Submitted by Renata Lopes (renatasil82@gmail.com) on 2016-09-26T15:01:16Z No. of bitstreams: 1 querenhapuquedecarvalho.pdf: 2151188 bytes, checksum: 46edf6e6c2cee8d6e5ff0064b7460f3d (MD5) / Approved for entry into archive by Diamantino Mayra (mayra.diamantino@ufjf.edu.br) on 2016-09-26T20:34:17Z (GMT) No. of bitstreams: 1 querenhapuquedecarvalho.pdf: 2151188 bytes, checksum: 46edf6e6c2cee8d6e5ff0064b7460f3d (MD5) / Made available in DSpace on 2016-09-26T20:34:17Z (GMT). No. of bitstreams: 1 querenhapuquedecarvalho.pdf: 2151188 bytes, checksum: 46edf6e6c2cee8d6e5ff0064b7460f3d (MD5) Previous issue date: 2016-06-21 / CAPES - Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / O crescimento da população idosa é um fenômeno mundial, e no Brasil, vem ocorrendo de forma acelerada. Atrelada a essa rapidez com que o processo de transição demográfica e epidemiológica vem ocorrendo, há uma série de questões cruciais para gestores e pesquisadores no que tange ao sistema de saúde, uma vez que, há uma crescente demanda por serviços de saúde, principalmente pela prevalência de Doenças Crônicas Não Transmissíveis (DCNT) nesta população. Nesse contexto, dentre os principais problemas que acometerão os idosos, está a demência, que apresenta incidência anual crescente com o envelhecimento, e associada à mesma está o déficit cognitivo progressivo, comumente observado no processo de envelhecimento natural e que representa um significativo problema de saúde pública. Nessa perspectiva, o presente trabalho tem por objetivo analisar a função cognitiva e fatores associados às transições dos perfis cognitivos em idosos residentes da Zona Norte da cidade de Juiz de Fora – MG, situada na Zona da Mata Mineira, por meio de um estudo de painel. Trata-se de um estudo longitudinal, realizado através de inquérito domiciliar, com duas coletas de dados realizadas no ano de 2010 e 2014/2015, respectivamente. O teste utilizado para avaliar a função cognitiva foi o Mini Exame do Estado Mental, teste mais utilizado em estudos que buscam avaliar alterações de nível cognitivo. A coorte foi composta por indivíduos com 60 anos ou mais de idade, de ambos os sexos, não institucionalizados, selecionados através de amostragem aleatória estratificada e por conglomerado, com base no tipo de cobertura assistencial ofertada pelo Sistema Único de Saúde. Para a análise dos dados, os mesmos foram processados por meio do software Statistical Package for Social Sciences® (SPSS) versão 17.0, e submetidos à análise descritiva bivariada e a análises de regressão logística multivariada. Para estas análises, utilizou-se a técnica de retirada uma a uma das variáveis com base no nível de significância (α<0,05). Como resultados, o painel apresenta maioria do sexo feminino (67,1%), 51,8% são casados ou têm união estável, 55,7% têm entre 1 a 4 anos de escolaridade e 60,5% pertencem a classe socioeconômica C. Com relação aos aspectos cognitivos, 72,4 é a idade média para ambos os sexos, proporcionalmente os homens apresentam maior comprometimento cognitivo (26,6%) do que as mulheres (18,5%) e 26,9% destes não frequentaram a escola. De acordo com as análises de regressão logística multivariada, a probabilidade de um idoso estar com comprometimento cognitivo na 2ª fase do estudo, ou seja, após 4 anos, é maior dentre os idosos com idade mais alta, de classe socioeconômica baixa ou alta, com autoavaliação da saúde e da visão insatisfatória. Dessa forma, os resultados deste estudo colaboram para um melhor entendimento dos possíveis fatores de predição à mudança do perfil cognitivo em idosos e com isso, ações de prevenção, promoção e recuperação de saúde, poderão ser tomadas com o intuito de reduzir possíveis danos advindos com o processo de envelhecimento e consequentemente, dos processos demenciais. / The growth of the elderly population is a worldwide phenomenon, and in Brazil, has been taking place in a very accelerated way. Linked to this fast growth is the epidemiologic demographic transition process which has been ocurring, and several crucial questions that managers and researchers must face about the health system, since there is an increasing demand on health services, mainly because of the prevalence of Chronic Noncommunicable diseases (CNCD) in the elderly population. In this context, within the main problems that will affect the elderly, it is dementia, that presents an annual incidence that increases markedly with age, and associated with dementia is the progressive cognitive deficit, commonly observed on the natural aging process and representing a significant problem of public health. In this perspective, the present research aims to evaluate the Cognitive Deficit and factors associated with transitions of cognitive profiles in elderly residents of the northern sector of Juiz de Fora – MG, located in the Zona da Mata Mineira, through a panel study. It is a longitudinal study, conducted through a household survey, with two data collections made in the years 2010 and 2014/1015, respectively. The test used to evaluate the Cognitive Impairment was the Mini-Mental State Examination, the most widely used test in studies that seek to evaluate cognitive level changes. The study cohort consisted of subjects aged 60 years or older, of both sexes, not institutionalized, selected through cluster sampling, based on the type of health care coverage offered by the United Health System. The collected data were entered into a database and analyzed by the software Statistical Package for the Social Sciences®17.0 (SPSS) version 17.0, and submitted to bivariate descriptive statistics and both binomial and multivariate logistic regression analysis. For these analyzes, we used the technique of removing variables one by one based on significance level (α<0,05). The panel is configured by the majority of women (67,1 %) , 51,8 % are married or have stable, 55,7 % have between 1 to 4 years of schooling and 60,5 % belong to socioeconomic class C. With respect to the cognitive aspects, 72,4 is the average age for both sexes in proportion men show greater cognitive impairment (26,6%) than women (18,5%) and 26,9 % these did not attend school. According to the Multivariate Logistic Regression Analysis results, the probability of an elderly living with cognitive impairment in the 2nd phase of the study, ie after 4 years, are higher among older people with older age, low or high socioeconomic class, with poor self-rated health and self-rated poor vision. Thus, the results of this study contribute for a better understanding of the possible predictive factors for the change in cognitive status in older adults and thus, prevention, health promotion and recovery, they may be taken in order to reduce possible damages arising with the aging process and consequently with dementia processes.

Made available in DSpace on 2014-08-20T14:30:16Z (GMT). No. of bitstreams: 1 Dissertacao_Marcos Britto Correa.pdf: 513868 bytes, checksum: 6b1c2a81e71b671e7afec86e13b96b8a (MD5) Previous issue date: 2009-02-19 / This study aimed to investigate the prevalence of use and needs of dental prosthesis at age 24 and determinants of needs of dental prosthesis in this population. The study was nested in a population-based birth cohort started in 1982 in Pelotas, Brazil. A representative sample of 720 young adults was evaluated. Dental examinations and interviews were performed at 15 and 24 years old. Use and need of prosthesis was the outcome. Exploratory variables included demographic and socioeconomic (at birth, 15 and 24 years old), oral health and dental service utilization patterns (at ages 15 and 24). Prevalence of outcomes was calculated and Poisson Regression was used to determine risk factors to needs of dental prosthesis. The prevalence of use and needs of dental prosthesis was 2.08% and 29.72%, respectively. Multivariable analysis showed that poorest family socioeconomic trajectory [PR= 1.56 IC95% (1.08-2.26)], lower mother s educational level at children birth (p=0.023), lower family income at children birth [RP= 1.37 (1.01-1.86)], no oral hygiene instruction by the dentist [RP=1.64 IC95% (1.11-2.41)] and caries presence (high DMFT tertile) (p<0,001) were associated with prosthetic treatment needs. The results of this study support the hypothesis that socieconomic, behavioral and clinical determinants were associated to the need of dental prosthesis / O objetivo do presente estudo foi verificar a prevalência do uso e da necessidade de prótese dentária em uma população de adultos jovens e avaliar sua associação com a trajetória econômica das famílias, as condições de saúde bucal e de utilização de serviços odontológicos ao longo da vida do indivíduo. Em 1982, todos os nascimentos hospitalares ocorridos em Pelotas foram identificados e estes indivíduos foram acompanhados por inúmeras vezes. Em 2006, uma amostra de 720 indivíduos pertencentes a esta coorte foi visitada para realização de exame bucal e aplicação de questionário incluindo questões de uso de serviços odontológicos, hábitos e comportamentos relativos à saúde bucal. Foi calculada a prevalência dos desfechos e utilizada Regressão de Poisson para estimar os fatores de risco à necessidade de prótese. A prevalência de necessidade e uso de prótese foi de 29,7% e 2,1% respectivamente. A análise multivariável mostrou que a trajetória de pobreza sempre ao longo da vida [RP= 1,56 IC95% (1,08-2,26)], a baixa escolaridade materna ao nascer (p=0,023), a baixa renda familiar ao nascimento [RP= 1,37 (1,01-1,86)], a não recepção de instrução de higiene oral por parte do dentista aos 15 anos [RP=1,64 IC95% (1,11-2,41)] e a presença de cárie pelo CPO-D (p<0,001) foram associadas à necessidade de prótese aos 24 anos. Os resultados deste estudo suportam a hipótese que fatores socioeconômicos, comportamentais e determinantes clínicos estão associados a necessidade de prótese dentária

Abstract Low back pain (LBP) is a common condition already in adolescence and seems to predict future symptoms. Most of the previous longitudinal studies on LBP have been conducted in adult populations. Among adolescents, LBP has been linked most convincingly to psychosocial factors and smoking. We are not aware of any previous studies on clustering of potential risk factors in adolescent LBP. The main objective of this study was to analyze the potential associations of mainly modifiable psychosocial and lifestyle factors with LBP in adolescence. The factors evaluated were overweight, smoking, physical workload, family’s socioeconomic status, and the co-occurrence of psychosocial and lifestyle factors. The present study used the database of the 16- to 18-year-old adolescent subcohort, Oulu Back Study (n=1,987), of the Northern Finland Birth Cohort 1986. Moreover, we evaluated whether the associations differ depending on the presence of LBP symptoms at baseline of the two-year follow-up period. LBP was common among cohort adolescents as, on average, every second adolescent reported LBP, girls reporting more symptoms. As a whole, more associations were found among girls than boys. Overweight was associated with new LBP in the follow-up, but the association was not strong. Prolonged habitual smoking was associated with LBP, particularly with symptoms at baseline and follow-up. High exposure to awkward trunk postures and an overall physically demanding job were especially associated with new LBP in the follow-up. Family’s socioeconomic status was not consistently associated with LBP. At 16 years, four latent class clusters of psychosocial and lifestyle factors were found in both genders. Clusters characterized by behavioural problems were associated with LBP among both genders. Especially girls with the combination of emotional and behavioural problems were at the greatest risk of seeking care for new LBP. The results of this study indicate that LBP in adolescence is associated with both psychosocial and lifestyle factors. The information obtained may be utilized for improving preventive measures and individual care for adolescent LBP. / Tiivistelmä Alaselkäkipu on yleistä jo nuorilla, ja varhain koetut oireet ennustavat myöhempiä selkäkipuoireita. Suurin osa alaselkäkivun seurantatutkimuksista on tehty aikuisväestöllä. Nuorten alaselkäkivun on esitetty vakuuttavimmin liittyvän psykososiaalisiin ongelmiin ja tupakointiin. Alaselkäkipuun liittyvien tekijöiden mahdollista kasautumista ei ole nuorilla tutkittu aiemmin. Tämän väitöstutkimuksen tarkoituksena oli selvittää, ovatko pääasiassa muunneltavissa olevat psykososiaaliset ja elämäntapatekijät yhteydessä nuoruudessa esiintyvään alaselkäkipuun. Arvioitavina tekijöinä olivat ylipaino, tupakointi, fyysinen työkuormitus, sosioekonomiset tekijät, sekä psykososiaalisten ja elämäntapatekijöiden kasautuminen. Tutkimusaineisto koostui Pohjois-Suomen syntymäkohortti 1986:n osaotoksesta (Oulun selkätutkimus), johon kuului 1987 16–18-vuotiasta nuorta. Mahdollisia yhteyksiä arvioitiin erikseen sen mukaan, raportoivatko nuoret seurannan alussa alaselkäkipuoireita vai ei. Alaselkäkipu oli nuorilla yleistä, ja tytöillä yleisempää kuin pojilla. Keskimäärin joka toinen nuori raportoi oireita. Tutkittujen tekijöiden ja alaselkäkivun välisiä yhteyksiä todettiin selvemmin tytöillä. Ylipaino ennusti seurannassa ilmaantuvaa alaselkäkipua, mutta yhteys ei ollut vahva. Säännöllinen tupakointi oli yhteydessä alaselkäkipuun ja etenkin toistuviin oireisiin. Tutkituista työkuormitustekijöistä etenkin hankalat työasennot ja fyysisesti monella tapaa raskaaksi luokiteltava työ altistivat seurannassa uudelle alaselkäkivulle. Sosioekonomiset tekijät eivät olleet selkeästi yhteydessä alaselkäkipuun. Latenttien luokkien analyysi tunnisti seurannan alkuvaiheessa neljä erilaista ryhmää (klusteria) psykososiaalisten ja elämäntapatekijöiden perusteella molemmilla sukupuolilla. Sekä tytöillä että pojilla alaselkäkipua esiintyi erityisesti niissä ryhmissä, joissa esiintyi runsaasti käyttäytymishäiriöitä. Tunne-elämän ja käyttäytymisen ongelmat ennustivat alaselkäkivun vuoksi hoitoon hakeutumista tytöillä. Tulosten perusteella nuorten alaselkäkipuun liittyy sekä psykososiaalisia että elämäntapatekijöitä. Tuloksia voidaan hyödyntää alaselkäkivun ennaltaehkäisyn ja nuorten yksilöllisen hoidon suunnittelussa.

Abstract High risk human papilloma virus (hrHPV) infection is a necessary but not a sufficient cause of cervical cancer. In Finland, since 1990 the incidence of cervical cancer has increased among women younger than 40 years of age despite a nationwide screening programme. In this thesis, the overall objective is to address the role of possible, earlier hrHPV epidemic in this increased incidence of cervical cancer. The target population includes all fertile-aged women in Finland during 1983–2006. The actual study population comprised all women with a minimum of two pregnancies within five years and under 32 years of age in 1983–1997 and under 29 years of age in 1995–2003 identified from the Finnish Maternity Cohort (FMC). From this subpopulation, two subcohorts were selected for hrHPV antibody analysis by random sampling stratified by age and calendar time. All cases of cervical cancer diagnosed for women under 50 years of age during 1983–2002 and 1986–2006 were identified from the Finnish Cancer Registry. The case-cohort design, used for estimating population attributable fractions (PAF) associated with hrHPV, included the cases of cervical cancer and the first subcohort of FMC. A steady annual increase of 0.7% per year in the incidence of HPV16 was estimated to have taken place in Finland from 1983 to 1997 among the 23–31-year-old women with at least two pregnancies. The estimated seroprevalence of HPV16 increased from 17% to 24%, respectively. The PAF of hrHPV exposures in squamous cell carcinoma of the uterine cervix (SCC) was estimated as 73% (95% CI: 13% to 93%). For 26–31-year-old women born in the 1960s and 1970s the incidence of SCC was roughly double compared with women born in the late 1950s. Mathematical modelling indicated that changes in the sexual behaviour partly accounted for the increase seen in the incidence of cervical cancer in the 1990s. The findings of this thesis indicate that growth in the background exposure to HPV16 preceded the increase of incidence of cervical cancer in Finland. At younger birth cohorts, the increase of the incidence of SCC is visible among fertile-aged women in Finland. Whether overall screening starting at 25 years of age, higher participation rate for cervical screening or HPV vaccination of early adolescents is the future solution to lowering the incidence of cervical cancer among young women remains to be seen. / Tiivistelmä Ihmisen papilloomaviruksen (HPV), erityisesti korkean riskin tyypin (hrHPV), aiheuttama infektio on kohdunkaulan syövän välttämätön, mutta ei riittävä syytekijä. Suomessa vuoden 1990 jälkeen kohdunkaulan syövän ilmaantuvuus on valtakunnallisesta seulonnasta huolimatta noussut alle 40-vuotiailla naisilla. Tämän väitöskirjan tavoitteena on osoittaa, mikä rooli mahdollisella aiemmalla hrHPV-epidemialla on kyseiseen kohdunkaulan syövän ilmaantuvuuden kasvuun. Tutkimuksen kohdeväestöön kuuluvat kaikki lisääntymisikäiset suomalaiset naiset. Varsinainen tutkimusväestö koostui kaikista vuosina 1983–97 alle 32-vuotiaana ja vuosina 1995–2003 alle 29-vuotiaana kaksi kertaa raskaana olleista naisista, jotka identifioitiin Suomen äitikohortista (FMC). Tästä joukosta valittiin satunnaisotannalla kaksi alikohorttia hrHPV-laboratorioanalyysejä varten. Kaikki vuosina 1983–2002 ja 1986–2006 kohdunkaulan syöpädiagnoosin alle 50-vuotiaana saaneet naiset poimittiin Suomen syöpärekisteristä. Tapaus-kohorttiasetelma, jota käytettiin hrHPV altistukseen liittyvien väestösyyosuuksien (PAF) estimoinnissa, sisälsi kohdunkaulan syöpätapaukset ja ensimmäisen alikohortin. Suomalaisten 23–31 -vuotiaiden, vähintään kahdesti raskaana olleiden, naisten vuosittainen HPV16-ilmaantuvuus kasvoi tasaisesti 0.7&#160;% per vuosi ajanjaksolla 1983–1997. Vastaavasti HPV16:n vallitsevuus kasvoi 17 prosentista 24 prosenttiin. Kohdunkaulan levyepiteelisyövän hrHPV-altistukseen liittyvän PAF:n estimoitiin olevan 73&#160;% (95&#160;%:n luottamusväli 13–93&#160;%). Levyepiteelisyövän ilmaantuvuus oli suunnilleen kaksinkertainen 1960- ja 1970-luvulla syntyneillä naisilla, heidän ollessaan 26–31 -vuotiaita, verrattuna 1950-luvulla syntyneisiin samanikäisiin naisiin. Matemaattisen mallinnuksen tulosten perusteella kohdunkaulan syövän ilmaantuvuuden nousu 1990- luvulla selittyy ainakin osittain sukupuolikäyttäytymisen muutoksilla. Tämän väitöskirjan tulokset osoittavat, että kasvanut HPV16-virukselle altistuminen edelsi kohdunkaulan syövän ilmaantuvuuden nousua Suomessa. Levyepiteelisyövän ilmaantuvuuden nousu nuorimmissa syntymäkohorteissa on nähtävissä lisääntymisikäisillä naisilla Suomessa. Tulevaisuudessa nähdään, onko seulonnan aloittaminen 25-vuotiaana, korkeampi seulontaan osallistumisosuus vai nuorten aikuisten HPV-rokottaminen ratkaisu nuorten naisten kohdunkaulan syövän ilmaantuvuuden vähentämiseksi.

Background: Reported rates of bipolar syndromes are highly variable between studies because of age differences, differences in diagnostic criteria, or restriction of sampling to clinical contacts. Method: In 1395 adolescents aged 14–17 years, DSM-IV (hypo)manic episodes (manic and hypomanic episodes combined), use of mental health care, and five ordinal subcategories representing the underlying continuous score of (hypo)manic symptoms (‘mania symptom scale’) were measured at baseline and approximately 1.5, 4 and 10 years later using the Munich-Composite International Diagnostic Interview (DIA-X/M-CIDI). Results: Incidence rates (IRs) of both (hypo)manic episodes and (hypo)manic symptoms (at least one DSM-IV core symptom) were far higher (714/105 person-years and 1720/105 person-years respectively) than traditional estimates. In addition, the risk of developing (hypo)manic episodes was very low after the age of 21 years [hazard ratio (HR) 0.031, 95% confidence interval (CI) 0.0050–0.19], independent of childhood disorders such as attention deficit hyperactivity disorder (ADHD). Most individuals with hypomanic and manic episodes were never in care (87% and 62% respectively) and not presenting co-morbid depressive episodes (69% and 60% respectively). The probability of mental health care increased linearly with the number of symptoms on the mania symptom scale. The incidence of the bipolar categories, in particular at the level of clinical morbidity, was strongly associated with previous childhood disorders and male sex. Conclusions: This study showed, for the first time, that experiencing (hypo)manic symptoms is a common adolescent phenomenon that infrequently predicts mental health care use. The findings suggest that the onset of bipolar disorder can be elucidated by studying the pathway from non-pathological behavioural expression to dysfunction and need for care.

No / OBJECTIVE: To describe the growth pattern from birth to 2 years of UK-born white British and Pakistani infants. DESIGN: Birth cohort. SETTING: Bradford, UK. PARTICIPANTS: 314 white British boys, 383 Pakistani boys, 328 white British girls and 409 Pakistani girls. MAIN OUTCOME MEASURES: Weight and length trajectories based on repeat measurements from birth to 2 years. RESULTS: Linear spline multilevel models for weight and length with knot points at 4 and 9 months fitted the data well. At birth Pakistani boys were 210 g lighter (95% CI -290 to -120) and 0.5 cm shorter (-1.04 to 0.02) and Pakistani girls were 180 g lighter (-260 to -100) and 0.5 cm shorter (-0.91 to -0.03) than white British boys and girls, respectively. Pakistani infants gained length faster than white British infants between 0 and 4 months (+0.3 cm/month (0.1 to 0.5) for boys and +0.4 cm/month (0.2 to 0.6) for girls) and gained more weight per month between 9 and 24 months (+10 g/month (0 to 30) for boys and +30 g/month (20 to 40) for girls). Adjustment for maternal height attenuated ethnic differences in weight and length at birth, but not in postnatal growth. Adjustment for other confounders did not explain differences in any outcomes. CONCLUSIONS: Pakistani infants were lighter and had shorter predicted mean length at birth than white British infants, but gained weight and length quicker in infancy. By age 2 years both ethnic groups had similar weight, but Pakistani infants were on average taller than white British infants.

No / The use of biomarkers of early genetic effects, predictive for cancer, such as micronuclei (MN) in lymphocytes, may help to investigate the association between diet and cancer. We hypothesised that the presence of mutagens in the diet may increase MN formation. A 'pooled' standardised analysis was performed by applying the same experimental protocol for the cytokinesis block micronucleus assay in 625 young healthy women after delivery from five European study populations (Greece, Denmark, UK, Spain and Norway). We assessed MN frequencies in mono- and binucleated T-lymphocytes (MNMONO and MNBN) and the cytokinesis blocked proliferation index using a semi-automated image analysis system. Food frequency questionnaires (FFQs) were used to estimate intake of fatty acids and a broad range of immunotoxic and genotoxic/carcinogenic compounds through the diet. Pooled difference based on delivery type revealed higher MNMONO frequencies in caesarean than in vaginal delivery (P = 0.002). Statistical analysis showed a decrease in MNMONO frequencies with increasing calculated omega-6 PUFA concentrations and a decrease in MNBN frequencies with increasing calculated omega-3 PUFA concentrations. The expected toxic compounds estimated by FFQs were not associated with MN formation in mothers after delivery. In pregnant women, an omega-3 and -6 rich diet estimated by FFQ is associated with lower MN formation during pregnancy and delivery.

No / Palliative care in the UK has been developed to meet the needs of predominantly middle aged and younger old people with cancer. Few data are available regarding the extent to which services respond to the specific needs of an older group of people with other illnesses. This paper draws on in-depth interviews conducted with 40 people (median age 77) with advanced heart failure and poor prognosis to explore the extent to which older people's views and concerns about dying are consistent with the prevalent model of the 'good death' underpinning palliative care delivery. That prevalent model is identified as the "revivalist" good death. Our findings indicate that older people's views of a 'good death' often conflict with the values upon which palliative care is predicated. For example, in line with previous research, many participants did not want an open awareness of death preceded by acknowledgement of the potential imminence of dying. Similarly, concepts of autonomy and individuality appeared alien to most. Indeed, whilst there was evidence that palliative care could help improve the end of life experiences of older people, for example in initiating discussions around death and dying, the translation of other aspects of specialist palliative care philosophy appear more problematic. Ultimately, the study identified that improving the end of life experiences of older people must involve addressing the problematised nature of ageing and old age within contemporary society, whilst recognising the cohort and cultural effects that influence attitudes to death and dying.

Introdução: Estudos avaliando a prevalência de síndrome de Evans (SE) no lúpus eritematoso sistêmico juvenil (LESJ) bem como possíveis fatores associados são restritos a poucos relatos de caso. Objetivos: Avaliar a prevalência de SE em uma grande população de LESJ, assim como sua possível associação com dados demográficos, manifestações clínicas, características laboratoriais, atividade/dano cumulativo da doença e tratamento. Métodos: Um estudo de coorte multicêntrico retrospectivo foi realizado em 10 serviços de Reumatologia Pediátrica provenientes do Grupo Brasileiro de Lúpus e incluiu 850 pacientes com LESJ. SE foi avaliada ao diagnóstico do LES e definida pela combinação de púrpura trombocitopênica autoimune (PTI) e anemia hemolítica autoimune (AHAI). Os pacientes foram divididos em dois grupos para a avaliação das associações propostas: pacientes que apresentaram SE e pacientes sem SE. Todos foram avaliados ao diagnóstico do LES. Resultados: SE foi observada em 11/850 pacientes de LESJ ao diagnostico (1,3%). A maioria deles tinha doença ativa (82%) e apresentaram manifestações hemorrágicas (58%). Todos os pacientes com SE foram hospitalizados e não houve nenhum óbito. As comparações entre pacientes LESJ com e sem SE ao diagnóstico demonstrou frequências similares do sexo feminino, envolvimento de múltiplos órgãos, perfil de auto-anticorpos semelhantes e complemento baixo (p > 0,05). Pacientes com SE tinham frequências menores de eritema malar (9% vs. 53%, p=0,003) e envolvimento músculo-esquelético (18% vs. 69%, p=0,001) do que aqueles sem esta complicação. A frequência de pulsoterapia com metilprednisolona (82% vs. 43%, p=0,013) e uso de gamaglobulina endovenosa (64% vs. 3%), p < 0,0001) foram significativamente maiores no grupo com SE, com dose atual de prednisona semelhante entre os dois grupos [1,1 (0,76-1,5) vs. 1,0 (0-30) mg/kg/dia, p=0,195]. Conclusões: Este foi o primeiro estudo que evidenciou a possível relação de SE como uma manifestação inicial rara e grave do LESJ, porém com bom prognóstico. O diagnóstico se torna o principal desafio devido à falta de sinais e sintomas característicos de lúpus e a dificuldade de se excluir diagnósticos diferenciais como infecção e imunodeficiência primária / Introduction: Studies evaluating the prevalence of Evans Syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) as well as possible associated factors has been rarely reported and restricted to case reports. Objectives: To evaluate the prevalence of ES in a large population of cSLE, and the association with demographic data, clinical manifestations, laboratory characteristics, disease activity, cumulative damage, and treatment. Methods: A retrospective multicenter cohort study was performed in 10 Pediatric Rheumatology services and included 850 patients with cSLE. ES was evaluated at the diagnosis of cSLE and defined as the combination of autoimmune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA). The patients were divided into two groups for the evaluation of the proposed associations: patients who presented ES and patients without ES. All were assessed at the cSLE diagnosis. Results: ES was observed in 11/850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (58%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multi-organ involvement, autoantibodies profile and low complement (p > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, p=0.003) and musculoskeletal involvement (18% vs. 69%, p=0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, p=0.013) and intravenous immunoglobulin use (64% vs. 3%, p < 0.0001) were significantly higher in the former group, with similar current prednisone dose between groups [1.1 (0.76-1.5) vs. 1.0 mg/kg/day (0-30), 0.195]. Conclusions: This was the first study that evidenced the possible relationship of ES as a rare and severe initial manifestation of cSLE, but with a good prognosis. Diagnosis becomes the main challenge due to the lack of signs and symptoms characteristic of lupus and the difficulty of excluding differential diagnoses such as infection and primary immunodeficiency

Introdução: As infecções são importantes causas de morbidade e mortalidade em pacientes com lúpus eritematoso sistêmico juvenil (LESJ). No entanto, estudos avaliando somente infecções fúngicas invasivas (IFI) em pacientes com LESJ são restritos a relatos de casos ou série de casos, sem qualquer avaliação sistemática dos possíveis fatores de risco ou desfechos associados. A escassez de dados referentes às IFI em pacientes com LESJ e seu impacto sobre as características da doença em uma grande população levou ao desenvolvimento deste estudo multicêntrico. Objetivos: Estudar a prevalência, fatores de risco e mortalidade de IFI em pacientes com LESJ. Método: Um estudo de coorte multicêntrico retrospectivo foi realizado com 852 pacientes com LESJ de 10 Serviços de Reumatologia Pediátrica do Estado de São Paulo. Uma reunião foi realizada e todos os pesquisadores foram treinados para o preenchimento do banco de dados. As IFI foram diagnosticadas de acordo com as definições revisadas pelo grupo de consenso EORTC/MSG (comprovadas, prováveis ou possíveis). Foram coletados dados acerca de dados demográficos, características clínico-laboratoriais, atividade da doença (SLEDAI-2K), dano cumulativo (SLICC/ACR-DI) e tratamento, além de características e complicações das IFI. Resultados: IFI foram diagnosticadas em 33/852 (3,9%) pacientes com LESJ. IFI comprovadas foram diagnosticadas em 22 pacientes, IFI prováveis em 5 e IFI possíveis em 6. Os tipos de IFI encontradas foram: candidíase em 20 pacientes, aspergilose em 9, criptococose em 2, histoplasmose disseminada em um e paracoccidioidomicose em um. A mediana de duração da doença foi menor (1,0 vs. 4,7 anos, p < 0,0001), com maiores escores de SLEDAI-2K atual [19,5 (0-44) vs. 2 (0-45), p < 0,0001] e dose atual de prednisona [50 (10-60) vs. 10 (2-90) mg/dia, p < 0,0001] em pacientes com IFI em comparação com os pacientes sem IFI. A frequência de óbito foi maior no grupo com IFI (51% vs. 6%, p < 0,0001). A análise de regressão logística revelou que SLEDAI-2K atual (OR=1,108, IC 95%=1,057- 1,163, p < 0,0001), dose atual de prednisona (OR=1,046, IC 95%=1,021-1,071; p < 0,0001) e duração da doença (OR=0,984, IC 95%=0,969-0,998, p=0,030) foram fatores de risco independentes para IFI (R2 Nagelkerke 0,425). Conclusão: Este foi o primeiro estudo que caracterizou IFI em pacientes com LESJ. Identificou-se que a atividade da doença e uso de glicocorticoides foram os principais fatores de risco para estas infecções potencialmente graves, principalmente nos primeiros anos de curso da doença e com uma elevada taxa mortalidade / Introduction: Infections are an important cause of morbidity and mortality in childhoodonset systemic lupus erythematosus (cSLE) patients. However, studies evaluating solely invasive fungal infections (IFI) in cSLE patients are restricted to case reports or case series without any systematic evaluation of the possible associated risk factors and outcome in pediatric lupus population. The scarcity of data regarding IFI in cSLE patients and its impact on disease characteristics in a large population led to the development of this multicenter study. Objective: To study the prevalence, risk factors and mortality of IFI in cSLE patients. Methods: A retrospective multicenter cohort study was performed in 852 cSLE patients from 10 Pediatric Rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to EORTC/MSG Consensus Group criteria (proven, probable and possible). Demographic data, clinical, laboratorial, disease activity (SLEDAI-2K), cumulative damage (SLICC/ACR-DI) and treatment were collected. IFI were characterized and its outcome were also evaluated. Results: IFI were observed in 33/852 (3.9%) cSLE patients. Proven IFI was diagnosed in 22 cSLE patients, probable IFI in 5 and possible IFI in 6. Types of IFI were: 20 candidiasis, 9 aspergillosis, 2 cryptococcosis, one disseminated histoplasmosis and one paracoccidioidomycosis. The median of disease duration was lower (1.0 vs. 4.7 years, p < 0.0001), with a higher current SLEDAI-2K [19.5 (0-44) vs. 2 (0-45), p < 0.0001] and current prednisone dose [50 (10-60) vs. 10 (2-90) mg/day, p < 0.0001] in patients with IFI compared to those without IFI. The frequency of death was higher in the former group (51% vs. 6%, p < 0.0001). Logistic regression analysis revealed that current SLEDAI-2K (OR=1.108; 95%CI=1.057-1.163; p < 0.0001), prednisone current dose (OR=1.046; 95%CI=1.021-1.071; p < 0.0001) and disease duration (OR=0.984; 95%CI=0.969-0.998; p=0.03) were independent risk factors for IFI (R2 Nagelkerke 0.425). Conclusion: This was the first study that characterized IFI in cSLE patients. We identified that disease activity and glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course and with a high rate of fatal outcome