High microbiome variability in pediatric tracheostomy cannulas in patients with similar clinical characteristics

Abstract Objectives: To evaluate the bacterial microbiome found in tracheostomy cannulas of a group of children diagnosed with glossoptosis secondary to Robin Sequence (RS), and its clinical implications. Methods: Pediatric patients were enrolled in the study at the time of the cannula change in the hospital. During this procedure, the removed cannula was collected and stored for amplicon sequencing of 16s rRNA. DNA extraction was performed using DNeasy PowerBiofilm Kit (QIAGEN® - Cat nº 24000-50) while sequencing was performed with the S5 (Ion S5™ System, Thermo Fisher Scientific), following Brazilian Microbiome Project (BMP) protocol. Results: All 12 patients included in the study were using tracheostomy uncuffed cannulas of the same brand, had tracheostomy performed for over 1-year and had used the removed cannula for approximately 3-months. Most abundant genera found were Aggregatibacter, Pseudomonas, Haemophilus, Neisseria, Staphylococcus, Fusobacterium, Moraxella, Streptococcus, Alloiococcus, and Capnocytophaga. Individual microbiome of each individual was highly variable, not correlating to any particular clinical characteristic. Conclusion: The microbiome of tracheostomy cannulas is highly variable, even among patients with similar clinical characteristics, making it challenging to determine a standard for normality. © 2022 Associa¸c˜ao Brasileira de Otorrinolaringologia e Cirurgia C´ervico-Facial. Published by Elsevier Editora Ltda. This is an open access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/).

Prophylaxis in hemophilia

Hemophilia is an inherited X-linked coagulopathy defined by a deficiency or abnormality in the clotting function of factor VIII (Hemophilia A) or factor IX (Hemophilia B). Prophylaxis ­ the regular administration of therapeutic products to maintain hemostasis and prevent bleeding ­ is the mainstream of treatment. Addressing the development and scientific evidence for administrating prophylaxis is the goal of this review. Prophylaxis is the therapeutic modality of choice for people with severe hemophilia, being considered, in principle, a lifelong treatment. It should have an early onset, ideally as a primary, or at least secondary. Even lifelong tertiary prophylaxis seems to offer benefit, although further studies are still lacking. Individualized strategies should lead to an optimization of the dilemma between better joint outcomes versus involved costs.

Hemophilia: a biography on therapeutical approaches

The history of hemophilia is ancient, with descriptions dated to the 2nd century AD. The first modern narratives appeared in 1800s, when total blood transfusion was the only available treatment and life expectancy was remarkably low. Advances occurred with the use of plasma and cryoprecipitate, but only the discovered of factor concentrates revolutionized the treatment. The implantation of prophylaxis allowed hemophilic patients to prevent bleeding and the development of chronic arthropathy, although with a significant burdensome with the regular infusions. In the past 20 years, this field has witnessed major improvements, including the development of gene therapy and other pharmacological approaches.

Translation and validation of the drooling impact scale questionnaire into Brazilian Portuguese / Tradução e validação do questionário da drooling impact scale para o português do Brasil

Abstract Introduction Inadequate drooling can cause serious clinical, functional and social problems. Validated questionnaires to evaluate drooling impact on quality of life are lacking in Brazilian Portuguese. Objectives To translate and validate the drooling impact scale to Brazilian Portuguese. Methods The drooling impact scale was translated to Brazilian Portuguese and back- translated to English to assess potential conceptual differences. Brazilian Portuguese version of drooling impact scale was applied to a 40 patients' sample of sialorrhea presenting pediatric patients (up to 20 years of age). Chronbach's alpha, exploratory factorial analysis and confirmatory factorial analysis were then proceeded with data collected. Results The mean drooling impact scale value for the whole population was 51.77 (SD = 16.13). The internal consistency obtained with Cronbach's alpha indicated a value of 0.72 for the entire sample. The Bartlett's test of sphericity was significant (p< 0.0001), confirming correlation among variables tested. Kaiser-Meyer-Olkin measure of sampling adequacy revealed a value of 0.72, indicating that the correlation matrix was reasonably suitable for factor analysis. Regarding exploratory factorial analysis, parallel analysis suggested a two-factor solution that was used for confirmatory factorial analysis. The first factor was responsible for 33.78% of the variance with an Eigenvalue of 3.38. The second factor explained 16.1% of the variance with an Eigenvalue of 1.61. At confirmatory factorial analysis, the two-factor model showed consistently better adjustments parameters than the one-factor model. Conclusion The drooling impact scale has been successfully translated to Brazilian Portuguese language, showing adequate internal validity. Validation of this instrument allows physicians and other personnel involved in the care of these patients to perform a better management of patients experiencing drooling. With this tool, we are now able to guide routines and provide guidelines both before and after the different kinds of treatments in order to improve the general well-being of the patient and his family.

Resumo Introdução A produção inadequada de saliva pode causar sérios problemas clínicos, funcionais e sociais. Questionários validados para avaliar o impacto da salivação na qualidade de vida em português do Brasil são necessários. Objetivos Traduzir e validar a Drooling Impact Scale para o português do Brasil. Método A Drooling Impact Scale foi traduzida para português do Brasil e retrotraduzida para o inglês para avaliar possíveis diferenças conceituais. A versão em português do Brasil da Drooling Impact Scale foi aplicada a uma amostra de 40 pacientes pediátricos que apresentavam sialorreia (até 20 anos). Alfa de Cronbach, análise fatorial exploratória e análise fatorial confirmatória foram então feitos com os dados coletados. Resultados O valor médio da Drooling Impact Scale para toda a população foi de 51,77 (DP = 16,13). A consistência interna obtida com o alfa de Cronbach indicou um valor de 0,72 para toda a amostra. O teste de esfericidade de Bartlett foi significante (p < 0,0001), confirmou a correlação entre as variáveis testadas. A medida de adequação da amostra de Kaiser-Meyer-Olkin revelou um valor de 0,72, indicou que a matriz de correlação era razoavelmente adequada para a análise fatorial. Em relação à análise fatorial exploratória, a análise paralela sugeriu uma solução de dois fatores, que foi usada para a análise fatorial confirmatória. O primeiro fator foi responsável por 33,78% da variância com um autovalor de 3,38. O segundo fator explicou 16,1% da variância com um autovalor de 1,61. Na análise fatorial confirmatória, o modelo de dois fatores mostrou parâmetros de ajustes consistentemente melhores do que o modelo de um fator. Conclusão A Drooling Impact Scale foi traduzida com sucesso para o português do Brasil, apresentou validade interna adequada. A validação desse instrumento permite que médicos e outras pessoas envolvidas no cuidado desses pacientes façam um melhor manejo dos pacientes com sialorreia. Com essa ferramenta, agora somos capazes de orientar rotinas e fornecer orientações antes e depois dos diferentes tipos de tratamentos, a fim de melhorar o bem-estar geral do paciente e de sua família.

Validation of blood components transport through a pneumatic tube system

ABSTRACT Introduction: Urgent blood component transfusions may be life-saving for patients in hemorrhagic shock. Measures to reduce the time taken to provide these transfusions, such as uncrossmatched transfusion or abbreviated testing, are available. However, transport time is still an additional delay and the use of a pneumatic tube system (PTS) may be an alternative to shorten the transport time of blood components. Objectives: To assess pneumatic tube system transportation of blood components based on a validation protocol. Methods: Pre- and post-transport quality control laboratory parameters, visual appearance, transport time and temperature of the packed red blood cells (RBCs), thawed fresh plasma (TFP), cryoprecipitate (CR), and platelet concentrate (PC) were evaluated. Parameters were compared between transport via pneumatic tube and courier. Results: A total of 23 units of RBCs, 50 units of TFP, 30 units of CR and ten units of PC were evaluated. No statistically significant differences were found between pre- and post-transport laboratory results. There was also no difference in laboratory parameters between transport modalities (PTS versus courier). All blood components transported matched regulatory requirements for quality criteria. The temperature during transport remained stable and the transport time via PTS was significantly shorter than the courier's transport time (p < 0.05). Conclusion: The PTS was considered a fast, safe and reliable means of transportation for blood components, also securing quality prerequisites.

Prevalence of thrombophilia-associated genetic risk factors in blood donors of a regional hospital in southern Brazil

ABSTRACT Introduction: Thromboembolic events occur due to an imbalance in the hemostasis and some factors associated with this condition can be inherited. In order to evaluate the frequency of genotypes considered to be common hereditary risk factors for thrombophilia associated with venous thrombosis (g.1691G>A and g.20210G>A) and hyperhomocysteinemia (g.677C>T and g.1298A>C), samples from voluntary healthy blood donors at the Hospital de Clínicas de Porto Alegre were tested. Methods: We examined 325 blood samples from blood donors collected from October 2017 to July 2018. Blood was collected on filter paper and the DNA was extracted for single nucleotide polymorphisms (SNPs) analysis using the qualitative real time polymerase chain reaction. Results: The calculated frequencies of each genetic variant in heterozygosity were 4% for the FV gene (g.1691G> A), 4% for the F2 gene (g.20210G> A) and 42% and 39% for methylenetetrahydrofolate reductase (MTHFR), g.677C>T and g.1298A>C, respectively. Only the genetic variants of MTHFR were found in homozygosity, with frequencies of 14% and 6% (g.677C>T and g.1298A>C), respectively. Discussion: Altogether, these results describe the frequencies of genetic variants associated with venous thrombosis and hyperhomocysteinemia in the analyzed group and are important to enhance our current knowledge about the genetic profiles of Brazilian blood donors.

Epidemiological characteristics of hemophilia in the pre-primary prophylaxis era: a historical cohort

Introduction: Epidemiological studies on hemophilia in the Brazilian population are historically scarce. Despite the continuous effort made by the National Program of Inherited Bleeding Disorders to map this condition, little information is available, especially on the period prior to program conception. Therefore, the present study aims to assess the epidemiological, serological, and clinical characteristics of patients with hemophilia in the state of Rio Grande do Sul, Brazil. Methods: A total of 455 patients had their medical records reviewed from January 1, 2003 to December 31, 2007. Results: We observed a remarkable prevalence of hepatitis C virus (HCV) infection in patients with both hemophilia A and B, and this prevalence significantly increased along with age (p < 0.001). No positive anti-HCV results were observed among children younger than 5 years old. There was a significant correlation between the severity of hemophilia and the number of arthropathies in all age categories. Considering the presence of inhibitors, a significant difference was observed between age groups, as older patients had higher inhibitor titers. There was a significant correlation between mean coagulation factor consumption and the number of arthropathies in patients over 5 years old. Conclusions: This profile analysis of patients with hemophilia reflects a gradual improvement in treatment safety and efficiency, as well as the need for continued investment in this population. (AU)

Cost of chronic red blood cell transfusion in the Brazilian private healthcare sector from a payer perspective / Custo de transfusão crônica de concentrado de hemácias na perspectiva do sistema de saúde suplementar brasileiro

Objective: In the private healthcare sector in Brazil, the dearth of information regarding transfusion cost may compromise the management of conditions requiring long-term transfusion. Therefore, the present study aimed to estimate the cost of chronic red blood cell (RBC) transfusion in this context. Methods: A payer perspective was chosen for the analysis. A survey performed by an expert advisory board gathered information on the amounts reimbursed by health plans to blood centers per outpatient transfusion of a single RBC bag in multitransfused patients. Survey results were contrasted to RBC transfusion cost calculated using Brazilian Hierarchical Classification of Medical Procedures (CBHPM) parameters from 2018 and 2010, the latter suggested by the advisory board as more accurately reflecting market prices. Results: Six blood centers in the South and Southeast of Brazil were surveyed. The median amount reimbursed per RBC unit was R$ 1,066.44 (interquartile range: R$ 665.00-1,252.00). The mean amount reimbursed was R$ 959.54 ± R$ 337.14 (minimum: R$ 295.00 ­ maximum: R$ 1,980.00). Using 2018 CBHPM parameters, the cost of transfusing one RBC unit was calculated as R$ 1,905.18. Using 2010 CBHPM parameters, the cost was R$ 1,119.69 per RBC unit. Conclusions: Analyses using 2018 CBHPM parameters may lead to overestimation of transfusion cost. The best estimate for outpatient transfusion of one RBC bag in the private health care sector in Brazil lies between the observed reimbursed values and 2010 CBHPM cost. The present results provide valuable information for future cost-effectiveness analyses focusing on disorders whose treatment involves routine RBC transfusion.

A carência de informações sobre custo de transfusão sanguínea na saúde suplementar brasileira pode comprometer análises econômicas e eventuais decisões relacionadas a esse procedimento no longo prazo. O objetivo deste estudo foi estimar o custo de transfusão crônica de concentrado de hemácias (CH) nesse contexto. Métodos: Foi escolhida a perspectiva do pagador. Uma pesquisa foi realizada com especialistas para levantamento de valores reembolsados para transfusão de uma bolsa de CH em pacientes politransfundidos. Os resultados da pesquisa foram comparados ao custo de transfusão de CH calculado utilizando a Classificação Brasileira Hierarquizada de Procedimentos Médicos (CBHPM), edições de 2018 e 2010, sendo a edição mais antiga sugerida pelos especialistas como estando mais de acordo com a realidade de mercado. Resultados: Seis hemocentros localizados no Sul e Sudeste do Brasil foram incluídos. Os valores mediano e médio reembolsados aos hemocentros foram R$ 1.066,44 (amplitude interquartil: R$ 665,00-1.252,00) e R$ 959,54 ± R$ 337,14 (mínimo: R$ 295,00 ­ máximo: R$ 1.980,00), respectivamente. Com base nos parâmetros da CBHPM 2018 e 2010, os custos de transfusão calculados para uma bolsa de CH foram R$ 1.905,18 e R$ 1.119,69, respectivamente. Conclusões: Análises utilizando parâmetros da CBHPM 2018 podem superestimar os custos de transfusão de CH. Na perspectiva do sistema de saúde suplementar brasileiro, a melhor estimativa de custo de transfusão por bolsa de CH está entre o valor reembolsado e o valor calculado utilizando a CBHPM 2010. O presente estudo apresenta informações importantes para futuras análises de custo-efetividade de patologias que envolvem tratamentos à base de politransfusão de CH.

Infecção por SARS-CoV-2 em paciente com agamaglobulinemia ligada ao X tratado com altas doses de imunoglobulina endovenosa e plasma de convalescente / SARS-CoV-2 infection in a patient with X-linked agammaglobulinemia treated with high doses of intravenous immunoglobulin and convalescent plasma

A pandemia pelo vírus SARS-CoV-2 atingiu adultos, crianças e penalizou indivíduos idosos e com comorbidades como diabetes, doença cardíaca, hipertensão e obesidade. A maioria dos infectados são assintomáticos ou têm sintomas leves, entretanto 15% podem apresentar pneumonia e 5% síndrome respiratória aguda grave. Apresentamos um caso de agamaglobulinemia ligada ao X (XLA) em paciente masculino de 27 anos que se infectou com SARS-CoV-2. Os pacientes com XLA não possuem linfócitos B e não produzem anticorpos devido a uma mutação no gene Bruton tirosino-quinase (BTK), responsável pela maturação dos linfócitos B. Ele infectou-se e foi internado em hospital de Ivoti/RS. A evolução da pneumonia foi rápida, necessitando transferência para o Hospital de Clínicas de Porto Alegre (HCPA) no 10° dia de evolução. Iniciou com infusão de imunoglobulinas, tendo utilizado o total de 400 gramas devido ao intenso catabolismo da IgG, mantendo-se sua concentração entre 700-900 mg/dL. Necessitou de ventilação mecânica, oxigenação por membrana extracorpórea (ECMO) e hemodiálise. Foi administrado plasma de convalescente (PC), 300 mL, por duas vezes, com melhora clínico-radiológica e retirada da ventilação mecânica. Piorou e repetiu outras 4 infusões de PC (total de 1717 mL), negativando o vírus na orofaringe (RT-PCR). Em 3 ocasiões teve sepse, debelada rapidamente. Apresentou anemia, com necessidade de transfusão frequente. Identificou-se linfopenia de CD3, CD4, CD8, NK e ausência de linfócitos B. A linfopenia foi revertida com a recuperação clínica e a alta hospitalar aconteceu no 70° dia de internação.

The SARS-CoV-2 pandemic has affected adults and children and penalized older people and those with comorbidities such as diabetes, heart disease, hypertension and obesity. Most of those infected are asymptomatic or have mild symptoms, but 15% may have pneumonia and 5% acute respiratory distress syndrome. We report a case of X-linked agammaglobulinemia (XLA) in a 27-yearold man who was infected with SARS-CoV-2. XLA patients do not have B lymphocytes and do not produce antibodies because of mutations in the Bruton tyrosine-kinase gene, responsible for the maturation of B cells. This patient was infected and then admitted to a hospital in Ivoti, southern Brazil. Pneumonia progressed rapidly, requiring transfer to the Hospital de Clínicas de Porto Alegre on the 10th day. Intravenous immunoglobulin infusions were initiated, using a total of 400 grams because of an intense catabolism of IgG, and the concentration was kept around 700- 900 mg/dL. Mechanical ventilation, extracorporeal membrane oxygenation and hemodialysis were necessary. Convalescent plasma (CP) was administered (2x300 mL) and then followed by clinical and radiological improvement and interruption of mechanical ventilation. Then he got sicker and had to return to invasive support and received 4 extra CP infusions (total of 1717 mL), until a negative reverse-transcriptase polymerase chain reaction (RT-PCR) for SARS-CoV-2 was obtained. On 3 occasions he had sepsis, promptly managed. He had anemia, requiring frequent transfusion, and lymphopenia (CD3, CD4, CD8, NK), with absence of B lymphocytes. Lymphopenia was reverted during recovery, and he was discharged from the hospital on the 70th day.

Quantification of peripheral blood CD34+ cells prior to stem cell harvesting by leukapheresis: a single center experience

ABSTRACT Background: Due to laboratory logistic issues, our center has traditionally scheduled peripheral blood stem cell harvests based on timing from the start of mobilization. This has proved to be useful in some cases, but also resulted in many fruitless harvests due to poor mobilization. In order to improve the efficiency of collections and compare the effectiveness of peripheral blood CD34+ cells as a predictor with data from other reports, this study analyzed the implementation of this routine. Methods: Peripheral blood and leukapheresis samples were quantified by flow cytometry and the association between these parameters was assessed. Results: Sixty-six consecutive leukapheresis samples were collected from 34 patients after the collection of peripheral blood samples for CD34+ quantification. A moderate positive correlation was observed between peripheral blood CD34+ cell count and total CD34+ cell count/kg (r = 0.596; p-value < 0.001). A multivariable regression model also confirmed this association and allowed the estimation that for every increase in five CD34+ cells/µL in the peripheral blood, a mean increase of 0.38 × 106 CD34+ cells/kg could be predicted. Demographic characteristics, baseline comorbidities and mobilization regimen did not influence final CD34+ cell count in this sample. Conclusions: As observed in other centers, quantification of peripheral blood CD34+ progenitor cells is a strong predictor of effectiveness to guide stem cell harvesting. Due to the results of this study, a modification in the peripheral blood stem cell harvesting logistics was implemented at our center in order to incorporate this routine.

Influence of dietary and physical activity restriction on pediatric adenotonsillectomy postoperative care in Brazil: a randomized clinical trial / Influência das restrições dietéticas e de atividade física no pós-operatório da adenotonsilectomia em pacientes pediátricos no Brasil: ensaio clínico randomizado

Abstract Introduction Although culturally food and physical activity restriction are part of the routine postoperative care of many Brazilian surgeons, current evidences from other countries support no such recommendations. Objective To determine whether dietary and physical restriction effectively lead to a decrease on postoperative complications of adenotonsillectomy in children when compared to no restriction. Methods We have designed a randomized clinical trial comparing two intervention: no specific counseling on diet or activity (Group A), and restriction recommendations on diet and physical activities (Group B). Caregivers completed a questionnaire on observed pain, diet and activity patterns, and medications administered. Parameters were compared at the 3rd and at the 7th postoperative day between intervention groups. Results We have enrolled a total of 95 patients, 50 in Group A and 45 in Group B. Fourteen patients were lost to follow up. Eventually, 41 patients in group A and 40 in Group B were available for final analysis. Mean age in months (A = 79.5; SD = 33.9/B = 81.1; SD = 32.6) and sex (A = 58% male; B = 64.4% male) were equivalent between groups. Pain, evaluated through visual analog scale in the 3rd (A = 2.0; IQR 1-6/B = 4.5; IQR 2-6; p = 0.18) and in the 7th (A = 1.0; IQR 1.0-4.5/B = 2.0; IQR 1.0-4.7; p = 0.29) postoperative days, was not different between groups, as was the amount of analgesics administered. Dietary and physical activity patterns also showed no statistically significant differences between groups. Conclusion Dietary and activity restriction after adenotonsillectomy does not seem to affect patients' recovery. Such information may impact considerably on the social aspects that involve a tonsillectomy, reducing the working days lost by parents and accelerating the return of children to school.

Resumo Introdução Embora culturalmente as restrições dietéticas e de atividade física sejam parte do cuidado pós-operatório de rotina de muitos cirurgiões brasileiros, evidências atuais de outros países não apoiam tais recomendações. Objetivo Determinar se as restrições dietéticas e físicas efetivamente levam a uma diminuição das complicações pós-operatórias da adenotonsilectomia em crianças quando comparadas com cuidados sem restrição. Método Realizamos um ensaio clínico randomizado comparando duas intervenções: nenhum aconselhamento específico sobre dieta ou atividade física (Grupo A) e recomendações de restrições dietéticas e de atividades físicas (Grupo B). Os cuidadores preencheram um questionário sobre a dor, a dieta e os padrões de atividade observados, e os medicamentos administrados. Os parâmetros foram comparados no 3° e no 7° dia do pós-operatório entre os grupos de intervenção. Resultados Avaliamos 95 pacientes, 50 no Grupo A e 45 no Grupo B; 14 foram perdidos no seguimento. Subsequentemente, 41 do grupo A e 40 do grupo B estavam disponíveis para a análise final. A média de idade em meses (A = 79,5, DP = 33,9/B = 81,1, DP = 32,6) e sexo (A = 58% do sexo masculino, B = 64,4% do sexo masculino) foram equivalentes entre os grupos. A dor, avaliada através da escala visual analógica no terceiro (A = 2,0; IIQ: 1-6/B = 4,5; IIR 2-6; p = 0,18) e no sétimo (A = 1,0; IIQ 1,0-4,5/B = 2,0; IIQR 1,0-4,7; p = 0,29) dia do pós-operatório, não foi diferente entre os grupos, assim como a quantidade de analgésicos administrados. Os padrões dietéticos e de atividade física também não mostraram diferenças estatisticamente significantes entre os grupos. Conclusão A restrição dietética e de atividade física após a adenotonsilectomia não parece afetar a recuperação dos pacientes. Tal informação pode ter um impacto considerável nos aspectos sociais que envolvem uma tonsilectomia, reduzir os dias de trabalho perdidos pelos pais e acelerar o retorno das crianças à escola.

Tracheostomy in children: a ten-year experience from a tertiary center in southern Brazil / Traqueostomia em crianças: uma experiência de dez anos em um centro terciário do sul do Brasil

Abstract Introduction: Children may require tracheostomy due to many different health conditions. Over the last 40 years, indications of tracheostomy have endorsed substantial modifications. Objective: To evaluate pediatric patients warranted tracheostomy at our Hospital, in regard to their indications, associated comorbidities, complications and decannulation rates. Methods: Retrospective study concerning patients under 18 years of age undergoing tracheostomy in a tertiary health care center, from January 2006 to November 2015. Results: 123 children required a tracheostomy after ENT evaluation during the study period. A proportion of 63% was male, and 56% was under one year of age. Glossoptosis was the most common indication (30%), followed by subglottic stenosis (16%) and pharyngomalacia (11%). The mortality rate was 31%. By the end of this review, 35 children (28.4%) had been decannulated, and the fewer the number of comorbidities, the greater the decannulation rate (0.77 ± 0.84 vs. 1.7 ± 1.00 comorbidities; p < 0.001). Conclusion: Tracheostomy in children is a relatively frequent procedure at our hospital. The most common indications are glossoptosis and subglottic stenosis. A high mortality rate was found, potentially substantiated by the high number of critical care patients with chronic neurological conditions in this cohort. Our decannulation rate is slightly below other series, probably because of the greater amount of patients with comorbidities.

Resumo Introdução: As crianças podem necessitar de traqueostomia devido a diferentes problemas de saúde. Ao longo dos últimos 40 anos, as indicações de traqueostomia passaram por mudanças substanciais. Objetivo: Avaliar pacientes pediátricos com traqueostomia no nosso hospital, no que diz respeito às suas indicações, comorbidades associadas, complicações e taxas de decanulação. Método: Estudo retrospectivo de pacientes com menos de 18 anos submetidos a traqueostomia em um centro de saúde terciário, de janeiro de 2006 a novembro de 2015. Resultados: 123 crianças precisaram de uma traqueostomia após avaliação otorrinolaringológica durante o período do estudo. Do total, 63% eram do sexo masculino e 56% menores de um ano. Glossoptose foi a indicação mais comum (30%), seguida por estenose subglótica (16%) e faringomalácia (11%). A taxa de mortalidade foi de 31%. Até o fim deste artigo, 35 crianças (28,4%) haviam sido decanuladas e quanto menor o número de comorbidades, maior foi a taxa de decanulação (0,77 ± 0,84 vs. 1,7 ± 1,00 comorbidades; p < 0,001). Conclusão: A traqueostomia em crianças é um procedimento relativamente frequente em nosso hospital. As indicações mais comuns são glossoptose e estenose subglótica. Uma alta taxa de mortalidade foi encontrada, potencialmente comprovada pelo elevado número de pacientes críticos com condições neurológicas crônicas nessa coorte. Nossa taxa de decanulação está ligeiramente abaixo de outras séries, provavelmente por causa da maior quantidade de pacientes com comorbidades.

Undersedation is a risk factor for the development of subglottic stenosis in intubated children / Subsedação é um fato de risco para o desenvolvimento de estenose subglótica em crianças intubadas

Abstract Objective: To analyze the level of sedation in intubated children as a risk factor for the development of subglottic stenosis. Methods: All patients between 30 days and 5 years of age who required endotracheal intubation in the pediatric intensive care unit between 2013 and 2014 were included in this prospective study. They were monitored daily and COMFORT-B scores were obtained. Flexible fiber-optic laryngoscopy was performed within eight hours of extubation, and repeated seven to ten days later if the first examination showed moderate to severe laryngeal injuries. If these lesions persisted and/or if the child developed symptoms in the follow-up period, microlaryngoscopy under general anesthesia was performed to evaluate for subglottic stenosis. Results: The study included 36 children. Incidence of subglottic stenosis was 11.1%. Children with subglottic stenosis had a higher percentage of COMFORT-B scores between 23 and 30 (undersedated) than those who did not develop subglottic stenosis (15.8% vs. 3.65%, p = 0.004). Conclusion: Children who developed subglottic stenosis were less sedated than children who did not develop subglottic stenosis.

Resumo Objetivo: Analisar o nível de sedação em crianças intubadas como um fator de risco para o desenvolvimento de estenose subglótica (ES). Métodos: Todos os pacientes entre 30 dias e cinco anos que necessitaram de intubação endotraqueal na Unidade de Terapia Intensiva Pediátrica entre 2013 e 2014 foram incluídos neste estudo prospectivo. Eles foram monitorados diariamente e foram obtidos os escores da escala Comfort-B. Foi feita laringoscopia com tubo flexível de fibra óptica em oito horas da extubação e repetida 7-10 dias depois, caso o primeiro exame tivesse mostrado lesões laríngeas moderadas a graves. Caso essas lesões tivessem persistido e/ou caso a criança tivesse desenvolvido sintomas no período de acompanhamento, foi feita microlaringoscopia sob anestesia geral para avaliar a ES. Resultados: Incluímos 36 crianças. A incidência da ES foi de 11,1%. As crianças com ES apresentaram um maior percentual de escores da escala Comfort-B entre 23 e 30 (subsedados) que os que não desenvolveram ES (15,8% em comparação com 3,65%, p = 0,004). Conclusão: As crianças que desenvolveram ES foram menos sedadas do que as que não desenvolveram.

Blood transfusion-related immunomodulation

The phenomenon of transfusion-related immunomodulation (TRIM) has been studied since the observation of a higher kidney allograft survival in patients who had received a higher number of transfusions. Conversely, it has been suggested as one of the possible causes related to the development of infections in patients with multiple blood transfusions and/or after a major surgery, and has been also associated with a decreased function of natural killer cells (NK) and antigen-presenting cells (APCs), reduced cell-mediated immunity, and increased regulatory T cells (Tregs). This review aimed to conceptualize TRIM and discuss some aspects related to its mechanisms and the prevention of immunomodulatory events.

Capillary hemangioma of the sphenoid sinus / Hemangioma capilar do seio esfenoidal

Hemangiomas of upper respiratory tract are uncommonly seen in clinical practice, and there are only a few reports of cases arising in the nasal sinuses. Specifically concerning capillary hemangiomas of the sphenoid sinus, as far as we know, there is only one case reported in the literature. This case report describes also a capillary hemangioma of the sphenoid sinus. A 26 year- old man complained of an intense headache and a retronasal bloody drainage. After some days in conservative treatment without any improvement, and after having an imaging study showing partial opacification of the sphenoid sinus, he underwent a left sphenoidectomy. The lesion identified in the region was ressected and sent to histopathological analysis, which revealed a capillary hemangioma. The patient is on follow-up for 12 months now and still asymptomatic. This case report emphasizes the importance of also considering this diagnosis when evaluating paranasal sinuses related complaints, even when faced with a clinical history and ancillary exams indicative of a common inflammatory disease. Despite the good result seen with the surgical management of this patient, this alternative should be carefully chosen, taking into account possible hemorrhagic complications of different hemangiomas.

Hemangiomas da via aérea superior são vistos raramente na prática clínica e há apenas alguns relatos de casos nos seios paranasais. Falando especificamente do hemangioma capilar do seio esfenoidal, pela nossa busca na literatura, há apenas um relato. Descrevemos aqui mais um caso de hemangioma capilar do seio esfenoidal. Paciente de 26 anos com cefaleia intensa e drenagem pós-nasal que, após alguns dias de tratamento conservador sem melhora, e tendo feito exames de imagem mostrando opacificação parcial do seio esfenóide, foi submetido a esfenoidectomia esquerda. A lesão identificada na região foi ressecada e enviada para análise histopatológica. A mesma revelou um hemangioma capilar. O paciente, com seguimento de 12 meses, permanece assintomático. Esse relato enfatiza a importância de considerar esse diagnóstico quando avaliadas queixas relacionadas aos seios paranasais, mesmo quando a história e exames indiquem uma patologia inflamatória comum. Apesar do bom resultado cirúrgico desse paciente, essa alternativa terapêutica deve ser considerada com cuidado, levando em conta as possíveis complicações hemorrágicas dos diferentes hemangiomas.

Rhinosinusitis in autologous and allogeneic bone marrow transplantation: a retrospective study on the performance of imaging studies on severity and prognostic evaluation / Rinossinusite em transplante de células-tronco hematopoéticas autólogo e alogênico: um estudo retrospectivo sobre o desempenho de estudos de imagem na avaliação de severidade e prognóstico

The objective of this work was to evaluate the diagnostic and prognostic performance of a traditional imaging staging system for rhinosinusitis in the bone marrow transplantation (BMT) scenario. A retrospective cohort study was carried out at a bone marrow transplantation referral center involving subjects who underwent allogeneic or autologous BMT from September 1st 2005 to September 31st 2007 and later evolved with rhinosinusitis during the BMT inpatient period. Patients who had a previous history of sinusal disease or otolaryngologic surgery were excluded from the study. Data concerning mortality, the treatment of rhinosinusitis and BMT outcomes were extracted from medical files. The collected parameters were compared to the Lund-Mackay tomographic staging system score which was calculated based on available tomography films of each patient. A total of 85 BMT were performed and 23 allogeneic and 14 autologous (43.5 percent) BMT patients evolved with rhinosinusitis during transplantation. A significant association with LMS was found for the absolute neutrophil count (ANC), with a higher ANC (>500/mm3) correlating with a higher LMS (Mean LMS for lower ANC 6.08 and higher ANC 9.71 points, p<0.05). Need for surgical management and post-BMT admissions, the resolution of the rhinosinusitis and overall mortality had no significant correlation with LMS. Patients with less than 500 neutrophils/mm3 are known to be prone to more severe infections, but paradoxically showed lower LMS when developing rhinosinusitis. However, there were no differences in the main outcomes between those with higher and lower LMS. This would possibly lead to an equivocal assumption of a less severe disease. Severely neutropenic patients are probably not able to mount an effective inflammatory response capable of inducing significant tomographic abnormalities. So, this imaging study would not be able to adequately evaluate the extent of sinusal involvement. We thus ...

O objetivo deste trabalho foi avaliar o desempenho diagnóstico e prognóstico de um escore de estadiamento de rinossinusite (RS) por tomografia em pacientes submetidos a transplante de medula óssea (TMO). Realizou-se um estudo de coorte retrospectivo de pacientes submetidos a transplante de medula óssea (autólogo e alogênico) de 1º de setembro de 2005 a 31 de setembro de 2007 que desenvolveram RS durante o período de internação do transplante. Pacientes com história prévia de doença sinusal ou cirurgia otorrinolaringológica foram excluídos do estudo. Dados relacionados à mortalidade, resolução da RS e desfechos do TMO foram extraídos do prontuário médico. Os parâmetros coletados foram correlacionados com o escore de estadiamento de Lund-Mackay (ELM), que foi calculado com base nas alterações tomográficas de cada paciente. Um total de 85 TMO foram realizados e 37 (23 alogênicos e 14 autólogos) destes pacientes desenvolveram RS durante o transplante. Uma correlação significativa com o ELM foi encontrada quando se considerava a contagem absoluta de neutrófilos (CAN), com uma CAN mais alta (>500/mcl) se associando com um ELM de maior valor (média de escore para CAN baixa 6,08 e CAN alta 9,71 pontos, p<0,05). A necessidade de intervenção cirúrgica e reinternações pós-TMO, resolução da RS e mortalidade geral não mostraram correlação com o ELM. Mesmo assumindo que a neutropenia severa é um fator de risco relevante para intercorrências infecciosas durante o TMO, paradoxalmente, os pacientes com menos de 500 neutrófilos/mcl mostraram um ELM de menor severidade, embora não tenham evoluído de maneira diferente daqueles com maior ELM. É provável que ELM mais alto esteja simplesmente ligado ao fato de uma CAN mais alta levar a uma maior reação inflamatória e consequente alteração tomográfica. Desta forma, o ELM não parece útil na avaliação de pacientes altamente imunossuprimidos como os do TMO. Rev. Bras. Hematol. Hemoter.

Análise do perfil de solicitações para transfusão de hemocomponentes no Hospital de Clínicas de Porto Alegre no ano de 2005 / Blood-based products request profile analysis in Hospital de Clínicas de Porto Alegre in 2005

O uso de hemocomponentes em hospitais de alta complexidade é elevado, sendo necessário controle e racionalização de sua utilização, dada sua escassez e alto custo. Para tal, muitos hospitais desenvolveram protocolos para uso de hemocomponentes. O objetivo deste trabalho foi descrever as características epidemiológicas das solicitações de hemoderivados e sua adequação em conformidade ao protocolo vigente no Hospital de Clínicas de Porto Alegre. Por meio de estudo retrospectivo transversal, foram avaliadas a freqüência de transfusões e suas indicações no ano de 2005 em três áreas (Internação Clínica, Cirúrgica e Centro de Terapia Intensiva). Analisaram-se as justificativas de cada solicitação em conformidade com o protocolo vigente. Foram identificadas 12.175 solicitações de hemoderivados no ano de 2005. O hemocomponente mais solicitado foi o concentrado de hemácias (6.578 solicitações), seguido do concentrado de plaquetas (4.133), plasma fresco (1.296) e do crioprecipitado (168). As perdas representaram 2,96 por cento dos eventos. As três áreas foram responsáveis por 59,77 por cento das solicitações de hemocomponentes. A internação clínica apresentou 85,57 por cento de solicitações em conformidade com o protocolo, seguida da CTI com 81,4 por cento e da cirurgia com 71,42 por cento. A maioria das solicitações de hemocomponentes esteve de acordo com o protocolo da instituição no ano de 2005. A internação clínica teve o perfil mais adequado de solicitações no ano de 2005, seguida do CTI e, por fim, da internação cirúrgica.

The use of blood-based products in a tertiary hospital is elevated so control and rational use is necessary. Many hospitals have protocols for the use of blood components. The aim of this work was to describe the epidemiological characteristics of blood-based product requests and their adjustment to the protocol of Hospital de Clínicas in Porto Alegre. Using a cross-sectional retrospective study, we analyzed the frequencies and indications of transfusion requests in 2005 in three different sectors (Clinical, Surgical and Intensive Care Unit). Each transfusion request was analyzed according to the current protocol. A total of 12,175 transfusion requests were analyzed. Requests for blood (6,578) were the commonest, followed by platelets (4,133), fresh frozen plasma (1296) and cryoprecipitate (168). Lost data represented 2.96 percent of the total events. The three sectors were responsible for 59.77 percent of blood-based product requests. The Clinical Sector requested 85.57 percent of its transfusions satisfactorily, followed by the ICU (81.4 percent) and finally the Surgical Sector (71.42 percent). The majority of transfusion requests in HCPA in 2005 were adequate according to the protocol. The Clinical Sector had the best request profile, followed by the ICU and the Surgical Sectort.