Predominantly antibody deficiency: case report / Deficiência predominantemente de anticorpos: relato de caso

Abstract Objectives: present a case of Inborn errors of immunity (IEI) as a potential diagnosis in pediatric patients with recurrent infections. Description: male patient, 13 years old, since he was eight months old had recurrent diarrhea, sinusitis, otitis, abscesses and urinary tract infections. At the age of ten, he presented mastoiditis progressing to meningitis, he was admitted to a tertiary hospital, where an immunological evaluation was performed, which led to the diagnosis of Predominantly Antibody Deficiency (PAD), with suspected X-linked Agammaglobulinemia (XLA). Treatment was initiated with administration of intravenous gamma globulin 400 mg/kg every four weeks, with a significant improvement of the condition. Discussion: usually, the diagnosis of XLA tends to be made in the first three years of life. However, in this report, although the first manifestations started at eight months of age, there was a delay of ten years before starting the treatment. In fact, the diagnosis of children and adults with IEI can be delayed if healthcare professionals are unable to find the true cause of recurrent infections. Therefore, the relevance of considering such pathologies in the presence of risk signs is highlighted, as early diagnosis being essential in treating and preventing morbidities.

Resumo Objetivos: apresentar um caso de Erro Inato da Imunidade (EII) como diagnóstico em potencial de pacientes pediátricos com infecções de repetição. Descrição: paciente masculino, 13 anos, desde os oito meses de idade apresentou quadros repetidos de diarreias, sinusites, otites, abscessos e infecções do trato urinário; destacando-se a otite, sinusite e diarreia pela maior recorrência. Aos dez anos, quando apresentou mastoidite evoluindo para meningite, foi internado em um hospital terciário, onde foi realizada avaliação imunológica, a qual levou ao diagnóstico de Deficiência Predominantemente de Anticorpos (DPAs), tendo como suspeita a agamaglobulinemia ligada ao cromossomo X (ALX). Foi iniciado tratamento com administração de gamaglobulina endovenosa 400 mg/kg a cada quatro semanas, ocorrendo melhora significativa do quadro. Discussão: normalmente, o diagnóstico da ALX tende a ser feito nos primeiros três anos de vida. Neste relato, entretanto, embora as primeiras manifestações tenham iniciado aos oito meses de idade, ocorreu um atraso de dez anos até o início do tratamento. De fato, o diagnóstico de crianças e adultos com EII pode ser retardado se os profissionais de saúde não conseguirem encontrar a causa das infecções recorrentes. Destaca-se, portanto, a relevância de se considerar tais patologias na vigência de sinais de riscos, pois o diagnóstico precoce é fundamental para tratar e prevenir morbidades.

Anafilaxia a alfa-gal: um relato de caso paraibano / Alpha-gal anaphylaxis: a case report from Paraíba, Brazil

O presente relato apresenta um caso de anafilaxia tardia ao carboidrato alfa-gal em um adolescente da cidade de Belém, na Paraíba, Brasil. O paciente desenvolveu reação tardia à ingesta de carne e vísceras de animais. Ele mora em fazenda e tem contato próximo com animais potencialmente contaminados por carrapatos. Essa causa de reação alérgica é nova, e estudos começaram a atribuí-la a casos antes ditos idiopáticos. A anafilaxia é uma reação potencialmente fatal, que deve ser prontamente diagnosticada e tratada. Sendo assim, a descoberta de seu fator desencadeante é um dos principais itens que direcionam o tratamento. No Brasil, nenhum caso de anafilaxia por alfa-gal foi antes descrito na literatura local.

This report presents a case of late anaphylaxis to alpha-gal carbohydrate in a teenager living in the city of Belém, Paraíba, Brazil. The patient developed a late reaction to eating meat and offal of animals; he lives on a farm and has close contact with animals potentially contaminated by ticks. This cause of allergic reaction is new, and studies have started to attribute it to cases previously said to be idiopathic. Anaphylaxis is a potentially fatal reaction that must be promptly diagnosed and treated. Thus, the discovery of its triggering factor is one of the main items that guide treatment. In Brazil, no case of alpha-gal anaphylaxis had been described in the local literature.

Biosynthesis of silver nanoparticles employing Trichoderma harzianum with enzymatic stimulation for the control of Sclerotinia sclerotiorum.

Biogenic synthesis of silver nanoparticles employing fungi offers advantages, including the formation of a capping from fungal biomolecules, which provides stability and can contribute to biological activity. In this work, silver nanoparticles were synthesized using Trichoderma harzianum cultivated with (AgNP-TS) and without enzymatic stimulation (AgNP-T) by the cell wall of Sclerotinia sclerotiorum. The nanoparticles were evaluated for the control of S. sclerotiorum. The specific activity of the T. harzianum hydrolytic enzymes were determined in the filtrates and nanoparticles. Cytotoxicity and genotoxicity were also evaluated. Both the nanoparticles exhibited inhibitory activity towards S. sclerotiorum, with no new sclerotia development, however AgNP-TS was more effective against mycelial growth. Both the filtrates and the nanoparticles showed specific enzymatic activity. Low levels of cytotoxicity and genotoxicity were observed. This study opens perspectives for further exploration of fungal biogenic nanoparticles, indicating their use for the control of S. sclerotiorum and other agricultural pests.

Characterization of Articaine-Loaded Poly(ε-caprolactone) Nanocapsules and Solid Lipid Nanoparticles in Hydrogels for Topical Formulations.

This work describes the development of poly-ε-caprolactone nanocapsules (PCL-NC) and solid lipid nanoparticles (SLN) aiming delivery for articaine (ATC), in order to improve its chemical stability in semi-solid preparations looking forward their use for skin delivery. The nanoparticles were characterized by size, polydispersity index, and pH. Cellular viability was evaluated using the MTT test and the in vitro release kinetics was determined using a two-compartment model. The hydrogels with nanoparticle suspensions were characterized considering their rheological aspects and in vitro permeation across artificial membranes. Colloidal stability was satisfactory, since the formulations did not present major alterations during 120 days. High ATC encapsulation was achieved (78% for PCL-NC and 65% for SLN). The release profile of PCL-NC-ATC was slower, compared to the free molecule and SLN-ATC. MTT experiments showed the nanosystems were capable to increase cellular viability compared with free ATC. The hydrogels showed good consistency, homogeneity, and stability and presented pseudoplastic behavior with thixotropy, improving drug efficacy in clinical applications. The gel based on PCL-NC showed faster onset of activity and flux of 35.68 ± 1.98 µg/cm2/h, which then continued for up to 8 h. This study opens up prospects for employment of nanoparticulate systems for modified release of ATC.

A relação mãe-filha, a devastação e a escrita / Mother-daughter relationship, devastation and writing

A leitura dos textos freudianos, lacanianos e de outros autores que teorizaram sobre a feminilidade, a sexualidade feminina, a escolha de objeto, a relação mãe-filha e a devastação, possibiltou a escrita deste texto artigo cujo título “A relação mãe-fiIha, a devastação e a escrita” sugere e marca a possibilidade do tornar-se mulher como saída diante da inexistência de um significante que dê conta do que é ser uma mulher. Em um percurso vasto, de Freud a Lacan, o enigma da feminilidade e o trabalho psíquico envolvido no devir da mulher são questões fundamentais. Onde Freud pergunta “O que quer uma mulher?”, Lacan permite um reajuste e diz se tratar do que deseja uma mulher. Freud fez da “inveja do pênis” o rochedo incontornável no fim da análise das mulheres e colocou do lado dos homens a ameaça de castração. Lacan concluiu que além da mulher não existir, não há relação sexual. Assim, sustentar o enigma, atravessar o fascínio e o ódio presentes na devastação, amar, gozar, desejar, são questões fundamentais que se colocam e que só podem ser elaboradas de forma única, isto é, por cada mulher, uma a uma. A escrita, que é real, está apontada, neste artigo, como uma possibilidade de dizer do feminino e só se torna viável enquanto escrita de uma impossibilidade, incluindo a falta, a perda e a causa(AU)

A reading of Freud’s texts, Lacan and other authors who theorized about femininity – female sexuality, choice of object, the mother-daughter relationship and devastation – enabled the writing of this article entitled “The mother-daughter relationship, the devastation and writing” suggests and marks the possibility of becoming a woman as output before the lack of a signifier which takes account of what being a woman means. In a broad way, from Freud to Lacan, the riddle of femininity and the psychic work involved in the woman’s becoming are key issues. Where Freud question “What does a woman want?” Lacan allows an adjustment and says it is about what the woman desires. Freud made the “penis envy” the unavoidable rock at the end of the analysis of women and placed the men’s side the threat of castration. Lacan concluded that besides the inexistence of the woman, there is no sexual relationship. Thus sustaining the puzzle, crossing the fascination and the hatred present in the ideas of devastation, love, enjoy, like, are fundamental questions that arise and that can only be developed in a unique way, that is, for each woman, one by one. The writing, which is real, is pointed out, in this work, as a possibility to say the female and only becomes viable as writing an impossibility, including lack, loss and cause(AU)

Biogenic silver nanoparticles based on trichoderma harzianum: synthesis, characterization, toxicity evaluation and biological activity.

White mold is an agricultural disease caused by the fungus Sclerotinia sclerotiorum, which affects important crops. There are different ways of controlling this organism, but none provides inhibition of its resistance structures (sclerotia). Nanotechnology offers promising applications in agricultural area. Here, silver nanoparticles were biogenically synthesized using the fungus Trichoderma harzianum and characterized. Cytotoxicity and genotoxicity were evaluated, and the nanoparticles were initially tested against white mold sclerotia. Their effects on soybean were also investigated with no effects observed. The nanoparticles showed potential against S. sclerotiorum, inhibiting sclerotia germination and mycelial growth. Nanoparticle characterization data indicated spherical morphology, satisfactory polydispersity and size distribution. Cytotoxicity and genotoxicity assays showed that the nanoparticles caused both the effects, although, the most toxic concentrations were above those applied for white mold control. Given the potential of the nanoparticles against S. sclerotiorum, we conclude that this study presents a first step for a new alternative in white mold control.

Qualidade de saneantes e antissépticos utilizados em hospitais da rede pública / Quality of the sanitizing and products used in public hospitals

Neste estudo, foi avaliada a qualidade de produtos saneantes e antissépticos utilizados em hospitais da rede pública, por terem papel relevante como medida de controle e prevenção das infecções e doenças. O presente estudo, proveniente do Programa de Monitoramento do Município de São Paulo, avaliou 54 produtos, dos quais 47 contidos em embalagens originais lacradas e sete previamente diluídos nos hospitais. Vinte e seis amostras de detergentes e desinfetantes foram avaliadas quanto aos parâmetros microbiológicos, pH e teor de substância ativa; 25 antissépticos e três produtos esterilizantes foram analisados somente quanto ao teor de componente ativo. Cinco das amostras de desinfetantes em embalagem original estavam em desacordo com a legislação vigente, no que diz respeito ao componente ativo, à ausência de atividade antimicrobiana e/ou rotulagem. Sobre as amostras de desinfetantes diluídos nos hospitais, 60 por cento não apresentaram atividade antimicrobiana. Das 13 amostras de detergentes, 30,8 por cento demonstraram contaminação microbiana, e em três amostras foi detectada a ocorrência de Pseudomonas, o que indica a necessidade de melhoria nos processos de fabricação. Os produtos antissépticos e detergentes foram considerados satisfatórios em relação aos demais ensaios realizados. Ressalta-se, ainda, a escassez de publicações de dados de programas de monitoramento de produtos dessas categorias no nosso país.

An illustrative case of Léri-Weill dyschondrosteosis

We report on a girl presenting Léri-Weill dyschondrosteosis (LWD) due to deletion of the SHOX gene. Her family included individuals with short stature alone or with both short stature and mesomelia or Madelung's deformity. The deletion was demonstrated through detection of hemizygosity for microsatellite markers SHOX-CA repeat, DXYS10092, DXYS10093 and DXYS10091 localized around the SHOX gene, with retention of paternal alleles in the proband and three of her sisters who had short stature as the only clinical feature. Hemizygosity for these loci was also observed in their mother, who had short stature too. The deletion in the proband was however larger, including locus DXY10083. The proband's only sister with normal height did not carry the deletion. Family history suggests transmission of the deletion from the proband's maternal great-grandfather to her grandfather via the Y chromosome, and from the grandfather to the proband's mother via the X chromosome after crossing-over in the pseudoautosomal region proximal to the SHOX gene.