Fibromyalgia and menopause: an open study on postmenopausal hormone therapy.

BACKGROUND: Fibromyalgia women (FM) seems to get worse at menopause suggesting some influence of estrogens on its pathophysiology. We aimed to study the influence of postmenopausal hormone therapy (HT) in FM, the relationship with sleep and FM impact. METHODS: We analyzed prospectively 69 menopausal women, divided in two groups, FM group (FMG; N.=32) and comparison group (CG; N.=28) submitted to HT for twelve weeks (1.2 mg/g transdermal estradiol, 100 mg micronized natural progesterone oral/daily). Data on Utian Quality of Life Questionnaire (UQOL) and Pittsburgh Sleep Quality Index (PSQI) were obtained in both groups, at entrance and twelve weeks after HT. FM patients also completed the Fibromyalgia Impact Questionnaire - Revised (FIQ-R) and fibromyalgia severity (FS). RESULTS: FM patients improved significantly the FIQ-R (P=0.0001, median FIQ-R score 30% lower), mainly the severity of FM, assessed by FS (P<0.0001). Both groups had improved quality of life and sleep (UQOL: P=0.0001; P=0.001, PSQI: P<0.0001; P=0.007, respectively). Differences between first and second PSQI were greater for CG than for FMG (P=0.008). CONCLUSIONS: HT improving sleep and quality of life in both groups; it was a significant clinical improvement seen by FIQ and FS in FM patients. These changes characterize improvement of functional status and symptoms severity.

Avaliação do luto familiar na perda gestacional e neonatal / Assessment of family grief over the gestational and neonatal loss

RESUMO: Objetivos: Avaliar o grau de luto causado pela perda gestacional ou neonatal em pais e mães, associando com va-riáveis sociodemográficas. Adicionalmente, comparar o grau de luto de acordo com o momento da perda. Métodos:Estudo transversal, realizado com aplicação de questionário sociodemográfico e questionário validado (Escala de Luto Perinatal­ELP) em pais que tiveram perda de seu filho em qualquer período gestacional ou no neonatal. Resultados: 542 pais e mães estavam aptos para participação do estudo e após serem convidados para responder à pesquisa, 104 (19,1%) concordaram em participar. Os 104 participantes foram divididos em dois grupos: perda no primeiro trimestre gestacional (76,9%), e demais trimestres somados ao período neonatal (23,1%). Evidenciou-se predomínio materno das respostas (89,4%) e idade média de 29,1±15,58 anos. A mediana do escore na ELP foi de 90 pontos, não havendo diferença entre as pontuações de acordo com o momento da perda. Primigestas e mulheres com idade <25 anos apresentaram pontuações maiores que as demais (p=0,042 e p=0,047). Ideação suicida foi relatada por 15,4% e 32,7% das mães que se culpam pela morte do bebê e têm escores significativamente maior do que aque-las que não tinham tal sentimento (p<0,0001). Estado civil, escolaridade, situação econômica, religião, realização de pré-natal, etnia e abortamento prévio não apresentaram associação significativa com os escores obtidos na ELP. Conclusão: O luto ocorreu independente da idade gestacional do momento da perda, sendo de maior intensidade nas mulheres mais jovens e naquelas com sentimento de culpa. Medidas devem ser tomadas para minimizar tal sofrimento. (AU)

ABSTRACT: Objectives: To evaluate the degree of grief caused by gestational or neonatal loss in parents, associating with socio-demographic variables. Additionally, compare the degree of grief according to the moment of loss. Methods: Cross-sectional study conducted with the application of a sociodemographic questionnaire and a validated questionnaire (Perinatal Grief Scale-PGS) in parents who lost their child at any time during pregnancy or in the neonatal period. Results: 542 fathers and mothers were able to participate in the study and after invited to respond, 104 (19.1%) were willing to participate. The 104 respondents were divided into two groups: loss in the first gestational trimester (76.9%), and other trimesters added to the neonatal period (23.1%). There was a predominance of maternal responses (89.4%) and a mean age was 29.1±15.58 years. The median PGS score was 90 points, with no difference between the scores according to the moment of loss. First pregnant and women under the age of 25 had higher scores than the others (p=0.042 and p=0.047) respectively. Suicidal ideation was reported by 15.4% and 32.7% of mothers who blame themselves for the death of the baby have significantly higher scores than those without such feelings (p <0.0001). Marital status, education, economic status, religion, prenatal care, ethnicity, and previous miscarriage were not significantly associated with the scores obtained in the PGS. Conclusion: Family grief occurred regardless of the moment of loss, being greater in younger women and those with feelings of guilt. Measures must be taken to minimize such suffering. (AU)

QUALITY OF LIFE EVALUATION IN CELIAC PATIENTS FROM SOUTHERN BRAZIL / Avaliação da qualidade de vida do paciente celíaco do Sul do Brasil

BackgroundRestrictions imposed by the gluten-free diet generate large changes in the daily habits of the celiac patient, causing a negative impact on quality of life.ObjetiveThis study aimed to evaluate the quality of life of patients with celiac disease on a capital in Southern Brazil.MethodsPatients older than 18 years were included, with confirmed celiac disease for at least 60 days in the period from June to October 2013. A validated questionnaire, with specific questions to assess the patient’s quality of life celiac was applied. A total score ranged from 20 to 100 points; the higher the score, worse quality of life.ResultsA total of 103 questionnaires were evaluated, 96 (93.2%) female, with average score 56.6±12.35 (28 to 88 points). The comparison between the questionnaire scores and family income was not significant (P=0.139). Patients diagnosed less than 1 year have poorer quality of life than those with more than 10 years (P=0.063). Patients older than 60 years had better quality of life compared with the younger ones (P=0.04).ConclusionThere was no association between quality of life and factors such as family income, length of diet and age at diagnosis. Chronological age greater than 60 years has positively influenced the quality of life of celiac patients.

ContextoRestrições impostas pela dieta isenta em glúten podem gerar grandes mudanças nos hábitos diários do paciente celíaco, causando um impacto negativo na sua qualidade de vida.ObjetivoEste estudo teve como objetivo avaliar a qualidade de vida de pacientes com doença celíaca, em uma capital do Sul do Brasil.MétodosPacientes maiores de 18 anos foram incluídos, com doença celíaca confirmada há mais de 60 dias, no período de junho a outubro de 2013. Um questionário validado, com perguntas específicas para avaliar a qualidade de vida do paciente celíaco foi aplicado. A pontuação total nesse questionário varia entre 20 a 100 pontos; quanto maior a pontuação, pior a qualidade de vida.ResultadosNo total 103 questionários foram avaliados, sendo 96 (93,2%) do sexo feminino, com pontuação média de 56,6±12,35 (28-88 pontos). A comparação entre os escores do questionário e renda familiar não foi significativa (P=0,139). Pacientes diagnosticados há menos de 1 ano, apresentam pior qualidade de vida do que aqueles com mais de 10 anos (P=0,063). Pacientes com mais de 60 anos apresentaram melhor qualidade de vida em comparação com os mais jovens (P=0,04).ConclusãoNão houve associação entre a qualidade de vida e fatores como renda familiar, tempo de dieta e idade no momento do diagnóstico. A idade cronológica superior a 60 anos influenciou positivamente a qualidade de vida de pacientes celíacos.

LOW BONE MINERAL DENSITY IN BRAZILIAN PATIENTS AT DIAGNOSIS OF CELIAC DISEASE / Alterações ósseas em celíacos brasileiros ao diagnóstico

BackgroundLow bone mineral density is considered an extra-intestinal manifestation of celiac disease with reduced bone mass, increased bone fragility, and risk of fractures. Celiac disease is considered a condition at high risk for secondary osteoporosis and the evaluation of bone density is very important in the clinical management of these patients.ObjectiveThe present study aimed to investigate bone alterations in celiac patients from Curitiba, South Region of Brazil at diagnosis, correlating the findings with age and gender.MethodsPatients who were included in the study were attended to in a private office of the same physician from January 2009 to December 2013. The diagnosis of celiac disease was done through clinical, serological and histological findings. All data were collected from the medical charts of the patients. After the diagnosis of celiac disease, evaluation for low bone mineral density was requested by dual-energy X-ray absorptiometry (DEXA). DEXA bone densitometer was used to estimate low bone mineral density at the lumbar spine and femur.ResultsA total of 101 patients, 82 (81.2%) female and 19 (18.8%) male subjects, with mean age of 39.0±3.03 years were included. At celiac disease diagnosis, 36 (35.6%) were younger than 30 years, 41 (40.6%) were between 31 and 50 years, and 24 (23.8%) were older than 50 years. Among the evaluated patients, 69 (68.3%) presented low bone mineral density, being 47% with osteopenia and 32% with osteoporosis. Patients who were older than 51 years and diagnosed with celiac disease presented low bone mineral density in 83.3% (20/24) of the cases. As expected, age influenced significantly the low bone mineral density findings. Among women, low bone mineral density was present with high frequency (60%) from 30 to 50 years. In patients diagnosed older than 60 years (n=8), all the women (n=5) and two of the three men had osteoporosis.ConclusionThis study demonstrated that 69% of Brazilian patients with celiac disease at diagnosis had low bone mineral density, being more frequent in women older than 50 years.

ContextoBaixa densidade mineral óssea é considerada uma manifestação extra-intestinal da doença celíaca com redução da massa óssea, aumento da fragilidade óssea e risco de fraturas. A doença celíaca é considerada uma condição de alto risco para a osteoporose secundária e a avaliação da densidade óssea é muito importante no manejo clínico dos pacientes.ObjetivoO presente estudo teve como objetivo investigar as alterações ósseas presentes em pacientes com doença celíaca de Curitiba-PR, no momento do diagnóstico, correlacionando os achados com a idade e gênero.MétodosOs pacientes incluídos no estudo foram atendidos por um só médico no período de janeiro/2009 a dezembro/2013. O diagnóstico da doença celíaca foi feito através das manifestações clínicas, sorologia específica e achados histológicos da mucosa duodenal. Todos os dados foram coletados a partir dos prontuários dos pacientes. Após o diagnóstico da doença celíaca, foi solicitada a avaliação de densidade mineral óssea por meio de densitometria (dual-energy X-ray absorptiometry DEXA). DEXA foi utilizada para estimar a densidade mineral óssea na coluna lombar e fêmur.ResultadosUm total de 101 pacientes, 82 (81,2%) mulheres e 19 (18,8%) homens, com idade média de 39,0±3,03 anos foram incluídos. No momento do diagnóstico de doença celíaca, 36 (35,6%) tinham menos de 30 anos, 41 (40,6%) tinham entre 31 e 50 anos, e 24 (23,8%) tinham mais de 50 anos. Entre os pacientes avaliados, 69 (68,3%) apresentaram baixa densidade mineral óssea, 47% deles com osteopenia e 32% com a osteoporose. Os pacientes maiores de 51 anos de idade apresentaram baixa densidade mineral óssea em 83,3% (20/24) dos casos. Como esperado, a idade influenciou significativamente os resultados da baixa densidade mineral óssea. Entre as mulheres, baixa densidade mineral óssea foi observada com alta frequência (60%) também na faixa etária entre 30 a 50 anos. Pacientes diagnosticados mais de 60 anos (n=8), todas as mulheres (n=5) e dois dos três homens tinham osteoporose.ConclusãoO presente estudo demonstrou que 69% dos pacientes brasileiros com doença celíaca no momento do diagnóstico apresentaram baixa densidade mineral óssea, sendo mais frequente em mulheres com mais de 50 anos.

Deposition of the lectin pathway of complement in renal biopsies of lupus nephritis patients.

BACKGROUND/AIMS: Lupus nephritis (LN) is one of the most serious manifestations of SLE occurring in 66-90% of these patients. The complement system is part of the innate immunity and modulator of inflammation and the adaptative immune response. Mannan-binding lectin (MBL) and Ficolin-2 (FCN-2) are important members of the lectin pathway of complement activation. Despite the significant participation of complement in the pathogenesis of the LN, there are few reports demonstrating "in situ" deposition of complement components in renal biopsy specimens in this disorder. The present study investigated the deposition of complement components in kidney specimens of LN patients. METHODS: Renal biopsies of 11 patients with SLE and LN were evaluated for immunofluorescence staining for IgG, IgA, IgM, C3, and C1q. Additionally, MBL, FCN-2 and C5b-9 were researched using monoclonal antibodies. RESULTS: All the biopsies were positive for IgG, C3, and C1q, eight were positive IgM and five had IgA deposition in glomerular tissue. The terminal complex of complement C5b9 was positive in all cases, MBL in nine (82%) cases; seven (63.6%) of them presenting concomitantly FCN-2 deposition. Patients presenting MBL deposition had higher mean of urinary proteins (9.0 g/day) than patients with negative MBL deposition (mean of 2.3g/day). CONCLUSIONS: In this study, we demonstrated in situ the participation of complement in the renal injury, including MBL and FCN-2 of the lectin pathway; also the strong role of C5b-9 in the pathogenesis of LN.

Autoantibodies in relatives of celiac disease patients: a follow-up of 6-10 years / Autoanticorpos em familiares de pacientes celíacos: seguimento por 6 a 10 anos

CONTEXT: Autoimmune diseases are 3 to 10 times more frequently in patients with celiac disease and their relatives than in the general population. OBJECTIVE: To investigate a broad spectrum of autoantibodies in celiac disease relatives from Southern Brazil, in a serological follow-up of 6-10 years, aiming to associate with other autoimmune diseases, degree of parentage, demographic and clinical data. METHODS: Serum samples of 233 relatives were analyzed in two different phases: n = 186 in phase I (1997-2000) and n = 138 (being 91 = follow-up group and 47 = newly tested) in phase II (2006-2007). As controls, 100 unrelated individuals were evaluated. Autoantibodies to smooth muscle, mitochondrial, liver-kidney microssome, parietal cell and thyroid microssome were tested by indirect immunofluorescence. RESULTS: A significant increase of autoantibodies, in both phases, was observed in the relatives when compared to the non-relatives (P = 0.0064), specifically to anti-thyroid microssome and anti-parietal cell. In both phases, the female/male proportion of autoantibodies was of 4:1 to 3:1 (P<0.041). The frequency of autoantibodies amongst 1st and 2nd degree relatives was 11.8% and 9.68% in phase I and 4% and 6.67% in phase II. CONCLUSION: Celiac disease relatives presented other autoantibodies and serological screening is a useful instrument for identifying autoimmune diseases along the years.

CONTEXTO: Doenças autoimunes são 3 a 10 vezes mais frequentes em pacientes com doença celíaca e em seus familiares que na população em geral. OBJETIVOS: Realizar amplo perfil de autoanticorpos em familiares de celíacos do sul do Brasil, em seguimento sorológico de 6-10 anos, visando associá-lo com outras doenças autoimunes, grau de parentesco, dados demográficos e clínicos desses indivíduos. MÉTODOS: Foram analisadas amostras de 233 familiares em duas etapas diferentes: n = 186 na etapa I (1997-2000) e n = 138 (91 recoleta e 47 novos familiares testados) na etapa II (2006-2007). Como controle foram avaliadas amostras de 100 não-familiares. Anticorpos antimúsculo liso, antimitocondrial, anticélula gástrica parietal, antimicrossomal de fígado e rim e antimicrossomal tireoidiano foram testados por imunofluorescência indireta. RESULTADOS: Foi observado um aumento significativo de positividade para os autoanticorpos em familiares de celíacos, quando comparados aos não-familiares (P = 0,0064), especificamente para o antimicrossomal tireoidiano e anticélula gástrica parietal. Entre os indivíduos com autoanticorpos positivos, a proporção do sexo feminino para o masculino foi de 4:1 e 3:1 em ambas as etapas (P<0,041). A frequência de autoanticorpos detectada entre familiares de primeiro e segundo graus foi de 11,8% e 9,68% na etapa I e 4% e 6,67% na etapa II. CONCLUSÃO: Familiares de pacientes celíacos apresentam autoanticorpos positivos e o acompanhamento sorológico desses indivíduos é utilizado como instrumento na identificação de doenças autoimunes ao longo dos anos.

Anti-cyclic citrullinated peptide antibodies in adult patients with juvenile idiopathic arthritis.

BACKGROUND: Antibodies to cyclic citrullinated peptide (anti-CCP) have been found in different proportions in the juvenile idiopathic arthritis (JIA) population. The majority of studies have been done in children or mixed population (children plus adults). AIM: The objective of the study was to study the prevalence of anti-CCP in JIA adult patients. METHODS: Anti-CCP3 was searched for in 49 adult patients with JIA and associated with clinical and demographics data. As comparisons, 156 patients with adult rheumatoid arthritis (RA) and 100 healthy volunteers were studied. RESULTS: Nine patients (18.3%) were positive for anti-CCP3. All of them had the polyarthritis form. This antibody was more common in JIA than in control subjects (P = 0.0002) and less common in JIA than in adult RA patients (P < 0.0001), but the rheumatoid factor polyarticular form of JIA had the same prevalence as in adult RA patients (P = 0.33).In JIA patients, anti-CCP had a positive association with the presence of rheumatoid factor (P < 0.0001), worse functional status (P = 0.04), need for orthopedic surgery (P = 0.01), and later disease onset (P = 0.0007). CONCLUSIONS: In adult patients with JIA, the prevalence of anti-CCP3 is 18%, and its presence may define a sample of patients with worse prognosis.

Association of anticyclic citrullinated peptide antibodies with extra-articular manifestations, gender, and tabagism in rheumatoid arthritis patients from southern Brazil.

Gender and environmental factors are known to influence the clinical heterogeneity and outcome of rheumatoid arthritis (RA). Some variables have been suggested to be associated with the severity of the disease, which can be of great value in the correct management of RA patients. The purpose of this study was to investigate the associations among anticyclic citrullinated antibody (anti-CCP2) positivity, extra-articular manifestations (EAM), gender, and tobacco exposure in a Brazilian RA population. We performed a transversal study comprising 156 RA patients which were investigated for EAM, functional class, presence of anti-CCP2, and IgM rheumatoid factor (IgM-RF). The determination of anti-CCP2 was performed using enzyme immunoassay (ELISA) kits and IgM-RF by latex agglutination test. Clinical and demographical data were obtained through review of charts. Anti-CCP positivity intensity was directly correlated with tobacco smoking, sex, and the development of rheumatoid nodules. Intense anti-CCP2 reaction was 19.8-fold higher in females vs. males, 2.7-fold higher in tobacco vs. non-tobacco users, 7.7-fold higher in female vs. male tobacco users, and 5.15-fold higher in patients with rheumatoid nodules. Tobacco smoking, gender, and rheumatoid nodules are significantly correlated with anti-CCP2 positivity in Brazilian RA patients.

Anti-cyclic citrullinated peptide antibodies and rheumatoid factor in rheumatoid arthritis patients and relatives from Brazil.

OBJECTIVES: To evaluate the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies and RF in RA patients and their relatives from Southern Brazil. METHODS: Anti-CCP2 and IgM-RF were evaluated in 156 RA patients and 200 relatives. Sera from 100 healthy unrelated individuals were used as control. The anti-CCP2 was detected by ELISA and the IgM-RF using the latex agglutination test. RESULTS: We identified 117 anti-CCP2 (75%)-positive and 106 RF (67.9%)-positive patients. Anti-CCP2 was increased in relatives (5.5%; 11/200) when compared with unrelated individuals (1%; P = 0.050). Titre of anti-CCP2 in RA patients did not differ from relatives [140.4 (75.7) vs 115.6 (84.2) U, respectively; P = 0.30]. Positive relatives were younger than patients for anti-CCP2 (P = 0.0081), RF (P < 0.001) and both concomitantly (P = 0.012), and although there was no difference for anti-CCP2 positivity according to gender, increased RF positivity and concomitant anti-CCP2/RF were observed in the female relatives (P = 0.067 and 0.082, respectively). No difference regarding the relative degree of tobacco use in relatives was detected. Among the 11 anti-CCP2-positive relatives, 2 females had RA diagnosis established and 6 individuals presented with joint symptoms suggestive of RA. CONCLUSION: The results demonstrate a significant positivity of anti-CCP2 in relatives of RA patients from Brazil and reinforce the importance of serological tools to identify undiagnosed RA.

Autoantibodies in silicosis patients and in silica-exposed individuals.

The aim of this study was to evaluate the prevalence of autoantibodies in silica-exposed patients with and without silicosis and without any known rheumatic disease. We studied 61 males exposed to silica for a mean time of 12.2 +/- 10.2 years of exposure. A total of 72.1% (44/61) of them presented with pulmonary silicosis. As control group we included 62 healthy males. In all samples we screened for rheumatoid factor (latex agglutination), antinuclear antibodies (indirect immunofluorescence), anti Scl-70 (ELISA) and ANCA (indirect immunofluorescence technique). One patient (1.6%) of the silica group had weakly positive ANA (titer 1:80, centromeric pattern); one (1.6%) had atypical ANCA and seven patients (11.4%) presented positive rheumatoid factor (values range from 8 to 32 UI/ml). One control patient had a positive RF and none of them had positive ANA or ANCA. All patients and controls were negative for anti-Scl-70. The finding of positive RF was higher in the silica-exposed patients (p = 0.032; Fisher). All patients with positive RF had pulmonary silicosis. In the silica-exposed group we could not find a relationship between the presence of RF and age (p = 0.21; Mann-Whitney), smoking habits (p = 0.25; Fisher) but a positive relationship was found with exposure time to silica dust (p = 0.005; Mann-Whitney). We conclude that there was 11.4% prevalence of low titer RF in the silica-exposed patients without known rheumatic disease. RF was more common in patients with longer exposure to silica dust and appeared only in those with silicosis. The presence of ANA, Scl-70 and ANCA was the same as in the control population.

Alterações do TSH em pacientes com síndrome de Down: uma interpretação nem sempre fácil / Alterations of TSH in Down's syndrome patients: a hard interpretation

OBJETIVO: Determinar as concentrações de hormônio estimulante da tireóide (TSH) e a presença de anticorpos antitireoperoxidase (anti-TPO) em pacientes com síndrome de Down (SD) atendidos no ambulatório do Hospital de Clínicas da Universidade Federal do Paraná. MÉTODOS: Foram incluídos no estudo 72 pacientes com SD, não aparentados e selecionados consecutivamente, com média de idade de 6,15 anos. Oitenta crianças sadias, pareadas com os pacientes, foram utilizadas como controles. Em todas as amostras foram determinadas as concentrações séricas de TSH e de anti-TPO, através do método de dosagem imunométrica. RESULTADOS: Trinta pacientes com SD (42,9 por cento) apresentaram alterações nas concentrações de TSH, sendo que 4,3 por cento tinham valores menores que 0,5æUI/ml e 38,6 por cento, valores superiores a 5æUI/ml (5,1 a 22) (média de 5,56 ± 4,18æUI/ml). Nos controles, a concentração média de TSH foi 2,76æUI/ml (± 1,14), evidenciando-se um aumento significativo nos níveis de TSH nos pacientes com SD (p < 0,001). De forma similar, caracterizou-se uma diferença significativa na positividade para o anti-TPO nos pacientes (15,4 por cento) em relação aos controles (0 por cento; p < 0,001). Observou-se ainda aumento significativo nas concentrações de TSH nos pacientes com idade superior a 9 anos (média de 6,86 ± 4,6æUI/ml) quando comparados aos menores de 9 anos (média de 5,24 ± 3,81æUI/ml; p = 0,006), bem como na positividade do anti-TPO (6/20 vs. 5/52; p = 0,041). CONCLUSÕES: Os resultados demonstraram alta prevalência de alterações das dosagens de TSH e de doença tireoidiana nos pacientes com SD, principalmente naqueles com idade superior a 9 anos. Os dados indicam que a avaliação da função tireoidiana nos pacientes com SD deve receber atenção especial dos profissionais de saúde que atendem esses pacientes.

OBJECTIVE: To evaluate the thyroid stimulating hormone (TSH) levels and the presence of antithyroperoxidase antibody (anti-TPO) in DownÆs syndrome (DS) patients from Hospital de Clínicas of Universidade Federal do Paraná (HC/UFPR). METHODS: Seventy-two DS patients, non-related and consecutively selected (mean age 6.15) were included in the study. Eighty matched healthy children were used as controls. The TSH measurement and the anti-TPO were determined by immunometric assay in all samples. RESULTS: Thirty patients with DS (42.9 percent) presented abnormal levels of TSH; 4.3 percent showed values below 0.5æIU/ml and 38.6 percent presented values higher than 5æIU/ml (range 5.1-22; mean 5.56 ± 4.18æIU/ml). The mean concentration of TSH in the controls was 2.76 ± 1.14æIU/ml, indicating a significant increase in TSH levels in the DS patients (p < 0.001). Similarly, a significant difference was observed in the anti-TPO positivity in the patientsÆ group (15.4 percent) when compared with the controls (0 percent; p < 0.001). In addition, the TSH levels of patients older than 9 years presented a significant increase (mean of 6.86 ± 4.6æIU/ml) when compared with the levels observed in patients younger than 9 years (mean of 5.24 ± 3.81æIU/ml; p = 0.006). The same pattern was observed in the positivity of anti-TPO (6/20 vs. 5/52; p = 0.041). CONCLUSIONS: The results demonstrated high prevalence of elevated TSH and anti-TPO in the patients from the DS ambulatory of HC/UFPR, with increased frequency in those older than 9 years. The data indicate that the evaluation of thyroid function in DS patients must receive special attention from health professionals who take care of these patients.

Celiac disease in children and adolescents with Down syndrome.

OBJECTIVES: High prevalence rates of celiac disease in patients with Down syndrome have been reported in several countries. However, in Brazil there is no data regarding this association. In this study we report the prevalence of celiac disease in Down syndrome children and adolescents from southern Brazil. METHODS: Seventy-one patients (32 female and 39 male, 2-18 years) from Curitiba, Brazil, were studied. Eighty young people (42 male and 38 female, 2-19 years) were used as controls. All subjects were screened for the IgA-antiendomysium antibody (EmA) and IgA anti-tecidual transglutaminase (anti-tTG). EmA was measured by an immunofluorescence assay using umbilical cord as the substrate and anti-tTG by ELISA with tecidual transglutaminase as the antigen. The total IgA serum level was determined by turbidimetry. RESULTS: Five DS patients (7%) were positive for EmA-IgA, with titers from 1/5 to 1/80 and 14 (17.5%) for anti-tTG (21-340 units). All EmA positive patients also presented anti-tTG antibodies simultaneously. Clinical and histological findings of the intestinal mucosa confirmed celiac disease diagnoses in four patients. The other EmA positive patient was asymptomatic and was not submitted to duodenal biopsy. Patients only positive for anti-tTG presented borderline values (< 25 units) and were asymptomatic. None of the controls were positive for EmA or anti-tTG. No Down syndrome patients or controls presented IgA deficiency. CONCLUSIONS: These data indicates a high prevalence (5.6%) of confirmed celiac disease in Down syndrome patients from southern Brazil.

Doença celíaca em crianças e adolescentes com síndrome de Down / Celiac disease in children and adolescents with Down syndrome

OBJETIVOS: Alta prevalência de doença celíaca em pacientes com síndrome de Down tem sido descrita em vários países. No entanto, no Brasil ainda não há relatos mostrando essa associação. O presente estudo teve como objetivo avaliar a prevalência de doença celíaca em crianças e adolescentes com síndrome de Down no sul do Brasil. MÉTODOS: Setenta e um pacientes (32 do sexo feminino e 39 masculino, 2-18 anos) provenientes de Curitiba, Brasil, foram estudados. Oitenta indivíduos (42 do sexo masculino e 38 feminino, 2-19 anos) foram utilizados como controles do estudo. Todas as amostras foram investigadas para anticorpo anti-endomísio (EmA) e anti-transglutaminase tecidual (anti-tTG). O EmA foi pesquisado através de imunofluorescência indireta usando cordão umbilical como substrato e o anti-tTG através da técnica de ELISA, utilizando transglutaminase tecidual como antígeno. As dosagens de IgA foram realizadas por turbidimetria. RESULTADOS: Cinco pacientes com síndrome de Down (7 por cento) foram positivos para EmA-IgA, com títulos entre 1/5 e 1/80 e catorze (17,5 por cento) para anti-tTG (21-340 unidades). Todos os pacientes positivos para EmA apresentaram simultaneamente positividade para o anti-tTG. Os achados clínicos e histológicos na mucosa intestinal confirmaram doença celíaca em quatro pacientes. O outro paciente EmA positivo não foi submetido a biópsia duodenal. Os pacientes positivos apenas para anti-tTG apresentaram valores limítrofes (< 25 unidades) e eram assintomáticos. Nenhum indivíduo do grupo controle foi positivo para EmA ou anti-tTG. Nenhuma amostra do estudo foi deficiente para IgA. CONCLUSÕES: Os dados do presente estudo mostram alta prevalência (5,6 por cento) de doença celíaca confirmada em crianças e adolescentes com síndrome de Down da região sul do Brasil.

Ativaçåo do sistema complemento causada pela circulaçåo extracorpórea em crianças submetidas à cirurgia cardíaca / Complement cascade activation due to cardiopulmonary bypass in children undergoing heart surgery

Complement activation was studied in 10 children undergoing heart surgery with (group 1) and in 9 children undergoing heart without cardiopulmonary bypass (group 2). Children with infectious disease and under corticotherapy as well as those with immunological disease were excluded. Cardiopulmonary bypass was performed with bubble oxygenators, primed with blood and crystalloid. Blood samples were drawn at hte beginning oh the operation and approximately 15 minutes before skin closure. Plasma concentrations of the third component factor (C3) and of its split product C3d were measured in all patients. C3 levels were expressed in mg/dl and C3d levels were expressed as a percentage of a normal control value. Plasma concentrations of C3 decreased significantly in group 1 (84.8 more or less 14.7 mg/dl and 34.2 more or less 8.2 mg/dl; p=0.007) and remained unchanged in group 2 (91 more or less 11.9 mg/dl and 85.9 more or less 13.4 mg/dl; p=0.13-NS). Plasma concentrations of C3d rose significantly in group 1 (12928.5 and 33867.5; p=0,005) but remained unchanged in group 2(86 more or less 15.1 and 93 more or less 21.3; p=0.10-NS). We concluded that the third componenet of the complement system C3 was consumed during the cardiopulmonary bypass and this consumption was due to activation