them come from a low socioeconomic population, where the demand for treatment has been occurring at a later age, further reinforcing the importance of the follow-up provided by the LPDC.KEY WORDS: Chagas disease; health services research; health profile; pharmaceutical care.

Mesmo entre os especialistas mais dedicados, a prática clínica da Neuroendocrinologia é conhecida por sua larga complexidade. Os temas e subtemas a partir dos quais se pode dividir a especialidade são inúmeros e precisam ser tratados com o devido cuidado, assegurando aos profissionais médicos referências confiáveis para as abordagens e aplicações mais adequadas em sua rotina. O Guia Prático em Neuroendocrinologia é o resultado da reunião dos principais nomes da área e uma iniciativa do Departamento de Neuroendocrinologia da Sociedade Brasileira de Endocrinologia e Metabologia (SBEM). Em 28 capítulos escritos em uma linguagem acessível, o guia oferece uma literatura que, ao assimilar a complexidade da Neuroendocrinologia, enfoca as lacunas de conhecimento de endocrinologistas de todo o país, visando transformá-las em informações consistentes e atualizadas para o melhor atendimento dos pacientes.

Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel–Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10–75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2 cm; P P = 0.001). All metastatic tumors were ≥2.8 cm. Codons 161 and 167 were hotspots for VHL germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off ≥2.8 cm, 44% and 91% for TVDT cut-off of ≤24 months). In 117 of 273 patients, PanNETs >1.5 cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs P = 0.020; 80% vs 50% at 10 years; P = 0.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8 cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.

Introduction: Ectopic ACTH syndrome is rare but is frequently a severe condition because of the intensity of the hypercortisolism. Patient management is complex and demands both strategies: the diagnosis and treatment of Cushing syndrome, and the specific management of neuroendocrine tumors. Therefore, management should be performed ideally by experienced endocrinology teams in collaboration with surgeons, oncologists, nuclear medicine, specialized hormonal laboratory and modern imaging platforms. Clinical Case: Female patient, born in 2000, at the age of 15 was diagnosed with Cushing’s syndrome secondary to ACTH secretion by mediastinal tumor. At diagnosis, ACTH 1152pg/ml was found (reference value Clinical Lesson: The reported case shows a rare location of an ACTH-producing tumor, emphasizing the severity of the presentation, the evolution of tumor de-differentiation, the different results identified in octreoscan and PET-DOTATOC, and the importance of multidisciplinary management.

Introduction: Multiple Endocrine Neoplasia type 1 (MEN1) is characterized by tumours of the parathyroid glands, neuroendocrine pancreatic tumour (PNET), and anterior pituitary. Studies suggest that MEN1 gen is not etiologically related to the oncogenesis of the papillary thyroid carcinoma (PTC). Expression of menin is preserved in human normal thyroid tissue and THc. Papillary thyroid carcinoma (PTC) combined with multiple endocrine neoplasia type 1 is a rare association. Cushing Syndrome (CS) is another rare associated condition with MEN-1. In patients with MEN1, CS can result from pituitary, adrenal, or other endocrine tumours. In large series of MEN-1 CS has low frequency (0.5%) or not described. Clinical Case: A.M.F.A, 49 years, female. In 2007, cytological study of thyroid nodules resulted in PTC, underwent total thyroidectomy. Anatomopathological (AP) confirmed PTC. She was followed with an excellent response to therapy. In 2018, abdominal ultrasound due to epigastric pain showed pancreatic lesion. MRI confirmed lesion in the head and body of the pancreas of 8.2cm. She underwent total gastroduodenopacreatectomy (TGDP), AP showed a well-differentiated PNET (KI67: 2%). Before TGDP, tests for MEN-1 were collected: IGF-1 of 450 ng/ml (RV for age: 90-249 ng/ml); calcium: 1,46 nmol/l (RV: 1,12-1,40), PTH: 91,7 pg/ml (RV: 15-65), prolactin 13,3 ng/ml (RV: 5-23), gastrin 22pg/ml (RV 13-115), and late night salivary cortisol (LNSC): 8,3 nmol/l (RV T mutation in heterozygosity. O3 family members had the same mutation. To investigate hypercortisolism, it was made basal ACTH (56,1 pg/ml (VR 46 pg/ml), urine free cortisol (UFC) of 261,0 mcg/24h, 1mg DST of 15,2 mcg/dl (RV

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.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.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.

Sheehan’s syndrome (SHS) is a rare condition related to the risk of osteoporosis and evaluation of bone texture imaging features on panoramic radiographs would be suitable for this condition, which was the aim of the present study. Fractal dimension, lacunarity, and trabecular morphologic aspects were significantly altered in these patients. SHS is an important public health problem particularly in developing countries. It is characterized as postpartum hypopituitarism secondary to obstetric complications-related ischemic pituitary necrosis that shows significant systemic metabolic repercussions. Thus, this study aimed to evaluate bone texture parameters in digital panoramic radiographs of patients with SHS. A case-control study was conducted with 30 SHS patients from an Endocrinology and Diabetology Service of reference in Brazil, and 30 age- and sex-matched healthy controls. A custom computer program measured fractal dimension, lacunarity, and some morphologic features in the following mandibular regions of interest (50 × 50 pixels): below the mental foramen (F1), between the first and second molars (M1), and at the center of the mandibular ramus (R1). The fractal analysis showed a statistically significant difference between the studied groups in all regions of interest. The fractal dimension in F1 (p = 0.016), M1 (p = 0.043), and R1 (p = 0.028) was significantly lower in SHS group, as well as lacunarity in R1 (p = 0.008). Additionally, several morphologic features were statistically significant in the SHS group (p

Objective: To propose a waist-to-height ratio (WtHR) cutoff point for metabolic risk in Brazilian Turner syndrome (TS) patients. Methods: Retrospective study of anthropometric, clinical and biochemical data related to the metabolic syndrome (MS) of 45 TS patients at a specialized center. The accuracy and area under the receiver operating characteristic (ROC) curve of body mass index (BMI), waist circumference (WC) and WtHR to identify the presence of at least one of the biochemical components of MS were calculated. Results: The average age was 23.8 years (4-61 years), 43% were overweight and 11% had MS. At least one component of MS was present in 80% of the patients, being more common central obesity, low levels of HDL and dysglycemia. None had diabetes. Patients with some of the biochemical changes in MS had higher WtHR than those without metabolic alterations (0.57 vs. 0.48, P = 0.001). In adult patients (20-61 years, n = 28) the WtHR > 0.5 had 87% sensitivity and 80% specificity to identify patients with some component of MS, demonstrating greater accuracy than BMI or WC. Conclusion: We propose a WtHR cutoff point of 0.5 for metabolic risk screening in Brazilian TS patients.

Background Sheehan’s syndrome (SS) is one of the leading causes of hypopituitarism in developing countries. It occurs after postpartum necrosis of the pituitary gland, and it is considered a significant public health problem. This paper, apparently unpublished, aimed to perform an analysis on oral aspects in patients with SS. Material and Methods A cross-sectional study was performed with 23 women diagnosed with SS at the Division of Endocrinology and Diabetes (Walter Cantídio University Hospital, Fortaleza, Brazil). Results Data on sociodemographic, dental and salivary flow aspects were collected through a clinical approach and a panoramic radiograph request. The mean age was 64 ± 11.5 years old, with the sample consisting mainly of married women (56.5%), socioeconomic class C2 or D / E (78.2%) and years of education up to 8 years (69.5%). The presence of horizontal bone loss (p

Introdução: a conversão de testosterona em estradiol ocorre pela ação da enzima aromatase. Inibidores da aromatase retardam o fechamento das epífises e prolongam o tempo de crescimento linear. Objetivo: relatar a evolução do tratamento com o inibidor da aromatase, anastrozol, em um paciente com baixa estatura idiopática e apresentar uma breve revisão sobre o uso desta classe terapêutica nesse contexto clínico. Relato do caso: paciente masculino, pré-púbere, 7,5 anos apresentava altura inicial 105 cm (-3,63 DP), altura alvo 159,6 cm (-1,79 DP) e idade óssea 5 anos. Exames laboratoriais e hormônio de crescimento após estímulo com clonidina estavam normais. Aos 16,5 anos, púbere (G3P3), com altura 151 cm (-3 DP) e idade óssea de 14 anos, foi iniciado tratamento com anastrozol. Após 2 anos, apresentava altura de 161,4 cm (-1,56 DP), idade óssea 15 anos e previsão de altura final 166,7 cm. A terapêutica foi bem tolerada sem alterações na coluna vertebral ou no perfil lipídico. Conclusão: a utilização de anastrozol demonstrou ser uma alternativa segura, eficaz e de baixo custo em relação ao uso de somatropina ao aumentar a previsão de altura final em portador de baixa estatura idiopática.

Neuroendocrine tumours are a heterogeneous group of diseases with a significant variety of diagnostic tests and treatment modalities. Guidelines were developed by North American and European groups to recommend their best management. However, local particularities and relativisms found worldwide led us to create Brazilian guidelines. Our consensus considered the best feasible strategies in an environment involving more limited resources. We believe that our recommendations may be extended to other countries with similar economic standards. Univ Sao Paulo, Inst Canc Estado Sao Paulo, BR-01246000 Sao Paulo, Brazil Univ Sao Paulo, Fac Med, Dept Radiol & Oncol, BR-01246903 Sao Paulo, Brazil Hosp Sirio Libanes, BR-01308050 Sao Paulo, Brazil Hosp Moinhos de Vento Porto Alegre, BR-90035000 Porto Alegre, RS, Brazil Oncoctr, BR-30360680 Belo Horizonte, MG, Brazil Univ Fed Rio Grande do Sul, Dept Cirurgia, BR-90040060 Porto Alegre, RS, Brazil Hosp Clin Porto Alegre, BR-90035903 Porto Alegre, RS, Brazil Univ Fed Ceara, Fac Med, Dept Fisiol & Farmacol, BR-60020180 Fortaleza, Ceara, Brazil Hosp Univ Walter Cantidio, BR-60430370 Fortaleza, Ceara, Brazil Inst Nacl Canc, BR-20230240 Rio De Janeiro, Brazil Univ Sao Paulo, Fac Med, Disciplina Endocrinol & Metabol, BR-01246903 Sao Paulo, Brazil AC Camargo Canc Ctr, Dept Surg, BR-01509010 Sao Paulo, Brazil Univ Sao Paulo, Fac Med, Dept Gastroenterol, Sao Paulo, Brazil Univ Fed Ciencias Saude Porto Alegre, BR-90050170 Porto Alegre, RS, Brazil Hosp Albert Einstein, BR-05652900 Sao Paulo, Brazil Hosp Base, Fac Med Sao Jose do Rio Preto, BR-15090000 Sao Paulo, Brazil Santa Casa Sao Jose do Rio Preto, BR-15025500 Sao Jose Do Rio Preto, Brazil Pontificia Univ Catolica Parana, Hosp Erasto Gaertner, BR-81520060 Curitiba, Parana, Brazil Univ Fed Rio Grande do Norte, BR-59300000 Natal, RN, Brazil Univ Sao Paulo, Inst Coracao, BR-05403900 Sao Paulo, Brazil AC Camargo Canc Ctr, Med Oncol, BR-01509010 Sao Paulo, Brazil Univ Fed Sao Paulo, Disciplina Gastroenterol, BR-04021001 Sao Paulo, Brazil Hosp Sao Rafael, BR-41253190 Salvador, BA, Brazil Hosp Canc Barretos, Dept Cirurgia Aparelho Digest Alto & Hepatobiliop, BR-14784400 Sao Paulo, Brazil Univ Sao Paulo, Fac Med, Dept Patol, BR-01246903 Sao Paulo, Brazil Clin AMO, BR-1950640 Salvador, BA, Brazil Hosp Sao Jose, BR-01323001 Sao Paulo, Brazil Univ Nove de Julho, BR-02111030 Sao Paulo, Brazil Univ Fed Sao Paulo, Disciplina Gastroenterol, BR-04021001 Sao Paulo, Brazil Web of Science

Many cases have been published showing a co-existence of autoimmune thyroid diseases (AITDs) and other autoimmune diseases. About a quarter of patients with achalasia have a concurrent thyroid disease, most commonly associated with hypothyroidism. Although relatively rare, the association of achalasia and hyperthyroidism requires attention. The physiopathology of Grave's Disease (GD) involves B- and T-mediator lymphocytes, which have an affinity for known thyroid antigens: thyroglobulin, thyroid-peroxidase, and thyrotrophin receptor. Currently, however, the real physiopathogenesis of achalasia continues to be unknown. Some important findings are suggestive of an autoimmune mechanism: significant infiltration of the myoenteric plexus by monocytes, presence of the class II-Human Histocompatibility Complex DQwl antigen and antibodies to myoenteric neurons. The present case reports a patient who, despite testing negative for Chagas' disease, had achalasia, progressed to developing significant wasting and worsening of his quality of life, was later diagnosed with hyperthyroidism. After endoscopic esophageal dilatation and radioiodine ablation of the thyroid gland, there was great improvement in the patient clinical condition. Arq Bras Endocrinol Metab. 2012;56(9):677-82 Muitos casos têm sido publicados mostrando uma coexistência entre as doenças autoimunes da tireoide (DAIT) e outras doenças autoimunes. Cerca de um quarto dos pacientes com acalasia têm doenças da tireoide concomitantemente, sendo a mais comum a associação com hipotireoidismo. Apesar de ser relativamente rara, a associação da acalasia e hipertireoidismo requer atenção. A fisiopatologia da doença de Graves (DG) envolve os linfócitos B e T-mediados, os quais têm afinidade pelos antígenos da tireoide: tireoglobulina, tireoperoxidase e receptor de tireotrofina. Atualmente, a real fisiopatogenia da acalasia continua desconhecida. No entanto, alguns importantes achados em análise são sugestivos de mecanismo autoimune: infiltração significativa do plexo mioentérico pelos monócitos, presença do antígeno-DQwl do Complexo Humano de Histocompatibilidade classe II e presença de anticorpos contra neurônios mioentéricos. Este presente caso aborda um paciente que, apesar de testes negativos para doença de Chagas, tem acalasia que progrediu para o desenvolvimento de significativa perda ponderal e piora da sua qualidade de vida, posteriormente, diagnosticado com hipertireoidismo. Após dilatação endoscópica esofágica e ablação da glândula tireoide com radioiodo, houve grande melhora na condição clínica do paciente. Arq Bras Endocrinol Metab. 2012;56(9):677-82

Introdução: Neoplasia Endócrina Múltipla Tipo 1 (NEM-1) é doença hereditária (DH) caracterizada pela presença de hiperparatireoidismo (HPT), adenoma de hipófise (AH) e tumor gastroenteropancreático (TGEP). Análise de heredogramas (HDGs) é estratégico nas DH. Objetivo: Construir, analisar HDGs e detectar comorbidades na NEM-1. Metodologia: Entrevista, aplicação de questionário, criação de HDGs (GenoPro®) e avaliação de parâmetros metabólicos. Parcerias: NPDM/HUWC-UFC. Resultados: Analisados 33 casos distribuídos em 3 famílias (F). Na F1, 18 casos:11 HPT, 10 AH e 08 TGEP. F2, 5 casos: 3 HPT, 3 TGEP e 1 AH. F3, 10 casos: 3 HPT, 8 AH e 4 TGEP. Detectado antecessor comum entre 2 famílias e presença de surdo-mudez congênita. Em 17 indivíduos, 10 mulheres, de diferentes famílias, observamos 41% de humor deprimido, 35% obesos (OB) e 59% de sobrepeso (SP). Conclusão: HDGs permitiram caracterizar o perfil intrafamiliar, relacionar famílias aparentemente distintas. Houve frequência elevada de alteração do humor, OB e SP.

Descrever o perfil microbiologico e os desfechos clinicos de ulceras graves em pes diabeticos de pacientes internados em um hospital universitario de atencao terciaria no estado do Ceara, Brasil. Metodos: Conduziu-se uma analise retrospectiva de dados obtidos nos prontuarios medicos de todos os pacientes diabeticos internados entre janeiro de 2006 a junho de 2007, nas enfermarias do Servico de Endocrinologia e Diabetes do Hospital Universitario Walter Cantidio (Universidade Federal do Ceara), por ulceras graves em pes diabeticos, com no minimo grau 2 da classificacao de Wagner, refratarias ao tratamento ambulatorial. Dados clinicos (sexo, idade, tempo de diabetes e co-morbidades) de cada paciente assim como as caracteristicas microbiologicas do material colhido das suas ulceras em pes ou das suas pecas cirurgicas (amputacoes) foram obtidos. Resultados: Foram identificados no periodo 17 diabeticos, todos tipo 2, com idade de 58,11±10,8 anos e 12,4±8,4 anos de doenca, 58,8% homens. Das ulceras, 41,1% eram grau 2, 35,2% grau 3, 11,7% grau 4 e 11,7% grau 5 de Wagner, 64,7% com menos de 3 meses de evolucao. Realizaram limpeza cirurgica 82,3% dos pacientes e amputacoes 47%, sendo identificada osteomielite em 47% dos casos. Antibioticoterapia empirica foi iniciada em todos os pacientes, sendo ciprofloxacina/ metronidazol o esquema mais usado (76,5%). Houve cultura negativa em 12,5% das realizadas. Nas positivas, os germes mais frequentes foram: S. aureus (57,1%); S. viridans (28,7%); P. aeruginosas (28,7%) e M. morganii (28,7%) A maioria (75%) dos S. aureus isolados eram meticilino-resistentes, mas sensiveis a vancomicina. Conclusao: Observouse a presenca de flora polimicrobiana com grande numero de patogenos multirresistentes e elevada prevalencia de osteomielite e amputacoes em diabeticos portadores de ulceras graves, neuropatia e doenca vascular periferica.

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.

Education is an essential aspect of diabetic care. For this, many strategies have been searched. The objectives of the present study were to describe “The Waiting Room Project”, a proposal based on using the time in which the patient waits for individual attendance, to promote Diabetes Education; and to evaluate the clinical and epidemiological profile of diabetic participants of this project. Twenty meetings were conducted from March to October 2006, with 350 patients followed-up at the Diabetes Clinics of Walter Cantídio University Hospital-UFC, and their companions. Of these, 76 randomly assigned patients were interviewed. The meetings occurred on each Friday, in 2 sessions of approximately half hour, which were coordinated by a multidisciplinary team, and took into account 25 patients each. At the end, a breakfast was served and used for nutritional education. Among the interviewed participants, women prevailed (85.5%), with mean age of 60.4±9.1 years, and average time of diagnosis of 10.6±5.9 years. In relation to treatment, 40.7% used insulin, 63.1% used oral anti-diabetics and 9.2% only adopted life style changes. Drugs compliance was present in only 47.8% of the participants and the majority (67.1%) did not practice physical activities. Regarding to the diet, only 31.5% fully adhered to it. More than 75% of the participants could read and write, and earned a minimum wage or less. From the observations derived from practice, one may perceive: a greater motivation of the patients after each meeting, a more active participation in doctor’s appointments and an increasing interest on their disease and care. To provide the participation of diabetic patients in their treatments, stimulating self-care, is a challenge to be reached by all health units, and that can be favored by strategies like this described.

Resumen pt: A educacao e um aspecto fundamental do cuidado do diabetico. Para tal, varias estrategias tem sido buscadas. Os objetivos do presente estudo foram descre...

Background: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. 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.