Surgical treatment of non-cystic fibrosis bronchiectasis in children and adolescents: A review.

OBJECTIVE: To discuss surgery for non-cystic fibrosis [CF] bronchiectasis in children and adolescents. SOURCES: Non-systematic review including articles in English, mainly from the last 5 years. SUMMARY OF FINDINGS: In this review, we present that in low- and middle-income countries [LMIC] clinical treatment fails in around 20% of cases due to low socioeconomic status and poor adherence. This causes the disease to progress and require surgery. We emphasize that the indications for surgery are not well defined and must be considered on an individual basis. The surgical treatment of bronchiectasis in children may be indicated in selected cases; especially in localized disease with frequent exacerbations despite an optimized clinical approach. Surgery can improve quality of life [QoL] and reduce exacerbations. It has few postoperative complications and low morbidity and mortality. Finally, we propose an algorithm for managing bronchiectasis, which takes into account LMIC settings with limited resources. CONCLUSION: We conclude that in LMICs, surgery is a treatment strategy for selected children/adolescents with bronchiectasis.

The wheezy infant: A viewpoint from low-middle income countries.

OBJECTIVE: To review the recent evidence in the literature of various aspects of recurrent/severe wheezing in children under 3 in low-middle income countries [LMICS]. SOURCES: A non-systematic review including articles in English. We mainly selected publications from the last 5 years. Studies on epidemiology, aetiology, diagnosis, treatment, and prevention were included in the search. We reviewed differential diagnoses of wheezing that focused on LMICS. We also reviewed aspects of prevention. SUMMARY OF THE FINDINGS: Many epidemiological studies have shown a variable but significant number of wheezy infants [WI] cases in LMICS when compared to other countries. The differential diagnosis of causes of wheezing in this age group is mandatory, taking into account local facilities. Few treatment options have been well studied for this age group. In LMICS, a pragmatic approach could be considered, as described in the article. It is difficult to study primary prevention for WI and secondary prevention (mainly environmental) may have some impact. A schematic approach for recurrent wheezers is presented, which takes into account settings with limited resources. CONCLUSION: Severely or recurrently wheezy children under 3 is a common clinical issue in LMICS. Studies on this age group are needed to reduce the significant morbidity. It may be possible to lower the high burden of wheezing in this age group by selecting the phenotype which may respond to inhaled steroids.

Challenges of COVID-19 in children in low- and middle-income countries.

As the coronavirus pandemic extends to low and middle income countries (LMICs), there are growing concerns about the risk of coronavirus disease (COVID-19) in populations with high prevalence of comorbidities, the impact on health and economies more broadly and the capacity of existing health systems to manage the additional burden of COVID-19. The direct effects of COVID are less of a concern in children, who seem to be largely asymptomatic or to develop mild illness as occurs in high income countries; however children in LMICs constitute a high proportion of the population and may have a high prevalence of risk factors for severe lower respiratory infection such as HIV or malnutrition. Further diversion of resources from child health to address the pandemic among adults may further impact on care for children. Poor living conditions in LMICs including lack of sanitation, running water and overcrowding may facilitate transmission of SARS-CoV-2. The indirect effects of the pandemic on child health are of considerable concern, including increasing poverty levels, disrupted schooling, lack of access to school feeding schemes, reduced access to health facilities and interruptions in vaccination and other child health programs. Further challenges in LMICs include the inability to implement effective public health measures such as social distancing, hand hygiene, timely identification of infected people with self-isolation and universal use of masks. Lack of adequate personal protective equipment, especially N95 masks is a key concern for health care worker protection. While continued schooling is crucial for children in LMICs, provision of safe environments is especially challenging in overcrowded resource constrained schools. The current crisis is a harsh reminder of the global inequity in health in LMICs. The pandemic highlights key challenges to the provision of health in LMICs, but also provides opportunities to strengthen child health broadly in such settings.

Childhood asthma in low and middle-income countries: Where are we now?

Pediatric asthma has been increasing in LMICs (Low Middle-Income Countries), leading to an important burden for both children and national health systems. Implementing measures to achieve control are influenced by the degree of organization health systems have, the availability and affordability of essential asthma medications, and the effective implementation of asthma programs and asthma guidelines. In this review authors give an updated view of the current situation of these components of asthma management in LMICs.

Pleural effusions in children from Southern Brazil.

Parapneumonic pleural effusions (PPE) are a relatively common (5-40%) complication of paediatric pneumonia. However, in clinical practice the majority of the effusions are small and do not need any further investigation or specific treatment apart from antibiotic therapy. A small number require drainage or surgical intervention. Rarely, significant effusions are associated with non-bacterial pneumonia in the paediatric population. Pleural tuberculosis in our hospital is the second highest cause of pleural effusions related to the high incidence of TB in our city. In the last 3 years we have had around 50 cases of pleural TB in children.

Surgical treatment of non-cystic fibrosis bronchiectasis in Brazilian children.

PURPOSE: To determine the clinical characteristics of patients submitted to surgical treatment for non-cystic fibrosis (CF) bronchiectasis, the indications for surgery, and the results obtained at a referral facility for pediatric thoracic surgery. METHODS: Between January 1998 and December 2009, we retrospectively reviewed the medical charts of 109 pediatric patients with non-CF bronchiectasis who underwent surgical treatment. These findings were subsequently analyzed by focusing on postoperative complications and long-term results. RESULTS: Of the 109 patients undergoing pulmonary resection, the mean age was 7.6 years (ranging from 1 to 15.5 y-o) with male predominance (59 %). The most common procedure was segmentectomy (43 %) followed by left lower lobectomy (38 %). Minor postoperative complications occurred in 36 % of the patients; the most common was transient atelectasis (26 %), followed by air leak (6 %), and postoperative pain (4 %). There was one death within the 30-day postoperative period, but it was unrelated to the procedure. Eighty-three children were followed after discharge, with a mean follow-up period of 667 days. Sixty-five (76 %) patients showed improvement of clinical symptoms after surgery. CONCLUSIONS: Lung resection for the treatment of non-CF bronchiectasis in children is a safe procedure, with no life-treating morbidity and low mortality. This procedure also leads to significant improvements in symptoms and quality of life.

Adverse early-life environmental exposures and their repercussions on adult respiratory health.

OBJECTIVE: To review in the literature the environmental problems in early life that impact the respiratory health of adults. SOURCES: Non-systematic review including articles in English. Search filters were not used in relation to the publication date, but the authors selected mainly publications from the last five years. SUMMARY OF THE FINDINGS: In this review, the authors present the exposure pathways and how the damage occurs depending on the child's stage of development; the authors describe the main environmental pollutants - tobacco smoke, particulate matter, air pollution associated with traffic, adverse childhood experiences and socioeconomic status; the authors present studies that evaluated the repercussions on the respiratory system of adults resulting from exposure to adverse environmental factors in childhood, such as increased incidence of Chronic Obstructive Pulmonary Disease (COPD), asthma and allergies; and, a decline in lung function. The authors emphasize that evidence demonstrates that adult respiratory diseases almost always have their origins in early life. Finally, the authors emphasize that health professionals must know, diagnose, monitor, and prevent toxic exposure among children and women. CONCLUSION: The authors conclude that it is necessary to recognize risk factors and intervene in the period of greatest vulnerability to the occurrence of harmful effects of environmental exposures, to prevent, delay the onset or modify the progression of lung disease throughout life and into adulthood.

Pleural tuberculosis: experiences from two centers in Brazil.

OBJECTIVE: This study aimed to describe the clinical and laboratory findings of patients diagnosed with pleural tuberculosis at two hospitals in southern Brazil. METHODS: Patients aged < 18 years were evaluated retrospectively. The patients' medical and epidemiological history, tuberculin skin test results, radiological and pathological findings, and pleural fluid analysis results were retrieved. RESULTS: Ninety-two patients with pleural tuberculosis were identified. The mean age was 10.9 years old. Twenty-one percent were children aged six years or less. The most common symptoms were fever (88%), cough (72%), and chest pain (70%). Unilateral pleural effusion was observed in 96% of the cases. Lymphocyte predominance was found in 90% of the pleural fluid samples. The adenosine deaminase activity of the pleural fluid was greater than 40 U/L in 85% of patients. A diagnosis of community-acquired pneumonia with antibiotic prescriptions was observed in 76% of the study population. CONCLUSIONS: Tuberculosis etiology must be considered in unilateral pleural effusion in a child with contact with a case of tuberculosis. Pleural fluid biomarkers contribute to the diagnosis of pleural tuberculosis in children and adolescents.

Histoplasmosis in children.

Histoplamosis is the most common primary systemic mycosis in the USA and is becoming more common as an opportunistic infection in HIV patients worldwide. In children the rate of asymptomatic infection is high. However, in infants with an immature immunological system, disseminated disease may occur. The clinical picture is variable depending on the immunological status. At the onset of the infection clinical manifestations are non specific (headache, fever, cough and nausea). Usually, these symptoms are self-limited and improve without treatment. However, patients with disseminated diseases present with prolonged fever, malaise, cough and weight loss. Hepatosplenomegaly is frequent in infants. Chest radiographs may be normal in 40 to 50% of patients with disseminated disease but findings such as lobar or diffuse infiltrates, cavitations, hilar adenopathy, or any combination of these may be found. Frequently, the clinical presentation is misdiagnosed as tuberculosis. Skin tests, serological reaction and specific cultures are used for diagnosis confirmation. Treatment indications and regimens are similar to those for adults, except that amphotericin B deoxycholate is usually well tolerated in children.

Assessment of nutritional status in children and adolescents with post-infectious bronchiolitis obliterans.

OBJECTIVE: To assess the nutritional status of children and adolescents with bronchiolitis obliterans and to analyze associations with clinical and nutritional factors. METHODS: The study included 57 patients. Nutritional status was assessed using z scores for weight/age, stature/age, weight/stature in children, and stature/age and body mass index percentiles in adolescents. Body composition was assessed via tricipital skin folds, subscapular skin folds, and the sum of both plus the muscular circumference of the arm; pulmonary function was also investigated in subjects over 8 years old. RESULTS: The high percentages of malnutrition and risk for malnutrition are noteworthy: 21.7 and 17.5%, respectively. Among children, weight/age and stature/age detected higher percentages of malnutrition (21.6 and 16.2%), while weight/stature underestimated this diagnosis. Among adolescents, body mass index detected a high percentage of malnutrition (25%) and of risk for malnutrition (20%). Body composition analysis detected 51% of patients with low muscle reserves, and the majority of patients had normal fat reserves. Compromised pulmonary function was associated with poor performance at exercise (r = 0.434; p = 0.024). Malnutrition and/or nutritional risk and low muscle reserves were significantly associated with the 6-minute walk test (p = 0.032; p = 0.030). There was no association between spirometry and the nutritional variables (p > 0.05). CONCLUSION: These results emphasize the need for nutritional intervention, and suggest that, in addition to using weight and height indices for nutritional assessment, it is necessary to combine these with an analysis of body composition, so that a larger number of patients with malnutrition and/or at an increased risk for developing malnutrition may be identified and correctly managed.

Functional capacity assessment in children and adolescents with post-infectious bronchiolitis obliterans.

OBJECTIVE: To assess functional capacity during exercise in children and adolescents with post-infectious bronchiolitis obliterans (PIBO). METHODS: 20 children with PIBO, aged 8-16 years old, and in follow-up at an outpatient clinic carried out cardiopulmonary exercise testing (CPET), a 6-minute walk test (6MWT) and pulmonary function tests (PFT), according to American Thoracic Society (ATS), European Respiratory Society (ERS) and American College of Chest Physicians (ACCP) guidelines. Results were expressed as percentages of predicted reference values: Armstrong's for CPET, Geiger's for 6MWT, Knudson's for spirometry, and Zapletal's for plethysmography. RESULTS: Mean age (+/- SD) was 11.4+/-2.2 years; 70% were boys; mean weight: 36.8+/-12.3 kg; mean height: 143.8+/-15.2 cm. When compared to reference values, PFT detected lower airflows (spirometry) and higher volumes (plethysmography). Eleven patients had reduced peak VO2 values in CPET (< 84% predicted). The mean distance walked (6MWT) was 77.0+/-15.7% of predicted (512+/-102 m). Peak VO2 was not correlated with 6MWT, but it was correlated with FVC (L) (r = 0.90/p = 0.00), with FEV1 (L) (r = 0.86/p = 0.00) and with RV/TLC, both in absolute values (r = -0.71/p = 0.02) and as percentages of predicted values (r = -0.63/p = 0.00). CONCLUSIONS: The majority of these post-infectious bronchiolitis obliterans patients exhibited reduced functional capacity, exhibited during both CPET and the 6MWT. Due to its greater feasibility, 6MWT could be an alternative where CPET is not available.

Adverse early-life environmental exposures and their repercussions on adult respiratory health

Abstract Objective: To review in the literature the environmental problems in early life that impact the respiratory health of adults. Sources: Non-systematic review including articles in English. Search filters were not used in relation to the publication date, but the authors selected mainly publications from the last five years. Summary of the findings: In this review, the authors present the exposure pathways and how the damage occurs depending on the child's stage of development; the authors describe the main environmental pollutants - tobacco smoke, particulate matter, air pollution associated with traffic, adverse childhood experiences and socioeconomic status; the authors present studies that evaluated the repercussions on the respiratory system of adults resulting from exposure to adverse environmental factors in childhood, such as increased incidence of Chronic Obstructive Pulmonary Disease (COPD), asthma and allergies; and, a decline in lung function. The authors emphasize that evidence demonstrates that adult respiratory diseases almost always have their origins in early life. Finally, the authors emphasize that health professionals must know, diagnose, monitor, and prevent toxic exposure among children and women. Conclusion: The authors conclude that it is necessary to recognize risk factors and intervene in the period of greatest vulnerability to the occurrence of harmful effects of environmental exposures, to prevent, delay the onset or modify the progression of lung disease throughout life and into adulthood.

Pleural tuberculosis: experiences from two centers in Brazil

Abstract Objective: This study aimed to describe the clinical and laboratory findings of patients diagnosed with pleural tuberculosis at two hospitals in southern Brazil. Methods: Patients aged < 18 years were evaluated retrospectively. The patients' medical and epidemiological history, tuberculin skin test results, radiological and pathological findings, and pleural fluid analysis results were retrieved. Results: Ninety-two patients with pleural tuberculosis were identified. The mean age was 10.9 years old. Twenty-one percent were children aged six years or less. The most common symptoms were fever (88%), cough (72%), and chest pain (70%). Unilateral pleural effusion was observed in 96% of the cases. Lymphocyte predominance was found in 90% of the pleural fluid samples. The adenosine deaminase activity of the pleural fluid was greater than 40 U/L in 85% of patients. A diagnosis of community-acquired pneumonia with antibiotic prescriptions was observed in 76% of the study population. Conclusions: Tuberculosis etiology must be considered in unilateral pleural effusion in a child with contact with a case of tuberculosis. Pleural fluid biomarkers contribute to the diagnosis of pleural tuberculosis in children and adolescents.

Pleural effusion with negative culture: a challenge for pneumococcal diagnosis in children.

BACKGROUND: Pneumococcal parapneumonic effusion seems to be increasing in children in the postvaccine era and is frequently associated with negative culture. Due to the low yield of culture, culture-independent tools are evaluated. METHODS: Culture-negative pleural fluid specimens from 38 children with parapneumonic effusion were examined for pneumococcal lytA by quantitative polymerase chain reaction (qPCR) and soluble antigen (C-polysaccharide) using an immunochromatographic test (BinaxNow Streptococcus pneumoniae). RESULTS: In 81% (30/37) and 63% (24/38) of the specimens, a positive result was obtained by qPCR and antigen detection, respectively. Most mismatches were observed in specimens with low quantities of pneumococcal DNA and a negative antigen test. CONCLUSIONS: Our results suggest an imperfect relationship between the 2 described methods. The immunochromatographic assay is a simple diagnostic tool, which can be used when resources are limited, and even after antibiotic use, but negative results may require confirmation through a more sensitive test, such as qPCR.

Evaluating bronchodilator response in pediatric patients with post-infectious bronchiolitis obliterans: use of different criteria for identifying airway reversibility.

OBJECTIVE: Post-infectious bronchiolitis obliterans (PIBO) is a clinical entity that has been classified as constrictive, fixed obstruction of the lumen by fibrotic tissue. However, recent studies using impulse oscillometry have reported bronchodilator responses in PIBO patients. The objective of this study was to evaluate bronchodilator responses in pediatric PIBO patients, comparing different criteria to define the response. METHODS: We evaluated pediatric patients diagnosed with PIBO and treated at one of two pediatric pulmonology outpatient clinics in the city of Porto Alegre, Brazil. Spirometric parameters were measured in accordance with international recommendations. RESULTS: We included a total of 72 pediatric PIBO patients. The mean pre- and post-bronchodilator values were clearly lower than the reference values for all parameters, especially FEF25-75%. There were post-bronchodilator improvements. When measured as mean percent increases, FEV1 and FEF25-75%, improved by 11% and 20%, respectively. However, when the absolute values were calculated, the mean FEV1 and FEF25-75% both increased by only 0.1 L. We found that age at viral aggression, a family history of asthma, and allergy had no significant effects on bronchodilator responses. CONCLUSIONS: Pediatric patients with PIBO have peripheral airway obstruction that is responsive to treatment but is not completely reversible with a bronchodilator. The concept of PIBO as fixed, irreversible obstruction does not seem to apply to this population. Our data suggest that airway obstruction is variable in PIBO patients, a finding that could have major clinical implications. OBJETIVO: A bronquiolite obliterante pós-infecciosa (BOPI) é uma entidade clínica que tem sido classificada como obstrução fixa e constritiva do lúmen por tecido fibrótico. Entretanto, estudos recentes utilizando oscilometria de impulso relataram resposta ao broncodilatador em pacientes com BOPI. O objetivo deste estudo foi avaliar a resposta broncodilatadora em pacientes pediátricos com BOPI, comparando critérios diferentes para a definição da resposta. MÉTODOS: Foram avaliados pacientes pediátricos com diagnóstico de BOPI tratados em um de dois ambulatórios de pneumologia pediátrica na cidade de Porto Alegre (RS). Parâmetros espirométricos foram medidos de acordo com recomendações internacionais. RESULTADOS: Foram incluídos 72 pacientes pediátricos com BOPI no estudo. As médias dos valores pré- e pós-broncodilatador foram claramente inferiores aos valores de referência para todos os parâmetros, especialmente FEF25-75%. Houve uma melhora pós-broncodilatador. Quando medidos como aumentos percentuais médios, VEF1 e FEF25-75% melhoraram em 11% e 20%, respectivamente. Entretanto, quando os valores absolutos foram calculados, as médias de VEF1 e FEF25-75% aumentaram somente em 0,1 l. Verificamos que a idade da agressão viral, história familiar de asma e alergia não tiveram efeitos significativos na resposta ao broncodilatador. CONCLUSÕES: Pacientes pediátricos com BOPI têm uma obstrução das vias aéreas periféricas que responde ao tratamento, mas não uma reversão completa com o broncodilatador. O conceito de BOPI como obstrução fixa e irreversível parece não se aplicar a essa população. Nossos dados sugerem que a obstrução de vias aéreas em pacientes com BOPI é variável, e esse achado pode ter importantes implicações clínicas.

Influência da intubação orotraqueal nas alterações miofuncionais orofaciais em lactentes / Influencia de la intubación orotraqueal sobre los cambios miofuncionales orofaciales en lactantes / Influence of orotracheal intubation on orofacial myofuncional changes in infants

Introdução: As alterações da deglutição pós-extubação são amplamente identificadas e estudadas, nas quais a disfagia é identificada nas fases oral e faríngea, acompanhada de penetração laríngea e aspiração traqueal. Entretanto, as alterações miofuncionais orofaciais em pacientes pós-extubação ainda não estão bem descritas em Pediatria. Objetivo: Verificar a influência da intubação orotraqueal (IOT) nas alterações miofuncionais orofaciais do lactente. Método: Estudo transversal, realizado em uma Unidade de Terapia Intensiva Pediátrica no período entre novembro de 2015 a setembro de 2016. Os participantes foram divididos em dois grupos: grupo estudo, com diagnóstico médico de cardiopatia congênita, pós-operatório de cirurgia cardíaca, em IOT por no mínimo 6 horas, e grupo controle composto por lactentes de 0 a 6 meses, previamente saudáveis, que não tiveram histórico de qualquer IOT anterior. Após a seleção, ambos os grupos foram submetidos ao Protocolo de Avaliação de Disfagia Pediátrica. Resultados: Postura e tônus labial, postura de língua, palato e qualidade vocal estiveram significativamente associados ao uso de IOT, pressão intraoral e padrão de sucção. Ao analisar a distribuição de frequência da comparação com e sem IOT, observa-se que não há um padrão específico que indique se a associação é prejudicial, protetora ou não interfere no padrão das características miofuncionais nesses casos. Conclusão: A influência da IOT foi encontrada nas estruturas miofuncionais orofaciais de bebês, quando comparados a bebês não intubados.

Introduction: Post-extubation swallowing changes are widely identified and studied, in which dysphagia is identified in the oral and pharyngeal phases, accompanied by laryngeal penetration and aspiration. However, orofacial myofunctional changes in post-extubation patients are still not well described in pediatrics. Objective: Verify the influence of orotracheal intubation on orofacial myofunctional changes in lactates. Methods: Cross-sectional study, performed in a Pediatric Intensive Care Unit from November 2015 to September 2016. Participants were divided into two groups: study group, with medical diagnosis of congenital heart disease, post-cardiac surgery, undergoing OTI for at least 6 hours, and the control group was composed of infants aged 0 to 6 months, previously healthy, who did not have any previous IOT. After selection, babies from both groups were submitted to the same assessment protocols. The clinical evaluation of the child's orofacial myofunctional structures was performed using the Pediatric Dysphagia Assessment Protocol. Results: Lip posture, lip tone, tongue posture, palate, and vocal quality were significantly associated with the use of OIT, intraoral pressure and suction pattern. When analyzing the frequency distribution of the comparison with and without OIT, it is observed that there is no specific pattern that indicates whether the association is harmful, protective or does not interfere in the pattern of myofunctional characteristics in these cases. Conclusion: OIT influence was found in orofacial myofunctional structures in infants, when compared to babies who were not intubated.

Introducción: Los cambios en la deglución post-extubación están ampliamente identificados y estudiados, en los que se identifica disfagia en las fases oral y faríngea, acompañada de penetración y aspiración laríngea. Sin embargo, los cambios miofuncionales orofaciales en pacientes post-extubación todavía no están bien descritos en pediatría. Objetivo: Verificar la influencia de la intubación orotraqueal sobre los cambios miofuncionales orofaciales en lactatos. Metodos: estudio transversal, realizado en una Unidad de Cuidados Intensivos Pediátricos desde noviembre de 2015 hasta septiembre de 2016. Los participantes se dividieron en dos grupos: grupo de estudio, con diagnóstico médico de cardiopatía congénita, postoperatorio cardíaco, sometidos a IOT durante al menos 6 horas y el grupo control, compuesto por lactantes de 0 a 6 meses, previamente sanos, que no tiene alguna IOT anterior. Después de la selección, los bebés de ambos grupos fueron sometidos a los mismos protocolos de evaluación. La evaluación clínica de las estructuras miofuncionales orofaciales del niño se realizó mediante el Protocolo de Evaluación de Disfagia Pediátrica. Resultados: La postura de los labios, el tono de los labios, la postura de la lengua, el paladar, la calidad vocal se asociaron significativamente con el uso de ITO, la presión intraoral y el patrón de succión. Al analizar la distribución de frecuencias de la comparación con y sin ITO, se observa que no existe un patrón específico que indique si la asociación es dañina, protectora o no interfiere en el patrón de características miofuncionales en estos casos. Conclusión: la influencia de la ITO se encontró en las estructuras miofuncionales orofaciales en los bebés, en comparación con los bebés que no fueron intubados.

Chronic obstructive pulmonary diseases in children.

OBJECTIVES: To verify and describe the main events related to the diagnosis and management of chronic obstructive pulmonary diseases in children (COPDC) and adolescents, considering the interrelated physiopathology, genetic, and environmental characteristics. SOURCES: Relevant literature from PubMed was selected and reviewed. SUMMARY OF THE FINDINGS: COPDC have an environmental and/or genetic origin and its manifestation has manifold genotypes, phenotypes, and endotypes. Although COPDC has no cure, it can be clinically controlled. Chronic cough is the main symptom and bronchiectasis can be present in several COPDC patients. The management of COPDC is more effective if based on guidelines and when treatment regimen adherence is promoted. Oral and inhaled corticosteroids, bronchodilators, inhaled antibiotics, and treatment of pulmonary exacerbation (PE) are the bases of COPDC management, and should be individualized for each patient. CONCLUSIONS: Correct diagnosis and knowledge of risk factors and comorbidities are essential in COPDC management. Procedures and drugs used should be based on specific guidelines for each COPDC case. Treatment adherence is critical to obtain the benefits of management. COPDC clinical control must be evaluated by the decrease in PEs, improved quality of life, reduction of pulmonary function loss, and lung structural damage. For most cases of COPDC, monitoring by interdisciplinary teams in specialized reference centers with surveillance strategies and continuous care leads to better outcomes, which must be evaluated by decreasing pulmonary function damage and deterioration, better prognosis, better quality life, and increased life expectancy.

Profile of cystic fibrosis in two reference centers in southern Brazil.

OBJECTIVE: to compare the characteristics of cystic fibrosis patients treated in two reference centers in southern Brazil in order to observe trends in the treatment and clinical outcomes that may produce changes in clinical conduct. METHODS: cross-sectional, retrospective study with 83 patients diagnosed with cystic fibrosis, aged one month to eighteen years. The variables analyzed were obtained through review of medical records, including: demographic and clinical characteristics, socioeconomic status, pulmonary function test, bacterial colonization profile, medication and physiotherapy. RESULTS: between the two centers there was significant difference in the variables, including admissions in the previous year (p<0.001), lifetime hospital admissions (p<0.001), use of Dornase alpha (p=0.003) and inhaled antibiotic therapy (p=0.006), which were higher at the Santo Antônio Children's Hospital (HCSA), while age at first colonization with Staphylococcus aureus (p=0.008), maternal age (p=0.030), clinical score (p=0.001), socioeconomic score (p=0.021) and use of hypertonic saline (p<0.001) were lower at HCSA compared to São Lucas Hospital (HSL). CONCLUSION: the study centers seem to receive a different population of patients, both in socioeconomic terms, as well as disease severity, which interferes with the choice of medication treatment. At the HCSA, preventive actions against infection due to the high incidence of Burkholderia cepacia Complex and careful research into early lung changes will be encouraged.

Disfagia orofaríngea em crianças com síndrome Cornélia De Lange: série de casos / Orofarineal dysfunction in children with the Cornélia De Lange Syndrome: series of cases / Disfagia orofaríngea en niños con síndrome de Cornelia De Lange: serie de casos

Introdução: A síndrome Cornélia De Lange (CdLS) é caracterizada por ser polimalformativa que envolve anomalias faciais, atraso de crescimento e desenvolvimento psicomotor, alterações comportamentais e malformações associadas. Sabe-se que as crianças acometidas por essa síndrome apresentam alterações de deglutição, mas são poucos os estudos apresentados na literatura devido à raridade da doença, sendo encontrado relato de um caso, e na maioria das vezes, com descrição dos achados. Objetivo: Identificar as alterações de deglutição em crianças com a Síndrome Cornélia de Lange, por meio da videofluoroscopia. Metodologia: Série de Casos, retrospectiva. Trata-se de uma amostra de conveniência com crianças, diagnosticadas com Síndrome Cornélia de Lange, que apresentassem videofluoroscopia da deglutição. Foram excluídos prontuários de pacientes que não estivessem completos. Os dados de caracterização da amostra foram obtidos através de prontuários físicos e os dados de desfecho do estudo através de laudos clínicos de videofluoroscopias da deglutição dos pacientes. Resultados: Dos 6 indivíduos, 5 do sexo masculino, em que 3 (50%) apresentaram aspiração laringotraqueal, de forma silente. A mediana de idade foi de 5,50 meses. Conforme os achados nas videofluoroscopias da deglutição, identificou-se dificuldades de deglutição como escape posterior prematuro de alimento, ejeção ineficiente e dificuldades de formação do bolo alimentar, como atraso no acionamento da reação faríngea, refluxo para nasofaringe, estase em valéculas e seios periformes e aspiração traqueal. Conclusão: Todas as crianças com Síndrome Cornélia de Lange deste estudo apresentaram disfagia em algum grau, e metade delas apresentou aspiração laringotraqueal de forma silente.

Introduction: Cornélia De Lange Syndrome (CdLS) is characterized by being polymalformative that involves facial anomalies, growth and psychomotor development retardation, behavioral changes and associated malformations. It is known that children affected by this syndrome have swallowing disorders, but there are few studies presented in the literature due to the rarity of the disease, with a case report being found and mostly with description of the findings. Objective: To identify swallowing disorders in children with Cornelia de Lange Syndrome, through videofluoroscopy. Methodology: Case series, retrospective. This is a convenience sample with children, diagnosed with Cornelia de Lange Syndrome, who had swallowing videofluoroscopy. Medical records of patients who were not complete were excluded. The sample characterization data were obtained from physical records and the study outcome data through clinical reports of patients' swallowing videofluoroscopies. Results: Of the 6 individuals, 5 were male, in which 3 (50%) had laryngotracheal aspiration, silently. The median age was 5.50 months. According to the findings in the swallowing videofluoroscopies, swallowing difficulties were identified, such as premature posterior escape of food, inefficient ejection and difficulties in the formation of the bolus, such as delay in triggering the pharyngeal reaction, reflux to the nasopharynx, stasis in the valleys and peripheral sinuses and tracheal aspiration. Conclusion: All children with Cornelia de Lange Syndrome in this study had dysphagia to some degree, and half of them had silent laryngotracheal aspiration.;Introducción: El síndrome de Cornélia De Lange (CdLS) se caracteriza por ser polimalformativo que involucra anomalías faciales, retraso del crecimiento y desarrollo psicomotor, cambios de comportamiento y malformaciones asociadas. Se sabe que los niños afectados por este síndrome presentan trastornos de la deglución, pero existen pocos estudios presentados en la literatura debido a la rareza de la enfermedad, encontrándose un reporte de caso y la mayoría de las veces con descripción de los hallazgos.

Objetivo: identificar los trastornos de la deglución en niños con síndrome de Cornelia de Lange, mediante videofluoroscopia. Metodología: Serie de casos, retrospectiva. Se trata de una muestra de conveniencia con niños, diagnosticados de Síndrome de Cornelia de Lange, que habían ingerido videofluoroscopia. Se excluyeron los registros médicos de los pacientes que no estaban completos. Los datos de caracterización de la muestra se obtuvieron de los registros médicos físicos y los datos de los resultados del estudio a través de informes clínicos de videofluoroscopias de deglución de los pacientes. Resultados: De los 6 individuos, 5 eran varones, de los cuales 3 (50%) tenían aspiración laringotraqueal, en silencio. La mediana de edad fue de 5,50 meses. De acuerdo con los hallazgos en las videofluoroscopias de deglución, se identificaron dificultades de deglución, como escape posterior prematuro de alimentos, eyección ineficiente y dificultades en la formación del bolo, como retraso en el desencadenamiento de la reacción faríngea, reflujo a la nasofaringe, estasis en los valles y senos periféricos y aspiración traqueal. Conclusión: Todos los niños con síndrome de Cornelia de Lange en este estudio tenían disfagia en algún grado y la mitad de ellos tenían aspiración laringotraqueal en silencio.