Abstract Objective The endoscopic release of the ulnar nerve reproduces a simple (in situ) procedure with smaller incisions, less soft tissue damage, and higher preservation of nerve vascularization. Endoscopy allows the clear visualization of the entire path of the nerve and surrounding noble structures. Moreover, it reveals any signs of compression and allows a safe release of 10cm distally or proximally to the medial epicondyle. Methods A retrospective survey revealed that 15 subjects (1 with a bilateral injury) underwent an ulnar nerve compression release at the elbow using the endoscopic technique with Agee (Micro-Aire Sugical Instruments, Charlottesville, VA, EUA) equipment from January 2016 to January 2020. Results Symptoms of ulnar nerve compression improved in all patients; on average, they resumed their work activities in 26.5 days. There was no recurrence or need for another procedure. In addition, there were no severe procedure-related complications, such as infection and nerve or vascular injury. One patient had transient paresthesia of the sensory branches to the forearm, with complete functional recovery in 8 weeks. Conclusion Our study shows that the endoscopic release of the ulnar nerve at the elbow with the Agee equipment is a safe, reliable technique with good outcomes.

Resumo Objetivo A liberação endoscópica do nervo ulnar permite reproduzir uma liberação simples (in situ), mas através de incisões menores e com menor lesão de partes moles e uma maior preservação da vascularização do nervo. A visualização clara através da endoscopia permite observar todo o trajeto do nervo e das estruturas nobres circundantes, mostrando os sinais de compressão, possibilitando realizar a liberação de forma segura em um trajeto de 10 cm nos sentidos distal e proximal ao epicôndilo medial. Método Foram encontrados, de forma retrospectiva, no período entre janeiro de 2016 e janeiro de 2020, 15 pacientes (sendo 1 com lesão bilateral) submetidos a liberação da compressão do nervo ulnar no cotovelo pela técnica endoscópica com equipamento de Agee (Micro-Aire Sugical Instruments, Charlottesville, VA, EUA). Resultados Todos os pacientes tiveram melhora dos sintomas de compressão do nervo ulnar e o período de retorno ao trabalho foi de em média 26,5 dias. Não houve recidivas e não houve a necessidade de outro procedimento. Também não houve complicações graves decorrentes do procedimento, como infecção, lesão nervosa ou vascular. Em um paciente, houve parestesia transitória dos ramos sensitivos para o antebraço, com retorno completo da função em 8 semanas. Conclusão Os resultados mostram que a liberação endoscópica do nervo ulnar no cotovelo comoequipamentodeAgeeéuma técnica segura, confiável e com bons resultados.

Resumen Introducción: Aunque COVID-19 (enfermedad por coronavirus 2019) en niños es usualmente leve, en casos excepcionales requieren hospitalización y cuidados intensivos. Los resultados adversos han sido observados principalmente en los niños con comorbilidades, justificando su vacunación. El objetivo de este estudio fue evaluar el riesgo de hospitalización y muerte en niños y adolescentes mexicanos con COVID-19 y comorbilidades. Métodos: Estudio transversal en 366,542 casos de COVID-19 confirmados, menores de 18 años y reportados por la Secretaría de Salud de México, hasta el 9 de julio del 2022. Se ejecutaron modelos multivariados de regresión logística. Resultados: El promedio de edad fue de 10.98 años, 50.6% de sexo masculino, y 7.3% reportaron al menos una comorbilidad. El porcentaje de hospitalización y muerte en pacientes con COVID-19 sin comorbilidades fue 3.52% y 0.20%, respectivamente; mientras que los pacientes con comorbilidades presentaron más elevados porcentajes de hospitalización (14.0%) y muerte (1.9%). La probabilidad de hospitalización fue 5.6 veces más en los pacientes con COVID-19 y comorbilidades, comparando con aquellos sin comorbilidades. Las comorbilidades que mostraron más riesgo fueron inmunosupresión (razón de momios (RM) 22.06), enfermedad renal crónica (ERC) (RM 11.36) y enfermedades cardiovasculares (RM 5.66). La probabilidad de muerte en los pacientes con comorbilidades fue 11.01 veces más que en aquellos sin enfermedades, y fue más elevado en aquellos con ERC (RM 12.57), enfermedades cardiovasculares (RM 6.87) y diabetes (RM 5.83). Conclusiones: Los pacientes pediátricos con comorbilidades presentaron mayor riesgo de COVID-19 severo, por lo que se sugiere promover con mayor énfasis la vacunación en ellos.

Abstract Background: Although COVID-19 (coronavirus disease 2019) in children is usually mild, they need hospitalization and intensive care in exceptional cases. Adverse outcomes have been observed mainly among children with comorbidities, justifying their vaccination. This study aimed to assess the risk of hospitalization and death in Mexican children and adolescents with COVID-19 and comorbidities. Methods: A cross-sectional study was performed on 366,542 confirmed COVID-19 cases under 18 years, reported by the Mexican Ministry of Health up to July 9, 2022. Logistic regression models were performed. Results: The mean age was 10.98 years, 50.6% were male, and 7.3% reported at least one comorbidity. The percentage of hospitalization and death in COVID-19 patients with and without comorbidities was 3.52%, and 0.20%, respectively; children with comorbidities presented a higher percentage of hospitalization (14.0%) and death (1.9%). The probability of hospitalization was 5.6 times greater in pediatric patients with COVID-19 and comorbidities, and the comorbidities that showed the greatest risk were immunosuppression (odds ratio (OR) 22.06), chronic kidney disease (CKD) (11.36), and cardiovascular diseases (5.66). The probability of death in patients with comorbidities was 11.01 times higher than in those without diseases, and the highest risk was observed in those with CKD (OR 12.57), cardiovascular diseases (6.87), and diabetes (5.83). Conclusions: Pediatric patients with comorbidities presented a higher risk of severe COVID-19. It is suggested that vaccination should be promoted with greater emphasis on pediatric patients with comorbidities.

RESUMO A salinidade causada pelo excesso de sais na solução do solo é um dos estresses ambientais mais limitantes na agricultura no mundo. Nesse cenário, dentre as estratégias para favorecer a expressão do potencial genético das plantas, destaca-se o uso de bioestimulantes. Objetivou-se avaliar a influência de um bioestimulante à base de extrato de algas marinhas no crescimento, produção de forragem, trocas gasosas e acúmulo de íons sódio e potássio em capim-pangolão Digitaria pentzii, submetido a condições de estresse salino. O experimento foi conduzido de março a julho de 2019, em delineamento de blocos casualizados, em esquema fatorial 2 × 3, com duas concentrações do bioestimulante (0 e 8 mL L-1) e três condutividades elétricas da água de irrigação (0,03; 2 e 4 dS m-1), com quatro repetições. O acúmulo de 50 µmol g-1 de sódio nas folhas correspondeu a uma redução de 0,3 g de matéria seca na produção de lâminas foliares por vaso. O bioestimulante não influenciou as características estruturais do capim-pangolão, acúmulo de fitomassa e condutância estomática, independentemente da salinidade. No nível de 4 dS m-1 na água de irrigação, a toxicidade do estresse iônico devido ao acúmulo de sais na parte aérea do capim-pangolão é mais severa. Esta é a primeira evidência da tolerância “moderada” à salinidade do capim-pangolão em regiões semiáridas.

ABSTRACT Salinity caused by excess salts in soil solutions is one of the most limiting environmental stresses in agriculture worldwide. In this scenario, among strategies that favor the expression of the genetic potential of plants, the use of biostimulants stands out. The objective of this study was to evaluate the influence of a seaweed-based biostimulant on growth, forage production, gas exchange, and accumulation of sodium and potassium ions in pangolão grass (Digitaria pentzii) under saline stress. The experiment was conducted from March to July 2019 using a randomized block design, in a 2 × 3 factorial scheme with two concentrations of biostimulant (0 and 8 mL L-1) and three electrical conductivities of irrigation water (0.03, 2 and 4 dS m-1), with four replicates. The accumulation of 50 µmol g-1 of sodium in leaves corresponded to a reduction of 0.3 g of dry matter in the leaf blade production per plot. The biostimulant did not influence the structural characteristics, phytomass accumulation, or stomatal conductance of the pangolão grass, regardless of salinity. At the level of 4 dS m-1 in irrigation water, the ionic stress toxicity due to the accumulation of salts in the aerial part of pangolão grass was more severe. This is the first evidence of the “moderate” salinity tolerance of pangolão grass in semi-arid regions.

Objectives: To re-evaluate a sample of older adults enrolled in a randomized controlled trial of lithium for amnestic mild cognitive impairment (MCI) after 11 to 15 years, re-assessing their current (or last available) global cognitive and functional state. Methods: We recalled all former participants of the Lithium-MCI trial conducted by our group between 2009 and 2012 to perform a single-blinded, cross-sectional evaluation of their global clinical state to compare the long-term outcome of those who received lithium vs. those who received placebo. Results: Of the original sample (n=61), we were able to reach 36 participants (59% of retention), of whom 22 had previously received lithium (61% of the recall sample) and 14 (39%) had received placebo. Since 30.5% of the recalled sample was deceased, psychometric data were collected only for 69.5% of the participants. We found statistically significant differences in current mean Mini Mental State Examination score according to previous treatment group (25.5 [SD, 5.3] vs. 18.3 [SD, 10.9], p = 0.04). The lithium group also had better performance in the phonemic Verbal Fluency Test than the control group (34.4 [SD, 14.4] vs. 11.6 [SD, 10.10], p < 0.001). Differences in these measures also had large effect sizes, as shown by Cohen’s d values of 0.92 and 1.78, respectively. Conclusion: This data set suggests that older adults with amnestic MCI who had been treated with lithium during a previous randomized controlled trial had a better long-term global cognitive outcome than those from a matched sample who did not receive the intervention.

Abstract Objective: To analyze the performance of the cystic fibrosis (CF) newborn screening (NBS) program over its first five years in a Brazilian northeastern state. Methods: A population-based study using a screening algorithm based on immunoreactive trypsinogen (IRT)/IRT. Data were retrieved from the state referral screening center registry. The program performance was evaluated using descriptive indicators such as the results of an active search, coverage, newborn’s age at the time of blood sampling, the time between sample collection and its arrival at the laboratory, and the child’s age at diagnosis of disease. Results: The public CF screening program covered 82.6% of the 1,017,576 births that occurred, with an accumulated five-year incidence of 1:20,767 live births. The median (25th-75th) age at diagnosis was 3.5 (2.3–7.3) months. The sampling before 7 days of life for the first IRT (IRT1) increased between 2013 and 2017 from 42.2 to 48.3%. Around 5% of IRT1 samples and 30% of the second samples were collected after 30 days of life. In the first and second stages of screening, 23.6% and 19.9% of the infants, respectively, were lost to follow-up. In both stages of screening, the samples were retained at the health units for a median (25th–75th) of 9.0 (7.0–13.0) days. Conclusions: The coverage by the CF-NBS program was satisfactory as compared to other Brazilian state rates and the percentage of IRT1 samples collected within the first week of life increased progressively. However, time of samples retention at the health units, inappropriate sampling, inherent methodological problems, and loss of follow-up need to improve.

ABSTRACT Objective: To investigate the detection of subsolid nodules (SSNs) on chest CT scans of outpatients before and during the COVID-19 pandemic, as well as to correlate the imaging findings with epidemiological data. We hypothesized that (pre)malignant nonsolid nodules were underdiagnosed during the COVID-19 pandemic because of an overlap of imaging findings between SSNs and COVID-19 pneumonia. Methods: This was a retrospective study including all chest CT scans performed in adult outpatients (> 18 years of age) in September of 2019 (i.e., before the COVID-19 pandemic) and in September of 2020 (i.e., during the COVID-19 pandemic). The images were reviewed by a thoracic radiologist, and epidemiological data were collected from patient-filled questionnaires and clinical referrals. Regression models were used in order to control for confounding factors. Results: A total of 650 and 760 chest CT scans were reviewed for the 2019 and 2020 samples, respectively. SSNs were found in 10.6% of the patients in the 2019 sample and in 7.9% of those in the 2020 sample (p = 0.10). Multiple SSNs were found in 23 and 11 of the patients in the 2019 and 2020 samples, respectively. Women constituted the majority of the study population. The mean age was 62.8 ± 14.8 years in the 2019 sample and 59.5 ± 15.1 years in the 2020 sample (p < 0.01). COVID-19 accounted for 24% of all referrals for CT examination in 2020. Conclusions: Fewer SSNs were detected on chest CT scans of outpatients during the COVID-19 pandemic than before the pandemic, although the difference was not significant. In addition to COVID-19, the major difference between the 2019 and 2020 samples was the younger age in the 2020 sample. We can assume that fewer SSNs will be detected in a population with a higher proportion of COVID-19 suspicion or diagnosis.

RESUMO Objetivo: Investigar a detecção de nódulos subsólidos na TC de tórax de pacientes ambulatoriais antes e durante a pandemia de COVID-19, bem como correlacionar os achados de imagem com dados epidemiológicos. Nossa hipótese foi a de que nódulos não sólidos (pré) malignos foram subdiagnosticados durante a pandemia de COVID-19 em virtude da sobreposição de achados de imagem de nódulos subsólidos e pneumonia por COVID-19. Métodos: Estudo retrospectivo no qual foram incluídas todas as imagens de TC de tórax realizadas em pacientes ambulatoriais adultos (com idade > 18 anos) em setembro de 2019 (antes da pandemia de COVID-19) e em setembro de 2020 (durante a pandemia de COVID-19). As imagens foram reavaliadas por um radiologista torácico, e os dados epidemiológicos foram extraídos de questionários preenchidos pelos pacientes e de encaminhamentos clínicos. Modelos de regressão foram usados para controlar fatores de confusão. Resultados: Foram reavaliadas 650 e 760 imagens de TC de tórax nas amostras de 2019 e 2020, respectivamente. Foram encontrados nódulos subsólidos em 10,6% dos pacientes que compuseram a amostra de 2019 e em 7,9% daqueles que compuseram a amostra de 2020 (p = 0,10). Nódulos subsólidos múltiplos foram encontrados em 23 e 11 dos pacientes que compuseram as amostras de 2019 e 2020, respectivamente. As mulheres constituíram a maioria da população do estudo. A média de idade foi de 62,8 ± 14,8 anos na amostra de 2019 e de 59,5 ± 15,1 anos na amostra de 2020 (p < 0,01). A COVID-19 foi responsável por 24% de todos os encaminhamentos para a realização de TC em 2020. Conclusões: Foram detectados menos nódulos subsólidos na TC de tórax de pacientes ambulatoriais durante a pandemia de COVID-19 do que antes da pandemia, embora a diferença não tenha sido significativa. Além da COVID-19, a principal diferença entre as amostras de 2019 e 2020 foi o fato de que a amostra de 2020 era mais jovem. Podemos supor que menos nódulos subsólidos serão detectados numa população com maior proporção de suspeita ou diagnóstico de COVID-19.

Abstract: At higher temperatures, lettuce seeds may not germinate, resulting in problems for crop establishment in the field and production. This study aimed to evaluate the maternal effect for thermotolerance on lettuce seeds and find enzymatic markers. We used the thermoinhibition tolerant cultivar ‘Everglades’, the sensitive ‘Verônica’, their reciprocal hybrids, F1 and F2 generation. Seeds were submitted to germination and enzymatic tests (catalase, superoxide dismutase, ascorbate peroxidase, and endo- β-mannanase). Germination (first and final counting) and speed index were compared through Tukey’s test. ‘Everglades’ and its reciprocal hybrids, have not been influenced by temperature regarding germination compared to the other treatments. Also, the maternal effect was observed for the F1 generation. Higher temperatures interfere with the activity of all enzymes evaluated, consequently in the physiological parameters. However, superoxide dismutase and endo- β-mannanase have shown direct and more expressive correlation with changes on the physiological parameters compared to the other enzymes. Thus, it is possible to conclude that those enzymes can be used as markers for thermotolerant genotype selection.

Resumo: Sob altas temperaturas, sementes de alface não germinam, resultando em problemas para o estabelecimento da cultura em condição de campo. Esse trabalho analisou a possibilidade de presença de efeito maternal na termotolerância em sementes de alface e buscou marcadores enzimáticos para tal. Usaram-se as cultivares Everglades (tolerante) e ‘Verônica’ (sensível), bem como seus híbridos recíprocos e a geração F2. As sementes foram analisadas quanto à germinação, bem como atividade enzimática (catalase, superóxido dismutase, ascorbato peroxidase e endo-β-mananase). O percentual de germinação; (primeira contagem e final), bem como a velocidade (IVG) foram analisados por meio de ANOVA e teste de Tukey. A cultivar Everglades e seus híbridos recíprocos não foram influenciados pela germinação em alta temperatura quando comparados com os outros tratamentos. Além disso, o efeito maternal foi observado na geração F1. Altas temperaturas interferem na atividade de todas as enzimas analisadas nesta pesquisa, consequentemente também nos parâmetros fisiológicos. Contudo, a superóxido dismutase e a endo-β-mananase tiveram suas variações altamente correlacionadas com as mudanças nos parâmetros fisiológicos. O presente trabalho evidenciou o efeito materno para a termotolerância em sementes de alface, bem como o uso da superóxido dismutase e endo-β-mananase como marcadores enzimáticos para tal característica.

Abstract Background Infectious endocarditis (IE) refers to infection of the endocardial surface of the heart and usually occurs in native or prosthetic valves. Objective This study aimed to raise IE data reflecting the surgical therapy in a University Hospital in the interior of the State of Sao Paulo–Brazil. Method Retrospective and observational approach of 328 patients with IE who underwent surgery between 1982 and 2020 Results The main data (n=121/37%), congestive heart failure (n=114/35%), valve disease (n=92/28%), diabetes mellitus (n=85/26%), chronic kidney disease (n=59/18%), and rheumatic fever (49/15%). Renal failure is one of the main and most relevant pre-surgical risk factors for a poor prognosis. Conclusion For a better clinical and surgical outcome, an early syndromic and etiological diagnosis of IE is necessary, especially in patients with multiple comorbidities.

Resumo Fundamento A endocardite infecciosa (EI) refere-se à infecção da superfície endocárdica do coração e geralmente ocorre em valvas nativas ou protéticas. Objetivo Este estudo teve como objetivo levantar dados de EI refletindo a terapêutica cirúrgica, em um Hospital Universitário do interior do estado de São Paulo – Brasil. Método Abordagem retrospectiva e observacional de 328 pacientes com EI operados entre 1982 e 2020 Resultados Os principais dados (n=121/37%), insuficiência cardíaca congestiva (n=114/35%), valvopatia (n=92/28%), diabetes mellitus (n=85/26%), doença renal crônica (n=59/18%) e febre reumática (49/15%). A insuficiência renal é um dos principais e mais relevantes fatores de risco pré-cirúrgicos para um mau prognóstico. Conclusão Para um melhor resultado clínico e cirúrgico é necessário o diagnóstico sindrômico e etiológico precoce da EI, principalmente em pacientes com múltiplas comorbidades.

Xerostomia is defined as the perception of dry mouth, and dysgeusia, as a change in taste. Both are common complaints in the elderly, especially among those making use of polypharmacy drug combinations. Aim This study aimed to determine the prevalence of xerostomia and dysgeusia and to investigate their association with polypharmacy in the elderly. Methods older people under follow-up at the Multidisciplinary Elderly Center of the University Hospital of Brasília were interviewed and asked about health problems, medications used, presence of xerostomia and dysgeusia. Descriptive statistics were used to determine the prevalence of the symptoms surveyed. The chi-square test was used to investigate the relationship between xerostomia and dysgeusia and polypharmacy. Secondary associations were performed using binomial logistic regression. Results Ninety-six older people were evaluated and of these, 62.5% had xerostomia and 21.1%, had dysgeusia. The average number of medications used was 4±3 medications per individual. Polypharmacy was associated with xerostomia but not dysgeusia. It was possible to associate xerostomia with the use of antihypertensive drugs. Conclusion Xerostomia was a frequent complaint among elderly people making use of polypharmacy, especially those using antihypertensives. Antihypertensives and antidepressants were used most drugs by the elderly and exhibited interactions with drugs most prescribed in Dentistry. Two contraindications were found between fluconazole and mirtazapine; and between erythromycin and simvastatin.

Abstract The pathogenesis of systemic lupus erythematosus (SLE) is complex. Few studies in Brazilian population have addressed cell phenotypes associated with immunological responses and their associations with SLE activity. The aim of this study is to investigate cell phenotypes associated to SLE diagnosis, treatment and activity. Twenty-eight SLE female patients (17 inactive, 11 active) and 10 healthy women were included in this study. Markers of natural killer (Nk), T and B cells in peripheral blood were evaluated by flow cytometry. Nkt cells were decreased only in SLE active patients. Activated CD4+, regulatory T FoxP3+ and B cells were decreased in both active and inactive SLE patients, compared to control group. The data corroborate the disruption of immune regulatory response in SLE patients and suggest phenotipic changes as possible biomarkers of SLE activity.

ABSTRACT Objective: To investigate the impact of impaired pulmonary function on patient-centered outcomes after hospital discharge due to severe COVID-19 in patients without preexisting respiratory disease. Methods: This is an ongoing prospective cohort study evaluating patients (> 18 years of age) 2-6 months after hospital discharge due to severe COVID-19. Respiratory symptoms, health-related quality of life, lung function, and the six-minute walk test were assessed. A restrictive ventilatory defect was defined as TLC below the lower limit of normal, as assessed by plethysmography. Chest CT scans performed during hospitalization were scored for the presence and extent of parenchymal abnormalities. Results: At a mean follow-up of 17.2 ± 5.9 weeks after the diagnosis of COVID-19, 120 patients were assessed. Of those, 23 (19.2%) reported preexisting chronic respiratory diseases and presented with worse lung function and exertional dyspnea at the follow-up visit in comparison with their counterparts. When we excluded the 23 patients with preexisting respiratory disease plus another 2 patients without lung volume measurements, a restrictive ventilatory defect was observed in 42/95 patients (44%). This subgroup of patients (52.4% of whom were male; mean age, 53.9 ± 11.3 years) showed reduced resting gas exchange efficiency (DLCO), increased daily-life dyspnea, increased exertional dyspnea and oxygen desaturation, and reduced health-related quality of life in comparison with those without reduced TLC (50.9% of whom were male; mean age, 58.4 ± 11.3 years). Intensive care need and higher chest CT scores were associated with a subsequent restrictive ventilatory defect. Conclusions: The presence of a restrictive ventilatory defect approximately 4 months after severe COVID-19 in patients without prior respiratory comorbidities implies worse clinical outcomes.

RESUMO Objetivo: Investigar o impacto do comprometimento da função pulmonar nos desfechos centrados no paciente após a alta hospitalar em pacientes sem doenças respiratórias preexistentes que foram hospitalizados em virtude de COVID-19 grave. Métodos: Trata-se de um estudo prospectivo de coorte em andamento, no qual pacientes com COVID-19 grave (com idade > 18 anos) são avaliados 2-6 meses depois da alta hospitalar. Avaliamos os sintomas respiratórios, a qualidade de vida relacionada à saúde, a função pulmonar e a distância percorrida no teste de caminhada de seis minutos. A definição de distúrbio ventilatório restritivo foi CPT abaixo do limite inferior da normalidade na pletismografia. As imagens de TC de tórax realizadas durante a hospitalização foram avaliadas quanto à presença e extensão de alterações parenquimatosas. Resultados: Em média 17,2 ± 5,9 semanas depois do diagnóstico de COVID-19, foram avaliados 120 pacientes. Destes, 23 (19,2%) relataram doenças respiratórias crônicas preexistentes e apresentaram pior função pulmonar e maior dispneia aos esforços na consulta de acompanhamento quando comparados aos outros participantes. Quando excluímos os 23 pacientes com doenças respiratórias preexistentes e mais 2 pacientes (sem medidas de volumes pulmonares), observamos distúrbio ventilatório restritivo em 42/95 pacientes (44%). Esse subgrupo de pacientes (52,4% dos quais eram do sexo masculino, com média de idade de 53,9 ± 11,3 anos) apresentou menor eficiência das trocas gasosas (DLCO), maior dispneia na vida diária e dessaturação de oxigênio ao exercício e redução da qualidade de vida relacionada à saúde em comparação com aqueles sem redução da CPT (50,9% dos quais eram do sexo masculino, com média de idade de 58,4 ± 11,3 anos). A necessidade de terapia intensiva e pontuações mais altas no escore de alterações parenquimatosas na TC de tórax apresentaram relação com distúrbio ventilatório restritivo subsequente. Conclusões: A presença de distúrbio ventilatório restritivo aproximadamente 4 meses depois da COVID-19 grave em pacientes sem comorbidades respiratórias prévias implica piores desfechos clínicos.

ABSTRACT Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.

RESUMO A fibrose cística (FC) é uma doença genética que resulta em disfunção da proteína reguladora de condutância transmembrana da FC (CFTR), que é um canal de cloro e bicarbonato expresso na porção apical de células epiteliais de diversos órgãos. A disfunção dessa proteína resulta em manifestações clínicas diversas, envolvendo primariamente os sistemas respiratório e gastrointestinal com redução da qualidade e expectativa de vida. A FC ainda é uma patologia incurável, porém o horizonte terapêutico e prognóstico é hoje totalmente distinto e muito mais favorável. O objetivo destas diretrizes foi definir recomendações brasileiras baseadas em evidências em relação ao emprego de agentes farmacológicos no tratamento pulmonar da FC. As perguntas PICO (acrônimo baseado em perguntas referentes aos Pacientes de interesse, Intervenção a ser estudada, Comparação da intervenção e Outcome [desfecho] de interesse) abordaram aspectos relativos ao uso de moduladores de CFTR (ivacaftor, lumacaftor + ivacaftor e tezacaftor + ivacaftor), uso de dornase alfa, terapia de erradicação e supressão crônica de Pseudomonas aeruginosa, e erradicação de Staphylococcus aureus resistente a meticilina e do complexo Burkholderia cepacia. Para a formulação das perguntas, um grupo de especialistas brasileiros foi reunido e realizou-se uma revisão sistemática sobre os temas, com meta-análise quando aplicável. Os resultados encontrados foram analisados quanto à força das evidências compiladas, sendo concebidas recomendações seguindo a metodologia GRADE. Os autores acreditam que o presente documento represente um importante avanço a ser incorporado na abordagem de pacientes com FC, objetivando principalmente favorecer seu manejo, podendo se tornar uma ferramenta auxiliar na definição de políticas públicas relacionadas à FC.