Rosicler Rocha Aiza Alvarez

HLA antigens and risk for development of pemphigus foliaceus (fogo selvagem) in endemic areas of Brazil

Endemic pemphigus foliaceus (EPF), is an autoimmune disease associated with production of IgG antibodies against epidermal antigens. We have tested 38 patients and 50 control subjects living in endemic areas to investigate whether HLA genes are associated with host factors that determine whether or not exposed individuals will develop this disease. A variant of HLA-DR1, an antigen common in Blacks (DRB1*0102), was found to be the main susceptibility factor (relative risk=7.3, P<0.0002). Two amino acids, in positions 85 and 86 of DRB1, distinguish DRB1*0102 from DRB1*0101. These residues appear to be involved in the formation of a functional epitope that causes T cell recognition and determines disease susceptibility. Moreover, subjects having DQw2 did not develop the disease, while the frequency of DQw2 in controls was 22% (RR=0.04, P<0.006). Thus HLA genes appear to play a crucial role in the response to an environmental factor which in this setting frequently leads to the development of autoimmune disease. An HLA-DQ allele, DQw2, appears to be associated with factors that prevent the development of the disease in exposed individuals.

Hanseníase em menores de quinze anos no município de Paracatu, MG (1994 a 2001)

O Municipio de Paracatu-MG e considerado prioritario para acoes de controle da hanseniase devido as suas taxas de deteccao e prevalencia desta nosologia, sobretudo em menores de quinze anos. Nos anos de 1998 e 1999, realizou-se um trabalho de esclarecimento sobre sinais e sintomas da doenca para uma populacao de cerca de oito mil escolares, o que contribuiu para o incremento da taxa de deteccao da doenca em menores de quinze anos, sendo o municipio classificado como hiperendemico entre os anos de 1997 e 2001. Realizou-se tambem um trabalho de desenho descritivo de quarenta e cinco pacientes diagnosticados e tratados no municipio de Paracatu entre 1994 e 2001. Cinquenta e tres por cento dos diagnosticos foram em criancas do sexo masculino, 75,5% dos casos se situavam entre dez e catorze anos de idade e a escolaridade da populacao deste estudo esta defasada em relacao a idade, sendo que 84% da clientela morava em zona urbana. Todos os inscritos (100%) foram casos novos, 56,0% de forma multibacilar, forma clinica Dimorfa, e 44% de formas paucibacilares, como a forma clinica Indeterminada (30,8%) e a Tuberculoide (13,2%). Todos foram tratados segundo esquema padrao, com 100% de cura. Vinte e quatro por cento dos doentes apresentaram reacoes no diagnostico e durante o tratamento, e 9% apresentaram reacoes no pos-alta. Vinte e dois por cento dos doentes apresentavam algum grau de incapacidade no diagnostico e 13% apresentavam algum grau de incapacidade na alta (indice considerado alto). Verificou-se que 62% dos pacientes tinham contatos intradomiciliares portadores de hanseniase, sendo que somente em 15,5% destes contatos foram verificadas as duas cicatrizes da vacina BCG. A hanseniase e um serio problema de saude publica no municipio estudado, e o trabalho realizado com escolares foi fundamental para o aumento da deteccao dos casos.

The profile of fogo selvagem (endemic pemphigus foliaceus) at the University Hospital of Brasilia–Brazil. Epidemiological and clinical considerations

Background  Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG4 subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasília (HUB) for more than 15 years.

Imunofluorescência indireta no pênfigo foliáceo endêmico. contribuição para sua padronização

The aim of the present research was to standardize the indirect immunofluorescence reaction for Endemic Pemphigus Foliaceus (Fogo Selvagem). We found that fresh human skin was the ideal substrate and could proceed from foreskin, head, neck, or anterior abdominal wall. PBS pre-washing of the skin preceding the incubation with the serum should be avoided since the antigenicity might be diminished. TAS-calcium pre-serves the Pemphigus antigenic properties of the skin and shall be preferred as the diluent for the sera. Albumin-coated slides are useful because they increase the adherence of the skin sections. The conjugate appropriate dilution is convenientely determined by the radial immunodiffusion test (Ouchterlony method). So far as the correlation between the antibody titer and the clinical activity is concerned, we concluded that a titer of 160 or more was of bad prognosis, since it was associated with the generalized form of the disease or with cases of the localized form refractory to the usual therapy. Nevertheless, this assumption needs confirmation by further studies involving an appropriate clinical approach.O proposito da presente investigacao foi padronizar a reacao de imunofluorescencia indireta para Penfigo Foliaceo Endemico (Fogo Selvagem). Verificamos que a pele humana normal e o substrato ideal e que pode proceder de prepucio, cabeca, pescoco ou da parede abdominal anterior. A lavagem previa da pele precedendo a incubacao com o soro deve ser evitada pois a antigenicidade pode ser diminuida. O TAS-calcio preserva as propriedades antigenicas da pele e deve ser preferido como diluente para os soros. Lâminas cobertas com albumina sao uteis porque aumentam a aderencia dos cortes de pele. A diluicao apropriada do conjugado e convenientemente determinada pelo teste de imunodifusao radial (metodo de Ouch-terlony). Com referencia a correlacao entre titulo de anticorpos e atividade clinica, concluimos que um titulo igual ou maior do que 160 era de mau prognostico pois estava associado a forma generalizada da doenca ou a casos de forma localizada refratarios a terapeutica usual. Contudo, esta obervacao requer confirmacao atraves de estudos que envolvam uma abordagem clinica apropriada.The aim of the present research was to standardize the indirect immunofluorescence reaction for Endemic Pemphigus Foliaceus (Fogo Selvagem). We found that fresh human skin was the ideal substrate and could proceed from foreskin, head, neck, or anterior abdominal wall. PBS pre-washing of the skin preceding the incubation with the serum should be avoided since the antigenicity might be diminished. TAS-calcium preserves the Pemphigus antigenic properties of the skin and shall be preferred as the diluent for the sera. Albumin-coated slides are useful because they increase the adherence of the skin sections. The conjugate appropriate dilution is convenientely determined by the radial immunodiffusion test (Ouchterlony method). So far as the correlation between the antibody titer and the clinical activity is concerned, we concluded that a titer of 160 or more was of bad prognosis, since it was associated with the generalized form of the disease or with cases of the localized form refractory to the usual therapy. Nevertheless, this assumption needs confirmation by further studies involving an appropriate clinical approach.

Pênfigo foliáceo endêmico (fogo-selvagem) no grupo indígena Xavánte, Mato Grosso, Brasil

Foi realizado inquerito soro-epidemiologico atraves de testes de imunofluorescencia indireta e imunoprecipitacao visando determinar a prevalencia do penfigo foliaceo endemico (PFE) na populacao indigena Xavante do Brasil Central. Do total de 163 amostras de soro testadas, 5 (3,1%) foram positivas a imunofluorescencia. Todos os soros positivos procediam de individuos com sintomas clinicos da doenca. Nao foram observadas diferencas do PFE entre os Xavante e a populacao em geral sob o ponto de vista imunologico, ja que se verificou uma predominância da IgG4 entre os anticorpos nos pacientes indigenas.

Active search for leprosy cases in Midwestern Brazil: a serological evaluation of asymptomatic household contacts before and after prophylaxis with bacillus Calmette-Guérin

Leprosy is a disease caused by Mycobacterium leprae that carries a high risk of disability, making early diagnosis mandatory. This study aimed to determine the applicability of anti-PGL-1 IgM antibody detection, using the ML FLOW technique, as an assistant tool for the detection of leprosy infection in asymptomatic household contacts (AHHC) of multibacillary leprosy index cases from Midwest Brazil. Serological changes induced by the prophylaxis of these household contacts with Bacillus Calmette-Guérin (BCG) were also verified. A total of 91 AHHC were assessed, among which, 18.68% (n = 17) presented both positive bacilloscopy and positive anti-PGL-1 IgM serology. Positivity concordance between these two laboratorial exams (Kappa Index = 1; p < 0.001) was indicated, however, one case did not demonstrate concordance between the semiquantitative assessment of anti-PGL-1 IgM and the bacilloscopy index (Kappa Index = 0.96; p < 0.001). Among the 17 AHHC with positive bacilloscopy, eight were reassessed after prophylaxis with BCG and two of them presented negative anti-PGL-1 IgM serology, being these patients who had presented a bacilloscopy index of < 2[+] in the initial assessment. This study shows that anti-PGL-1 IgM detection may be used as a tool to determine the bacillary load in AHHC and to detect immune changes related to prophylaxis by nonspecific vaccination.

Uso do teste ML Flow em escolares diagnosticados com hanseníase no município de Paracatu, Minas Gerais

A deteccao da hanseniase no municipio de Paracatu e elevada em menores de quinze anos, abrangendo cerca de 6,8/10.000 hab. em 2003 e e classificada como hiperendemica. O estudo objetiva analisar a aplicacao do teste sorologico do PGL-1 (ML Flow) em 56 de 68 pacientes escolares da rede publica, diagnosticados com hanseniase atraves da busca ativa de casos no municipio de Paracatu - MG (2004 a 2006), sendo 71%, paucibacilares. Cerca de 85,2% dos pacientes residiam na area urbana, 55,8% eram do sexo feminino e a doenca predominava no grupo de 10 a 14 anos (IC95%:0,49-0,89%) e χ2=7,376, sendo que 15 (26,7%) com resultado do ML Flow positivo. Cinco pacientes tinham incapacidades do Grau 1, da forma clinica Dimorfa (40% ML Flow positivo). O percentual de casos de hanseniase entre os contatos intradomiciliares foi de 46,4%, sendo que 44,9% deles com resultado do teste do ML Flow positivo. O estudo sugere incorporar o teste ML Flow nos servicos de saude, uma vez que o mesmo auxilia na classificacao operacional da doenca, controle de contatos intradomiciliares com resultado do teste positivo, visando a deteccao precoce dos casos suspeitos de hanseniase.

Dermatoses entre os Xavánte da área indígena Pimentel Barbosa, Mato Grosso (Brasil)

The authors present the results of a dermatological survey conducted among the Xavánte Indians from the Pimentel Barbosa Reservation, state of Mato Grosso, Brazil Scabies, pediculosis, and pioderma were the most frequent diseases, clearly related to poor hygiene. The finding of perleche indicates the presence of nutritional deficiency. Endemic pemphigus foliaceus (fogo selvagem) was the most serious dermatological affection found in this population.

Validation of the questionnaire on hand function assessment in leprosy

OBJECTIVE To validate the psychometric properties of the questionnaire on hand function assessment in leprosy. METHODS Study conducted with a convenience sample of 101 consecutive patients in Brasília (Central-Western Brazil), from June 2008 to July 2009. The individuals were adults affected by leprosy, with impairment of the ulnar, median and radial nerves. Interobservers and intraobserver reproducibility was analyzed through successive interviews, and construct validity was analyzed through association between age, clinical form of leprosy, duration of nerve injury, grip and pinch strength measured with a dynamometer, sensibility test performed with Semmes-Weinstein monofilaments and manual ability assessment using the Jebsen test of hand function. Pondered kappa coefficient was calculated and a Bland-Altman plot was constructed to assess the reproducibility of the instrument. For internal consistency, Cronbachs alpha coefficient was utilized. Pearsons correlation coefficient was calculated and a multiple regression model was used. RESULTS The pondered kappa values for interobservers and intraobserver assessments ranged from 0.86 to 0.97 and from 0.85 to 0.97, respectively. The value of Cronbachs alpha coefficient was 0.967. Pearsons correlation coefficient showed an association (p < 0.001) among duration of nerve injury, grip and pinch strength, cutaneous sensibility and mean score in the Jebsen Test. The mean score of the questionnaire on hand functional assessment in leprosy was associated with operational classification of leprosy, duration of nerve injury, grip strength, cutaneous sensibility and manual ability (p < 0.0001 for the model as a whole). CONCLUSIONS The questionnaire on hand functional assessment in leprosy presents almost perfect interobservers and intraobserver reproducibility, high internal consistency and correlation with operational classification of leprosy, duration of nerve injury, grip strength, cutaneous sensibility in the hands and manual ability.OBJETIVO: Validar as propriedades psicometricas do questionario de avaliacao funcional das maos em hanseniase. METODOS: Estudo realizado com amostra de conveniencia de 101 pacientes consecutivos em Brasilia, DF, de junho de 2008 a julho de 2009. As pessoas eram adultas afetadas pela hanseniase, com comprometimento nos nervos ulnar, mediano e radial. Foi analisada a reprodutibilidade interobservadores e intraobservador com entrevistas sucessivas e a validade do constructo com associacao entre idade, forma clinica da hanseniase, tempo de lesao do nervo, forcas de preensao e pincas realizadas com dinamometro, teste de sensibilidade realizado com monofilamentos de Semmes-Weinstein e avaliacao da habilidade manual, utilizando o teste de funcao manual de Jebsen. Calcularam-se os valores do indice kappa ponderado e construiu-se um grafico Bland-Altman para avaliar a reprodutibilidade do instrumento. Para a consistencia interna, utilizou-se o coeficiente alfa de Cronbach. Foi calculado o coeficiente de correlacao de Pearson e usado modelo de regressao multipla. RESULTADOS: Os valores de kappa ponderado para as avaliacoes interobservadores e intraobservador variaram de 0,86 a 0,97 e de 0,85 a 0,97, respectivamente. O valor do coeficiente alfa de Cronbach foi de 0,967. O coeficiente de correlacao de Pearson mostrou associacao (p < 0,001) entre tempo de lesao do nervo, forcas de preensao e pincas, sensibilidade cutânea e escore medio do teste de Jebsen. O escore medio do questionario de avaliacao funcional das maos em hanseniase associou-se com classificacao operacional da hanseniase, tempo de lesao do nervo, forca de preensao, sensibilidade cutânea e habilidade manual (p < 0,0001 para o conjunto do modelo). CONCLUSOES: O questionario de avaliacao funcional das maos em hanseniase apresenta reprodutibilidade quase perfeita interobservadores e intraobservador, alta consistencia interna e correlacao com classificacao operacional da hanseniase, tempo de lesao do nervo, forca de preensao, sensibilidade cutânea nas maos e habilidade manual.

Validação do questionário de avaliação funcional das mãos em hanseníase

OBJECTIVE To validate the psychometric properties of the questionnaire on hand function assessment in leprosy. METHODS Study conducted with a convenience sample of 101 consecutive patients in Brasília (Central-Western Brazil), from June 2008 to July 2009. The individuals were adults affected by leprosy, with impairment of the ulnar, median and radial nerves. Interobservers and intraobserver reproducibility was analyzed through successive interviews, and construct validity was analyzed through association between age, clinical form of leprosy, duration of nerve injury, grip and pinch strength measured with a dynamometer, sensibility test performed with Semmes-Weinstein monofilaments and manual ability assessment using the Jebsen test of hand function. Pondered kappa coefficient was calculated and a Bland-Altman plot was constructed to assess the reproducibility of the instrument. For internal consistency, Cronbachs alpha coefficient was utilized. Pearsons correlation coefficient was calculated and a multiple regression model was used. RESULTS The pondered kappa values for interobservers and intraobserver assessments ranged from 0.86 to 0.97 and from 0.85 to 0.97, respectively. The value of Cronbachs alpha coefficient was 0.967. Pearsons correlation coefficient showed an association (p < 0.001) among duration of nerve injury, grip and pinch strength, cutaneous sensibility and mean score in the Jebsen Test. The mean score of the questionnaire on hand functional assessment in leprosy was associated with operational classification of leprosy, duration of nerve injury, grip strength, cutaneous sensibility and manual ability (p < 0.0001 for the model as a whole). CONCLUSIONS The questionnaire on hand functional assessment in leprosy presents almost perfect interobservers and intraobserver reproducibility, high internal consistency and correlation with operational classification of leprosy, duration of nerve injury, grip strength, cutaneous sensibility in the hands and manual ability.OBJETIVO: Validar as propriedades psicometricas do questionario de avaliacao funcional das maos em hanseniase. METODOS: Estudo realizado com amostra de conveniencia de 101 pacientes consecutivos em Brasilia, DF, de junho de 2008 a julho de 2009. As pessoas eram adultas afetadas pela hanseniase, com comprometimento nos nervos ulnar, mediano e radial. Foi analisada a reprodutibilidade interobservadores e intraobservador com entrevistas sucessivas e a validade do constructo com associacao entre idade, forma clinica da hanseniase, tempo de lesao do nervo, forcas de preensao e pincas realizadas com dinamometro, teste de sensibilidade realizado com monofilamentos de Semmes-Weinstein e avaliacao da habilidade manual, utilizando o teste de funcao manual de Jebsen. Calcularam-se os valores do indice kappa ponderado e construiu-se um grafico Bland-Altman para avaliar a reprodutibilidade do instrumento. Para a consistencia interna, utilizou-se o coeficiente alfa de Cronbach. Foi calculado o coeficiente de correlacao de Pearson e usado modelo de regressao multipla. RESULTADOS: Os valores de kappa ponderado para as avaliacoes interobservadores e intraobservador variaram de 0,86 a 0,97 e de 0,85 a 0,97, respectivamente. O valor do coeficiente alfa de Cronbach foi de 0,967. O coeficiente de correlacao de Pearson mostrou associacao (p < 0,001) entre tempo de lesao do nervo, forcas de preensao e pincas, sensibilidade cutânea e escore medio do teste de Jebsen. O escore medio do questionario de avaliacao funcional das maos em hanseniase associou-se com classificacao operacional da hanseniase, tempo de lesao do nervo, forca de preensao, sensibilidade cutânea e habilidade manual (p < 0,0001 para o conjunto do modelo). CONCLUSOES: O questionario de avaliacao funcional das maos em hanseniase apresenta reprodutibilidade quase perfeita interobservadores e intraobservador, alta consistencia interna e correlacao com classificacao operacional da hanseniase, tempo de lesao do nervo, forca de preensao, sensibilidade cutânea nas maos e habilidade manual.