Infertility is one of the major complications of obesity. Studies have shown that administration of leptin modulated the expression of Β-catenin in the ovary and reversed obesity-induced infertility in rats. Coenzyme Q10 (CoQ10), an antioxidant, supplies the energy used for ovulation, oocyte and embryo development and prevents DNA damage that causes infertility. We hypothesized that leptin when combined with CoQ10 could greatly improve fertility. Twenty-one female Sprague-Dawley rats were used in this study and divided into five treatments groups. Group I rats was fed rat chow diet (RCD) while groups II to V were fed High-fat diet (HFD) for 14 weeks to induce infertility. Group 1 RCD and group II HFD control rats received 1 ml of saline intraperitoneally (i.p.) twice daily for 2 days, group III HFD rats received 1 ml of 100 µg of leptin i.p. twice daily for 2 days, group IV HFD rats received 10 mg/kg of CoQ10 i.p. for 2 weeks plus saline twice daily for 2 days. Group V HFD rats received 1 ml of 100 µg of leptin i.p. twice daily for 2 days plus 10 mg/kg of CoQ1o i.p. for 2 weeks. Estrous cycle was checked daily and food intake and body weight measured twice weekly before and after treatments. Fourteen days post treatment, all the animals were sacrificed. The blood and tissues were collected for analysis. The results show a significant decrease in food intake and body weight and regular estrous cycle restored in groups III and V rats. There was significant (p < 0.05) increase in spleen weight in groups IV and V. FSH level increased significantly (p < 0.05) in the leptin plus CoQ10 treated group while CoQ10 level was increased significantly (p < 0.05) in the leptin-treated group. Β-catenin expression was decreased in group IV and V, suggesting that Β-catenin expression may be downregulated by COQ10 administration. These results indicate that synergistic action of leptin and CoQ10 could delay the onset of obesity-induced infertility exhibited by the reduction of food intake and body weight. In conclusion, combinations of CoQ10 with leptin can improve fertility in obese infertile female rats and could provide a novel therapeutic strategy for the treatment of female infertility.

Metabolic syndrome (MetS) is a major risk factor for cardiovascular disease yet it has been little studied in Hispanic children of rural areas. This pilot study aimed to estimate the prevalence of MetS and its components (high waist circumference (WC), elevated blood pressure (BP), high triglycerides (TRI), low high-density lipoprotein (HDL), and hyperglycemia) in a sample of Hispanic children aged 2 to 10 years from northeast Tennessee (TN). Between June and October 2015, 46 Hispanic children were recruited during their well-child visit at a community health center in Johnson City, TN. Anthropometric data, blood pressure readings, and a blood sample were collected. Descriptive statistics were used to estimate the prevalence of MetS and its components. Chi-squared test or Fisher’s exact test was used to test differences of proportions. Results showed that 41.3% of Hispanic children (mean age:6.8 years; SD:2.5) were overweight or obese (Body Mass index (BMI) for age and sex ≥85th percentile), 15.2% had a high WC (≥90th percentile for age and sex), 30.4% had elevated BP (systolic or diastolic BP for age, sex and height ≥90th percentile), 13.0% had low HDL (≤5th percentile for age and sex), and 45.6% had high TRI (≥95th percentile for age and sex). Overall, 17 (37%) children were negative for every component of MetS, 17 (37%) were positive for one component, 6 (13%) for two components, 5 (11%) for three components, and 1 (2%) for four components. The prevalence of MetS (≥3 components) was 13%. While the prevalence of MetS did not vary by sex, it tended to be higher (33.3%) in children aged 4 or younger than in 5 to 10 (12.1%) year olds (P=0.05). The prevalence of having 2 or more positivities for MetS was significantly higher among overweight/obese children than in children with lean weight (47.4% vs. 11.1%, P=0.03). Findings provide evidence that Hispanic children are at high risk for MetS. Prevention efforts should begin early and target children with elevated BMI.

Mass spectrometry has emerged as having a vital role in various applications to biochemical fields. In this thesis, we have utilized a variety of mass spectrometry techniques for both bacteriophage proteomics and colostrum and milk lipidomics studies. Our first study was the proteome characterization of Great Salt Lake bacteriophage NS01 with SDS-PAGE GEL to separate the viral proteins and high performance liquid chromatography (HPLC) coupled with an LTQ Orbitrap to identify the proteins after in-gel digestion. In this project, we have successfully identified 11 proteins with high confidence, p-values < 0.01, including coat protein gp88 with a coverage of 91% and tail protein gp86 with a coverage of 40.96%, which facilitated the classification of NS01 as a T7-like phage. Our second study was the discovery of colostrum and milk biomarkers that can be used to predict the likelihood of development of production-related metabolic diseases (PRMDs) in dairy cows through a lipidomics approach. In this study, an electrospray ionization, time-of-flight mass spectrometer was applied to lipid profiling, quantification and significant biomolecule selection. A Q-Star quadrupole, orthogonal time-of-flight mass spectrometer and an Agilent 6530 accurate-mass quadrupole/time-of flight mass spectrometer were both used for lipid biomarker fragmentation and identification. According to linear discriminative statistical modeling, three panels of biomarkers were defined. A combination of 2 milk lipid predictors, including DG18:0/18:0 and TG 18:0/18:0/18:1, provided PRMD predictions with 75.0% sensitivity at 90.0% specificity. A combination of 3 colostrum lipid predictors, including TG16:0/18:1/18:3, DG16:0/16:0 and C40H60NO, provided PRMD prediction with 90.0% sensitivity at 86.4% specificity. Furthermore, a combination of 7 colostrum and milk biomarkers, including calculated differences between 'shared' markers found to be significantly different in both colostrum and milk, provided a predictive sensitivity of 87.5% at a specificity of 100%. Thus, three panels of lipid biomarkers have been discovered in 1-4 day postparturient dairy cow colostrum and milk that can be used to predict resistance or susceptibility prior to onset of clinically apparent PRMDs. These novel lipids could be used as important diagnostic predictors in the future. Therefore, mass spectrometry based proteomics and lipidomics approaches have been efficient tools in the biochemical research described in this thesis.

CHARACTERIZATION OF STAPHYLOCOCCAL NUCLEASE AND TUDOR DOMAIN CONTAINING PROTEIN 1 (SND1) AS A MOLECULAR TARGET IN HEPATOCELLULAR CARCINOMA AND NON-ALCOHOLIC STEATOHEPATITIS Nidhi Jariwala, PhD A dissertation submitted in partial fulfillment of the requirements for the degree of Doctor of Philosophy in Integrative Life Sciences Virginia Commonwealth University, 2017 Devanand Sarkar, M.B.B.S., PhD. Associate Professor, Department of Human and Molecular Genetics Virginia Commonwealth University Richmond, Virginia SND1, a subunit of the miRNA regulatory complex RISC, has been implicated as an oncogene in hepatocellular carcinoma (HCC). Oncoprotein SND1 regulates gene expression at a post-transcriptional level in multiple cancers including hepatocellular carcinoma (HCC). In the present study, we characterize oncogenic functions of SND1 in HCC employing a novel transgenic mouse model (Alb/SND1) and present SND1 as a potential molecular target in HCC management. We show that Alb/SND1 mice develop spontaneous HCC with partial penetrance and exhibit more highly aggressive HCC induced by chemical carcinogenesis. Livers from Alb/SND1 mice exhibit a relative increase in inflammatory markers and spheroid-generating tumor initiating cells (TiC). Mechanistic investigations defined roles for Akt and NF-κB signaling pathways in promoting TiC formation in Alb/SND1 mice. Intravenous administration of the selective SND1 inhibitor 3', 5'-deoxythymidine bisphosphate (pdTp) inhibited tumor formation without effects on body weight or liver function. We conclude that SND1 drives pro-oncogenic transcriptomic and proteomic changes in hepatocyte resulting in aggressive HCC. SND1 specific RNA interactome is identified with RNA immunoprecipitation sequencing (RIPSeq) approach. With an adjusted p value of2-fold enrichment over control, 282 mRNAs were identified to significantly associate with SND1 protein. We focused on the tumor suppressor Protein Tyrosine Phosphatase non-receptor type 23 (PTPN23) because its regulation by SND1 and its role in HCC are not known. In current study, we confirm that SND1 post-transcriptionally downregulates PTPN23. Pursuing functional studies with tetracycline inducible overexpression system, we validate that PTPN23 inhibits tyrosine kinase signaling, proliferation, epithelial to mesenchymal transition, migration, invasion and in vivo tumorigenesis. Alb/SND1 mice also manifest steatosis and fibrosis at one year of age. Coupled with a pro-inflammatory hepatic phenotype, we conclude that Alb/SND1 livers present NASH. High fat diet causes severe NASH and aggressive NASH induced HCC in Alb/SND1 mice. Serum and hepatic lipid profiling shows that hepatocyte specific SND1 overexpression associate with elevated triglyceride and cholesterol LDL levels. Contrarily, hepatocyte specific deletion of SND1 (SND1ΔHEP) in vivo, significantly protects against age dependent steatosis. Association of SND1 in NASH pathology is novel discovery and we present preliminary evidence confirming role of SND1 in promoting NASH.

Throughout the years, women have been the focus of eating disorders and body image research. With women being the focus of research in these areas, disorders such as Anorexia Nervosa, Bulimia Nervosa, and Body Dysmorphic Disorder (BDD) have caught the eyes of researchers. With this new discovery, there has been a recent surge of research on body image issues and men. Originally termed reverse anorexia or bigorexia, Muscle Dysmorphia (MD) has gained the attention of researchers for about a decade (Grieve, 2007; Pope, Katz & Hudson, 1993; Pope, Gruber, Choi, Olivardia & Phillips, 1997). The Muscle Dysmorphia Inventory (MDI) was created to measure the symptoms of MD and provide clinicians with an inventory that would aid them in the diagnosis and treatment of MD (Short, 2005). The MDI is a 34-item scale that uses the etiological model proposed by Grieve (2007) and assesses or evaluates the four proposed diagnostic criteria of MD (Short, 2005). Short (2005) developed questions using the four criteria of MD outlined by Olivardia (2001). While the MDI has shown good internal consistency (Cronbach’s alpha = .87; Short, 2005), no other psychometric properties have been assessed. The goal of this study was to assess the validity and reliability of the MDI so that the accuracy of the measure will be known and it may be used as a diagnostic tool in clinical settings. The hypothesis of the study was that the MDI is a reliable and valid measure. This study assessed the reliability and validity of the MDI through a correlational design. Test-retest reliability and Cronbach’s alpha were used to identify the reliability of the MDI. The MDI was compared to tests that measure traits of MD and to self-reported behaviors, which assessed concurrent and predictive validity of the MDI. The MDI appears to be a good diagnostic tool for clinicians to use when looking at MD. This study examined the reliability and the validity of the MDI. This study shows that the MDI has adequate test-retest reliability, and good internal consistency. The results indicate that the MDI has good concurrent validity when compared to other tests that measure symptoms of MD. Under predictive validity, there was some support to suggest that the MDI can predict MD, however, the predictive validity of the MDI needs to be examined more in-depth. The MDI appears to measure more state symptoms of MD than trait symptoms. This may make it easier for clinicians to detect an emerging problem before it becomes harder for the individual to overcome.

Obesity is an ever-growing concern in the developed world that carries with it a plethora of health issues. For example, obesity increases an individual’s risk for Type 2 Diabetes and cardiovascular disease. Pregnancy is a vital time for a woman to maintain optimal health, both for her own benefit as well as that of her offspring, and yet almost half of women in the United States who are of age to bear children are overweight or obese. In mice, we found that offspring born to dams fed a high fat diet did not have impaired glucose tolerance, contrary to our hypothesis. In addition, we challenged the offspring with a high fat diet, and found no difference in glucose tolerance as a result of maternal diet. Exercise is at the opposite end of the wellness spectrum – individuals who exercise experience many health benefits. Even overweight or obese individuals who exercise without losing weight have improved insulin sensitivity, for example. Studies have previously used voluntary running and found that offspring born to exercised dams have improved glucose tolerance. With the goal of controlling variable running times and distances, we developed a novel model of controlled exercise and have shown that it is a safe intervention that warrants further study. In addition, many individuals choose to take dietary supplements for various reasons. Branched chain amino acids (BCAAs) are a common dietary supplement that have been shown to increase lean mass, and may be implicated in glucose metabolism. We supplemented female mice with BCAAs for 16 weeks and found that exercise plus BCAAs improved body composition compared to sedentary control-diet fed animals, when exercise alone did not. In summary, we herein explore a number of health behaviors in female mice, both negative treatments such as consumption of a high fat diet and positive interventions such as exercise and BCAA supplementation, and the impact that they may have on the female animal and/or her offspring.

Diss. (sammanfattning) Uppsala : Uppsala universitet, 2007. / Härtill 4 uppsatser.

By facilitating timely diagnosis and treatment initiation, population-wide newborn screening programs have led to important reductions in morbidity and mortality for many rare diseases, including medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency. Newborn screening has also expanded the spectrum of disease severity for MCAD and VLCAD deficiencies to include a higher proportion of milder cases, raising questions about appropriate disease management. To date there has been no systematic attempt to characterize best management practices in terms of the guidance that is available to those who provide care for MCAD and VCLAD deficiencies; nor has there been an attempt to understand the extent to which current practices align with such currently available guidance. The two projects that are part of this thesis sought to address these research gaps with a particular focus on two key disease-specific management practices we identified in advance as priorities: the use of carnitine supplementation and the recommended duration of fasting. The objective of the first project was to systematically review the quality and content of clinical practice guidelines and/or recommendations for the diagnosis and management of both MCAD and VLCAD deficiencies. Two independent reviewers assessed the eligibility of citations retrieved from a comprehensive search of the peer-reviewed and grey literature. We appraised the quality of the reviewed guidance and extracted information on the content of recommendations. From the 25 guidance documents that met our inclusion criteria, only 7 incorporated evidence reviews, indicating that guidance in this field does not generally meet established methodological standards for the rigorous development of clinical practice guidelines. With respect to content, we identified unclear and inconsistent recommendations regarding fasting times and the use of carnitine supplementation. Further empirical evidence in these areas is necessary to inform the development of future rigorous guidelines. The objective of the second project was to identify actual practices in the management of MCAD deficiency. We conducted a scoping review of published literature on treatment practices around the world and a secondary analysis of data documenting treatments received by participants in a Canadian pediatric cohort study. For the scoping review, citations retrieved from our comprehensive search strategy were screened by two independent reviewers. We extracted information on study characteristics and disease management. Our secondary analysis included longitudinal data for Canadian children with MCAD deficiency, born between 2006 and 2015 and enrolled in a cohort study at one of 13 centres. For both project components, we described carnitine supplementation and fasting times, overall and according to potential indicators of disease severity (genotype, biochemical phenotype). We identified 5 relevant publications in the scoping review and analyzed data for 107 children participating in the Canadian cohort. Management practices related to carnitine supplementation and fasting times for MCAD deficiency were highly variable based on both data sources. There was some evidence of an association between genotype and carnitine use, which, based on the scoping review, may be due to a relationship between genotype and carnitine deficiency. While actual practice was in some ways aligned with the guidance we reviewed in the first project, these results underscore the need for further evidence to address areas of uncertainty that have been prioritized by patients and families, clinicians, and health researchers, including questions regarding the potential to tailor treatment to predicted disease severity and an emphasis on controversial therapies such as carnitine supplementation.

Submitted by Ana Maria Fiscina Sampaio (fiscina@bahia.fiocruz.br) on 2016-06-21T16:51:44Z No. of bitstreams: 1 Roberta Souza Freitas Fatores associados... 2016.pdf: 1075660 bytes, checksum: a789e9068c829f9e0fce4cded6a358e6 (MD5) / Approved for entry into archive by Ana Maria Fiscina Sampaio (fiscina@bahia.fiocruz.br) on 2016-06-21T16:51:58Z (GMT) No. of bitstreams: 1 Roberta Souza Freitas Fatores associados... 2016.pdf: 1075660 bytes, checksum: a789e9068c829f9e0fce4cded6a358e6 (MD5) / Made available in DSpace on 2016-06-21T16:51:58Z (GMT). No. of bitstreams: 1 Roberta Souza Freitas Fatores associados... 2016.pdf: 1075660 bytes, checksum: a789e9068c829f9e0fce4cded6a358e6 (MD5) Previous issue date: 2016 / Made available in DSpace on 2016-07-22T14:09:07Z (GMT). No. of bitstreams: 3 Roberta Souza Freitas Fatores associados2016.pdf.txt: 162291 bytes, checksum: 3b09f9bee896b38be74dad496f96b13a (MD5) Roberta Souza Freitas Fatores associados2016.pdf: 1075660 bytes, checksum: a789e9068c829f9e0fce4cded6a358e6 (MD5) license.txt: 2991 bytes, checksum: 5a560609d32a3863062d77ff32785d58 (MD5) Previous issue date: 2016 / Fundação Gonçalo Moniz, Instituto de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil / Objetivo: Estimar a prevalência de cintura hipertrigliceridêmica (CH) dos participantes do Estudo Longitudinal da Saúde do Adulto (ELSA-Brasil), identificar fatores associados à CH e comparar com outros indicadores de risco cardiovascular e metabólico. Métodos: Dados da linha de base de uma coorte composta por servidores públicos. O fenótipo CH é definido pela presença simultânea de circunferência da cintura (CC) aumentada e hipertrigliceridemia em jejum. O indicador foi construído segundo pontos de corte da CC definidos pelo National Cholesterol Education Program (NCEP) e International Diabetes Federation (IDF). A associação entre as variáveis independentes e CH foi testada por meio de modelos de regressão logística multivariada. A CH foi comparada também com outros indicadores de risco cardiovascular e metabólico por meio de testes de correlação, índice Kappa, sensibilidade e especificidade. Resultados: Foram avaliados 12.811 funcionários, 54,2% do sexo feminino, a prevalência da CH-IDF foi de 24,7% e a da CH-NCEP de 13,3%. CH foi associado a consumir álcool de modo excessivo, ser ex-fumante, ter hipertensão, diabetes, HDL baixo, não-HDL alto e PCR aumentado, independente do sexo ou critério de definição. A CH também foi associada a auto percepção negativa do estado de saúde entre homens e mulheres classificadas por meio do critério NCEP; a prática de atividade física no tempo livre entre homens; a união atual ou passada entre mulheres com classificação de CH-IDF; e a cor/raça branca e ter seguro de saúde com CH-NCEP no sexo masculino. A síndrome metabólica apresentou maior correlação com CH entre os participantes de ambos os sexos. Conclusões: Em decorrência de suas associações e da correlação com síndrome metabólica, o indicador CH pode ser utilizado como ferramenta de triagem para indivíduos com risco cardiometabólico na prática clínica. / Objective: To estimate the prevalence of hypertriglyceridemic waist (HW) of participants in the Longitudinal Study of Adult Health (ELSA-Brasil), identify risk factors and compare with other cardiovascular and metabolic risk indicators. Methods: Baseline data from a cohort of civil servants. The CH phenotype was defined by the simultaneous presence of increased waist circumference (WC) and hypertriglyceridemia fasting. The indicator was built in cut-off points of the WC defined by the National Cholesterol Education Program (NCEP) and International Diabetes Federation (IDF). The association between the independent variables and HW were tested using hierarchical multivariate logistic regression models. The CH was compared with other cardiovascular and metabolic risk indicators through correlation tests, Kappa, sensitivity and specificity. Results: A total of 12.811 employees, 54,2% female, with a prevalence of HW-IDF was 24,7% and the HW-NCEP 13,3%. CH was associated with consuming alcohol excessively, being a former smoker, have high blood pressure, diabetes, low HDL, non-HDL high and increased CRP, regardless of sex or defining criterion. The CH was also associated with negative self-perception of health status between men and women classified by the NCEP criteria; physical activity during leisure time for males; the marital status in women for the HW-IDF classification; and color / race and health insurance in males for HW-NCEP. Metabolic syndrome was correlated to HW in both sexes. Conclusions: The HW can be used as a screening tool for patients with cardiometabolic risk in clinical practice once it was associated with these risks factors and it was correlated with metabolic syndrome.

Introdução: Beribéri é uma doença causada pela deficiência de tiamina (vitamina B1) que, apesar de facilmente tratável, pode levar a óbito. Há pelo menos setenta anos não se tinha referência de surtos de beribéri no Brasil. Objetivo: Descrever o perfil epidemiológico dos casos de beribéri e óbitos notificados no Estado do Maranhão. Método: Estudo descritivo-retrospectivo que analisou 1.207 casos notificados de beribéri e 40 óbitos ocorridos no Maranhão no período de 2006 a 2008. As informações foram obtidas do banco de dados da Vigilância Epidemiológica e fichas de notificação. Resultados: Os casos distribuíram-se em 434 (36,0%) em 2006, 551 (45,6%) em 2007 e 222 (18,4%) em 2008. Afetou dois terços das Unidades Regionais de Saúde e 26,3% dos municípios localizados nas regiões centro-oeste, noroeste e sudoeste do Estado. As notificações foram maiores nos meses de março a agosto, com pico em junho. O coeficiente de incidência no período foi de 4,32/10.000 hab. Homens foram mais acometidos (81,9%), com concentração de casos na faixa etária de 20-40 anos (57,0%). Predominou a ocorrência do beribéri seco (84,6%) e o tempo decorrido entre os primeiros sintomas e a notificação foi inferior a três meses para dois terços dos casos. A hospitalização ocorreu para 50% dos casos em 2006, 30% em 2007 e 15% em 2008. O consumo de álcool foi referido por 53,2% dos acometidos em 2006, com proporção discretamente menor nos anos subseqüentes, e o hábito de fumar por cerca de um terço dos acometidos. Sintomas mais comuns foram: diminuição da força, dormência e edema das pernas, dificuldade para caminhar e dor na panturrilha. Em relação à escolaridade, constatou-se que mais da metade (57,2%) tinha quatro anos ou menos de estudo, dois terços (66,2%) desempenhava atividade laboral pesada e 72,9% tinham renda familiar inferior a um salário mínimo. Foram registrados 40 óbitos (3,3% do total de casos notificados) apenas em 2006, concentrados no mês de junho (61,9%) e distribuídos em 21 municípios (9,7%). A taxa de mortalidade para o Estado foi de 0,45/10.000 hab. Praticamente a totalidade dos óbitos ocorreu no sexo masculino (97,5%), sendo 72,5% na faixa etária de 20-30 anos. Para dois terços, o tempo decorrido entre os primeiros sintomas e a notificação foi inferior a três meses e a hospitalização ocorreu para 42,5%. Hábito de consumir álcool e fumar foi elevado entre aqueles que foram a óbito, 75,0% e 66,7%, respectivamente. Conclusão: O estudo se destaca por abranger análise de todas as notificações de beribéri do estado do Maranhão. Constatou-se que os casos e óbitos apresentaram distribuição espacial e temporal relacionado à realidade do estado. A ausência de óbitos em 2007 e 2008 e redução dos casos em 2008 sugerem efeitos positivos das ações de enfretamento governamentais implementadas. Esta avaliação descritiva é importante para nortear as atividades e áreas a serem priorizadas no planejamento das ações de combate, tanto no nível central (federal, estadual e municipal), como para os profissionais da atenção básica e especializada, no direcionamento de suas práticas. Os resultados contribuem também para o delineamento de outras pesquisas que possam auxiliar no estudo da determinação do beribéri no Brasil. / Introduction: Beriberi is a disease caused by a deficiency of thiamine (vitamin B1) which, although easily treatable, may lead to death. There had been no register of beriberi outbreaks in Brazil for at least seventy years. Objective: To describe the epidemiological profile of reported beriberi cases in the State of Maranhão. Method: A descriptive retrospective study of 1,207 reported beriberi cases and 40 deaths in the State of Maranhão from 2006 to 2008. The information was obtained from the database of Epidemiological Surveillance and the reporting forms. Results: The cases were distributed in 434 (36.0%) in 2006, 551 (45.6%) in 2007 and 222 (18.4%) in 2008. It affected two thirds of the Regional Health Units and 26.3% of the municipalities located in the Central-Western, Northwestern and Southwestern of the State. The notifications were higher in the months from March to August, peaking in June. The incidence rate in the period was 4.32 / 10,000 inhabitants. Men were more affected (81.9%), with more concentration of cases in the age group 20-40 years (57.0%). Dry beriberi was predominant (84.6%) and the elapsed time between first symptoms and reporting was less than three months in two thirds of cases. Hospitalization occurred for 50.0% of cases in 2006, 30.0% in 2007 and 15.0% in 2008. Alcohol consumption was reported by 53.2% of patients in 2006 and slightly lower proportion in subsequent years, and smoking was reported for about one-third of patients. Main symptoms were loss of strength, numbness and swelling of the legs, difficulty in walking and calf pain. Regarding education it was found that more than half (57.2%) had four or fewer years of study, two thirds (66.2%) performed heavy labor activity and 72.9% had a family income lower than minimum wage. Forty deaths were recorded (3.3% of all reported cases) only in 2006, concentrated in the month of June (61.9%) and distributed in 21 municipalities (9.7%). The mortality rate for the State was 0.45 per 10,000 inhabitants. Almost all the deaths occurred in males (97.5%), and 72.5% aged 20-30 years. For two-thirds of the patients who died, the elapsed time between first symptoms and reporting was less than three months and hospitalization occurred in 42.5%. Alcohol consumption and smoking were higher among those who died, 75.0% and 66.7% respectively. Conclusion: This study stands out for including analysis of all reported beriberi cases in the State of Maranhão. It was found that cases and deaths showed spatial and temporal distribution related to the social reality of the State. The absence of deaths in 2007 and 2008 and reduction of cases in 2008 suggest positive effects of the implemented governmental actions. This descriptive evaluation is important to guide the activities and areas to be prioritized in the planning of actions at the central level (federal, state and municipal), as well as for guiding the practice of primary and specialized healthcare professionals. The results also contribute to the design of other research that may support the study of beriberi in Brazil.