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Christiane Bezerra Rocha Liberato, 1, 2 Natália Bitar da Cunha Olegario, 1, 2 Virginia Oliveira Fernandes, 1–3 Ana Paula Dias Rangel Montenegro, 2 Grayce Ellen da Cruz Paiva Lima, 1, 2 Lívia Aline de Araújo Batista, 1, 2 Lívia Vasconcelos Martins, 1, 2 Jaquellyne Gurgel Penaforte-Saboia, 1, 2 Ivan Lucas Rocha Liberato, 2 Larissa Ferreira Lopes, 2 Catarina Brasil d’Alva, 1, 2 Frederico Luís Braz Furtado, 1, 2 Ricardo Luiz De Medeiros Lima, 4 Lucia Helena Coelho Nóbrega, 4 Josivan Gomes Lima, 4 Renan Magalhães Montenegro Junior 1–3 Brazilian Group for the Study of Inherited and Acquired Lipodystrophies (BRAZLIPO) 1Department of Clinical Medicine, Federal University of Ceará, Fortaleza, Brazil; 2Clinical Research Unit, Walter Cantidio University Hospital, Federal University of Ceará, Fortaleza, Brazil; 3Department of Community Health, Federal University of Ceará, Fortaleza, Brazil; 4Department of Clinical Medicine, Onofre Lopes University Hospital, Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, BrazilCorrespondence: Renan Magalhães Montenegro Junior Rua Professor Costa Mendes, 1608, Fortaleza, Ceará 60416-200, BrazilTel +55 85 3366-8600Fax +55 85 3366-8619Email renanmmjr@gmail.comPurpose: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by the absence of functional adipocytes resulting in ectopic lipid storage, metabolic disorders and early cardiovascular disease. Two-dimensional speckle-tracking (2D-STE) allows the detection of early abnormalities in myocardial function. We aimed to evaluate myocardial deformation in a large sample of CGL patients using 2D-STE.Patients and Methods: A cross-sectional study of 22 patients with CGL and 22 healthy subjects, matched for sex and age, was conducted from 2013 to 2018. All participants had undergone standard conventional echocardiography (ECHO) and 2D-STE. Determination of blood glucose, lipids, insulin, and leptin were performed in all CGL patients.Results: In the CGL group the mean age was 14.6± 10.7 years where 68.2% (n=15) were younger than 18 years old. All the patients had hypoleptinemia, 95.4% (21/22) low HDL-c, 86.4% (19/22) hypertriglyceridemia, 68.2% (15/22) diabetes, 50% (11/22) hepatic steatosis, 41% (9/22) insulin resistance, 41% (9/22) hypercholesterolemia, and 18.2% (4/22) hypertension. ECHO showed that 36.6% (8/22) of CGL patients presented diastolic dysfunction, 31.8% (7/22) left ventricular hypertrophy (LVH), 27.3% (6/22) increased left atrial volume index (LAVI), and 18.2% (4/22) increased left ventricular systolic diameter (LVDS) but normal ejection fraction (EF), whether using 2D-STE, 68.2% (15/22) of CGL patients showed abnormal global longitudinal strain (GLS) (p< 0.01), and in almost LV segments. Positive association between abnormal GLS and A1c (r=0.57, p=0.005), glucose (r=0.5, p=0.018) and basal insulin (r= 0.69, p= 0.024), and negative association with leptin (r = − 0.51, p = 0.005) were found in these patients.Conclusion: The 2D-STE revealed precocious left ventricular systolic dysfunction in a young CGL population with normal systolic function by ECHO. Early exposure to common metabolic abnormalities as insulin resistance, hyperglycemia, and hypoleptinemia must be involved in myocardial damage in these patients.Keywords: Berardinelli-Seip congenital lipodystrophy, global longitudinal strain, cardiac function, early detection

Background: Familial Partial Lipodystrophy (FPLD) is a disease with wide clinical and genetic variation, with seven different subtypes described. Until genetic testing becomes feasible in clinical practice, non-invasive tools are used to evaluate body composition in lipodystrophic patients. This study aimed to analyze the different anthropometric parameters used for screening and diagnosis of FPLD, such as thigh skinfold thickness (TS), Köb index (Köbi), leg fat percentage (LFP), fat mass ratio (FMR) and leg-to-total fat mass ratio in grams (LTR), by dual-energy X-ray absorptiometry, focusing on determining cutoff points for TS and LFP within a Brazilian population., Methods: Thirty-seven patients with FPLD and seventy-four healthy controls matched for body mass index, sex and age were studied. Data were collected through medical record review after signing informed consent. All participants had body fat distribution evaluated by skinfolds and DXA measures. Fasting blood samples were collected to evaluate glycemic and lipid profiles. Genetic studies were carried out on all patients. Two groups were categorized based on genetic testing and/or anthropometric characteristics: FPLD+ (positive genetic test) and FPLD1 (negative genetic testing, but positive clinical/anthropometric criteria for FPLD)., Results: Eighteen (48.6%) patients were classified as FPLD+, and 19 (51.4%) as FPLD1. Unlike what is described in the literature, the LMNA variant in codon 582 was the most common. Among the main diagnostic parameters of FPLD, a statistical difference was observed between the groups for, Köbi, TS, LFP, FMR, and LTR. A cutoff point of 20 mm for TS in FPLD women was found, which is lower than the value classically described in the literature for the diagnosis of FPLD. Additionally, an LFP < 29.6% appears to be a useful tool to aid in the diagnosis of these women., Conclusion: Combining anthropometric measurements to assess body fat distribution can lead to a more accurate diagnosis of FPLD. This study suggests new cutoff points for thigh skinfold and leg fat percentage in women with suspected FPLD in Brazil. Further studies are needed to confirm these findings., (© 2024. The Author(s).)

Introduction: Prader-Willi syndrome (PWS) is a genetic disorder characterized by hypothalamic-pituitary deficiencies including hypogonadism. In girls with PWS, hypogonadism can present early in childhood, leading to genital hypoplasia, delayed puberty, incomplete pubertal development, and infertility. In contrast, girls can present with premature activation of the adrenal axis leading to early pubarche and advanced bone age. We aim to evaluate the progression of puberty and adrenarche signals in girls with PWS., Methodology: A longitudinal retrospective cohort study included girls with PWS followed at a Pediatric Endocrinology Outpatient Clinic in a Tertiary University Hospital in Sao Paulo, Brazil from 2002 to 2022. Data collected via chart review included clinical information on birth history, breast and pubic hair Tanner stages, presence of genital hypoplasia, age at menarche, regularity of menstrual cycles, body mass index (BMI) z-score, final height, age of initiation of estrogen replacement and growth hormone replacement, as well as results for PWS genetic subtype; biochemical investigation (LH, FSH, estradiol, DHEA-S); radiographic bone age and pelvic ultrasound., Results: A total of 69 girls were included in the study and the mean age of puberty onset was 10.2 years in those who started puberty after the age of 8 years. Breast Tanner stage IV was reached by 29.1% girls at a mean age of 14.9 years. Spontaneous menarche was present in 13.8% and only one patient had regular menstrual cycles. Early adrenarche was seen in 40.4% of cases., Conclusion: Our study demonstrated in a large sample that girls with PWS often present with delayed onset of puberty despite frequent premature adrenarche. Based on our results, we suggest an estrogen replacement protocol for girls with PWS to be started at the chronological age or bone age of 12-13 years, taking into consideration the uterus size. Further prospective studies are needed., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Passone, Aragão, Franco, Leite, Gonzalez, Schil, Ybarra, Damiani, Kerkhof, Montenegro Junior and Silva.)

Introduction: Congenital Generalized Lipodystrophy (CGL) is a rare autosomal recessive disease caused by mutations in genes responsible for the formation and development of adipocytes. Bone abnormalities are described. However, there is a scarcity of data., Objective: To describe bone characteristics in a large CGL1 and 2 case series., Methods: Cross-sectional study that assessed bone radiological features of CGL patients of a reference hospital in Fortaleza (CE), Brazil. Patients underwent clinical and bone mineral metabolism evaluation, radiographs of the axial and appendicular skeleton and bone mineral density (BMD) assessment by DEXA (dual energy X-ray absorptiometry)., Results: Nineteen patients were included, fourteen were CGL1 and 5, CGL2. Median age was 20 years (8-42) and 58% were women. Median BMI and percentage of body fat were, respectively, 21 Kg/m² (16-24), and 10.5% (7.6-15). The median leptin concentration was 1 ng/mL (0.1-3.3). Diabetes mellitus and dyslipidemia were present in 79% and 63% of patients, respectively. Median calcium and phosphate were normal in almost all patients (95%). Median parathyroid hormone and 25-OH-vitamin D were 23 pg/mL (7-75) and 28 ng/mL (18-43). Osteolytic lesions, osteosclerosis and pseudo-osteopoikylosis, were present in 74%, 42% and 32% of patients, respectively. Lytic lesions were found predominantly in the extremities of long bones, bilaterally and symmetrically, spine was spared. Osteosclerosis was present in axial and appendicular skeleton. Pseudo-osteopoikilosis was found symmetrically in epiphyses of femur and humerus, in addition to the pelvis. BMD Z-score greater than +2.5 SD was observed in 13 patients (68.4%). BMD was higher in CGL1 compared to CGL2 in lumbar spine and total body in adults. No associations were found between high BMD and HOMA-IR (p=0.686), DM (p=0.750), osteosclerosis (p=0.127) or pseudo-osteopoikilosis (p=0.342), and, between pain and bone lesions. Fractures were found in 3 patients., Conclusion: Bone manifestations are prevalent, heterogeneous, and silent in CGL1 and CGL2. Osteolytic lesions are the most common, followed by osteosclerosis and pseudo-osteopoikilosis. Bone mass is high in most cases. There was no pain complaint related to bone lesions. Thus, systematic assessment of bone manifestations in CGL is essential. Studies are needed to better understand its pathogenesis and clinical consequences., Competing Interests: RMMJ has received grant support and has served as an advisor and/or a consultant to Amryt Pharma, PTC Therapeutics and NovoNordisk. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Freire, Brasil d’Alva, Madeira, Lima, Fernandes, Aguiar, Portella, Galvão Ozório, Ponte, Montenegro and Montenegro Junior.)

Background: Graft loss increases the risk of patient death after simultaneous pancreas-kidney (SPK) transplantation. The relative risk of each graft failure is complex due to the influence of several competing events., Methods: This retrospective, single-center study compared 4-year patient survival according to the graft status using Kaplan-Meier (KM) and Competing Risk Analysis (CRA). Patient survival was also assessed according to five eras (Era 1: 2001-2003; Era 2: 2004-2006; Era 3: 2007-2009; Era 4: 2010-2012; Era 5: 2012-2015)., Results: Between 2000 and 2015, 432 SPK transplants were performed. Using KM, patient survival was 86.5% for patients without graft loss (n = 333), 93.4% for patients with pancreas graft loss (n = 46), 43.7% for patients with kidney graft loss (n = 16), and 25.4% for patients with pancreas and kidney graft loss (n = 37). Patient survival was underestimated using KM versus CRA methods in patients with pancreas and kidney graft losses (25.4% vs. 36.2%), respectively. Induction with lymphocyte depleting antibodies was associated with 81% reduced risk (HR.19, 95% CI.38-.98, p = .0048), while delayed kidney function (HR 2.94, 95% CI 1.09-7.95, p = .033) and surgical complications (HR 2.94, 95% CI 1.22-7.08, p = .016) were associated with higher risk of death. Four-year patient survival increased from Era 1 to Era 5 (79% vs. 87.9%, p = .047)., Conclusion: In this cohort of patients, kidney graft loss, with or without pancreas graft loss, was associated with higher mortality after SPK transplantation. Compared to CRA, the KM model underestimated survival only among patients with pancreas and kidney graft losses. Patient survival increased over time., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Cryptococcosis in HIV-negative patients can be an opportunistic or endemic disease. There are no published studies on the use of the finger-prick whole blood (point-of-care) cryptococcal antigen lateral flow assay (CrAg LFA) for diagnosing cryptococcosis in HIV-negative patients. We conducted a case series study of HIV-negative patients with cryptococcosis in two centers in São Paulo, Brazil. The objectives were to identify the sensitivity of a finger-prick whole blood CrAg LFA and to describe the main characteristics of this population. We identified 30 HIV-negative patients with cryptococcosis [19 (63%), male; median age, 47 years]. Ten (33%) patients were immunosuppressed, ten (33%) had other comorbidities, and ten (33%) were apparently immunocompetent and without comorbidities. The distribution of the sites of cryptococcosis was as follows: the central nervous system, 90% (n = 27); pulmonary, 43% (n = 13); and other extrapulmonary sites, 40% (n = 12). The sensitivity of the finger-prick whole blood CrAg LFA for the diagnosis of cryptococcosis was 97% (29/30). Among 26 participants with cryptococcal meningitis, the sensitivity of testing cerebrospinal fluid was as follows: CrAg latex agglutination, 77% (20/26); CrAg LFA, 96% (25/26); and culture, 81% (21/26). Culture speciation identified Cryptococcus gattii in 16 (62%) cases, and all had a positive finger-prick whole blood CrAg LFA. This test presented high sensitivity to the diagnosis of cryptococcosis in HIV-negative patients, including those caused by C. gattii.

Introduction: Scaphoid is the most fractured carpal bone, with a 5-10% nonunion rate. Treatment challenges include choosing the implant and graft that best corrects humpback deformity and carpal malalignment with higher chances of bony healing., Objective: Compare cortico-cancellous and cancellous grafts between two groups of patients treating scaphoid nonunion with locking plates using autologous bone graft and evaluate bone healing rates and radiographic, tomographic, and functional parameters before and after surgery., Methods: Non-randomized prospective study including 20 cases of scaphoid nonunion. Groups were divided into Group A (ten patients treated with cortico-cancellous iliac graft) and Group B (ten patients treated with cancellous iliac graft). Patients underwent pre- and postoperative radiographs, computed tomography, and functional evaluation., Results: In postoperative analysis, Group A showed a statistically significant difference in intrascaphoid angle (p = 0.002) and scapholunate angle (p = 0.011) correction when comparing the pre- and postoperative periods. Group B showed a statistically significant difference in intrascaphoid angle (p = 0.002) and scapholunate angle correction (p = 0.0018), grip strength (p = 0.002), and tip pinch strength (p = 0.001) when comparing the pre- and postoperative periods. By comparing both groups, Group B showed a statistically significant difference in intrascaphoid angle correction (p = 0.002), grip strength (p = 0.002), tip pinch strength (p = 0.002), and radial deviation (p = 0.0003). There was no statistical difference when comparing bony healing between groups., Conclusion: Scaphoid nonunion treatment with a locking plate was effective, showing a high bony healing rate and improved carpal alignment in imaging tests for both graft types. However, results for intrascaphoid angle correction, grip strength, tip pinch strength, and radial deviation were better in Group B., Level of Evidence: IV, case series., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background : Noninvasive ventilation (NIV) provides positive pressure through different interfaces. A multifunctional full-face mask prototype was developed to provide NIV from three sources: ICU ventilators, portable ventilators, and high-flow medical gas pipeline systems. This study aimed to evaluate the usability of this prototype mask. Methods : This was a quantitative experimental study, conducted in two phases: the development of a full-face mask prototype NIV interface, and the evaluation of its usability by health professionals (evaluators) using a heuristic approach. The Wolf Mask prototype is a multifunctional full-face mask that makes it possible to deliver positive pressure from three different sources: microprocessor-controlled ICU ventilators, portable ventilators with single-limb circuits, and high-flow medical gas. The evaluation was conducted in three stages: presentation of the prototype to the evaluators; skills testing via simulation in a clinical environment; and a review of skills. Results : The prototype was developed by a multidisciplinary team and patented in Brazil. The evaluators were 10 health professionals specializing in NIV. Seven skills related to handling the prototype were evaluated. Three of the ten evaluators called for (non-urgent) changes to improve recognition of the components of the prototype. Only one evaluator called for (non-urgent) changes to improve recognition of the pieces, assembly, and checking the mask. Conclusions : The newly developed multifunctional full-face mask prototype demonstrated excellent usability for providing noninvasive ventilation from multiple sources. Minor modifications may further improve the design.

We aimed to identify HLA-DRB1 , -DQA1 , and -DQB1 alleles/haplotypes associated with European, African, or Native American genomic ancestry (GA) in admixed Brazilian patients with type 1 diabetes (T1D). This exploratory nationwide study enrolled 1599 participants. GA percentage was inferred using a panel of 46 ancestry informative marker-insertion/deletion. Receiver operating characteristic curve analysis (ROC) was applied to identify HLA class II alleles related to European, African, or Native American GA, and showed significant ( p < 0.05) accuracy for identifying HLA risk alleles related to European GA: for DRB1*03:01 , the area under the curve was (AUC) 0.533; for DRB1*04:01 AUC = 0.558, for DRB1*04:02 AUC = 0.545. A better accuracy for identifying African GA was observed for the risk allele DRB1*09:01 AUC = 0.679 and for the protective alleles DRB1*03:02 AUC = 0.649, DRB1*11:02 AUC = 0.636, and DRB1*15:03 AUC = 0.690. Higher percentage of European GA was observed in patients with risk haplotypes ( p < 0.05). African GA percentage was higher in patients with protective haplotypes ( p < 0.05). Risk alleles and haplotypes were related to European GA and protective alleles/haplotypes to African GA. Future studies with other ancestry markers are warranted to fill the gap in knowledge regarding the genetic origin of T1D in highly admixed populations such as that found in Brazil.

Objective: To evaluate whether ultrasound abdominal fat measurements in the first and second trimesters can predict adverse gestational outcomes, particularly gestational diabetes mellitus (GDM), and identify early patients at higher risk for complications., Methods: A prospective cohort study of 126 pregnant women at 11-14 and 20-24 weeks of gestation with normal fasting glucose levels during early pregnancy. From 126 participants with complete data, 13.5% were diagnosed with GDM, based on the cutoffs established for the peripherical blood glucose. Subcutaneous, visceral, and maximum preperitoneal abdominal fat were measured using ultrasound techniques. GDM status was determined by oral glucose tolerance test (OGTT) with 75 g glucose overload, and the following values were considered abnormal: fasting glucose ≥92 mg/dl and/or 1 h after overload ≥180 mg/dl and/or 2 h after overload ≥153 mg/dl. The receiver operator characteristic (ROC) curve was used to determine the optimal threshold to predict GDM., Results: Maximum preperitoneal fat measurement was predictive of GDM, and subcutaneous and visceral abdominal fat measurements did not show significant differences in the prediction of GDM. According to the ROC curve, a threshold of 45.25 mm of preperitoneal fat was identified as the optimal cutoff point, with 87% sensitivity and 41% specificity to predict GDM. The raw and adjusted odds ratios for age and pre-pregnancy body mass index were 0.730 (95% confidence interval [CI], 0.561-0.900) and 0.777 (95% CI, 0.623-0.931), respectively., Conclusion: The use of a 45.25 mm threshold for maximum preperitoneal fat, measured by ultrasound to predict the risk of GDM, appears to be a feasible, inexpensive, and practical alternative to incorporate into clinical practice during the first trimester of pregnancy.

Background: Previous studies suggest intestinal dysbiosis is associated with metabolic diseases. However, the causal relationship between them is not fully elucidated. Gut microbiota evaluation of patients with congenital generalized lipodystrophy (CGL), a disease characterized by the absence of subcutaneous adipose tissue, insulin resistance, and diabetes since the first years of life, could provide insights into these relationships., Methods: A cross-sectional study was conducted with patients with CGL (n = 17) and healthy individuals (n = 17). The gut microbiome study was performed by sequencing the 16S rRNA gene through High-Throughput Sequencing (BiomeHub Biotechnologies, Brazil)., Results: The median age was 20.0 years old, and 64.7% were female. There was no difference between groups in pubertal stage, BMI, ethnicity, origin (rural or urban), delivery, breastfeeding, caloric intake, macronutrient, or fiber consumption. Lipodystrophic patients presented a lower alpha diversity (Richness index: 54.0 versus 67.5; p = 0.008). No differences were observed in the diversity parameters when analyzing the presence of diabetes, its complications, or the CGL subtype., Conclusion: In this study, we demonstrate for the first time a reduced gut microbiota diversity in individuals with CGL. Dysbiosis was present despite dietary treatment and was also observed in young patients. Our findings allow us to speculate that the loss of intestinal microbiota diversity may be due to metabolic abnormalities present since the first years of life in CGL. Longitudinal studies are needed to confirm these findings, clarifying the possible causal link between dysbiosis and insulin resistance in humans., (© 2022. The Author(s).)

Objective: Studies of subcutaneous and visceral abdominal fat thickness evaluated by ultrasound as a predictor of gestational diabetes mellitus (GDM) have been published, but the best technique and standardization are unknown. To identify, critically evaluate, and analyze studies using subcutaneous and visceral abdominal fat as a model for predicting GDM in the first and second trimesters of pregnancy and evaluate their methodological quality., Methods: PubMed, Scopus, and Web of Science databases were searched from May to July 2019. We included studies of any sample size performed for any duration and in any configuration. Model development and validation studies were eligible for inclusion. Two authors independently performed the eligibility assessment of the studies by reviewing the titles and abstracts. Data on study design, gestational age, diagnostic criteria for GDM, device, ultrasound fat measurement technique, and cutoff point for GDM prediction were extracted., Results: The electronic search resulted in 1331 articles, of which 14 were eligible for systematic review. Different criteria for diagnosing GDM and fat measurement techniques were used. The cutoff point for subcutaneous, visceral, and total abdominal fat for predicting GDM in the first and second trimesters varied between the studies., Conclusion: No study validated the model for predicting GDM using subcutaneous and visceral abdominal fat measurements. External validation studies are recommended to improve the generalization of this GDM predictor in clinical practice.

The purpose of this paper was to conduct a systematic review and meta-analysis of studies that compared muscle hypertrophy and strength gains between resistance training protocols employing very low (VLL < 30% of 1-repetition maximum (RM) or >35RM), low (LL30%-59% of 1RM, or 16-35RM), moderate (ML60%-79% of 1RM, or 8-15RM), and high (HL ≥ 80% of 1RM, or ≤7RM) loads with matched volume loads (sets × repetitions × weight). A pooled analysis of the standardized mean difference for 1RM strength outcomes across the studies showed a benefit favoring HL vs. LL and vs. ML and favoring ML vs. LL. The LL and VLL results showed little difference. A pooled analysis of the standardized mean difference for hypertrophy outcomes across all studies showed no differences between training loads. Our findings indicate that when the volume load is equal between conditions, the highest loads induce superior dynamic strength gains. Alternatively, hypertrophic adaptations were similar irrespective of the load magnitude. Novelty: Training with higher loads elicits greater gains in 1RM muscle strength when compared to lower loads, even when the volume load is equal between conditions. Muscle hypertrophy is similar irrespective of the magnitude of the load, even when the volume load is equal between conditions.

Introduction: The radiographic and surgical findings, and treatment of radiocarpal fracture dislocations, were analyzed retrospectively in 40 patients., Materials and Methods: All patients were classified according to Dumontier´s radiological classification and compared with the surgical findings. Based on this analysis, a new classification and treatment are proposed., Results: From 1995 to 2018, 40 patients with radiocarpal fracture dislocation underwent surgery. Thirty-six were males and four were females. The mean age was twenty-four years (range: 18-45). Three dislocations were volar dislocations and 37 were displaced dorsally. Initially, 8 (20%) patients were classified as group I, 29 (72.5%) as group II, and 3 (7.5%) remained unclassified. The main variations occurred in group II. Seven fractures were stable after radial styloid fixation and 6 remained unstable. Sixteen fractures presented articular fragments or an interposed capsule, which prevented anatomical reduction using conservative maneuvers., Conclusion: Based in our intraoperative observations and surgical results, we believe that a more detailed classification should be adopted. Level of Evidence IV; Therapeutic Studies; Case Series., Competing Interests: All authors declare no potential conflict of interest related to this article.

Brazil has been severely affected by the COVID-19 pandemic. The high numbers of confirmed cases and deaths have continued unabated since the first reported case, with no flattening or downward turn in the curve. In this context, healthcare workers have been exposed uninterruptedly to stress factors throughout a year of the pandemic. The study´s aim was to identify and analyze healthcare workers´ perceptions of their feelings and concerns that have surfaced in responding to the pandemic., Method: This was a cross-sectional online qualitative survey study of 554 healthcare personnel working in the state of Rio de Janeiro during the COVID-19 pandemic. Recruitment occurred from July 20 to September 30, 2020, using an online survey, preceded byfree informed consent term. Data were analyzed with the Iramuteq software., Results: Through a dendrogram, the words with the highest chi-square were highlighted and grouped into four classes: healthcare workers´ fear of falling ill to COVID-19 and infecting their family members; work/labor issues; feelings of powerlessness and need for public policies for government action; and fatigue and burnout in the pandemic. Each word class was also illustrated by a similarity tree., Conclusion: The study revealed healthcare workers´ exacerbated fear of infection and transmission of COVID-19 to their family members, besides financial losses and feelings of powerlessness and abandonment., Competing Interests: The authors have declared that no competing interests exist.

Although used by resistance-trained individuals, it is unknown if increasing muscle strength prior to hypertrophy training leads to greater muscle growth and strength gains. We investigated muscle thickness and maximum strength in 26 resistance-trained men who were randomly assigned to either: STHT, consisted in a 3-week strength-oriented training period (4x1-3 repetition maximum [RM]) prior to a 5-week hypertrophy-oriented period (4x8-12RM), or HT, which comprised an 8-week hypertrophy-oriented training period. Vastus lateralis muscle thickness, and back squat and leg-press 1-RM were assessed at pre, third week, and after 8 weeks of training. When pre-to-post changes are compared, STHT induced greater muscle growth (p = 0.049; 95%CI = 0.15-3.2%; d = 0.81) and strength gains in the back squat (p = 0.015; 95%CI = 1.5-13%; d = 1.05) and leg-press 45° (p = 0.044; 95%CI = 0.16-9.9%; d = 0.79) compared to HT. Our results support the use of a period to increase muscle strength prior to an HT to increase muscle thickness and maximum strength in resistance-trained men.

Congenital Generalized Lipodystrophy (CGL) is a rare syndrome characterized by the almost total absence of subcutaneous adipose tissue due to the inability of storing lipid in adipocytes. Patients present generalized lack of subcutaneous fat and normal to low weight. They evolve with severe metabolic disorders, non-alcoholic fatty liver disease, early cardiac abnormalities, and infectious complications. Although low body weight is a known risk factor for osteoporosis, it has been reported that type 1 and 2 CGL have a tendency of high bone mineral density (BMD). In this review, we discuss the role of bone marrow tissue, adipokines, and insulin resistance in the setting of the normal to high BMD of CGL patients. Data bases from Pubmed and LILACS were searched, and 113 articles published until 10 April 2021 were obtained. Of these, 76 were excluded for not covering the review topic. A manual search for additional literature was performed using the bibliographies of the studies located. The elucidation of the mechanisms responsible for the increase in BMD in this unique model of insulin resistance may contribute to the understanding of the interrelationships between bone, muscle, and adipose tissue in a pathophysiological and therapeutic perspective.

Summary: Background. Patients with chronic urticaria (CU) often report an impaired quality of life (QoL). Although a positive effect of addressing spirituality in health care has been proved in several chronic diseases, its potential role in CU has received no attention. Objective. We aim to evaluate spirituality and QoL in CU subjects. Methods. In a single-centre observational study, 100 CU subjects were investigated using Functional Assessment of Chronic Illness Therapy-Spiritual Well-Being (FACIT-Sp-12) scale, Chronic Urticaria Quality of life Questionnaire (CU-Q2oL) and Urticaria Control Test (UCT). Results. Of 100 subjects, 82 were female and 18 were male. It was observed that subjects with poorly controlled CU presented FACIT Sp-12 meaning/peace (p = 0.004) significantly lower, and CU-Q2oL (p less than 0.0001) significantly higher (worst QoL) than subjects with controlled CU. There was no difference in the FACIT Sp-12 faith (p = 0.43) between groups. There was moderate direct correlation between FACIT Sp-12 faith and FACIT Sp-12 meaning/peace (r = 0.483; p less than 0.0001; n = 100). There was a significant strong inverse correlation between the CU-Q2oL and the UCT (r = - 0.762; p less than 0.0001; n = 100). No correlation was found between the FACIT Sp-12 faith and CU-Q2oL, neither with UCT. Conclusions. No study has ever investigated the role of spirituality in managing patients with urticaria. Our findings support the impact of poorly controlled urticaria in spiritual well-being and QoL. Therefore, clinicians should pay more attention to spirituality among CU patients. We suggest that urticaria guidelines should include specific recommendations on spirituality assessment.

Background: A new strain of human coronavirus (HCoV) spread rapidly around the world. Diabetes and obesity are associated with a worse prognosis in these patients. Congenital Generalized Lipodystrophy (CGL) patients generally have poorly controlled diabetes and require extremely high doses of insulin. There is no documentation in the literature of cases of COVID in CGL patients. Thus, we aimed to evaluate the prevalence of SARS-CoV-2 infection in CGL patients, and the association of their clinical and metabolic characteristics and outcomes., Methods: This is a cross-sectional study carried out between July and October 2020. Clinical data collected were respiratory or other flu-like symptoms, need of hospitalization in the last three months, CGL comorbidities, and medications in use. Cholesterol, triglycerides, glycohemoglobin A1c levels, anti-SARS-CoV-2 antibodies and nasopharyngeal swab for RT-qPCR were also obtained in all CGL patients. Mann-Whitney U test was used to analyze the characteristics of the participants, verifying the non-adherence of the data to the Gaussian distribution. In investigating the association between categorical variables, we used Pearson's chi-square test and Fisher's exact test. A significance level of 5% was adopted., Results: Twenty-two CGL patients were assessed. Eight subjects (36.4%) had reactive anti-SARS-CoV-2 antibodies. Only one of these, also presented detectable RT-qPCR. Five individuals (62.5%) were women, median age of 13.5 years (1 to 37). Symptoms like fever, malaise, nausea, diarrhea and chest pain were present, and all asymptomatic patients were children. All subjects had inadequate metabolic control, with no difference between groups. Among positive individuals there was no difference between those with AGPAT2 (75%) and BSCL2 gene mutations (25%) (p > 0.05). No patient needed hospitalization or died., Conclusions: We described a high prevalence of SARS-CoV-2 infection in CGL patients with a good outcome in all of them. These findings suggest that at least young CGL patients infected by SARS-COV-2 are not at higher risk of poor outcome, despite known severe metabolic comorbidities.

Purpose: In upper and chronic brachial plexus injuries for which neurological surgery is not a good treatment option, one possibility for gaining elbow flexion is free functional muscle transfer. The primary aim of our study was to evaluate the elbow flexion gain achieved by free gracilis muscle transfer with partial ulnar nerve neurotization., Methods: This surgery was performed in 21 patients with upper and chronic (> 12 months) brachial plexus injuries. The level of injury, patient age, the time between trauma and surgery, the affected side, and the aetiology of the lesion were recorded. The primary outcome evaluated was elbow flexion muscle strength, which was measured using the British Medical Research Council (BMRC) scale, in patients with a minimum follow-up period of 12 months. The criterion used to classify elbow flexion as good was a grade of M4 or higher., Results: An M4 elbow flexion strength gain was observed in 61.9% of the patients. A gain of M2 or higher was observed in 95.2% of the patients. The mean range of active motion was 77° (range 10 minimum-110 maximum)., Conclusion: In patients with upper and chronic brachial plexus injuries, free gracilis muscle transfer with ulnar nerve neurotization yields a satisfactory gain in elbow flexion strength and is therefore a good treatment option.

Type 1 diabetes mellitus (T1DM) results from the immune cell-mediated destruction of functional pancreatic β-cells. In the presymptomatic period, T1DM is characterized by the presence of two or more autoantibodies against the islet cells in patients without glycemic decompensation. Therapeutic strategies that can modify the autoimmune process could slow the progression of T1DM. Dipeptidyl peptidase-4 (DPP-4) or CD26, a multifunctional serine protease with a dual function (regulatory protease and binding protein), can modulate inflammation and immune cell-mediated β-cell destruction. CD26 is involved in T-cell co-stimulation, migration, memory development, thymic maturation, and emigration patterns. DPP-4 degrades the peptide hormones GLP-1 and GIP. In addition to regulating glucose metabolism, DPP-4 exerts anti-apoptotic, regenerative, and proliferative effects to promote β-cell mass expansion. GLP-1 receptor signaling may regulate murine lymphocyte proliferation and maintenance of peripheral regulatory T-cells. In patients with T1DM, the serum DPP-4 activity is upregulated. Several studies have suggested that the upregulated DPP-4 activity is correlated with T1DM pathophysiology. DPP-4, which is preferentially expressed on the Th1 surface, can promote the polarization of Th1 immunity, a prerequisite for T1DM development. CD26 inhibition can suppress T-cell proliferation and Th1 cytokine production and stimulate tumor growth factor beta-1 (TGF-β1) secretion, which plays an important role in the regulation of autoimmunity in T1DM. Studies on humans or animal models of T1DM have suggested that DPP-4 inhibitors can improve β-cell function and attenuate autoimmunity in addition to decreasing insulin dependence. This review summarizes the emerging roles of DPP-4 inhibitors in potentially delaying the progression of T1DM., Competing Interests: The authors report no conflicts of interest for this work., (© 2021 Gurgel Penaforte-Saboia et al.)

A literature review on the clinical, laboratory, and treatment features of type B insulin resistance syndrome (TBIRS). Data from PubMed, the Virtual Health Library and Cochrane database were selected and analyzed using the REDCap application and R statistical program. From 182 papers, 65 were selected, which assessed 119 clinical cases, 76.5% in females and 42.9% in African-Americans, with an average age of 44 years. A common feature of TBIRS is co-occurrence of autoimmune diseases, such as systemic lupus erythematosus (most frequently reported). Hyperglycemia of difficult control was the mostly reported condition. Tests for anti-insulin receptor antibodies were positive in 44.2% of the cases. Disease management comprised fractional diet, insulin therapy (maximum dose given was 57 600 IU/day), plasmapheresis and immunosuppression with several classes of drugs, mainly glucocorticoids. Remission occurred in 69.7% of cases, in 30.3% of these spontaneously. The mortality rate was 15.38%. There was an inverse relationship between anti-insulin antibodies and remission (p = 0.033); and a positive correlation between combined immunosuppressive therapy and remission (p = 0.002). Relapse occurred in 7.6% of the cases. This rare syndrome has difficult-to-control diabetes, even with high doses of insulin, and it is usually associated with autoimmune diseases. Therapeutic advances using immunomodulatory therapy have led to significant improvements in the rate of remission.

Objective This study aimed to determine the thyroid-stimulating hormone (TSH) reference interval (RI) and to assess the influence of the use of thyroid ultrasonography (TUS) on reference individual selection from a healthy adult population in Fortaleza, Brazil. Subjects and methods This cross-sectional study recruited patients (N = 272; age = 18-50 years) with normal thyroid function (NTF) and placed them in three groups according to their test results: NTF (n = 272; all participants), TUS (n = 170; participants who underwent thyroid US), RI (n = 124; reference individuals with normal TSH levels). TSH, FT4, TT3, TgAb, and TPOAb concentrations were determined by electrochemiluminescence assay. TUS was performed using a 7-12 MHz multifrequency linear transducer by two radiologists. The 2.5th and 97.5th percentiles of the distribution curve corresponded to lower and upper TSH RI levels, respectively. Results The mean TSH level was 1.74 ± 0.96 mIU/L, and TSH range was 0.56-4.45 mIU/L. There was no difference in the TSH concentrations between men and women nor between the groups. TUS did not appear to be an essential tool for the reference group selection. Conclusion The upper limit of TSH was comparable to the reference interval provided by the assay manufacturer (4.45 vs. 4.20 mIU/L) but the lower limit was not (0.56 vs. 0.27 mIU/L). This finding may have a clinical impact since these values may lead to the misdiagnosis of euthyroid patients with subclinical hyperthyroidism.

Anastrepha fraterculus (Wiedemann, 1830) is the main pest of fruit in southern Brazil. The use of toxic baits is one of the alternatives for its management. In this study, the toxic baits Anamed + malathion (10,000 mg/liter), Flyral 1.25% + malathion (2,000 mg/liter), and Gelsura (alpha-cypermethrin, 2,000 and 4,000 mg/liter) were highly toxic to the adults of A. fraterculus (lethal time [LT50] < 7 h). In contrast, Success 0.02 CB had an LT50 of 48.4 h. In the absence of rain, all the formulations had residual effects (>90% mortality) on A. fraterculus adults up to 21 d after treatment (DAT). In the presence of 5, 25, and 50 mm of rainfall, there was a significant reduction in the residual effect over time. However, with up to 50 mm of rain, Anamed + malathion and Gelsura 2,000 and 4,000 mg/liter caused between 43.0 and 79.0% of mortality. In the field, during two consecutive seasons (2015/2016 and 2016/2017), applications of Gelsura 2,000 mg/liter (four applications/season) caused population suppression of the pest throughout the apple fruiting period. However, in the 2016/2017 season, in the area using Gelsura, a higher percentage (≈12%) of apple fruits damaged by A. fraterculus females was observed when compared with the area with insecticide application (damage <3%). The toxic bait Gelsura (2,000 and 4.000 mg/liter) was shown to be promising for use in the management of A. fraterculus, with results similar to those with the application of synthetic insecticides., (© The Author(s) 2020. Published by Oxford University Press on behalf of Entomological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by the near-total loss of subcutaneous adipose tissue soon after birth, resulting in ectopic fat deposition and severe metabolic disturbances. Most cases are caused by AGPAT2 or BSCL2 gene mutations. We aimed to report two unrelated CGL patients with a novel frameshift mutation in AGPAT2 (p.Leu124Serfs*26)., Methods: Clinical features and laboratory were obtained by medical interview and medical records review. DNA was extracted, amplified and sequenced. Mutation Taster was used to estimate the potential biological impact of the AGPAT2 mutations on the protein function., Results: Patient 1: a 30-year-old woman with lipodystrophy phenotype at birth and diagnosis of diabetes at age 13 presented with severe hypertriglyceridemia and pancreatitis at age 17, hypertension and albuminuria at age 18, proliferative diabetic retinopathy with visual loss at age 25, and an acute myocardial infarction due to multivessel coronary disease during a hospitalization for forefoot amputation at age 29. At this time, she required hemodialysis due to end-stage renal disease. Patient 2: a 12-year-old girl with lipodystrophy phenotype and hypertriglyceridemia detected in the first year of life and abnormalities in the global longitudinal strain, evaluated by speckle-tracking echocardiography last year. Molecular analysis identified a c.369&#95;372delGCTC (p.Leu124Serfs*26) AGPAT2 mutation in both unrelated patients, a compound heterozygous mutation in Patient 1, and homozygous mutation in Patient 2., Conclusion: We describe two unrelated patients with type 1 CGL due to Leu124Serfs*26, a novel AGPAT2 frameshift mutation, presenting as early cardiovascular disease. These findings suggest an association between Leu124Serfs*26 and a more aggressive phenotype., Competing Interests: Competing interestsThe authors declare that they have no competing interests., (© The Author(s) 2020.)

Objective: To systematically review current literature to determine the effectiveness of the ischemic compression (IC) technique on pain and function in individuals with shoulder pain., Methods: This review was conducted according to recommendations of Preferred Reporting Items for Systematic Reviews and Meta-Analyses and the Cochrane Collaboration for Systematic Reviews; a search was performed in the electronic databases PubMed, Cumulative Index to Nursing and Allied Health Literature, SPORTDiscus, Physiotherapy Evidence Database, and Web of Science. Randomized clinical trials and quasi-randomized clinical trials were included, and the methodological quality was evaluated through the Physiotherapy Evidence Database scale., Results: The search found 572 studies; of these, 71 were selected by title and, subsequently, 29 were selected through abstract analyses. After critical analyses, 5 studies were included. The methodological quality ranged from 4 (reasonable) to 9 (excellent) points. Pain was assessed by all studies using the visual analog scale, Global Perceived Effect scale, Numerical Rating Scale, pressure pain threshold, or Perceived Amelioration Numerical Scale. Function was evaluated by 3 studies through the Shoulder Pain and Disability Index; Neck Disability Index; American Shoulder and Elbow Surgeons Standardized Shoulder Assessment; and Disabilities of the Arm, Shoulder, and Hand questionnaires. The studies showed that the IC technique produces immediate and short-term positive effects for pain, and positive short-term effects for shoulder function in individuals with shoulder pain., Conclusion: The IC technique seems to be beneficial for pain and shoulder function. However, caution is needed when considering this evidence owing to the limited quality of some studies, the few articles found, and the lack of standardization of the application parameters of the technique to facilitate its reproducibility., (Copyright © 2020. Published by Elsevier Inc.)

Diachasmimorpha longicaudata (Ashmead, 1905) (Hymenoptera: Braconidae) is considered one of the main biological control agents of Ceratitis capitata (Wiedemann) (Diptera: Tephritidae). However, the application of toxic baits for the management of C. capitata might exert side effects on the parasitoid. The objective of this study was to evaluate the side effects of toxic bait formulations on D. longicaudata. The food attractants Anamed, 3% Biofruit, 1.5% CeraTrap, 1.25% Flyral, 3% Isca Samaritá, 3% Isca Samaritá Tradicional, and 7% sugarcane molasses mixed with an organophosphate insecticide [malathion, 2.0 grams of active ingredient (g a.i.) L -1 ] and the commercial formulation Gelsura (2.0 and 4.0 g a.i. L -1 alpha-cypermethrin) showed high toxicity to D. longicaudata adults (>90% mortality) after 96 h and were thus classified as harmful (Class 4). Similarly, 3% Isca Samaritá Tradicional and 7% sugarcane molasses in formulations with the insecticides spinosad and spinetoram (0.096 g a.i. L -1 or kg) were moderately harmful (Class 3). In contrast, the food attractants Anamed, 3% Biofruit, 1.5% CeraTrap, 1.25% Flyral, and 3% Isca Samaritá Tradicional in combination with spinosad and spinetoram and the formulation Success 0.02CB (0.096 g a.i. L -1 spinosad) were classified as harmless (<10% mortality up to 96 h, Class 1). Additionally, these formulations did not reduce the parasitism and emergence rate of the F 1 generation of D. longicaudata in C. capitata larvae. Formulations of toxic baits based on spinosyn are suitable for the management of C. capitata together with the parasitoid D. longicaudata.

Background: Handheld dynamometers (HHD) provide quick and low-cost assessments of muscle strength and their use has been increasing in clinical practice. There is no available data related to the validity of HHD for this measurement., Objective: To verify the concurrent validity of scapular protraction measurements using an HHD., Methods: Individuals with traumatic anterior glenohumeral instability were allocated in Instability Group (n=20), healthy swimmers were allocated in Athletes Group (n=19) and healthy subjects were allocated in Sedentary Group (n=21). Concurrent validity was verified by the Pearson correlation test between HHD and isokinetic measurements. The agreement between instruments was verified by Bland-Altman plots, for each of the two HHD positions., Results: A moderate correlation was observed between seated (r=0.59) and lying supine HHD (r=0.54) and isokinetic dynamometer measurements for the all groups. Separated group analysis exhibited a strong correlation between seated HHD and isokinetic dynamometer measurements in the Instability Group (r=0.80), Sedentary Group (r=0.79) and Athletes Group (r=0.76). The Bland-Altman plot showed greater agreement in the seated position than the lying supine position when comparing measurements with the HHD and isokinetic in both the general sample and separated groups., Conclusion: The HHD may be considered a valid tool for assessing scapular protraction muscle strength among healthy athletes, non-athletes and subjects with shoulder instability. We recommend to assess subjects in the seated position and to be aware that the HHD tends to overestimate the peak force, compared with the gold-standard isokinetic dynamometer., (Copyright © 2018 Associação Brasileira de Pesquisa e Pós-Graduação em Fisioterapia. Publicado por Elsevier Editora Ltda. All rights reserved.)

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.

Several functional hybrid materials have been reported as immobilized porphyrin derivatives in various organic and inorganic host materials (polymers, mineral clays, silica, etc.), with potential applications in various fields, such as photochemistry, electrochemistry and heterogeneous catalysis. Layered double hydroxides (LDHs), commonly known as hydrotalcite-like materials, have also been analyzed for use as supports for metallocomplexes. Recently, nanocomposite materials with a core-shell structure produced by combining two kinds of nanometer-size materials have received considerable attention, since the use of these materials is a promising strategy to prevent the aggregation and self-oxidation of molecules, reducing the catalytic activity. In this study, monodispersed hierarchical layered double hydroxides on silica spheres (LDH@SiO 2 ) with core-shell structures were developed for metalloporphyrin immobilization and the materials were used as the oxidant catalysts of different substrates.

Background: Acute kidney injury (AKI) is a common complication in the immediate postoperative period of patients undergoing liver transplantation., Objective: The aim of this study was to evaluate preoperative risk factors for AKI after liver transplantation., Methods: A cross-sectional study was conducted with adults submitted to orthotopic liver transplantation at a reference hospital in Fortaleza, Northeast of Brazil, from January to December 2016. Preoperative risk factors were evaluated for AKI development in the immediate postoperative period. AKI was defined according to the Kidney Disease: Improving Global Outcomes (KDIGO) criteria., Results: A total of 40 patients were included in the study. AKI was found in 85% of patients in the first 24 hours after transplantation, most of them (40%) classified in KDIGO stage 1. Preoperative data indicate that serum albumin levels were lower in the KDIGO stage 3 group compared to the non-AKI group, as well as the hematocrit levels. Direct bilirubin (DB) was higher in the KDIGO stage 3 group compared to the group without AKI, as well as alkaline phosphatase (AP) and gamma-glutamiltransferase (GGT). In a logistic regression analysis independent risk factors for AKI were increase levels of AP, GGT and DB and decrease level of serum albumin., Conclusion: Low levels of serum albumin, and elevated levels of DB, AP and GGT in the preoperative period are risk factors for AKI development after liver transplantation.

Background: A premature myocardial infarction (PMI) is usually associated with a familial component. This study evaluated cardiovascular risk factors in first-degree relatives (FDR) of patients with PMI not presenting the familial hypercholesterolemia phenotype., Methods: A cross-sectional study comprising FDR of non-familial hypercholesterolemia patients who suffered a myocardial infarction <45-years age matched for age and sex with individuals without family history of cardiovascular disease. Subjects were evaluated for presence of the metabolic syndrome and its components, lifestyle, statin therapy, and laboratory parameters., Results: The sample was composed of 166 FDR of 103 PMI patients and 111 controls. The prevalence of smoking (29.5 vs. 6.3%; p < 0.001), prediabetes (40.4 vs. 27%; p < 0.001), diabetes (19.9 vs. 1.8%; p < 0.001), metabolic syndrome (64.7 vs. 36%; p < 0.001), and dyslipidemia (84.2 vs. 31.2%; p = 0.001) was greater in FDR. There was no difference on the prevalence of abdominal obesity between groups. In addition, FDR presented higher triglycerides (179.0 ± 71.0 vs. 140.0 ± 74.0 mg/dL; p = 0.002), LDL-cholesterol (122.0 ± 36.0 vs. 113.0 ± 35 mg/dL; p = 0.031), non-HDL-cholesterol (157.0 ± 53.0 vs. 141.0 ± 41.0 mg/dL; p = 0.004), and lower HDL-cholesterol (39.0 ± 10.0 vs. 48.0 ± 14.0 mg/dL; p < 0.001) than controls. Thyrotropin levels (2.4 ± 1.6 vs. 1.9 ± 1.0 mUI/L; p = 0.002) were higher in FDR. The risk factor pattern was like the one of index cases. Only 5.9% (n = 10) of FDR were in use of statins., Conclusions: FDR of non-familial hypercholesterolemia patients with PMI presented an elevated prevalence of metabolic abnormalities, inadequate lifestyle and were undertreated for dyslipidemia.

Purpose: It has been suggested that flexibility training may reduce the total volume of training during resistance trainings. The purpose of this study was to compare the effect of flexibility training immediately before resistance training (FLEX-RT) versus resistance training without flexibility training (RT) on maximum strength and the vastus lateralis muscle cross-sectional area (CSA)., Methods: Participants had each leg assigned to RT or FLEX-RT. Both groups performed four sets of leg extensions to voluntary failure of 80% of one repetition maximum (1RM); however, FLEX-RT performed two sets of 25 s of static stretching before resistance training. Number of repetitions and total volume were calculated during weeks 1-5 and 6-10. Vastus lateralis muscle CSA, 1RM, and flexibility were assessed at baseline and after 10 weeks., Results: The number of repetitions and total training volume were greater for RT than FLEX-RT for weeks 1-5 and 6-10. Regarding the vastus lateralis muscle CSA, a main time effect was observed, however, greater change was observed for RT than FLEX-RT (12.7 and 7.4%, respectively). A main time effect for 1RM was also observed with similar changes for RT and FLEX-RT (12.7 and 12.9%, respectively). Flexibility was increased pre- to post-training for FLEX-RT with greater change for FLEX-RT (10.1%) than RT (2.1%)., Conclusion: These results show that performing flexibility training immediately before resistance training can contribute to a lower number of repetitions, total volume, and muscle hypertrophy.

Myosin Va (MyoVa) is an actin-based molecular motor abundantly found at the centrosome. However, the role of MyoVa at this organelle has been elusive due to the lack of evidence on interacting partners or functional data. Herein, we combined yeast two-hybrid screen, biochemical studies and cellular assays to demonstrate that MyoVa interacts with RPGRIP1L, a cilia-centrosomal protein that controls ciliary signaling and positioning. MyoVa binds to the C2 domains of RPGRIP1L via residues located near or in the Rab11a-binding site, a conserved site in the globular tail domain (GTD) from class V myosins. According to proximity ligation assays, MyoVa and RPGRIP1L can interact near the cilium base in ciliated RPE cells. Furthermore, we showed that RPE cells expressing dominant-negative constructs of MyoVa are mostly unciliated, providing the first experimental evidence about a possible link between this molecular motor and cilia-related processes.

Objective: To present the preliminary results from treating patients with Legg-Calvé-Perthes Disease (LCPD) by means of hip arthrodiastasis using a monolateral external fixator applied to the hip and to succinctly describe the surgical technique used, in a prospective study., Methods: Prospective study on 18 patients with LCPD who underwent surgical treatment by means of the hip arthrodiastasis technique using a monolateral external fixator. There were 13 male and five female patients of mean age 8.5 years, ranging from five to 13 years. All the patients presented unilateral hip impairment: nine on the right side and nine on the left. The results were evaluated at maturity using clinical and radiological criteria., Results: All the patients evolved with improvement of joint mobility, and pain relief was achieved in 88.9% of them. Reossification of the femoral epiphysis occurred within the first three months of the treatment. The hips operated at the necrosis stage of the disease did not passed through the fragmentation stage, thus shortening the evolution of the disease. The results were 77.8% satisfactory and 22.2% unsatisfactory., Conclusion: Hip arthrodiastasis with a monolateral external fixator during the active phase of LCPD improved the degree of joint mobility. Use of the arthrodiastasis technique at the necrosis stage or at the fragmentation stage (active phase of the disease) presented satisfactory results from treatment of LCPD.

Objective: Flexor tendon tenolysis on zone 2 is a difficult and really challenging for hand surgery. With the objective of comparing the results obtained between tenolyses with intraoperative awakening, performed with locoregional anesthesia (group 1), from those obtained with traditional tenolysis performed under general anesthesia or total blockage of the brachial plexus (group 2), the authors conducted a prospective and controlled study., Methods: 22 patients with 39 fingers with flexor tendon injuries on zone 2 evolving to adherences were assessed. All patients were operated after three months and before one year of the primary tendinous suture. All patients showed limited active motion not improved by rehabilitation. Groups 1 and 2 showed to be homogenous concerning patients' age and gender, preoperative compromising, and absence of associated injuries or pathologies. All patients were assessed according to active motion (TAMs) both preoperatively and at 6 months postoperatively., Results: The statistical analysis of data obtained for groups 1 and 2 shows that the tenolysis performed with both techniques produce good results. By comparing the results for total active motion after six months of the tenolysis, group 1 patients (treated by the intraoperative awakening technique) were found to present better outcomes., Conclusions: Flexor tenolysis on zone 2 provide good results in terms of joint range of motion. The intraoperative awakening technique with locoregional anesthesia provides improved outcomes when compared to the traditional technique.

Background: There have been few studies on pentamidine in the Americas; and there is no consensus regarding the dose that should be applied., Objectives: To evaluate the use of pentamidine in a single dose to treat cutaneous leishmaniasis., Methods: Clinical trial of phase II pilot study with 20 patients. Pentamidine was used at a dose of 7 mg/kg, in a single dose. Safety and adverse effects were also assessed. Patients were reviewed one, two, and six months after the end of treatments., Results: there was no difference between the treatment groups in relation to gender, age, number or location of the lesions. Pentamidine, applied in a single dose, obtained an effectiveness of 55%. Mild adverse events were reported by 17 (85%) patients, mainly transient pain at the site of applications (85%), while nausea (5%), malaise (5%) and dizziness (5%) were reported in one patient. No patient had sterile abscess after taking medication at a single dose of 7mg/kg., Conclusions: Clinical studies with larger samples of patients would enable a better clinical response of pent amidine at a single dose of 7mg, allowing the application of more powerful statistical tests, thus providing more evidences of the decrease in the effectiveness of that medication. Hence, it is important to have larger studies with new diagrams and/or new medications.

Background: In type 1 diabetes mellitus (T1DM) management, enhancing health-related quality of life (HRQoL) is as important as good metabolic control and prevention of secondary complications. This study aims to evaluate possible regional differences in HRQoL, demographic features and clinical characteristics of patients with T1DM in Brazil, a country of continental proportions, as well as investigate which variables could influence the HRQoL of these individuals and contribute to these regional disparities., Methods: This was a retrospective, cross-sectional, multicenter study performed by the Brazilian Type 1 Diabetes Study Group (BrazDiab1SG), by analyzing EuroQol scores from 3005 participants with T1DM, in 28 public clinics, among all geographical regions of Brazil. Data on demography, economic status, chronic complications, glycemic control and lipid profile were also collected., Results: We have found that the North-Northeast region presents a higher index in the assessment of the overall health status (EQ-VAS) compared to the Southeast (74.6 ± 30 and 70.4 ± 19, respectively; p < 0.05). In addition, North-Northeast presented a lower frequency of self-reported anxiety-depression compared to all regions of the country (North-Northeast: 1.53 ± 0.6; Southeast: 1.65 ± 0.7; South: 1.72 ± 0.7; Midwest: 1.67 ± 0.7; p < 0.05). These findings could not be entirely explained by the HbA1c levels or the other variables examined., Conclusions: Our study points to the existence of additional factors not yet evaluated that could be determinant in the HRQoL of people with T1DM and contribute to these regional disparities.

Background: To evaluate the determinants of intensive insulin regimens (ITs) in patients with type 1 diabetes (T1D)., Methods: This multicenter study was conducted between December 2008 and December 2010 in 28 public clinics in 20 Brazilian cities. Data were obtained from 3,591 patients (56.0% female, 57.1% Caucasian). Insulin regimens were classified as follows: group 1, conventional therapy (CT) (intermediate human insulin, one to two injections daily); group 2 (three or more insulin injections of intermediate plus regular human insulin); group 3 (three or more insulin injections of intermediate human insulin plus short-acting insulin analogues); group 4, basal-bolus (one or two insulin injections of long-acting plus short-acting insulin analogues or regular insulin); and group 5, basal-bolus with continuous subcutaneous insulin infusion (CSII). Groups 2 to 5 were considered IT groups., Results: We obtained complete data from 2,961 patients. Combined intermediate plus regular human insulin was the most used therapeutic regimen. CSII was used by 37 (1.2%) patients and IT by 2,669 (90.2%) patients. More patients on IT performed self-monitoring of blood glucose and were treated at the tertiary care level compared to CT patients (p < 0.001). The majority of patients from all groups had HbA1c levels above the target. Overweight or obesity was not associated with insulin regimen. Logistic regression analysis showed that economic status, age, ethnicity, and level of care were associated with IT (p < 0.001)., Conclusions: Given the prevalence of intensive treatment for T1D in Brazil, more effective therapeutic strategies are needed for long term-health benefits.

Introduction: Subclinical hypothyroidism (SCH), defined as elevated concentrations of thyroid stimulating hormone (TSH) despite normal levels of thyroid hormones, is highly prevalent in Brazil, especially among women and the elderly. Although an increasing number of studies have related SCH to an increased risk of coronary artery disease and mortality, there have been no randomized clinical trials verifying the benefit of levothyroxine treatment in reducing these risks, and the treatment remains controversial., Objective: This consensus, sponsored by the Thyroid Department of the Brazilian Society of Endocrinology and Metabolism and developed by Brazilian experts with extensive clinical experience with thyroid diseases, presents these recommendations based on evidence for the clinical management of SCH patients in Brazil., Materials and Methods: After structuring the clinical questions, the search for evidence in the literature was initially performed in the MedLine-PubMed database and later in the Embase and SciELO - Lilacs databases. The strength of evidence was evaluated according to the Oxford classification system and established based on the experimental design used, considering the best available evidence for each question and the Brazilian experience., Results: The topics covered included SCH definition and diagnosis, natural history, clinical significance, treatment and pregnancy, and the consensus issued 29 recommendations for the clinical management of adult patients with SCH., Conclusion: Treatment with levothyroxine was recommended for all patients with persistent SCH with serum TSH values > 10 mU/L and for certain patient subgroups.

Background: Renal complications in acromegaly include glomerular hyperfiltration, insulin resistance, hypercalciuria and urolithiasis. The aim of this study was to investigate whether urinary calcium (U(Ca)) excretion is a direct consequence of growth hormone secretion or secondary to hyperfiltration and/or insulin resistance., Methods: We performed a cross-sectional study of 58 patients diagnosed with acromegaly. Demographic data were obtained, serum analysis was performed, including insulin-like growth factor (IGF)-1, and 24-h urine collection, to measure urinary protein excretion, U(Ca) and phosphate excretion, as well as fractional excretion of sodium and potassium. We also calculated the homeostasis model assessment of insulin resistance (HOMA-IR)., Results: Patients were predominantly male (60.3%), and their mean age was 45.9 ± 14 years. Hypercalciuria was present in 24% of patients. Patients with higher HOMA-IR had higher IGF-1 levels, a trend toward higher body mass index and higher U(Ca) excretion. In univariate analysis, U(Ca) excretion was associated with HOMA-IR (r = 0.472, P = 0.001), phosphaturia (r = 0.457, P = 0.001), IGF-1 (r = 0.398, P = 0.002) and creatinine clearance (r = 0.394, P = 0.001). HOMA-IR and phosphaturia were independently associated with U(Ca) excretion. No independent associations were found between phosphaturia and HOMA-IR or IGF-1., Conclusions: The present study revealed an association between hypercalciuria and insulin resistance in patients with acromegaly. Further studies are required to fully understand the pathogenesis of these abnormalities in patients with acromegaly.

Pregnancy affects both maternal and fetal metabolism, and even in non-diabetic women, it exerts a diabetogenic effect. Among pregnant women, 2% to 14% develop gestational diabetes. Pregnancy can also occur in women with preexisting diabetes, which may predispose the fetus to many alterations in organogenesis, restrict growth, and the mother, to some diabetes-related complications, such as retinopathy and nephropathy, or to acceleration of the course of these complications, if they are already present. Women with gestational diabetes generally start their treatment with diet and lifestyle changes; when these changes are not enough for optimal glycemic control, insulin therapy must then be considered. Women with type 2 diabetes using oral hypoglycemic agents are advised to change to insulin therapy. Those with preexisting type 1 diabetes should start intensive glycemic control. As basal insulin analogues have frequently been used off-label in pregnant women, there is a need to evaluate their safety and efficacy. The aim of this review is to report the use of both short- and long-acting insulin analogues during pregnancy and to enable clinicians, obstetricians, and endocrinologists to choose the best insulin treatment for their patients.