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Background: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal, chronic, progressive disease. It is a rare hereditary amyloidosis, which manifests as a sensorimotor neuropathy and autonomic dysfunction. It begins during adulthood., Aims and Methods: Our aim is to evaluate psychopathological dimensions in a population attending a consultation center for TTR-FAP. Two hundred and nine subjects (symptomatic and asymptomatic carriers), 84 men and 127, women participated in the study. Most subjects were married (67.1%) and most of them were still working; 33% were retired from work or on a sick leave. A sociodemographic questionnaire and The Brief Symptom Inventory (BSI) were applied. Statistical analysis was performed (descriptive analysis, Mann-Whitney, Wilcoxon, and Spearman tests)., Results: The Global Symptom Index (GSI) was significantly higher in patients ( p = 0.001). Considering GSI, 32.7% of total subjects were above the median for general population. When subgroups were evaluated, 25.6% of symptomatic carriers, 26.3% of subjects without established diagnosis, and 39.1% of patients were above median. GSI was significantly higher in patients ( p = 0.001). Some BSI dimensions were also significantly higher in the patient group (somatization, depression, anxiety, and psychoticism) when compared with carriers. Women scored higher than men. Sick women scored higher for all dimensions except somatization. Asymptomatic carriers scored statistically higher for phobic anxiety ( p = 0.01), interpersonal sensitivity, anxiety, and depression. In patients, most dimensions and GSI (rho = 0.33, p = 0.002) had positive correlations with years of disease., Conclusions: TTR-FAP patients and carriers are a very vulnerable group for psychological distress and psychopathological problems. Women and patients are at higher risk., (Copyright © 2017 by S. Karger AG, Basel.)

Background: The aim of the present systematic review and meta-analysis was to assess the efficacy of antimicrobial photodynamic therapy (aPDT) as a therapeutic protocol for oral decontamination., Methods: In order to address the focused question: Is aPDT a useful therapeutic protocol for oral decontamination?, an electronic search without time or language restrictions was conducted up to July 2017 in indexed databases using the combination of different key words including photochemotherapy, lasers, photodynamic therapy, disinfection, mouth, saliva and oral. The exclusion criteria included reviews, case-reports, case-series, commentaries, letters to the editor, interviews, and updates. Four randomized control trials were included and processed for data extraction., Results: All studies reported that aPDT was effective in reducing the overall oral microbial load in saliva. Considering the effects of aPDT+photosensitizer (PS) compared with PS alone, there was no heterogeneity noticed for aPDT+PS (Q value=0.15, P=0.69, I 2 =0%). The overall mean difference for bacterial count in CFU/ml between aPDT+PS and PS alone was also not significant (weighted mean difference=-0.41, 95% CI=-1.12 to 0.29, p=0.24) at follow-up., Conclusion: The efficacy of aPDT for oral decontamination remains unclear. Further well-designed randomized clinical trials assessing the efficacy of aPDT reducing the oral microbial load are need., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The aim of the present study was to assess the relationship between prenatal maternal cigarette smoking (PMCS) and early childhood caries (ECC) through a systematic review of currently available scientific evidence., Material and Methods: To address the focused question: "Is there an association between PMCS and ECC?" an electronic literature search without time or language restrictions was conducted till May 2017 in indexed databases using various key words including dental caries, pregnancy, smoking, tobacco products and child. Letters to the editor, commentaries, reviews, case reports and case series and studies in which, ECC was investigated without clinical dental examination, were excluded., Results: Eight observational cross-sectional studies were included. The number of participants ranged between 1102 and 76920 children with age ranging between 24 months and 72 months. Seven studies reported a positive association between PMCS and ECC. One study reported that children whose mother smoked at least five cigarettes/day during pregnancy presented a higher caries severity level compared with to those whose mothers did not smoke. One study showed no association between ECC and PMCS., Conclusions: The association between PMCS and ECC remains debatable. Further well-designed longitudinal studies are needed in this regard. Key words: Cigarette, early childhood caries, pregnancy, risk factors, smoking., Competing Interests: Conflict of interest statement:None.

This study aimed to examine the differences between mothers of children with and without respiratory problems in variables related to psychological, parental, and marital functioning and to determine which contributed more to parenting stress, because there is a lack of information in this field. Participants were 459 mothers of children attending kindergartens, who accepted to participate. The instruments were The International Study of Asthma and Allergies in Childhood questionnaire, Hospital Anxiety and Depression Scale, Parenting Stress Index-Short Form, Parenting Stress Index-Long Form, and Escala de Avaliação da Satisfação em Áreas da Vida Conjugal . Anxiety, depression, and parenting stress were higher in the more symptomatic children and parenting stress was associated with anxiety, depression, and marital satisfaction. Findings support the relevance of children's respiratory-related variables to mothers' psychological, parental, and marital functioning., Competing Interests: Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

To determine the prevalence of hypogonadotropic hypogonadism (HH) among patients with non-functioning pituitary adenomas (NFPA) and the post-surgery outcome on pituitary gonadotropins secretion (PGS); to determine the prevalence of erectile dysfunction (ED) on male patients with NFPA, to evaluate the impact of testosterone replacement therapy (TRT) in those with HH. Retrospective evaluation of gonadal function in 109 NFPA patients (45 males), with a mean age of 51.8 years, diagnosed on the last 10 years. ED questionnaire applied to 34 male patients. Male patients with NFPA were significantly older (males 58.1±15.8 vs. females 47.4±16.94; p=0.001). Most patients had macroadenomas (67%; p=0.001) and only a minority were incidentalomas (19%; p<0.001). Prevalence of HH was 40% (60% on males, 25% on females; p<0.001). Surgery was performed in 54% of all patients (71% of males, 42% of females; p<0.003). After intervention, 14% became HH, 69% maintained previous function and 17% improved. On the questionnaire, 76% reported having ED, 54% of which had HH and 21% were under TRT. Of the patients under TRT, 79% still had ED. Median age of patients with ED was significantly higher [with ED 65 vs. without 49 years; p=0.012). There was no BMI difference between patients with or without TRT (28.0 vs. 27.4 Kg/m 2 ). NFPA was more frequent in older rather than younger patients. Males were older, had more HH and surgery. There was no significant improvement of pituitary function with surgery (17%) and 13% became iatrogenic HH. TRT had a low efficacy to improve ED in these patients., Competing Interests: Conflict of interest: The authors have no conflict of interest to disclose., (© Georg Thieme Verlag KG Stuttgart · New York.)

Prostate cancer antigen 3 (PCA3) is a prostate-specific long noncoding RNA (lncRNA) involved in the control of prostate cancer (PCa) cell survival, through modulating androgen receptor (AR) signaling. To further comprehend the mechanisms by which PCA3 modulates LNCaP cell survival, we characterized the expression patterns of several cancer-related genes, including those involved in epithelial-mesenchymal transition (EMT) and AR cofactors in response to PCA3 silencing. We also aimed to develop a strategy to stably silence PCA3. Small interfering RNA (siRNA) or short hairpin RNA (shRNA) was used to knock down PCA3 in LNCaP cells. The expression of 84 cancer-related genes, as well as those coding for AR cofactors and EMT markers, was analyzed by quantitative real-time PCR (qRT-PCR). LNCaP-PCA3 silenced cells differentially expressed 16 of the 84 cancer genes tested, mainly those involved in gene expression control and cell signaling. PCA3 knockdown also induced the upregulation of several transcripts coding for AR cofactors and modulated the expression of EMT markers. LNCaP cells transduced with lentivirus vectors carrying an shRNA sequence targeting PCA3 stably downregulated PCA3 expression, causing a significant drop (60 %) in the proportion of LNCaP cells expressing the transgene. In conclusion, our data provide evidence that PCA3 silencing modulates the expression of key cancer-related genes, including those coding for AR cofactors and EMT markers. Transducing LNCaP cells with an shRNA sequence targeting PCA3 led to loss of viability of the cells, supporting the proposal of PCA3 knockdown as a putative therapeutic approach to inhibit PCa growth.

Background: Obesity is associated with higher leukocyte counts, whereas weight loss decreases these counts. It is unknown if different bariatric surgery (BS) types have different effects on leukocytes., Objectives: The aim of the study was to determine predictors of leukocyte and their subset count variation in patients submitted to BS., Setting: Tertiary care university hospital, Porto, Portugal., Methods: This was a retrospective analysis of patients submitted to Roux-en-Y gastric bypass (RYGB), laparoscopic adjustable gastric banding (LAGB), or sleeve gastrectomy (SG). Leukocyte and subset counts were compared between baseline and 1-year postsurgery and between BS types. A multivariate linear regression model was built to study determinants of leukocyte and subset variation., Results: We analyzed 764 patients: 238 submitted to LAGB; 452 to RYGB, and 74 to SG. Mean age was 42 years and 86.6% were women. All BS types were associated with a decrease in leukocyte and neutrophil counts but the variation in body mass index and homeostatic model assessment (HOMA-IR) were the only variables independently associated with a decrease 1-year postsurgery. Monocytes increased in patients submitted to LAGB and decreased in those who underwent RYGB and SG. The BS type was independently associated with monocyte variation. Patients who underwent RYGB and SG had a decrease in monocyte counts of 77/µL and 62/µL, respectively, compared with LAGB., Conclusion: Weight and insulin resistance are the main predictors of leukocyte and neutrophil variation after BS. The specific type of BS is a determinant of monocyte count variation independent of the amount of weight loss or the degree of insulin resistance improvement., (Copyright © 2016 American Society for Bariatric Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: While attending to higher education, multiple changes occur in the lives of young students. These changes make higher education students particularly more exposed and vulnerable to mental health problems, and therefore more likely to present suicidal behaviors., Objectives: The aim of this study was to assess suicidal ideation in higher education students, and its relationship with some psychosocial variables., Methods: Exploratory, quantitative and descriptive study, applied on a sample of 1074 students of a higher education institution in Portugal., Results: Some significant associations with suicidal ideation were found with self-concept factors (self-acceptance, self-effectiveness and impulsivity); stress, anxiety and clinical depression; linkage anxiety and social support activities dimensions; intimacy and total social support., Conclusion: In our sample we found 84 students with potential suicidal risk, and through the result analysis, the biopsychosocial profile of the student at risk for suicidal ideation exhibits the following traits: female, age 18/19, low self-concept, insecure linkage patterns and little involvement in social activities and intimacy relationships. All facts considered, higher education institutions should provide programs that promote mental health and suicide prevention in academic environments. The results of this study also have implications for policy makers, clinical practice, suicide prevention and higher education institutions., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Human lipodystrophies are uncommon disorders, with important clinical consequences, which are often undiagnosed. The Barraquer-Simons syndrome is a form of partial symmetric lipodystrophy of unknown etiology, characterized by the loss of subcutaneous adipose tissue, limited to upper part of the body. Insulin resistance and metabolic complications are less common than with other lipodystrophy subtypes. Patients usually have decreased serum complement-component 3 levels, associated with complement activation by the alternative pathway, which may indicate the presence of renal involvement., Case Presentation: The authors report a case of a 31-year-old woman with progressive loss of subcutaneous fat, limited to the face, neck and thorax. She presented no severe metabolic complications, neither signs of insulin resistance. Laboratory tests revealed mild dyslipidemia, and low serum levels of complement-component 3. Clinical and biochemical characteristics were consistent with the diagnosis of Barraquer-Simons syndrome., Conclusion: The present case illustrates the importance of recognizing the clinical features of this lipodystrophic syndrome, which may present potentially severe consequences and psychological distress. A brief overview is made, addressing the clinical signs of the disease, its course, and how to manage it.

Objective: The aim of this study was to analyze the prevalence of thyroid disorders in patients with a positive biopsy for breast cancer prior to specific antitumor treatment., Methods: The frequency and pattern of thyroid disorders were evaluated in 112 patients with breast cancer (G1) and 125 control patients (G2) by analyzing serum thyroid-stimulating hormone (TSH), anti-thyroid peroxidase antibodies, and anti-thyroglobulin antibodies. In addition, the expression of estrogen receptors, progesterone receptors, and human epidermal growth factor receptor 2 (HER2) was assessed in the breast biopsies by immunohistochemistry., Results: The frequency of thyroid disorders, such as changes in TSH levels and/or the presence of thyroid antibodies, was not different between the 2 groups examined (30.4% in G1 versus 28.0% in G2) (P = .69). However, a family history of thyroid disease was more frequent in patients with breast cancer (50.5% in G1 versus 28.2% in G2) (P = .001). Regarding the clinical stage of breast cancer, there was no difference between women with autoimmune thyroiditis and those without thyroid dysfunction (P = .316). Similarly, there were no differences in hormone receptor (estrogen or progesterone) and HER2 expression between patients who tested positive and those who tested negative for anti-thyroid antibodies (P = .052 and P = .549, respectively)., Conclusion: The data obtained in this study did not reveal a higher frequency of autoimmune thyroid disease in patients with breast cancer compared to controls. A family history of thyroid disease was more common in those with breast cancer.

Permanent neonatal diabetes (PNDM) can result from activating heterozygous mutations in KCNJ11 gene, encoding the Kir6.2 subunit of the pancreatic ATP-sensitive potassium channels (KATP). Sulfonylureas promote KATP closure and stimulate insulin secretion, being an alternative therapy in PNDM, instead of insulin. Male, 20 years old, diagnosed with diabetes at 3 months of age. The genetic study identified a novel heterozygous mutation in exon 1 of the KCNJ11 gene - KCNJ11:c1001G>7 (p.Gly334Val) - and confirmed the diagnosis of PNDM. Therefore it was attempted to switch from insulin therapy to sulfonylurea. During glibenclamide institution C-peptide levels increased, however the suboptimal glycemic control lead us to restart an intensive insulin scheme. This new variant of KCNJ11 mutation had a phenotypic lack of response to sulfonylurea therapy. Age, prior poor metabolic control and functional change of KATP channel induced by this specific mutation may explain the observed unsuccessful switch to sulfonylurea. Interestingly, C-peptide levels raise during glibenclamide administration support some degree of improvement in insulin secretory capacity induced by the treatment. Understanding the response to sulfonylurea is crucial as successful treatment may be life-changing in these patients.

Background: The aim of this study was to assess the relationship between health-related quality of life (HRQoL) and the presence or absence of hypertension and diabetes-related chronic complications in type 2 diabetes, and also the association between HRQoL and the number of chronic complications., Methods: One hundred patients with type 2 diabetes were interviewed. HRQoL was evaluated using the age-adjusted Short-Form 36 dimensions (physical functioning, role-physical, bodily pain, general health, vitality, social functioning, role-emotional, and mental health)., Results: The mean age of the study population was 62.7±8.7 years; 54.0% were male, and 51.0% were receiving only oral hypoglycemic agents. Chronic complications were related to worse HRQoL in different dimensions: peripheral neuropathy and cardiovascular disease (all, except bodily pain), retinopathy (physical functioning, general health, vitality, and mental health), peripheral arterial disease (physical functioning, role-physical, and general health), and nephropathy (general health and vitality). Hypertension was related to worse general health and vitality. An increased number of chronic complications was associated with worse HRQoL in all dimensions of Short-Form 36 except for the bodily pain dimension., Conclusion: The presence and increased number of diabetes-related chronic complications, and the presence of hypertension were related to worse age-adjusted HRQoL. Peripheral neuropathy and cardiovascular disease were more strongly related to age-adjusted HRQoL.

The study seeks to analyze the topic of Chronic Non Communicable Diseases (NCDs) and the role of intersectorial actions in tackling these diseases. It involved a narrative review through searches on Medline, Pubmed and Lilacs databases, and documents from the World Health Organization (WHO), the Pan American Health Organization (PAHO) and the Brazilian government. The link between the theme of intersectoriality and NCD is reviewed in the Global Conferences and Agendas on Health Promotion, and the role of other sectors in relation to effective interventions for NCDs. Some experiences of intersectorial activities for tackling NCDs are presented. The conclusion drawn is that the implementation of intersectorial action in conjunction with sectorial health policies in tackling NCDs has become a political priority, involving coordinated actions, negotiations forums, integrated planning and sustainable funding. It is necessary to seek the active participation of social actors in the advocacy, monitoring and accountability process, coupled with training processes for managers and other professionals, which will give sustainability to the social processes and required changes. Intersectorial partnerships are fundamental.

The objective of this study was to describe situations related to sexual health of adolescents, according to the National Survey of School Health (PeNSE). It is a cross-sectional study performed by the Brazilian Institute of Geography and Statistics (IBGE), in partnership with the Ministry of Health that involved 60,973 students and 1,453 public and private schools. Data analysis points out that 30.5% (95%CI 29.9-31.2) of the adolescents had already had sexual relations sometime in their lives, being more frequent for males (43.7%; 95%CI 42.7-44.7) than females (18.7%; 95%CI 18.0-19.4), especially those who go to public schools (33.1%; 95%CI 32.4-33.9), aged 15 years or older (47.3%; 95%CI 45.7-48.9) and 16 years (63.5%; 95%CI 61,5-65.4). The sexual initiation age was early and 40.1% (95%CI 38.8-41.4) reported having had only one partner in life. The use of condoms in the last sexual relation was high both for protective (75.9%; CI95% 74.8-76.9) and contraceptive methods (74.7%; 95%CI 73.6-75.7). It is necessary to emphasize actions for promoting sexual health towards adolescents in order to minimize vulnerabilities.

Acquired digital fibrokeratomas (ADF) are benign and uncommon lesions consisting of collagenous papules and nodules covered by hyperkeratotic epidermis. These tumors occur mainly on the fingers and toes and infrequently on the palms and soles. They may possibly be triggered by a reaction to a trauma, ADF usually present as small and solitary dome-shaped lesions with a collarete of slightly raised skin at the base. We report a rare case of fibrokeratoma of the heel, presenting as a large and pedunculated nodule.

Introduction: The metabolic syndrome (MS) consists of the conjoint presence of risk factors for coronary. Several study groups have developed diagnostic criteria for MS, standing out those from the "Adult Treatment Panel III" (ATPIII), due to their wide utilization. Recently, the International Diabetes Federation (IDF) developed new diagnostic criteria for MS., Objectives: To evaluate the prevalence of MS in a group of women with severe obesity. To evaluate the prevalence of each one of the diagnostic criteria for MS. To compare the results obtained with the utilization of the ATPIII and the IDF criteria. To relate the results with the patients' age and Body Mass Index (BMI)., Sample and Methods: We evaluated 128 women (mean age = 38 years, sd = 11) with BMI equal to or higher than 35.0 kg/m2 (mean BMI = 46.5 kg/m2, sd = 6.5) on anthropometric measures (weight, height and waist circumference), analytically (fasting serum levels of triglycerides, HDL cholesterol and glucose), being also registered the values of systolic and diastolic blood pressure and age., Results: The prevalence of MS in our sample following the ATPIII and the IDF criteria was, respectively, of 66.4% e 70.3%. The most frequently filled in criteria are those referring to waist circumference (ATPIII: 99.2%; IDF: 100%), blood pressure (77.3%) and HDL cholesterol (69.5%). Older patients and those with higher BMI fill in more criteria, being the diagnostic of MS associated to higher mean age and BMI., Conclusion: The prevalence of MS in women with severe obesity is high, being similar when the ATPIII or the IDF diagnostic criteria are used.