Pesquisa de mutações CFTR em crianças e adolescentes com Bronquiolite Obliterante Pós-Infecciosa/

Apêndice e anexoOrientador: Nelson Augusto Rosário FilhoTese(doutorado) - Universidade Federal do Paraná, Setor de Ciências da Saúde, Programa de Pós-Graduação em Saúde da Criança e do Adolescente. Defesa: Curitiba, 20 de dezembro de 2006.Inclui bibliografiaÁrea de concentração: Doenças respiratória

Prevalência de asma, rinoconjuntivite alérgica e eczema atópico, em escolares de 13 e 14 anos : mudanças após 6 anos em Curitiba

Orientador: Dr. Nelson Augusto Rosário FilhoDissertação (mestrado) - Universidade Federal do Paraná, Setor de Ciências da Saúde, Programa de Pós-Graduação em Pediatria. Defesa : Curitiba, 13/12/2001Inclui referências: p. 71-80Resumo: O ISAAC (International Study of Asthma and Allergies in Childhood) é um projeto internacional para avaliar a prevalência de asma e doenças alérgicas em crianças em vários países do mundo. Em Curitiba (1995), 2.946 alunos de 13 e 14 anos, da rede pública e particular de ensino, selecionados de forma aleatória, responderam ao questionário escrito (QE) do projeto ISAAC. Após 6 anos, foi repetido o mesmo protocolo (agora validado para a população de Curitiba), com o mesmo método, com o objetivo de verificar mudanças na prevalência de asma e doenças alérgicas. Foram entrevistados 4.007 escolares de 13 e 14 anos. O número de questionários devolvidos e preenchidos de forma correta foi 3.628, que corresponde a uma taxa resposta de 90,5%. Para o diagnóstico de PA (provável asma) foram considerados os seguintes critérios: os escolares com quatro ou mais crises nos últimos 12 meses; 1 a 3 crises de sibilos, com sono interrompido por crise; 1 a 3 crises associadas a tosse noturna e ou sibilos após exercício físico. A participação foi maior para alunos do sexo feminino, 1.919 (53%). A prevalência de PA, em 1995 e 2001, foi 11,6% e 12,4%, respectivamente. Sintomas de asma (sibilância nos últimos 12 meses), de rinoconjuntivite (sintomas nasais e oculares sem estar gripado) e de eczema (grosseirão de pele com coceira, com localização em pregas), para 1995 e 2001, tiveram prevalências de (18,5% e 18,9%), (14,2% e 17,2%) e (3,8% e 3,7%), respectivamente. Existe uma ampla variação na prevalência de asma em vários centros em diversos países. Nossos resultados encontram-se neste intervalo de variação e diferente de outros estudos, não houve aumento da prevalência dos sintomas de asma e eczema após 6 anos. Este aumento foi significativo apenas para sintomas de rinoconjuntivite (RC). Esses achados sugerem que fatores de risco diferentes são necessários para a expressão clínica de doenças alérgicas e que fatores semelhantes podem ter respostas clínicas diferentes. Palavras-chave: Asma; Doenças alérgicas; Prevalência; ISAAC.Abstract: The ISAAC (International Study of Asthma and Allergies in Childhood) was designed to allow comparison between populations in different countries. It is an international project to evaluate worldwide variation in the prevalence of symptoms of asthma and allergic diseases. In Curitiba (1995), 2,946 schoolchildren, 13-14 year old, from private and public schools were randomly selected to self-reported ISAAC written questionnaire (WQ). After 6 years, with the same method, the ISAAC (WQ) was applied again to 4,007 adolescents. The objective was to verify time trends in the prevalence of asthma, allergic rhinoconjunctivitis and atopic eczema. Response rate for a complete questionnaire was 90.5% (3,628). Probable asthma was considered for 4 or more attacks in the last 12 mos; 1-3 attacks with sleep disturbance due to wheezing; 1-3 attacks with dry cough at night or wheezing after exercise. There was a higher participation of female schoolchildren, 1919 (53%). In 1995 and 2001, the prevalence of probable asthma was 11.6% and 12.4%, respectively; of symptoms of asthma (wheezing in the last 12 months) was 18.5% and 18.9%, respectively; of rhinoconjunctivitis (nasal and eyes symptoms in the last 12 mos, when did not have a cold or the flu) it was 14.2% and 17.2%, respectively; of eczema (rash and pruritus in the last 12 mos that had affected the skin creases) it was 3.8% and 3.7%, respectively. There was a worldwide variation in the prevalence of allergic diseases symptoms between populations in different centres and countries. Our results show an increase in the prevalence of rhinoconjunctivitis symptoms and no changes for asthma and eczema. This results suggest that different risk factors were necessary to express allergic diseases and that similar factors can result in different clinical features. Key words: Asthma, Allergic disease, Prevalence, ISAAC

ANGIOEDEMA HEREDITÁRIO: UMA ATUALIZAÇÃO.

Objective: Hereditary angioedema is a genetic disease with autosomal dominance inheritance characterized by quantitative and qualitative deficiency of C1 inhibitor, resulting in edema of multiples organs. The present study provides a literature review and update on diagnosis, classification and treatment of this pathology. Method: Review of recent and relevant literature, original articles and national and international guidelines. Results: Hereditary angioedema clinical manifestations are abdominal pain, vomiting, diarrhea and swelling of limbs, face, larynx and genitals. The diagnosis is made by history, physical exam and laboratory tests such as serum C4 and enzyme inhibitor C1-esterase. Classification is based on deficiency or absence of C1 esterase. Therapeutic approach follows guidelines to avoid triggering factors, medications for the management of attacks and prophylactic treatment. Androgens attenuated are used for preventive and antifibrinolytic treatment and fresh plasma are used in acute attacks. New drugs for inhibiting bradykinin and concentrated plasma-derived C1 have been used in the treatment and prevention of acute episodes. Conclusions: Hereditary angioedema is a serious disease with high morbidity and mortality. The correct diagnosis of this condition by several health professionals is essential for its proper management. Objetivo: O angioedema hereditário é uma doença genética, de herança autossômica dominante caracterizada pela deficiência quantitativa e qualitativa do inibidor de C1 resultando em edema de vários órgãos. O presente estudo tem como objetivo uma revisão da literatura, visando atualização do diagnóstico, classificação e tratamento desta patologia.Método: Revisão da literatura recente de artigos originais e diretrizes nacionais e internacionais.Resultados: As manifestações clínicas de angioedema hereditário são dor abdominal, vômitos, diarreia e edema de membros, face, laringe e genitais. O diagnóstico se faz por anamnese, exame físico e exames laboratoriais, entre eles dosagem sérica de C4 e do inibidor da C1-esterase. A classificação é baseada na deficiência ou não da enzima C1 esterase. A abordagem terapêutica segue orientações gerais para evitar fatores desencadeantes, medicamentos para o manejo das crises e tratamento profilático. Andrógenos atenuados são utilizados para tratamento preventivo e antifibrinolíticos e plasma fresco são empregados nas crises. Novas drogas inibidoras de bradicinina e concentrados de C1 derivados de plasma têm sido utilizados no tratamento e prevenção das crises. Conclusões: O angioedema hereditário é doença grave, com alta morbidade e mortalidade. O diagnóstico correto desta condição pelos profissionais da saúde é essencial para seu manejo adequado

Prevalência de asma, rinoconjuntivite alérgica e eczema atópico, em escolares de 13 e 14 anos : mudanças após 6 anos em Curitiba

Orientador: Dr. Nelson Augusto Rosário FilhoDissertação (mestrado) - Universidade Federal do Paraná, Setor de Ciências da Saúde, Programa de Pós-Graduação em Pediatria. Defesa : Curitiba, 13/12/2001Inclui referências: p. 71-80Resumo: O ISAAC (International Study of Asthma and Allergies in Childhood) é um projeto internacional para avaliar a prevalência de asma e doenças alérgicas em crianças em vários países do mundo. Em Curitiba (1995), 2.946 alunos de 13 e 14 anos, da rede pública e particular de ensino, selecionados de forma aleatória, responderam ao questionário escrito (QE) do projeto ISAAC. Após 6 anos, foi repetido o mesmo protocolo (agora validado para a população de Curitiba), com o mesmo método, com o objetivo de verificar mudanças na prevalência de asma e doenças alérgicas. Foram entrevistados 4.007 escolares de 13 e 14 anos. O número de questionários devolvidos e preenchidos de forma correta foi 3.628, que corresponde a uma taxa resposta de 90,5%. Para o diagnóstico de PA (provável asma) foram considerados os seguintes critérios: os escolares com quatro ou mais crises nos últimos 12 meses; 1 a 3 crises de sibilos, com sono interrompido por crise; 1 a 3 crises associadas a tosse noturna e ou sibilos após exercício físico. A participação foi maior para alunos do sexo feminino, 1.919 (53%). A prevalência de PA, em 1995 e 2001, foi 11,6% e 12,4%, respectivamente. Sintomas de asma (sibilância nos últimos 12 meses), de rinoconjuntivite (sintomas nasais e oculares sem estar gripado) e de eczema (grosseirão de pele com coceira, com localização em pregas), para 1995 e 2001, tiveram prevalências de (18,5% e 18,9%), (14,2% e 17,2%) e (3,8% e 3,7%), respectivamente. Existe uma ampla variação na prevalência de asma em vários centros em diversos países. Nossos resultados encontram-se neste intervalo de variação e diferente de outros estudos, não houve aumento da prevalência dos sintomas de asma e eczema após 6 anos. Este aumento foi significativo apenas para sintomas de rinoconjuntivite (RC). Esses achados sugerem que fatores de risco diferentes são necessários para a expressão clínica de doenças alérgicas e que fatores semelhantes podem ter respostas clínicas diferentes. Palavras-chave: Asma; Doenças alérgicas; Prevalência; ISAAC.Abstract: The ISAAC (International Study of Asthma and Allergies in Childhood) was designed to allow comparison between populations in different countries. It is an international project to evaluate worldwide variation in the prevalence of symptoms of asthma and allergic diseases. In Curitiba (1995), 2,946 schoolchildren, 13-14 year old, from private and public schools were randomly selected to self-reported ISAAC written questionnaire (WQ). After 6 years, with the same method, the ISAAC (WQ) was applied again to 4,007 adolescents. The objective was to verify time trends in the prevalence of asthma, allergic rhinoconjunctivitis and atopic eczema. Response rate for a complete questionnaire was 90.5% (3,628). Probable asthma was considered for 4 or more attacks in the last 12 mos; 1-3 attacks with sleep disturbance due to wheezing; 1-3 attacks with dry cough at night or wheezing after exercise. There was a higher participation of female schoolchildren, 1919 (53%). In 1995 and 2001, the prevalence of probable asthma was 11.6% and 12.4%, respectively; of symptoms of asthma (wheezing in the last 12 months) was 18.5% and 18.9%, respectively; of rhinoconjunctivitis (nasal and eyes symptoms in the last 12 mos, when did not have a cold or the flu) it was 14.2% and 17.2%, respectively; of eczema (rash and pruritus in the last 12 mos that had affected the skin creases) it was 3.8% and 3.7%, respectively. There was a worldwide variation in the prevalence of allergic diseases symptoms between populations in different centres and countries. Our results show an increase in the prevalence of rhinoconjunctivitis symptoms and no changes for asthma and eczema. This results suggest that different risk factors were necessary to express allergic diseases and that similar factors can result in different clinical features. Key words: Asthma, Allergic disease, Prevalence, ISAAC

Utility of Asthma Control Questionnaire 7 in the assessment of asthma control

Our objective was to evaluate the reproducibility of Asthma Control Questionnaire 7 (ACQ-7) in asthma patients, comparing our results against those obtained with the Global Initiative for Asthma (GINA) criteria. We evaluated 52 patients. Patients completed the ACQ-7, underwent spirometry, and were clinically assessed to determine the level of asthma control according to the GINA criteria, in two visits, 15 days apart. The ACQ-7 cutoff for uncontrolled asthma was a score of 1.5. The ACQ-7 showed good reproducibility, with a correlation coefficient of 0.73. The ACQ-7 identified a greater number of patients with uncontrolled asthma than did the GINA criteria; according to the GINA criteria, 47 patients (90.4%) presented with partially controlled asthma

Systematic review on fecal calprotectin in cystic fibrosis

Objectives: Fecal calprotectin is an inflammatory marker used for monitoring intestinal diseases. It has been studied as a marker of intestinal inflammation in cystic fibrosis (CF), a multi-systemic genetic disease caused by alterations to the CFTR gene. Manifestations of the disease favor a systemic inflammation not limited to the respiratory tract, therefore, calprotectin is a non-invasive and effective diagnostic method. The aim of the study was to perform a systematic review of the literature with a qualitative synthesis of studies. Sources: The articles were selected from PubMed, Web of Science, Scielo and Lilacs. Summary of the findings: Nine studies were selected for that qualitative synthesis, one was a randomized clinical trial, and eight were case-control or cohort designs. Most studies have indicated that calprotectin is a marker of systemic inflammation in CF and not just intestinal inflammation. Calprotectin is an aid in monitoring inflammatory bowel conditions in patients with cystic fibrosis. Conclusion: Further studies should be conducted to investigate the role of this marker in the systemic inflammation of CF

Spirometric values in children and adolescents with short stature

BACKGROUND: Several factors influence the pulmonary function values considered normal. In children of short stature, there are difficulties in interpreting the pulmonary function. OBJECTIVE: To assess spirometric values in children and adolescents with short stature and to identify a correction factor to adequately predict the expected values for this population. METHOD: A prospective selection of 77 patients was made, all with short stature and no respiratory disease. These patients were submitted to spirometry, transcutaneous hemoglobin oxygen saturation, chest perimeter measurement, and immediate hypersensitivity testing. Bone age was assessed by wrist X-rays. The data obtained by spirometry (FVC, FEV1, and FEF25-75%) were compared with those of Polgar and Promadhat (1971), predicted in three ways: a) by actual height; b) by height estimated at the 50th percentile for chronological age (CA); c) by height estimated at the 50th percentile for bone age (BA). RESULTS: The mean height was 133.3 ± 13.2 cm, and the deficit in relation to the third percentile was 5.4 ± 6.0 cm. The values obtained for FVC, FEV1, FEF25-75%, were significantly higher than those predicted by actual height. The mean FEV1 obtained was 2.42 ± 0.71 L, and the predicted (actual height) was 2.10 ± 0.64 L; according to the height estimated by BA and CA, the values were 2.27 and 2.86 L, respectively. The mean FVC1 was 2.20 ± 0.6 L, and the predicted was 1.90 ± 0.55 L. With the height estimated for bone age and chronologic age, the predicted values were 2.10 and 2.60 L, respectively. CONCLUSION: Children and adolescents with short stature have higher spirometric values than predicted for their actual height. These findings suggest that the height estimated at the 50th percentile for bone age can be used to evaluate pulmonary function