High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening

OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.

Methicillin-resistant Staphylococcus aureus in cystic fibrosis patients: do we need to care? A cohort study

ABSTRACT CONTEXT AND OBJECTIVE: The prevalence of a variety of potentially pathogenic microorganisms in cystic fibrosis patients, such as methicillin-resistant Staphylococcus aureus (MRSA), has increased over the past decade. Given the increasing prevalence of MRSA and the few data available in the literature, better understanding of the clinical repercussions of colonization by this bacterium in cystic fibrosis patients becomes essential. This study aimed to evaluate the repercussions of chronic colonization by MRSA in cystic fibrosis patients. DESIGN AND SETTING: Retrospective cohort study from January 2004 to December 2013 in a cystic fibrosis reference center. METHODS: Each patient with cystic fibrosis was evaluated for nutritional status (body mass index, BMI, and BMI percentile), pulmonary function and tomographic abnormalities (modified Bhalla scores) at the time of chronic colonization by MRSA or methicillin-susceptible Staphylococcus aureus (MSSA) and throughout the study period. RESULTS: Twenty pairs of patients were included. There were no significant differences between the groups regarding nutritional characteristics. Spirometric data showed a trend towards greater obstruction of the airways in patients with MRSA. Patients with MRSA presented greater structural damage to their lungs, demonstrated not only by the total Bhalla score but also by its parameters individually. CONCLUSIONS: Patients colonized by MRSA presented greater functional and structural respiratory impairment at the time of chronic colonization. Disease progression was also faster in patients chronically colonized by MRSA than in those with MSSA. This was shown through comparisons that avoided possible confounding variables.

Draft genome sequence of Acinetobacter pittii ST643 shared by cystic fibrosis patients

Acinetobacter pittii has emerged as an important hospital pathogen that is associated with outbreaks and drug resistance. In cystic fibrosis (CF) patients, the detection of Acinetobacter spp. is rare; however, we isolated the A. pittii sequence type ST643 in several Brazilian CF patients treated in the same centre. The current study describes the draft genome of A. pittii ST643.

Escore tomográfico em pacientes com fibrose cística colonizados por Pseudomonas aeruginosa ou Staphylococcus aureus / High-resolution computed tomography scores in cystic fibrosis patients colonized with Pseudomonas aeruginosa or Staphylococcus aureus

OBJETIVO: Comparar achados de TCAR em pacientes com fibrose cística (FC) colonizados cronicamente por Pseudomonas aeruginosa ou Staphylococcus aureus, empregando o escore de Bhalla modificado, e avaliar as confiabilidades intraobservador e interobservador do método. MÉTODOS: Estudo transversal retrospectivo incluindo 41 pacientes portadores de FC, 26 dos quais colonizados cronicamente por P. aeruginosa (grupo Pa) e 15 por S. aureus (grupo Sa). Dois radiologistas analisaram independentemente em duas ocasiões, as imagens de TCAR desses pacientes e aplicaram o escore de Bhalla modificado. As confiabilidades intra e interobservador foram avaliadas segundo o coeficiente de correlação intraclasse (CCI). RESULTADOS: Houve boa concordância intraobservador e interobservador (CCI > 0,8). Os resultados dos escores do grupo Pa foram mais elevados que os do grupo Sa para o observador 1 (média de 13,50 ± 3,90 e mediana de 13,5 vs. média de 5,0 ± 5,28 e mediana de 3,0) e para o observador 2 (média de 11,96 ± 5,07 e mediana de 12,0 vs. média de 5,07 ± 5,65 e mediana de 5,0). Alterações tomográficas, como bronquiectasias, espessamento das paredes brônquicas, formação de tampões mucosos, comprometimento de gerações de divisões brônquicas e padrão de atenuação em mosaico, foram mais prevalentes no grupo colonizado por P. aeruginosa. CONCLUSÕES: O escore de Bhalla modificado se mostrou reprodutível e confiável para a avaliação de TCAR e permitiu a diferenciação entre os pacientes incluídos nos dois grupos. Escores mais altos no grupo Pa evidenciaram maior comprometimento estrutural pulmonar nesse grupo.

OBJECTIVE: To compare HRCT findings in cystic fibrosis (CF) patients chronically colonized with Pseudomonas aeruginosa or Staphylococcus aureus using the modified Bhalla CT scoring system, as well as to evaluate intraobserver and interobserver reliability of the method. METHODS: This was a retrospective cross-sectional study involving 41 CF patients, 26 of whom were chronically colonized with P. aeruginosa (Pa group), and 15 of whom were colonized with S. aureus (Sa group).Two independent radiologists evaluated the HRCT scans of these patients using the modified Bhalla CT scoring system in two different moments. Intraobserver and interobserver reliability was calculated using the intraclass correlation coefficient (ICC). RESULTS: There was good intraobserver and interobserver agreement (ICC > 0.8). Scores were higher in the Pa group than in the Sa group for observer 1 (mean, 13.50 ± 3.90; median, 13.5 vs. mean, 5.00 ± 5.28; median, 3.0) and for observer 2 (mean, 11.96 ± 5.07; median, 12.0 vs. mean, 5.07 ± 5.65; median, 5.0). In addition, HRCT findings, such as bronchiectasis, bronchial wall thickening, mucus plugging, generation of bronchial divisions, and mosaic attenuation/perfusion pattern, were more prevalent in the Pa group. CONCLUSIONS: The modified Bhalla CT scoring system was reproducible and reliable for use in the evaluation of HRCT scans, allowing distinctions to be drawn between the two groups of patients under study. The higher scores in the Pa group provided evidence of greater pulmonary impairment in that group.

Comparison of the worldwide transmissible Pseudomonas aeruginosa with isolates from brazilian cystic fibrosis patients

Cross-infection with Pseudomonas aeruginosa among cystic fibrosis (CF) patients is a rare occurrence. However, the emergence of transmissible strains has been reported between unrelated individuals. We analyzed the genetic relationship among P. aeruginosa isolates from Brazilian CF patients and transmissible clones which are worldwide spread. The data does not indicate the presence of closely related variant clones.

Infecção pulmonar por Pseudomonas aeruginosa na fibrose cística: diagnótico sorológico e conduta / Pseudomonas aeruginosa pulmonary infection in cystic fibrosis: serologic diagnosis and therapeutic / Infección pulmonar por Pseudomonas aeruginosa en la fibrosis cística: diagnóstico serológico y conducta

Objetivo: avaliar o diagnóstico da infecção e colonização pulmonar por Pseudomonas aeruginosa (Pa), através da detecção de anticorpos séricos específicos em pacientes fibrocísticos.Fontes pesquisadas: foi revisada a base de dados Medline, no período de 1986 a 2007, com os descritores fibrose cística e pseudomonas. Síntese dos dados: as alterações no transporte de íons nas células epiteliais levam à obstruçãocrônica das vias aéreas, com infecções secundárias. A Pa mucóide é o principal agente que aumenta a morbidade e a mortalidade nos pacientes fibrocísticos. A erradicação da bactéria é possível com antibioticoterapia precoce, queaumenta a sobrevida dos pacientes. A cultura de secreções respiratórias apresenta limitação como método diagnóstico, pela dificuldade decoleta da amostra. Parece promissora a detecção de anticorpos específicos no soro para Pa, que, em muitos casos, precede o isolamento bacterianoem cultura. O método mais utilizado é ode ELISA, com pesquisa de IgG ou IgG, IgM, e IgA. A positividade da sorologia não discrimina entre colonização e infecção pulmonar e, a condutaantibiótica não é uniforme nessa situação. Conclusões: a pesquisa sorológica de anticorpos para Pa pode ser útil no tratamento do pacientefibrocístico. Complementa a avaliação clínica, permite reavaliações não invasivas durante a evolução e possibilita a antibioticoterapia precoceem parte dos casos.

Objective: to evaluate the diagnosis of lung infection and colonization by Pseudomonas aeruginosa (Pa) through the detection of specific seric antibodies in fibrocystic patients. Data sources: the Medline database, was searched in the period 1986 to 2007, with the keywords cystic fibrosisand pseudomonas. Data synthesis: the epithelium cells disturb in ion transport cause a chronic airways obstruction, with secondary infections. Themucoid Pa is the main agent that increases morbidity and mortality in fibrocystic patients. The Objective: to evaluate the diagnosis of lung infectionand colonization by Pseudomonas aeruginosa (Pa) through the detection of specific seric antibodies in fibrocystic patients. Data sources: the Medline database, was searched in the period1986 to 2007, with the keywords cystic fibrosis and pseudomonas. Data synthesis: the epithelium cells disturb in ion transport cause a chronic airwaysobstruction, with secondary infections. The mucoid Pa is the main agent that increases morbidity and mortality in fibrocystic patients. The bacteria eradication is possible with early antibiotic therapy, which increases patients’ survival period. Respiratory secretions culture is limited as a diagnostic method, due to the difficulty incollecting samples. The detection of specific antibodies in serum for Pa, which in many cases precedesthe bacteria isolation in culture, seems to be promising. The most used method is the ELISA, with survey of IgG or IgG, IgM, and IgA. The serological positive result does not make a distinctionbetween lung colonization and infection, and the antibiotic procedure is not uniform in such situation. Conclusions: the serological antibodies survey for Pa can be useful in the treatment of fibrocystic patients. It completes the clinical evaluation, allowing non invasive reevaluations during the evolution as well as an early antibiotictherapy in some cases.

Objetivo: evaluar el diagnostico de la infección y colonización pulmonar por Pseudomonas aeruginosa (Pa) en pacientes con fibrosis cística, mediante la detección de anticuerpos sericosespecíficos. Fuente de los datos: fueron seleccionados los artículos en la base de datos Medline publicados entre 1986 y 2007, con las palabras clave fibrose cistica y pseudomonas. Síntesis delos datos: las alteraciones en el transporte de los iones de las células epiteliales llevan a la obstruccióncrónica de las vias aereas, con infecciones secundarias. La Pa mucoide es el principal agente que aumenta la morbidad y mortalidad de los pacientes fibrocisticos. La eliminación de labacteria es posible con antibióticoterapia precoz, qui determina mayor sobrevida de los pacientes.Lo cultivo de secreciones respiratorias presenta limitación como método diagnóstico, determinada por la dificultad de recolectar la muestra. Ladetección de anticuerpos específicos en el suero para Pa en muchos casos precede el islamiento bacteriano en cultura. Lo metodo más utilizadoes el de ELISA, con pesquisa de IgG, o IgG IgM y IgA. El resultado positivo non discrimina entre la ocurrencia de colonización o infección pulmonary, por lo tanto, a conducta non es uniforme en esta situación. Conclusiones: la pesquisa serologicade anticuerpos para Pa puede ser util en el tratamiento del paciente con fibrose cística. Complementa la evaluación clinica, permite reevaluaciones non invasivas, y posibilita la antibioticoterapia precoz en parte dos casos.

Chronic infection of cystic fibrosis patient airways by a single clone of Burkholderia cepacia: replacement of non-mucoid to mucoid morphotype

O presente trabalho descreve a emergência de cepas mucoides do complexo B. cepacia em um paciente com Fibrose Cística dentro de um acompanhamento bacteriológico prospectivo de nove anos. Os dados clínicos sugerem a associação entre o isolamento do morfotipo mucoide e a deterioração clínica do paciente. Apesar da variação fenotípica, os testes moleculares mostraram que o paciente manteve-se cronicamente infectado por cepas de mesma origem clonal.

Chronic infection of cystic fibrosis patient airways by a single clone of Burkholderia cepacia: replacement of non-mucoid to mucoid morphotype

Mucoid Burkholderia cepacia morphotype emerged within a nine year follow-up of a cystic fibrosis patient. Clinical data suggested a linkage between the mucoid phenotype isolation and the deterioration of the patient's condition. Despite of the phenotypic variation, molecular typing showed that the patient was chronically infected with B. cepacia complex isolates belonging to a same genetic clone.

O presente trabalho descreve a emergência de cepas mucoides do complexo B. cepacia em um paciente com Fibrose Cística dentro de um acompanhamento bacteriológico prospectivo de nove anos. Os dados clínicos sugerem a associação entre o isolamento do morfotipo mucoide e a deterioração clínica do paciente. Apesar da variação fenotípica, os testes moleculares mostraram que o paciente manteve-se cronicamente infectado por cepas de mesma origem clonal.

Chronic infection of cystic fibrosis patient airways by a single clone of Burkholderia cepacia: replacement of non-mucoid to mucoid morphotype

Mucoid Burkholderia cepacia morphotype emerged within a nine year follow-up of a cystic fibrosis patient. Clinical data suggested a linkage between the mucoid phenotype isolation and the deterioration of the patient's condition. Despite of the phenotypic variation, molecular typing showed that the patient was chronically infected with B. cepacia complex isolates belonging to a same genetic clone.

O presente trabalho descreve a emergência de cepas mucoides do complexo B. cepacia em um paciente com Fibrose Cística dentro de um acompanhamento bacteriológico prospectivo de nove anos. Os dados clínicos sugerem a associação entre o isolamento do morfotipo mucoide e a deterioração clínica do paciente. Apesar da variação fenotípica, os testes moleculares mostraram que o paciente manteve-se cronicamente infectado por cepas de mesma origem clonal.