Abstract Objective: To evaluate idiopathic musculoskeletal pain, musculoskeletal pain syndromes, and use of electronic devices in adolescents with asthma and healthy controls. Methods: Cross-sectional study was conducted on 150 asthmatic adolescents and 300 controls. Adolescents completed a self-administered questionnaire regarding painful symptoms, use of electronic devices, and physical activity. Seven musculoskeletal pain syndromes were evaluated, and Asthma Control Test (ACT) was assessed. Results: Musculoskeletal pain (42% vs. 61%, p = 0.0002) and musculoskeletal pain syndromes (2.7% vs. 15.7%, p = 0.0006) were significantly lower in asthmatic adolescents than in controls. The frequency of pain in the hands and wrists was reduced in asthmatic than in controls (12.6% vs. 31.1%, p = 0.004), in addition to cell phone use (80% vs. 93%, p < 0.0001), simultaneous use of at least two electronic media (47% vs. 91%, p < 0.0001), myofascial syndrome (0% vs. 7.1%, p = 0.043), and tendinitis (0% vs. 9.2%, p = 0.008). Logistic regression analysis, including asthma with musculoskeletal pain as the dependent variable, and female sex, ACT > 20, simultaneous use of at least two electronic devices, cell phone use, and weekends and weekdays of cell phone use, as independent variables, showed that female sex (odds ratio [OR], 2.06; 95% confidence interval [CI], 1.929–6.316; p = 0.0009) and ACT ≥ 20 (OR, 0.194; 95% CI, 0.039–0.967; p = 0.045) were associated with asthma and musculoskeletal pain (Nagelkerke R2 = 0.206). Conclusion: Musculoskeletal pain and musculoskeletal pain syndromes were lower in adolescents with asthma. Female sex was associated with musculoskeletal pain in asthmatic, whereas patients with asthma symptoms and well-controlled disease reported a lower prevalence of musculoskeletal pain.

RESUMO Relato de caso de paciente com imunodeficiência que necessitava de reposição regular de imunoglobulina endovenosa. Ela apresentou um episódio de lesão pulmonar aguda relacionada à transfusão após uso de produto de imunoglobulina diferente daquele que recebia habitualmente. Evoluiu com alterações respiratórias (hipóxia, dispneia e alteração de ausculta pulmonar) minutos após o fim da infusão, necessitando de suporte respiratório não invasivo. A paciente recebeu alta hospitalar após 36 horas, com boa evolução. Obteve recuperação total dos sintomas, sem mais reações nas infusões subsequentes de imunoglobulina (sendo optado por não mais prescrever o produto que foi usado quando ocorreu o episódio de lesão pulmonar aguda relacionada à transfusão). Apesar de rara, essa reação é potencialmente grave, não possui tratamento específico além de terapia de suporte, e há pouca informação na literatura sobre o risco de recorrência. A decisão sobre o seguimento da terapia com imunoglobulina após esse efeito adverso deve ser analisada individualmente, avaliando os possíveis riscos e benefícios para o paciente.

ABSTRACT Case report of a patient with an immunodeficiency who demands regular replacement of intravenous immunoglobulin. She presented an episode of transfusion-related acute lung injury shortly after using an immunoglobulin product different than the one she usually received. The patient evolved with respiratory changes (hypoxia, dyspnea, change in pulmonary auscultation) minutes after the end of the infusion, and received non-invasive respiratory support. She was discharged after 36 hours with good outcome. The patient achieved full recovery, showing no further reactions in subsequent immunoglobulin infusions (no longer receiving the product that was used when she had the episode of transfusion-related acute lung injury). Although rare, this reaction is potentially serious and has no specific treatment other than supportive therapy. The literature is scarce regarding the risk of recurrence. The decision on whether to proceed with immunoglobulin therapy after this adverse effect should be analyzed individually, assessing the possible risks and benefits for the patient.

Resumo Objetivo: descrever características clínicas, tomográficas e de função pulmonar em pacientes pediátricos com hipogamaglobulinemia primária (HP). Método: estudo de coorte retrospectivo de crianças com HP que recebiam gamaglobulina endovenosa (GEV) e antibiótico profilático entre 2005 e 2010. As características epidemiológicas, clínicas, os achados de tomografia e espirometria foram comparadas adotando níveis de significância de 5%. Resultados: foram avaliados 30 pacientes com HP. Após o início da reposição de GEV, houve redução da frequência de pneumonias (p<0,001). Os 11 pacientes que apresentavam bronquiectasias na primeira tomografia computadorizada (TC) eram mais velhos ao diagnóstico (p=0,001) e tiveram maior atraso no diagnóstico (p=0,001) quando comparados aos pacientes sem bronquiectasias. Ao final do estudo, 18 pacientes apresentavam bronquiectasias e 27/30 também apresentaram outras alterações pulmonares, isoladas ou concomitantes. O escore de Bhalla foi aplicado à última TC de 16/30 pacientes, com mediana do escore de 11 (variação 7-21), com correlação positiva entre o escore e o número de pneumonias após o início do tratamento (r=0,561; p=0,024). O escore foi ainda correlacionado com valores de volume expiratório forçado no primeiro segundo (VEF1) e capacidade vital forçada (CVF) obtidos por espirometria de 13/16 pacientes, com correlação negativa com VEF1 pré- (r=-0,778; p=0,002) e pós-broncodilatador (r=-0,837; p<0,001) e CVF (r=-0,773; p=0,002). Conclusão: complicações pulmonares foram frequentes nesta coorte, apesar da diminuição na frequência de pneumonias com o tratamento. A investigação precoce de pacientes com infecções de repetição para imunodeficiências primárias pode reduzir a frequência dessas complicações. A monitorização de alterações na espirometria pode indicar a necessidade de investigação radiológica.

Summary Objective: To describe clinical features, tomographic findings and pulmonary function in pediatric patients with primary hypogammaglobulinemia (PH). Method: A retrospective cohort study of children with PH who received intravenous immunoglobulin (IVIG) and prophylactic antibiotics between 2005 and 2010. Epidemiological and clinical features, computed tomography (CT) findings, and spirometric data were compared, assuming a 5% significance level. Results: We evaluated 30 patients with PH. After the start of IVIG replacement, there was a decline in the frequency of pneumonia (p<0.001). The 11 patients with bronchiectasis in their first CT scan were older at diagnosis (p=0.001) and had greater diagnostic delay (p=0.001) compared to patients without bronchiectasis. At the end of the study, 18 patients had bronchiectasis and 27 also had other lung disorders, alone or in combination. The Bhalla score was applied to the last CT scan of 16 patients, with a median score of 11 (range 7-21), with a positive correlation between the score and the number of pneumonias after the start of treatment (r=0.561; p=0.024). The score was also correlated with forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) values in 13/16 patients, with negative correlation to FEV1 previously to bronchodilator (r=-0.778; p=0.002) and after bronchodilator (r =-0.837; p<0.001) and FVC (r=-0.773; p=0.002). Conclusion: Pulmonary complications were common in this cohort, despite the decrease in the frequency of pneumonia with treatment. Early investigation of patients with recurrent infections for primary immunodeficiencies can reduce the frequency of these complications. The monitoring of changes in spirometry may indicate the need to carry out radiological investigation.

Resumo Objetivo: avaliar o diâmetro da pápula do teste cutâneo alérgico (TCA) com extrato de leite de vaca (LV) comparando pacientes tolerantes e persistentes. Método: estudo de coorte retrospectivo de análise de banco de dados de crianças com diagnóstico de alergia à proteína do leite de vaca (APLV) mediada pela imunoglobulina E, em ambulatório especializado, que realizaram TCA de forma evolutiva, sendo alocados em dois grupos: tolerantes ou persistentes, entre janeiro de 2000 e julho de 2015. As comparações foram realizadas ao diagnóstico e evolutivamente entre tolerantes e persistentes, pelos testes de Fisher, Mann-Whitney ou Wilcoxon, utilizando níveis de significância de 5%. Resultados: aplicando critérios de inclusão e exclusão, a amostra incluiu 44 pacientes (29 tolerantes e 15 que persistiram com APLV). No grupo tolerante, as medianas do TCA foram: ao diagnóstico, de 6 mm, e, no desenvolvimento de tolerância, de 2 mm, com diferença significante (p<0,0001). No grupo persistente, a mediana do TCA ao diagnóstico foi de 7 mm e no momento do último TCA, de 5 mm, sem diferença estatística (p=0,173). A comparação das medianas no último TCA entre os grupos mostrou-se significante (p=0,001), com redução maior de 50% no valor do TCA no grupo tolerante. Conclusão: os TCA seriados foram úteis para o diagnóstico, e a redução maior que 50% em seu diâmetro pode indicar o momento para realização de testes de provocação oral (TPO), auxiliando na detecção de tolerância na APLV.

Summary Objective: To evaluate the wheal diameter in allergy skin-prick tests (SPT) with cow’s milk extract (CM) comparing tolerant and persistent patients. Method: A retrospective cohort study involving database analysis of children with diagnosis of cow’s milk protein allergy (CMPA) mediated by immunoglobulin E in a specialized outpatient clinic that regularly performed SPT between January 2000 and July 2015. Patients were allocated into two groups: tolerant or persistent. Comparisons were made at diagnosis and over time between tolerant and persistent patients using Fisher’s, Mann-Whitney or Wilcoxon tests and significance level at 5%. Results: After applying inclusion and exclusion criteria, the sample includes 44 patients (29 tolerant and 15 who persisted with CMPA). In the tolerant group, the medians of SPT were: 6 mm at diagnosis and 2 mm at the development of tolerance; a significant difference (p<0.0001) was found. In the persistent group, the median SPT at diagnosis was 7 mm, while in the last SPT it was 5 mm, with no statistical difference (p=0.173). The comparison of medians in the last SPT between groups was significant (p=0.001), with a reduction greater than 50% in SPT in the tolerant group. Conclusion: Serial SPTs were useful for diagnosis, and a decrease higher than 50% in diameter can indicate the moment to perform oral food challenge (OFC) tests, helping to detect tolerance in CMPA.

OBJETIVO: Revisar o papel dos inibidores da calcineurina no tratamento das dermatoses alérgicas, com ênfase nos mecanismos de ação, eficácia e efeitos adversos tópicos e sistêmicos. FONTES DOS DADOS: Artigos de língua inglesa publicados na MEDLINE, considerando as palavras chave: pimecrolimus, tacrolimo, calcineurin inhibitors. Foram selecionados os artigos originais que apresentaram estudos controlados e estudos abertos para avaliação da eficácia, tolerabilidade e eventos adversos. Também foram avaliados artigos de revisão e relatos e série de casos, sendo estes últimos considerados apenas para avaliação de efeitos adversos. Foram consultados os sites oficiais da Food and Drug Administration e dos fabricantes de inibidores da calcineurina. SÍNTESE DOS DADOS: Os dados mostraram que inibidores de calcineurina são eficientes no tratamento da dermatite atópica leve a grave, levando a melhora dos sintomas, diminuição do número de crises e necessidade de corticoterapia tópica. Apresentam boa tolerabilidade e poucos efeitos adversos tópicos. Até o momento, não há evidências que sustentem a maior prevalência de neoplasias nos pacientes que utilizam esses medicamentos; entretanto, um adequado sistema de farmacovigilância está montado para avaliar esse aspecto. CONCLUSÕES: Os inibidores de calcineurina são uma nova classe de medicamentos para o tratamento das dermatoses alérgicas. São eficazes, tolerados e com poucos efeitos adversos. Devem ser sempre utilizados de acordo com as orientações preconizadas, e os pacientes devem ser sempre acompanhados pelo médico durante e após sua administração.

OBJECTIVE: To review the role of calcineurin inhibitors in the treatment of allergic dermatitis, focusing on mechanisms of action, efficacy and topical and systemic adverse effects. SOURCES: Articles written in English and published in MEDLINE using the following keywords: pimecrolimus, tacrolimus, calcineurin inhibitors. Original articles that presented controlled and open studies for assessing efficacy, tolerability and adverse effects were selected. Review articles and case series were also evaluated; the latter was only considered for assessing adverse effects. The official websites of the Food and Drug Administration and of manufacturers of calcineurin inhibitors were also used. SUMMARY OF THE FINDINGS: The data showed that calcineurin inhibitors are efficient in the treatment of mild to severe atopic dermatitis, leading to improvement in symptoms, reduction in number of attacks and need of topical corticotherapy. Calcineurin inhibitors have good tolerability and few topical adverse effects. To date, there has been no evidence to support higher prevalence of neoplasia in patients using these drugs; however, an adequate pharmacovigilance system has been set up to assess this aspect. CONCLUSIONS: Calcineurin inhibitors, which are a new drug class in the treatment of allergic dermatitis, are efficient, well tolerated and have few adverse effects. Calcineurin inhibitors should always be used according to recommended guidelines, and patients should always be followed by the physician during and after their administration.