Ekaterini Goudouris

Clinical Features of Candidiasis in Patients With Inherited Interleukin 12 Receptor β1 Deficiency

BACKGROUND Interleukin 12Rβ1 (IL-12Rβ1)-deficient patients are prone to clinical disease caused by mycobacteria, Salmonella, and other intramacrophagic pathogens, probably because of impaired interleukin 12-dependent interferon γ production. About 25% of patients also display mucocutaneous candidiasis, probably owing to impaired interleukin 23-dependent interleukin 17 immunity. The clinical features and outcome of candidiasis in these patients have not been described before, to our knowledge. We report here the clinical signs of candidiasis in 35 patients with IL-12Rβ1 deficiency. RESULTS Most (n = 71) of the 76 episodes of candidiasis were mucocutaneous. Isolated oropharyngeal candidiasis (OPC) was the most common presentation (59 episodes, 34 patients) and was recurrent or persistent in 26 patients. Esophageal candidiasis (n = 7) was associated with proven OPC in 2 episodes, and cutaneous candidiasis (n = 2) with OPC in 1 patient, whereas isolated vulvovaginal candidiasis (VVC; n = 3) was not. Five episodes of proven invasive candidiasis were documented in 4 patients; 1 of these episodes was community acquired in the absence of any other comorbid condition. The first episode of candidiasis occurred earlier in life (median age±standard deviation, 1.5 ± 7.87 years) than infections with environmental mycobacteria (4.29 ± 11.9 years), Mycobacterium tuberculosis (4 ± 3.12 years), or Salmonella species (4.58 ± 4.17 years) or other rare infections (3 ± 11.67 years). Candidiasis was the first documented infection in 19 of the 35 patients, despite the vaccination of 10 of these 19 patients with live bacille Calmette-Guérin. CONCLUSIONS Patients who are deficient in IL-12Rβ1 may have candidiasis, usually mucocutaneous, which is frequently recurrent or persistent. Candidiasis may be the first clinical manifestation in these patients.

II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies

RESUMO Nos ultimos anos, novas imunodeficiencias primarias e defeitos geneticos tem sido descritos. Recentemente, produtos de imunoglobulina, com aprimoramento em sua composicao e para uso por via subcutânea, tornaram-se disponiveis em nosso meio. Com o objetivo de orientar o medico no uso da imunoglobulina humana para o tratamento das imunodeficiencias primarias, os membros do Grupo de Assessoria em Imunodeficiencias da Associacao Brasileira de Alergia e Imunologia produziram um documento que teve por base uma revisao narrativa da literatura e sua [...]

Tecnologias de informação e comunicação e ensino semipresencial na educação médica

Medical education is facing challenges to modify teaching practices and curriculum structure. Information and Communication Technology (ICT) can facilitate tackling several of these challenges. In this scenario, this study aimed at identifying forms of adopting an Internet based course authoring tool in blended learning formative processes in undergraduate, graduate, and continuing medical education at UFRJ. Based on the analyses it was possible to identify a profile of ICT usage and to discuss the results according to the following categories derived from the Virtual Learning Environments developed by professors: academic unit affiliation, disciplines and educational levels involved, field of knowledge of the blended learning courses, formation of the teaching staff team for the courses, content and communication resources used and educational activities adopted.

Increased sensitization to several allergens over a 12-year period in Brazilian children

2. Ruokolainen L, von Hertzen L, Fyhrquist N, et al. Green areas around homes reduce atopic sensitization in children. Allergy. 2015;70:195-202. 3. von Hertzen L, Beutler B, Bienenstock J, et al. Helsinki alert of biodiversity and health. Ann Med. 2015;47:218-225. 4. Lehtimäki J, Karkman A, Laatikainen T, et al. Patterns in the skin microbiota differ in children and teenagers between rural and urban environments. Sci Rep. 2017;7:45651. 5. Maechler M, Rousseeuw P, Struyf A, et al. cluster: “Finding Groups in Data”: Cluster Analysis Extended. https://cran.r-project.org/web/ packages/cluster/index.html. Accessed January 2, 2016. 6. Oksanen J, Blanchet FG, Kindt R, et al. vegan: Community Ecology Package. R package version 2.3-2. 2015. https://CRAN.R-project. org/package=vegan. Accessed January 2, 2016. 7. Anders S, Huber W. Differential expression analysis for sequence count data. Genome Biol. 2010;11:R106. 8. Lax S, Smith DP, Hampton-Marcell J, et al. Longitudinal analysis of microbial interaction between humans and the indoor environment. Science. 2014;345:1048-1052. 9. Kembel SW, Jones E, Kline J, et al. Architectural design influences the diversity and structure of the built environment microbiome. ISME J. 2012;6:1469-1479. 10. Pekkarinen P, von Hertzen L, Laatikainen T, et al. A disparity in the association of asthma, rhinitis, and eczema with allergenspecific IgE between Finnish and Russian Karelia. Allergy. 2007;62: 281-287. 11. Ruokolainen L, Lehtimäki JM, Karkman AV, Haahtela TK, von Hertzen L, Fyhrquist NT. Holistic view on health. Two protective layers of biodiversity. Ann Zool Fenn. 2017;54:39-49. 12. Haahtela T, Valovirta E, Bousquet J, Mäkelä M. The Finnish Allergy Programme 2008–2018 works. Eur Respir J. 2017;49:1700470. 13. Prescott S, Larcombe D, Logan A, et al. The skin microbiome: impact of modern environments on skin ecology, barrier integrity, and systemic immune programming. World Allergy Organ J. 2017; 10:29.

II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies (vol 15, pg 1, 2017)

[This corrects the article doi: 10.1590/S1679-45082017AE3844].

Comment to: II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies. einstein (Sao Paulo). 2017; 15(1): 1-16

Caro editor, Em relação ao “II Consenso Brasileiro sobre o uso de imunoglobulina humana em pacientes com imunodeficiências primárias”, publicado na revista einstein (São Paulo), em 2017, volume 15, numero 1,(1) gostaríamos de atualizar uma informação fornecida na página 6, a respeito do uso de imunoglobulina subcutânea facilitada pela hia luronidase. No momento da redação do texto, a informação, devidamente referenciada, era de que o produto não se encontrava aprovado em crianças e gestantes, mesmo nos países em que estava disponível comercialmente. No entanto, o produto está liberado na Europa para uso em crianças de qualquer idade desde julho de 2016.(2-4) O uso em gestantes ainda se encontra em investigação.(4) Consideramos relevante corrigir esta informação de maneira a garantir que o texto por nós redigido forneça as informações mais atualizadas possível, ao mesmo tempo que garantimos que os pacientes tenham acesso a mais este recurso terapêutico, assim que esta medicação seja liberada para uso em nosso meio.