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OBJECTIVE: To identify the characteristics of children with cystic fibrosis with low initial forced expiratory volume in 1 second (FEV1) % predicted and to investigate their outcome. STUDY DESIGN: Patients were categorized into low or high initial FEV1 groups using cluster analysis. Comparisons of the demographic and clinical data were performed between the 2 groups. RESULTS: From 122 children, 21 clustered into the low and 101 into the high FEV1 group. The mean FEV1 was 69% ± 12% predicted for the low and 95% ± 12% predicted for the high FEV1 group (P < .001). The low FEV1 group had lower body mass index percentiles (P = .003), were hospitalized more frequently (P = .001), and had been on dornase alfa longer (P = .006). Low FEV1 group had more patients with positive cultures for Pseudomonas aeruginosa (P = .002) and Stenotrophomonas maltophilia (P < .001) and had more total number of cultures positive for mucoid P. aeruginosa (P = .009) and methicillin resistant Staphylococcus aureus + P. aeruginosa (P = .005). The low FEV1 group continued to have low FEV1 measurements, their FEV1 declined slower, required more hospitalizations per year (P = .01), and had more cultures for mucoid (P = .003) and nonmucoid P. aeruginosa (P = .02) ± methicillin resistant S. aureus (P = .002) in comparison with the high FEV1 group. Poor adherence was associated with lower initial FEV1 values in females, and early, rapid decline of FEV1 in males. CONCLUSIONS: Some children with cystic fibrosis may present with poor lung function early in life and continue to have subnormal lung function associated with reduced body mass index, more frequent hospitalization, and higher rates of infection. Such children may benefit from careful evaluation and close follow-up.

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INTRODUCTION: Flexible bronchoscopy is a valuable diagnostic and therapeutic tool. The objective of the study is to describe the experience with flexible bronchoscopy in a Pulmonology Section of a Pediatric tertiary care hospital associated to the university. POPULATION AND METHODS: Studies performed between 01/2002 and 12/2005 were reviewed. Olympus(R) bronchoscopes (external diameters 2.8 and 4.8 mm) were used. Bronchoalveolar lavage was performed with 3 aliquots of 1 ml/kg of warmed sterile saline solution. RESULTS: One hundred and two fiberoptic bronchoscopies (49 males, 42 outpatients) and 77 bronchoalveolar lavage were performed. Patient's age ranged from 2 months to 18 years. Fifty one studies were done in an endoscopy suite, 47 in the operating room and 4 in the intensive care unit. Patient's diagnosis were: chronic lung disease (recurrent/ persistent pneumonia, bronchiectasis, primary ciliary dyskinesia) 65% (n: 66); immune deficiencies 18% (n: 18); cystic fibrosis 7% (n: 7), difficult airway 5% (n: 6) and noisy breathing 5% (n: 5). Ten patients developed mild complications (7 hypoxemia, 2 upper airway obstruction, 1 cardiac arrhythmia), and 1 patient a severe one (severe bronchial obstruction). Eighty five (65/77) bronchoalveolar lavage samples, 100% (12/12) bronchial brushing and 100% (6/6) endobronchial biopsies were representative. Procedures helped the decision-making process in 75% (76/102) of the patients studied. CONCLUSIONS: In this carefully selected population, flexible bronchoscopy and bronchoalveolar lavage were useful for their diagnosis and treatment.

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La fibrobroncoscopia constituye una herramienta diagnóstico-terapéutica de gran valor.El objetivo del presente estudio es describir la experienciacon fibrobroncoscopia en un servicio deneumonología de un hospital de niños urbano asociadoa la Universidad.Material y métodos. Se revisaron todos los procedimientosde fibrobroncoscopia realizados entre01/2002 y 12/2005. Se utilizaron broncoscopiosOlympus« (diámetro externo 2,8 mm y 4,8 mm). El lavado broncoalveolar se realizó con 3 alícuotas de1 ml/kg de solución salina entibiada.Resultados. Se efectuaron 102 fibrobroncoscopias(49 varones, 42 pacientes ambulatorios), y en 77 casosse realizó lavado broncoalveolar. El intervalo deedad fue de 2 meses a 18 años. Cincuenta y unestudios se realizaron en la sala de procedimientosde la unidad, 47 en quirófano y 4 en la unidad decuidados intensivos. Los diagnósticos de los pacientesfueron: enfermedad pulmonar crónica (neumoníarecurrente/persistente, bronquiectasias, sospechade disquinesia ciliar) 65% (n: 66); huésped inmunocomprometido18% (n: 18); fibrosis quística 7% (n:7), intubación dificultosa 5% (n: 6) y respiraciónruidosa 5% (n: 5). Diez pacientes tuvieron complicacionesleves (7 hipoxemia, 2 obstrucción laríngea, 1arritmia cardíaca), y 1 paciente complicación grave(obstrucción bronquial graves). Ochenta y cinco porciento (65/77) de las muestras de lavado broncoalveolar,100% (12/12) de los cepillados bronquiales y100% (6/6) de las biopsias de mucosa fueron representativas.Los procedimientos fueron de utilidadclínica en 75% (76/102) de los pacientes estudiados.Conclusiones. En esta población cuidadosamenteseleccionada, los resultados de la fibrobroncoscopiay el lavado broncoalveolar fueron útiles para sudiagnóstico y tratamiento.(AU)

Introduction. Flexible bronchoscopy is a valuable diagnostic and therapeutic tool. The objective of the study is to describe the experience with flexible bronchoscopy in a Pulmonology Section of a Pediatric tertiary care hospital associated to the university. Population and methods. Studies performed between 01/2002 and 12/2005 were reviewed. Olympus« bronchoscopes (external diameters 2.8 and 4.8 mm) were used. Bronchoalveolar lavage was performed with 3 aliquots of 1 ml/kg of warmed sterile saline solution. Results. One hundred and two fiberoptic bronchoscopies (49 males, 42 outpatients) and 77 bronchoalveolar lavage were performed. Patientãs age ranged from 2 months to 18 years. Fifty one studies were done in an endoscopy suite, 47 in the operating room and 4 in the intensive care unit. Patientãs diagnosis were: chronic lung disease (recurrent/persistent pneumonia, bronchiectasis, primary ciliary dyskinesia) 65% (n: 66); immune deficiencies 18% (n: 18); cystic fibrosis 7% (n: 7), difficult airway 5% (n: 6) and noisy breathing 5% (n: 5). Ten patients developed mild complications (7 hypoxemia, 2 upper airway obstruction, 1 cardiac arrhythmia), and 1 patient a severe one (severe bronchial obstruction). Eighty five (65/77) bronchoalveolar lavage samples, 100% (12/12) bronchial brushing and 100% (6/6) endobronchial biopsies were representative. Procedures helped the decision-making process in 75% (76/102) of the patients studied. Conclusions. In this carefully selected population, flexible bronchoscopy and bronchoalveolar lavage were useful for their diagnosis and treatment.(AU)

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La fibrobroncoscopia constituye una herramienta diagnóstico-terapéutica de gran valor.El objetivo del presente estudio es describir la experienciacon fibrobroncoscopia en un servicio deneumonología de un hospital de niños urbano asociadoa la Universidad.Material y métodos. Se revisaron todos los procedimientosde fibrobroncoscopia realizados entre01/2002 y 12/2005. Se utilizaron broncoscopiosOlympus® (diámetro externo 2,8 mm y 4,8 mm). El lavado broncoalveolar se realizó con 3 alícuotas de1 ml/kg de solución salina entibiada.Resultados. Se efectuaron 102 fibrobroncoscopias(49 varones, 42 pacientes ambulatorios), y en 77 casosse realizó lavado broncoalveolar. El intervalo deedad fue de 2 meses a 18 años. Cincuenta y unestudios se realizaron en la sala de procedimientosde la unidad, 47 en quirófano y 4 en la unidad decuidados intensivos. Los diagnósticos de los pacientesfueron: enfermedad pulmonar crónica (neumoníarecurrente/persistente, bronquiectasias, sospechade disquinesia ciliar) 65% (n: 66); huésped inmunocomprometido18% (n: 18); fibrosis quística 7% (n:7), intubación dificultosa 5% (n: 6) y respiraciónruidosa 5% (n: 5). Diez pacientes tuvieron complicacionesleves (7 hipoxemia, 2 obstrucción laríngea, 1arritmia cardíaca), y 1 paciente complicación grave(obstrucción bronquial graves). Ochenta y cinco porciento (65/77) de las muestras de lavado broncoalveolar,100% (12/12) de los cepillados bronquiales y100% (6/6) de las biopsias de mucosa fueron representativas.Los procedimientos fueron de utilidadclínica en 75% (76/102) de los pacientes estudiados.Conclusiones. En esta población cuidadosamenteseleccionada, los resultados de la fibrobroncoscopiay el lavado broncoalveolar fueron útiles para sudiagnóstico y tratamiento.

Introduction. Flexible bronchoscopy is a valuable diagnostic and therapeutic tool. The objective of the study is to describe the experience with flexible bronchoscopy in a Pulmonology Section of a Pediatric tertiary care hospital associated to the university. Population and methods. Studies performed between 01/2002 and 12/2005 were reviewed. Olympus® bronchoscopes (external diameters 2.8 and 4.8 mm) were used. Bronchoalveolar lavage was performed with 3 aliquots of 1 ml/kg of warmed sterile saline solution. Results. One hundred and two fiberoptic bronchoscopies (49 males, 42 outpatients) and 77 bronchoalveolar lavage were performed. Patient’s age ranged from 2 months to 18 years. Fifty one studies were done in an endoscopy suite, 47 in the operating room and 4 in the intensive care unit. Patient’s diagnosis were: chronic lung disease (recurrent/persistent pneumonia, bronchiectasis, primary ciliary dyskinesia) 65% (n: 66); immune deficiencies 18% (n: 18); cystic fibrosis 7% (n: 7), difficult airway 5% (n: 6) and noisy breathing 5% (n: 5). Ten patients developed mild complications (7 hypoxemia, 2 upper airway obstruction, 1 cardiac arrhythmia), and 1 patient a severe one (severe bronchial obstruction). Eighty five (65/77) bronchoalveolar lavage samples, 100% (12/12) bronchial brushing and 100% (6/6) endobronchial biopsies were representative. Procedures helped the decision-making process in 75% (76/102) of the patients studied. Conclusions. In this carefully selected population, flexible bronchoscopy and bronchoalveolar lavage were useful for their diagnosis and treatment.

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