Search

Abstract One of the measures for monitoring microbial resistance is the calculation of the defined daily dose of antimicrobial agents. For this calculation, the weight of an adult of 70 kg is used as a standard, so that application in neonatology is not possible. The aim of this study is to describe the use profile and calculate the defined daily dose (DDD) of antimicrobials in a neonatal intensive care unit (NICU) of a public hospital in the interior of Bahia, Brazil. From March 2020 to December 2021, the medical records of 712 newborns admitted to a NICU between September 2018 and June 2020 were analyzed. A total of 410 newborns diagnosed with neonatal sepsis were included. The most used antimicrobials per patient were gentamicin (408/410; 99.5%), ampicillin (407; 99.3%), amikacin (29; 7.1%) and oxacillin (21; 5.1%), with a mean (SD) treatment duration of 9.8 (3.9) days. The most commonly used combination of antimicrobials was ampicillin with gentamicin, which was used in 406 patients (99.0%). The values for neonatal DDDs were on average 26 times lower than those for adult DDDs. The neonatal DDDs were similar to those observed in other studies. Ampicilin and cefepime were the antimicrobials for which the greatest differences were observed in neonatal DDDs compared with adult DDDs, which differed mainly between maintenance doses, reflecting the lack of international standards in neonatology. Standardization of DDDs as a surveillance measure has the potential to clarify the pattern of antimicrobial use in neonatal patients worldwide and, in particular, to prevent indiscriminate use and bacterial resistance.

Introdução Nas últimas décadas, alguns medicamentos foram desenvolvidos para o tratamento da Doença Macular Relacionada à Idade (DMRI), tendo como alvo principal o bloqueio do fator de crescimento do endotélio vascular (VEGF). No Brasil, o uso de anti-VEGF foi incorporado ao SUS, em 2018, para uso em pacientes com DMRI exsudativa, com melhor AV corrigida igual ou superior a 20/400 e igual ou inferior a 20/3. Apesar da eficácia da terapia ter sido consistentemente demonstrada em ensaios clínicos, existem lacunas acerca da efetividade do uso da terapia anti-VEGF no mundo real. Assim, este estudo avaliou a efetividade dos medicamentos Bevacizumabe ou Aflibercepte em pacientes do SUS com DMRI. Métodos Estudo retrospectivo longitudinal, com dados de prontuário de 54 pacientes com DMRI que fizeram uso de Bevacizumabe ou Aflibercepte. Os desfechos em saúde utilizados foram as medidas de acuidade visual (AV) e de espessura central da retina obtidas pelo OCT. Pacientes com AV < 20/400 foram denominados como “pior visão”. Resultados Dentre os 60 olhos, o medicamento utilizado com maior frequência foi o Bevacizumabe (55,0%). A primeira injeção intraocular foi utilizada, em média (DP) aos 74.7 (9.4) anos e foram aplicadas, em média, 6.4 (4.3) injeções. Não foi observada diferença (p=0.262) entre o percentual de resposta satisfatória de acordo com o uso de Bevacizumabe (16/33; 48.5%) ou Aflibercepte (17/27; 63.0%). Os desfechos de AV segundo Snellen, ganho de letras e espessura retiniana demonstraram melhora ou estabilidade em 55%, 32.8% e 78.3% dos casos, respectivamente. Não houve diferença significativa entre o número médio (DP) de injeções anti-VEGF aplicadas entre os grupos com e sem resposta terapêutica (5.8 [3.7] vs. 7.0 [4.8]; p = 0.315), nem no tempo decorrido entre a primeira e a última injeção, em dias, nos grupos com e sem resposta (274.3 [317.1] vs. 434.8 [514.6]; p = 0.262). Observou-se uma tendência de melhor resposta à medida que a classificação da acuidade visual piorava (ptend= 0.062). Entre os olhos classificados como “pior visão” (AV

Abstract: The Brazilian Unified National Health System (SUS) has incorporated newborn screening for cystic fibrosis since 2001. The protocol involves two samples of immunoreactive trypsinogen (IRT1/IRT2). This study aims to analyze fixed and floating values at the first and second IRT (IRT1/IRT2) cutoff points and assess the accuracy of the IRT/IRT methodology in a population from Northeastern Brazil. Descriptive, individual-level data from the newborn screening reference service data system (2013-2017) were used in this observational population study. The sensitivity, specificity, and positive predictive values (PPV) for the protocol were calculated. The best cutoff point was determined using the Youden’s index. The previous year’s cut-off values for the IRT1 and IRT2 99.4-, 99.5-, 99.6-, and 99.7-percentiles were utilized for the floating cutoff. During the studied period, 840,832 newborns underwent screening for cystic fibrosis, obtaining 49 cystic fibrosis diagnoses: 39 by newborn screening (79.6%) and 10 (20.4%) by clinical suspicion (false negative). The sensitivity, specificity, and PPV of the protocol totaled 79.6%, 99.9%, and 6.1%, respectively. No proposed cutoff for IRT1 performed better than the current one. IRT2 performed similarly to the current protocol at a cutoff point of 90ng/mL, showing the appropriate sensitivity and specificity while reducing the frequency of false positives. The protocol to screen newborns for cystic fibrosis had low sensitivity, a predictive positive value, and a high number of false positives and negatives. A floating cut point for IRT1 or IRT2 seems to constitute no viable option. However, changing the IRT2 cut point from 70ng/mL to 90ng/mL seems to have advantages and should undergo consideration.

ABSTRACT Objective: To characterize the effectiveness of anti-vascular endothelial growth factor drugs in exudative age-related macular degeneration. Methods: Retrospective longitudinal study of 54 patients with age-related macular degeneration receiving bevacizumab or aflibercept. Demographic data, visual acuity, and central retinal thickness measurements were collected. Improvement/stability of visual acuity and reduction in retinal thickness configured satisfactory responses. Results: Among the 60 eyes studied, there was no difference (p = 0.262) in satisfactory response when using bevacizumab (48.5%) or aflibercept (63.0%). Snellen’s visual acuity, letter gain, and retinal thickness showed improvement or maintenance in 55.0%, 32.8%, and 78.3% of cases, respectively. The percentage of improvement/maintenance was higher in eyes with an initial visual acuity of < Snellen 20/400 (70.0% versus 40.0%; p = 0.002). Conclusion: A higher percentage of improvement/stabilization of visual acuity and macular thickness was observed in patients with age-related macular degeneration, with better response in patients with visual acuity worse than Snellen 20/400.

Abstract Objective To compare memory outcomes after surgery for unilateral hippocampal sclerosis (HS)‐associated epilepsy in patients with unilateral and bilateral ictal electrographic involvement. Methods We prospectively evaluated HS patients, aged 18‐55 years and IQ ≥70. Left (L) and right (R) surgical groups underwent noninvasive video‐EEG monitoring and Wada test. We classified patients as Ipsilateral if ictal EEG was restricted to the HS side, or Bilateral, if at least one seizure onset occurred contralaterally to the HS, or if ictal discharge evolved to the opposite temporal region. Patients who declined surgery served as controls. Memory was evaluated on two occasions with Rey Auditory‐Verbal Learning Test and Rey Visual‐Design Learning Test. Baseline neuropsychological test scores were compared between groups. Pre‐ and postoperative scores were compared within each group. Reliable change index Z‐scores (RCI) were obtained using controls as references, and compared between surgical groups. Results We evaluated 64 patients. Patients were classified as: L‐Ipsilateral (9), L‐Bilateral (15), L‐Control (9), R‐Ipsilateral (10), R‐Bilateral (9), and R‐Control (12). On preoperative evaluation, memory performance did not differ among surgical groups. Right HS patients did not present postoperative memory decline. L‐Ipsilateral group presented postoperative decline on immediate (P = 0.036) and delayed verbal recall (P = 0.011), while L‐Bilateral did not decline. L‐Ipsilateral had lower RCI Z‐scores, indicating delayed verbal memory decline compared to L‐Bilateral (P = 0.012). Significance Dominant HS patients with bilateral ictal involvement presented less pronounced postoperative verbal memory decline compared to patients with exclusive ipsilateral ictal activity. Surgery was indicated in these patients regardless of memory impairment on neuropsychological testing, since resection of the left sclerotic hippocampus could result in cessation of contralateral epileptiform activity, and, therefore, improved memory function.

Abstract Background HTLV-1-associated uveitis (HAU) is an inflammatory reaction of the choroid, retina, optic nerve and vitreous that can lead to vision impairment. The worldwide prevalence of HAU varies widely. Objective To determine the prevalence of HAU in patients from Salvador, Bahia-Brazil, and describe uveitis type and associated symptoms. Methods Cross-sectional analytical study to determine the prevalence of uveitis in HTLV-1-infected patients recruited in Bahia, Brazil, a region considered endemic for HTLV-1. Patients were enrolled at a local reference center for HTLV (infected) and at an outpatient ophthalmology clinic (noninfected group). All patients were examined by the same ophthalmologist following a single protocol. Prevalence ratios (PR) were calculated. Results A total of 168 consecutively examined HTLV-1-infected patients and 410 noninfected patients (randomly selected) were included. Females predominated (82.1%) in the HTLV-1-infected group (versus 64.4% in the uninfected group) (p

Background Flexibility is crucial to the harmonious execution of joint movements. While skeletal muscle dysfunction in patients with HTLV-1 can interfere with mobility, it is unclear whether these patients experience reduced flexibility.

IntroductionFamilial clustering of HTLV-1 and related diseases has been reported in Brazil. However, intrafamilial transmission of HTLV-1 based on molecular analysis has been studied only in a few communities of Japanese immigrants and African-Brazilians.ObjectiveTo investigate the familial clustering of HTLV-1 infection and to determine the likely routes of transmission through epidemiological and genetic analyzes.MethodsMedical records of 1,759 HTLV-1+ patients from de the Center for HTLV in Salvador, Brazil, were evaluated to identify first-degree relatives previously tested for HTLV-1. Familial clustering was assumed if more than one member of the same family was HTLV-1+. LTR regions of HTLV-1 sequences were analyzed for the presence of intrafamilial polymorphisms. Family pedigrees were constructed and analyzed to infer the likely transmission routes of HTLV-1.ResultsIn 154 patients at least one other family member had tested positive for HTLV-1 (a total of 182 first-degree relatives). Of the 91 couples (182 individuals), 51.6% were breastfed, and 67.4% reported never using a condom. Of the 42 mother-child pairs, 23.8% had a child aged 13 years or younger; all mothers reported breastfeeding their babies. Pedigrees of families with 4 or more members suggests that vertical transmission is a likely mode of transmission in three families. Three families may have had both vertical and sexual transmission routes for HTLV-1. The genetic signatures of the LTR region of 8 families revealed 3 families with evidence of vertical transmission, another 3 families (spouses) with sexual transmission, and one family with both transmission routes. HTLV-1 sequences belonged to Cosmopolitan subtype HTLV-1a Transcontinental subgroup A.ConclusionSexual and vertical transmission routes contribute to the intrafamilial spread of HTLV-1 in the state of Bahia.

Brazil is home to the highest absolute number of human T-cell lymphotropic virus type-1 (HTLV-1)-infected individuals worldwide; the city of Salvador, Bahia, has the highest prevalence of HTLV-1 infection in Brazil. Due to the complex nature of several diseases associated with this retrovirus, a multidisciplinary health care approach is necessary to care for people living with HTLV-1. The Bahia School of Medicine and Public Health’s Integrative Multidisciplinary HTLV Center (CHTLV) has been providing support to people living with HTLV and their families since 2002, striving to ensure physical and mental well-being by addressing biopsychosocial aspects, providing clinical care and follow-up, including to pregnant/postpartum women, as well as comprehensive laboratory diagnostics, psychological therapy, and counseling to family members. To date, CHTLV has served a total of 2,169 HTLV-infected patients. The average patient age is 49.8 (SD 15.9) years, 70.3% are female, most are considered low-income and have low levels of education. The majority (98.9%) are HTLV-1 cases, and approximately 10% have been diagnosed with tropical spastic paraparesis/HTLV-1-associated myelopathy (TSP/HAM), while 2.2% have infective dermatitis and 1.1% have adult T-cell lymphoma. In all, 178 pregnant/postpartum women [mean age: 32.7 (±6.5) years] have received care at CHTLV. Regarding vertical transmission, 53% of breastfed infants screened for HTLV tested positive in their second year of life, nearly 18 times the rate found in non-breastfed infants. This article documents 20 years of experience in implementing an integrative and multidisciplinary care center for people living with HTLV in Bahia, Brazil. Still, significant challenges remain regarding infection control, and HTLV-infected individuals continue to struggle with the obtainment of equitable and efficient healthcare.

Background: The prevalence of keratoconjunctivitis sicca (KCS) associated with Human T-Cell Lymphotropic Virus Type 1 (HTLV-1) (HTLV-1/KCS) has been estimated at around 37%, but its clinical manifestations are poorly described. Purpose: To determine the prevalence and associated factors of HTLV-1/KCS in a large cohort of HTLV-1-infected individuals living in Salvador, Brazil. Methods: A cross-sectional study was conducted between June 2004 and September 2017 at the Integrative and Multidisciplinary Center for HTLV in Salvador, Bahia-Brazil. Data from 758 HTLV-1-infected patients was collected. A complete ophthalmologic examination was performed in both eyes. Lacrimal function was evaluated by breakup time, Rose Bengal and Schirmer I Tests. KCS diagnosis was considered in the presence of at least two out of three positive tests. HTLV-1 proviral load Crude and Adjusted Prevalence Rates (PR) with 95% Confidence Intervals (95% CI) were estimated using multivariate Poisson Regression with robust error variance. Results: The overall prevalence of KCS was 31.7%, with higher rates observed in HTLV-1-associated myelopathy/tropical spastic paraparesis patients (crude PR: 1.84; CI95%: 1.50–2.26) even after adjusting for age, sex, time of HTLV-1 diagnosis and schooling (adjusted PR: 1.63; CI95%: 1.31–2.02). Proviral load, low corrected visual acuity, burning and/or pain and itching were all significantly higher in patients with KCS. Conclusion: Burning and/or pain and itching and low corrected visual acuity were the most common alterations of HTLV-1/KCS. High Proviral load was found to be associated with the presence of KCS. It is strongly recommended that HTLV-1 patients undergo periodic ophthalmologic examination to promote the early diagnosis of KCS and prevent the consequences associated with dry eye disease. Keywords: HTLV-1, Keratoconjunctivitis sicca, Prevalence, Dry eye syndromes

ABSTRACT Objective: To characterize metabolic control and verify whether it has any relation with socioeconomic, demographic, and body composition variables in children and adolescents with phenylketonuria (PKU) diagnosed in the neonatal period. Methods: This cohort study collected retrospective data of 53 phenylketonuric children and adolescents. Data on family income, housing, and mother’s age and schooling level were collected, and anthropometric measures of body composition and distribution were taken. All dosages of phenylalanine (Phe) from the last five years (2015-2019) were evaluated and classified regarding their adequacy (cutoffs: 0-12 years: 2-6 mg/dL; 12-19 years: 2-10 mg/dL). Adequate metabolic control was considered if ≥7%) of the dosages were within desired ranges. Results: The mean (±standard deviation) age in the last year was 10.1±4.6 years. Most of them were under 12 years old (33/53; 62.3%) and had the classic form of the disease (39/53; 73.6%). Better metabolic control was observed among adolescents (68.4 versus 51.4%; p=0.019). Overweight was found in 9/53 (17%) and higher serum Phe levels (p

Introduction. Acute Physiology and Chronic Health Evaluation (APACHE) II and III and Sequential Organ Failure Assessment (SOFA) are prognostic scores commonly used in the intensive care unit (ICU). Their accuracy in predicting mortality has not been adequately evaluated in comparison to prognostic scores commonly used in critically ill cirrhotic patients with acute decompensation (AD) or acute-on-chronic liver failure (ACLF). Aims. This study was conducted to evaluate the performance of prognostic scores, including APACHE II, SOFA, Chronic Liver Failure Consortium (CLIF-C) SOFA, Child–Turcotte–Pugh (CPS), Model for End-Stage Liver Disease (MELD), MELD-Na, MELD to serum sodium ratio (MESO) index, CLIF-C organ failure (CLIF-C OF), CLIF-C ACLF, and CLIF-C AD scores, in predicting mortality of cirrhotic patients admitted to the ICU. Patients and Methods. A total of 382 patients (280 males, mean age 67.3 ± 10.6 years) with cirrhosis were retrospectively evaluated. All prognostic scores were calculated in the first 24 hours of ICU admission. Their ability to predict mortality was measured using the analysis of the area under the receiver operating characteristic curve (AUC). Results. Mortality was observed in 31% of the patients. Analysis of AUC revealed that CLIF-C OF (0.807) and CLIF-SOFA (0.776) had the best ability to predict mortality in all patients, but CLIF-C OF (0.749) had higher prognostic accuracy in patients with ACLF. CLIF-SOFA, SOFA, and CLIF-C AD had the highest AUC values in patients with AD, with no statistical difference (p=0.971). Conclusions. When compared to other general or liver-specific prognostic scores, CLIF-C OF, CLIF-SOFA, SOFA, and CLIF-C AD have good accuracy to predict mortality in critically ill patients with cirrhosis and patients with AD. According to the clinical scenario, different scores should be used to provide prognosis to patients with cirrhosis in the ICU.

Introduction: Human T-lymphotropic virus (HTLV)-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a neurological disorder that mostly affects women. This disease is characterized by a progressive loss of motor function and disruptions in sensory function in the lower limbs. HTLV-1 is also associated with isolated neurologic dysfunctions, overactive bladder, and erectile dysfunction. The occurrence of sexual dysfunction in HTLV-1–infected women remain unclear. Aim: To investigate associations between HTLV-1 infection and sexual dysfunction in both asymptomatic infected women and those diagnosed with HAM/TSP compared with uninfected women. Methods: HTLV-1–infected and uninfected women were assessed for sexual dysfunction using the Female Sexual Function Index instrument. Sexual dysfunction was considered if global Female Sexual Function Index scores were

Resumo Objetivo Investigar a frequência de sintomas otoneurológicos em crianças com hipotireoidismo congênito e relacioná-los com os aspectos clínicos. Métodos Estudo exploratório de caráter descritivo seccional. A casuística foi composta pelos pais e/ou cuidadores de 105 crianças com diagnóstico de hipotireoidismo congênito e com 5 anos ou mais de idade. A coleta de dados utilizou um questionário estruturado elaborado e testado pelos pesquisadores. As informações clínicas e laboratoriais foram obtidas dos prontuários médicos. Resultados A maioria dos indivíduos (72,4%) demonstrou sintomas relacionados à disfunção vestibulococlear, na qual a tontura/vertigem correspondeu a 56,2% dos sintomas, seguida pela hipoacusia (43,8%) e pelo zumbido (12,4%). Houve correlação estatística entre os sintomas de hipoacusia (p=0,016) e a idade para o teste de rastreio neonatal, bem como os sintomas de tontura/vertigem com a etiologia do hipotireoidismo congênito (p=0,003). Na percepção da hipoacusia (RP=0,49; IC=0,31-0,77), a exposição ao fator demonstrou associação com a probabilidade de 95%. Conclusão Os achados sugerem significativa prevalência e associação entre hipotireoidismo congênito e sintomas otoneurológicos, especialmente tontura, hipoacusia e zumbido.

This study described a broad clinical characterization of classical homocystinuria (HCU) in Brazil. This was a cross-sectional, observational study including clinical and biochemical data from 72 patients (60 families) from Brazil (South, n = 13; Southeast, n = 37; Northeast, n = 8; North, n = 1; and Midwest, n = 1). Parental consanguinity was reported in 42% of families. Ocular manifestations were the earliest detected symptom (53% of cases), the main reason for diagnostic suspicion (63% of cases), and the most prevalent manifestation at diagnosis (67% of cases). Pyridoxine responsiveness was observed in 14% of patients. Only 22% of nonresponsive patients on treatment had total homocysteine levels

BACKGROUND: In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/ß0-thalassemia.OBJECTIVE: To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia.METHODS: This is a retrospective study of 242 children aged between two and six years with diagnoses of Hb SS or Hb SC. The hemoglobin was evaluated using high performance liquid chromatography and alpha thalassemia [3.7 kb deletion (-a3.7)] was detected by polymerase chain reaction. Patients were classified as homozygous (-a3.7/-a3.7), heterozygous (-a3.7/a), or homozygous wild-type. Analysis of variance was used to compare the mean Hb A2 values between the alpha thalassemia groups.RESULTS: The mean (± standard deviation) Hb A2 concentrations in the Hb SS group (n = 135) was 3.68 ± 0.65%. The mean values for individuals with Hb SS and heterozygous (n = 28) or homozygous for alpha thalassemia (n = 3) were 3.98 ± 0.64% and 4.73 ± 0.25%, respectively. The mean Hb A2 of all the Hb SC patients (n = 107) was 4.01 ± 0.507 with 4.29 ± 0.41% and 4.91 ± 0.22% in individuals heterozygous (n = 23) and homozygous for alpha thalassemia (n = 7), respectively. All patients homozygous for alpha thalassemia had Hb A2 levels above 3.5%. However, Hb A2 values above 5.2% were seen in patients with Hb SS and Hb SC, independently of alpha thalassemia.CONCLUSION: Hb A2 levels are elevated in patients with Hb S or Hb C, and are directly influenced by the alpha thalassemia genotypes.

OBJECTIVE: To study the breastfeeding history (BF) and the anthropometric status of children with Sickle Cell Disease (SCD). METHODS: A cross-sectional study of 357 children with SCD aged between 2 and 6 years, regularly followed at a Newborn Screening Reference Service (NSRS) between November 2007 and January 2009. The outcome was anthropometric status and the exposures were: BF pattern, type of hemoglobinopathy and child's age and gender. RESULTS: The mean (SD) age was 3.7 (1.1) years, 52.9% were boys and 53.5% had SCA (hemoglobin SS). The prevalence of exclusive breastfeeding (EBR) up to six months of age was 31.5%, the median EBR times (p25-p75) was 90.0 (24.0-180.0) days and the median weaning ages (p25-p75) was 360.0 (90.0-720.0) days respectively. Normal W/H children experienced EBR for a mean duration almost four times longer than malnourished children (p=0.01), and were weaned later (p

Objective: To investigate the role of demographic variables in the relationship between the presence of HAM/TSP and current major depression. Methods: It is a cross-sectional study of 108 HTLV-1 infected patients (47 with TSP/HAM) resident of Salvador, Brazil. The Mini International Neuropsychiatric Interview, Brazilian Version 5 was used to evaluate the presence of depression. Prevalence ratios were used to describe relationship between HAM/TSP and depression. The HAM/TSP classification was carried out according to the criteria proposed by Castro-Costa et al. Results: Prevalence of depression was 37.96%. No association was observed between presence of HAM/TSP and diagnosis of current major depression in the global analysis of patients (PR: 0.94; CI 95%: 0.57–1.55). In the stratified analysis, however, greater prevalence of depression was observed amongst individuals with HAM/TSP in the 18–39 age group (PR: 2.59; CI 95%: 1.36–4.95). Conclusion: Our findings suggest that age is an effect modifier in the relationship between HAM/TSP and depression, and this aspect should be considered in future studies on the topic. Keywords: Depression, Human T-lymphotropic virus 1, Age factors, HTLV-1 associated myelopathies

Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA.This study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6-18 years. All study subjects were evaluated for endothelial function using Doppler ultrasonography. In addition, a number of laboratory assays were performed, including reticulocyte and leukocyte counts as well as measurement of circulating levels of total bilirubin, C-reactive protein (CRP), glucose, lipoproteins and peripheral oxyhemoglobin saturation. These parameters were also compared between SCA patients who were undertaking hydroxyurea (HU) and those who were not.Flow-mediated vasodilation (FMD) values were found to be reduced in SCA patients compared with those detected in healthy controls. SCA individuals with lower FMD values exhibited higher number of hospital admissions due to vaso-occlusive events. Additional analyses revealed that patients who had decreased FMD values exhibited higher odds of acute chest syndrome (ACS) episodes. A preliminary analysis with limited number of individuals failed to demonstrate significant differences in FMD values between SCA individuals who were treated with HU and those who were not.Children and adolescents with SCA exhibit impaired endothelial function. Reductions in FMD values are associated with ACS. These findings underline the potential use of FMD as screening strategy of SCA patients with severe prognosis at early stages.

BACKGROUND:Salvador is the city with the highest number of HTLV-1 infected individuals in Brazil, yet the main route of HTLV-1 transmission is unknown. OBJECTIVE:To investigate the association of syphilis infection as a proxy for sexual transmission of HTLV-1 infection in the general population of this city. METHODS:A cross sectional population-based study was conducted with 3,451 serum samples obtained by a representative simple random sampling. Data on gender, age, income, and years of education were collected by questionnaire and the presence of HTLV, HIV and Treponema pallidum infection was determined by serology. Logistic regression analysis was used to evaluate the independent effect of the potential explanatory variables to HTLV-1 infection and Odds Ratios (OR) and 95% CI were calculated. RESULTS:The majority of studied individuals were female (56.4%), had less than 7 years of education (55.3%) and earned two or less minimum wages (52.0%). The overall prevalence of HTLV-1 was 1.48% (51/3,451; 95% CI: 1.10%- 1.94%), which increased with age. Only three persons younger than 17 (3/958; 0.31%; CI 95% 0.06-0.91) years were infected by HTLV-1. Among the 45 syphilis positives, 12 (26.7%) were HTLV positive, while among 21 HIV positives, only one (4.8%) was HTLV positive. HTLV-1 infection was found to be associated with syphilis infection (ORADJUSTED 36.77; 95% CI 14.96-90.41). CONCLUSION:The data presented herein indicate that horizontal transmission between adults is the main route of HTLV-1 infection in the general population of Salvador and that this is likely to occur through sexual contact.

Objetivo: Descrever e comparar as características e os desfechos clínicos de pacientes com lesão renal aguda séptica e não séptica. Métodos: Coorte aberta com 117 pacientes graves com lesão renal aguda consecutivamente admitidos em unidade de terapia intensiva, sendo excluídos aqueles que apresentavam doença renal crônica em estágio avançado, transplante renal, internação ou morte em um período inferior a 24 horas. Presença de sepse e óbito intra-hospitalar representaram, respectivamente, a exposição e o desfecho principal. Análise de confundimento foi realizada com a regressão logística. Resultados: Não houve diferenças na média de idade entre os grupos com lesão renal aguda séptica e não séptica [65,30±(21,27) anos versus 66,35±12,82 anos; p=0,75]. Nos dois grupos, similarmente, observou-se predomínio do sexo feminino (57,4% versus 52,4%; p=0,49) e de afrodescendentes (81,5% versus 76,2%; p=0,49). Os pacientes com sepse apresentaram maiores médias de escore Acute Physiology and Chronic Health Evaluation II [21,73±7,26 versus 15,75± (5,98; p18,5 (OR: 9,77; IC95%: 3,73-25,58) foram associados ao óbito. Conclusão: Sepse foi um preditor independente para óbito. Existem diferenças entre as características e desfechos clínicos dos pacientes com lesão renal aguda séptica versus não séptica.

Introduction: Screening for vertically transmitted infection is mandatory and must be conducted at the first prenatal consultation. The most vulnerable women's groups are those at the lowest socio-economic level. Dried blood spot testing on filter paper could represent a secure way to screen pregnant women in the prenatal period. Methods: A cross-sectional study was conducted between November 2009 and March 2010, in the Metropolitan Region of Salvador, Bahia, Brazil, to compare the accuracy of the dried blood spot in filter paper and venipuncture serological as screening methods for HIV, HTLV, VHB, VHC, Treponema pallidum, and Toxoplasma gondii during prenatal period. Results of the venous blood sample collected in tubes were considered the gold standard. Results: Serum samples and dried blood spot were obtained from 692 pregnant women aged between 14 and 42 years, with a median age of 26. Thirteen women were seropositive for T. gondii (1.88%; 95% CI: 0.60–2.71%), five for T. pallidum (0.72%; 95% CI: 0.15–1.61%), two for HBV (0.29%; 95% CI: 0.050.95%) and one for HTLV-1 (0.14%; 95% CI: 0.01–0.71%). No one was positive for HCV and HIV. The dried blood spot accuracy for syphilis and HTLV were 100% (95% CI: 99.25–100) and 100% (95% CI: 99.45–100%), respectively. The average time between blood collection and recording of the sample in the reference laboratory was 4.93 (3.82) days and between dried blood spot processing and active search for pregnant women was 3.44 (4.27) days. Conclusions: The use of dried blood spot may represent a secure way to expedite access to results of vertically transmitted diseases in the prenatal period, particularly in regions with scarce healthcare resources. Keywords: Prenatal care, Infectious diseases, Seroprevalence, Dried blood spot testing

Objective: The purpose of this study was to evaluate the oral hygiene status in pediatric obese patients. Methods: A cross-sectional study was conducted from 2011 to 2012, which evaluated 180 Brazilian pediatric patients, 6-14 years old, girls and boys, recruited according to two Body Mass Index (BMI) categories: obese and non-obese (healthy weight). For the evaluation the oral hygiene status, the study used Oral Hygiene Index (OHI) and Gingival Bleeding Index (GBI). Results: According to the total sample, 5/60 obese (8.3%) and 57/120 non-obese (47.5%) had good OHI, while 23/60 obese (38.4%) and 3/120 non-obese (2.5%) were classified in a low level of OHI, with a significance between the groups (p < 0.001), even after sorting by age. According to the classification of GBI, 60/60 obese (100.0%) and 89/120 non-obese (74.2%) had GBI 1 (bleeding gingiva), and 0/60 obese and 31/120 non-obese (25.8%) were classified as GBI 0 (healthy gingiva), with a significance between the groups (p < 0.001), even after sorting by age. Conclusions: This study indicated that OHI and GBI were significantly higher in the obese children group.

OBJECTIVE: The present study aims to describe ocular alterations in sickle cell disease patients in Bahia, a Northeast state, with the highest prevalence of the disease in Brazil. METHODS: We carried out a cross-sectional study in a group of 146 (292 eyes) sickle cell disease patients (90 HBSS and 56 HBSC). Ophthalmologic examination including indirect binocular ophthalmoscopy was performed. Examination was completed by fluorescein angiography to detect retinal lesions. RESULTS: The most frequent ocular lesions identified were "vascular tortuosity" and "black sunburst". Proliferative retinopathy was found in 22 (12.2%) eyes of HBSS patients and 25 (22.3%) eyes of HBSC patients (OR=2.06; CI95%: 1.5-4.06, p=0.022); Its frequency was higher among HBSS patients aged 20-39 years, while in HBSC patients, it peaked after 40 years (35.7% and 42.8%) and dropped sharply afterwards. CONCLUSION: Proliferative retinopathy was described as early as 10 years of age in both patients groups. Proliferative sickle retinopathy can result in blindness and the knowledge of the most prevalent ocular alterations and age risk will be important to establish a protocol of ophthalmologic follow-up, in order to prevent a severe visual loss and increase patient's life quality.OBJETIVO: O presente estudo teve como objetivo descrever as alterações oculares em pacientes portadores de doença falciforme, na Bahia, um estado do Nordeste, que tem a maior prevalência da doença no Brasil. MÉTODOS: Nós conduzimos um estudo de corte transversal em um grupo de 146 (292 olhos) pacientes com Doença Falciforme (90 HBSS e 56 HBSC). Para exame oftalmológico foi realizada oftalmoscopia binocular indireta complementada pela retinografia fluorescente para detecção de lesões retinianas decorrentes da Doença Falciforme. RESULTADOS: As lesões mais frequentemente encontradas foram o aumento da tortuosidade vascular e "black sumburst" Retinopatia proliferativa foi encontrada em 22 (12,2%) olhos de pacientes HBSS e 25 (22,3%) olhos de pacientes HBSC (OR=2.06; CI95%: 1.5-4.06, p=0. 022); essa frequência foi maior entre os pacientes HBSS com idade entre 20 - 39 anos, enquanto que nos pacientes HBSC foi maior nos acima de 40 anos (35.7% e 42.8%), decaindo abruptamente após essa idade. CONCLUSÃO: Retinopatia proliferativa foi descrita por volta dos dez anos de idade em ambos os grupos. A prevalência da retinopatia falciforme proliferativa pode resultar em cegueira e o conhecimento das alterações oculares mais prevalentes e idade de risco destas em pacientes com Doença Falciforme será importante para estabelecer um protocolo de acompanhamento oftalmológico, para prevenir um dano visual clinicamente grave, aumentando a qualidade de vida destes pacientes.

In order to determine the prevalence of ocular lesions in HTLV-1 infected patients in Salvador Bahia, a transversal study was conducted on 140 HTLV-1 infected patients (90 asymptomatic and 50 tropical spastic paraparesis/HTLV-1-associated myelopathy) between June 2004 and November 2005. The ophthalmological examination included visual acuity measurement, ocular motility, biomicroscopy of the anterior and posterior chambers, intraocular pressure and evaluation of lachrymal secretion. Observation verified 4 (2.8%) out of 140 patients with uveitis (two patients had intermediate uveitis and two had pan-uveitis) and 39 (36.4%) out of 107 patients with keratoconjunctivitis sicca. The prevalence of Keratoconjunctivitis sicca was significantly higher among the TSP/HAM patients (OR age adjusted=3.64; 95%CI 1.59-8.32). Uveitis and corneal opacities were also important findings, indicating the strong need for periodic ophthalmological follow-up in all HTLV-1 subjects.Com o objetivo de determinar a prevalência de lesões oculares, em portadores de HTLV-1 em Salvador, Bahia, foi realizado um estudo transversal em 140 pacientes (90 assintomático e 50 com paraparesia espática tropical/mielopatia associada ao vírus linfotrópico de células T humanas) entre junho de 2004 e novembro de 2005. O exame oftalmológico incluiu medida da acuidade visual, exame da motilidade ocular, biomicroscopia anterior e posterior, pressão intraocular e avaliação do filme lacrimal. Observamos 4.0 (2.8%) pacientes com uveites (dois com uveíte intermediária e dois com panuveíte) e 39 (36,4%) pacientes com ceratoconjuntivite seca. A prevalência de Ceratoconjuntivite seca foi significantemente mais elevada entre os pacientes com TSP/HAM (RC ajustada para idade = 3,64; IC 95% 1,59-8,32). As uveítes e opacidades corneanas foram também, patologias importantes, indicando a necessidade de acompanhamento oftalmológico periódico nos portadores de HTLV-1.

OBJETIVO: Descrever e comparar a percepção materna e a autopercepção da criança/adolescente do seu estado nutricional, identificando fatores associados a erro na percepção. MÉTODOS: Estudo transversal realizado em Salvador (BA) com 1.741 estudantes entre 6 e 19 anos, classificados de acordo com os percentis do índice de massa corporal (IMC) em baixo peso (IMC < p5), eutróficos (p5 ≤ IMC < p85), risco para sobrepeso (p85 ≤ IMC < p95) e sobrepeso (IMC > p95). Perguntas acerca da percepção do peso, padrão de atividade física e realização de dieta foram respondidas por alunos e genitoras. Foram avaliados, através de análise multivariada, fatores associados a erro na autopercepção e percepção materna. RESULTADOS: Houve acerto de 64,7% para a autopercepção e de 75,3% para a percepção das genitoras. O principal fator associado a erro na autopercepção foi a faixa etária entre 6 e 9 anos (OR = 1,59; IC95% 1,15-2,20). Entre as meninas, ter excesso de peso e fazer atividade física foram as características associadas à melhor percepção. Para os meninos, a presença de excesso de peso resultou em maior risco de erro na autopercepção. Entre as genitoras, filho com excesso de peso (OR = 3,02; IC95% 2,05-4,46) e faixa etária de 6 a 9 anos (OR = 1,88; IC95% 1,28-2,76) foram os fatores associados a erro na percepção. CONCLUSÃO: A falta de percepção adequada do peso ocorreu com muita freqüência entre as crianças e suas genitoras, em especial quando havia excesso de peso, fatores que podem representar obstáculos ao correto reconhecimento de alterações nutricionais.OBJECTIVE: To describe and compare maternal perception and the self-perception of children/adolescents of their nutritional status, identifying factors associated with incorrect perceptions. METHODS: Cross-sectional study carried out in Salvador, BA, Brazil with 1,741 students aged 6 to 19 years, classified according to body mass index (BMI) percentiles as underweight (BMI < p5), well-nourished (p5 ≤ BMI < p85), at risk of overweight (p85 ≤ BMI < p95) or overweight (BMI > p95). Students and their mothers answered questions on perception of weight, patterns of physical exercise and dieting. By means of multivariate analysis, factors associated with incorrect maternal and self- perceptions were analyzed. RESULTS: Self-perceptions were correct in 64.7% of cases and 75.3% of maternal perceptions were correct. The principal factor associated with incorrect self-perception was age between 6 and 9 years (OR = 1.59; 95%CI 1.15-2.20). Among girls, being overweight and practicing physical exercise were characteristics associated with better perception. For boys, the presence of overweight resulted in an increased risk of incorrect self-perception. Among mothers, having an overweight child (OR = 3.02; 95%CI 2.05-4.46) and a child aged from 6 to 9 years (OR = 1.88; 95%CI 1.28-2.76) were associated with incorrect perception. CONCLUSIONS: A failure to correctly perceive weight was very frequent among children and their mothers, especially when children were overweight. These factors could represent obstacles to correctly recognizing nutritional abnormalities.

O objetivo foi descrever as características sociodemográficas, epidemiológicas e comportamentais de mulheres infectadas (64) e não infectadas (66) pelo HTLV-1 em Salvador, Bahia. O diagnóstico sorológico foi obtido por ELISA, WB e Imunofluorescência. Dados epidemiológicos e sociodemográficos foram coletados utilizando questionário padronizado. O Qui-quadrado ou teste de Fisher foi usado para dados categóricos e ANOVA ou Kruskall Wallis (3 grupos) e teste T ou Mann Whitney (2 grupos) para os dados contínuos. As variáveis associadas foram ajustadas por regressão logística. Mais da metade (57,8%) das mulheres soropositivas eram assintomáticas. As sintomáticas (com PET/MAH) tinham menor escolaridade. A comparação entre mulheres soropositivas e soronegativas mostrou que a hemotransfusão, a prática de sexo anal, coitarca antes dos 18 anos e ter mais de 3 parceiros sexuais na vida foram fatores de risco para infecção pelo HTLV-1. A prevenção da transmissão sexual e vertical (aleitamento materno) deve ser reforçada e triagem no pré-natal mandatória.The objective was to describe the sociodemographic, epidemiological and behavioral characteristics of women infected with HTLV-1 (64) and uninfected women (66) in Salvador, Bahia. The serological diagnosis was obtained via Elisa, Western Blot and Immunofluorescence. Epidemiological and sociodemographic data were collected using a standardized questionnaire. The chi-squared or Fisher test was used for categorical data and ANOVA or Kruskal-Wallis (3 groups) and the T-test or Mann-Whitney (2 groups) were used for continuous data. Associated variables were adjusted using logistic regression. More than half (57.8%) of the seropositive women were asymptomatic. The symptomatic women (with HAM/TSP) had fewer years of education. Comparison between seronegative and seropositive women showed that blood transfusion, anal sex practices, first sexual intercourse before the age of 18 years and three or more sexual partners over women’s lifetime were risk factors for HTLV-1 infection. The prevention of both sexual transmission and vertical transmission (breastfeeding) should be reinforced. Prenatal screening is of paramount importance.

OBJETIVOS: descrever as características clínicas dos pacientes com hiperfenilalaninemia acompanhados no Serviço de Referência em Triagem Neonatal (SRTN) do estado da Bahia. MÉTODOS: estudo descritivo transversal, tendo como amostra todos os pacientes com diagnóstico conhecido de Hiperfenilalaninemia residentes no estado da Bahia e acompanhados no SRTN até setembro de 2005. Tal população é composta de 46 famílias, num total de 51 pacientes. A análise dos dados foi descritiva, incluindo medidas de tendência central e dispersão. RESULTADOS: houve discreto predomínio do gênero feminino (52,9%). A maioria dos pacientes (78,4%) teve seu diagnóstico estabelecido através da triagem neonatal, tendo, portanto, tratamento precoce. Consangüinidade foi registrada em 32,6% das famílias. A média de início do tratamento entre os pacientes diagnosticados pela triagem neonatal foi de 56,6 37,8 dias, enquanto que entre os pacientes com diagnóstico tardio, foi de 7,1 anos. CONCLUSÕES: o estudo descreve um grupo de pacientes representativo de uma patologia incluída no Programa Nacional de Triagem Neonatal (PNTN), sendo, portanto, de relevância para a saúde pública. Entre os dados clínicos, chama a atenção a média de idade do início do tratamento, superior ao recomendado na literatura, alertando para a necessidade de um maior enfoque no diagnóstico precoce.OBJECTIVES: to describe clinical aspects of hyper-phenylalaninemia in patients followed up on the Reference Service for Neonatal Screening (SRTN) in the state of Bahia. METHODS: cross-sectional study that enrolled all patients living in the state of Bahia and followed up on the SRTN who had a definite diagnosis of hyper-phenylalaninemia prior to September, 2005. That population was made up of 46 families, with 51 patients. The analysis of data was descriptive, using measures of central tendency and dispersion. RESULTS: a discrete predominance of females was found (52.9%). Most patients (78,4%) were diagnosed on neonatal screening, thus receiving early treatment. Consanguinity was registered in 32.6% of the families. The average age at the adoption of therapy was 56.6 37.8 days on the patients diagnosed on neonatal screening and 7.1 years among those with a late diagnosis. CONCLUSIONS: the relevance of this study to public health lies upon the fact that it describes clinical features of a group of patients with a disease included on the National Neonatal Screening Program. Among clinical data, we highlight the average age at the adoption of therapy, which is higher than the recommended by medical literature, stressing the need of reinforcing the importance of early diagnosis.

ABSTRACT Objective: To evaluate the candidate genes PAX-8, NKX2-5, TSH-R and HES-1 in 63 confirmed cases of thyroid dysgenesis. Subjects and methods: Characterization of patients with congenital hypothyroidism into specific subtypes of thyroid dysgenesis with hormone levels (TT4 and TSH), thyroid ultrasound and scintigraphy. DNA was extracted from peripheral blood leukocytes and the genetic analysis was realized by investigating the presence of mutations in the transcription factor genes involved in thyroid development. Results: No mutations were detected in any of the candidate genes. In situ thyroid gland represented 71.1% of all cases of permanent primary congenital hypothyroidism, followed by hypoplasia (9.6%), ectopia (78%), hemiagenesis (6.0%) and agenesis (5.5%). The highest neonatal screening TSH levels were in the agenesis group (p < 0.001). Conclusions: Thyroid dysgenesis is possibly a polygenic disorder and epigenetic factors could to be implicated in these pathogeneses.