Problemi non correnti
Il bambino con tosse cronica e bronchiectasie: inquadramento e proposte terapeutiche
BRONCHIECTASIS IN CHILDREN
CESARE BRAGGION, UGO PRADAL, ANTONELLA DELMARCO, MARIA CARLI
Servizio Malattie Respiratorie e Digestive Croniche dell’Età Evolutiva, Centro Regionale Veneto Fibrosi Cistica, Ospedale Civile Maggiore, Azienda Ospedaliera di Verona
Giugno 2003 - pagg. 371 -380
Abstract
Little is known about the prevalence of bronchiectasis (BR) in paediatric age. However, outside the
case of cystic fibrosis (CF), they seem to be rare. The BR can be localised or disseminated. For the
former, the most common etiology is post-infectious (adenovirus, mycoplasma, pertussis, tuberculosis).
Most of the latter are again post-infectious, other causes being CF, the immotile cilia
syndrome, immunological deficiencies and congenital malformations of the lungs. The cause remains
undetermined in 40-50 percent of cases (idiopathic forms). BR must be suspected when a
productive cough persits several weeks from an acute lower respiratory infection (ALRI) or in the
presence of repeated ALRI, particularly if localised in the same area. X-ray may not be sufficient
and CAT scan is often required to identify BR. Early diagnosis is important to increase the efficacy
of treatment, although for many of the proposed treatments (physiotherapy, antibiotics, oral or nebulised
steroids) conclusive evidence of effectiveness is lacking.
Parole chiave
Suggerite dall'AI
Classificazione MeSH
Bibliografia
2002;346:1383-93.
2. Callahan CW, Redding GJ. Bronchiectasis in children: orphan disease or persistent problem? Pediatr Pulmonol 2002;33:492-6.
3. Davis PB, Drumm M, Konstan MW. Cystic fibrosis. State of the art. Am J Respir Crit Care Med 1996;154:1229-56.
4. Padoan R, Pardo F, Giglio L, Bossi A. Regional differences in the incidence of cystic fibrosis in Italy. Ital J Pediatr 2001; 27:876-886.
5. Cavaillon OE, Chadelat K, Boule M, et al. Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease. Eur Respir J 1999;14:339-46.
6. Angrill J, Augusti C, De Celis R, et al. Bronchial inflammation and colonization in patients with clinically stable bronchiectasis. Am J Respir Crit Care Med 2001;164:1628-32.
7. Zheng L, Tipoe G, Lam WK, et al. Up-regulation of circulating adhesion molecules in bronchiectasis. Eur Respir J 2000;16:691-6.
8. Shum DKY, Chan SCH, Ip MSM. Neutrophil- mediated degradation of lung proteoglycans: stimulation by tumor necrosis factoralpha in sputum of patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1925- 31.
9. Zach MS. Lung disease in cystic fibrosis. An updated concept. State of the art review. Pediatr Pulmonol 1990;8:188-202.
10. Godfrey S, Springer C, Novinski N, et al. Exercise but not methacoline differentiates asthma from chronic lung disease in children. Thorax 1991;46:488-92.
11. Kim CK, Chung CY, Sook J, et al. Late abnormal findings on high-resolution computed tomography after Mycoplasma pneumoniae. Pediatrics 2000;105:372-8.
12. Pasteur MC, Helliwell SM, Houghton SJ, et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1277-84.
13. Kauffman HF, Tomee JFC, van der Werf TS, et al. Review of fungus-induced asthmatic reactions. Am J Respir Crit Care Med 1995; 151:2109-16.
14. Bush A, Cole P, Hariri M, et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J 1998;12:982-8.
15. Jones VF, Eid NS, Franco SM, et al. Familial congenital bronchiectasis: Williams-Campbell syndrome. Pediatr Pulmonol 1993;16:263- 7.
16. Rosen FS, Cooper MD, Wedgwood RJP. The primary immunodeficiencies. N Engl J Med 1995;333:431-40.
17. De Gracia J, Rodrigo MJ, Morell F, et al. IgG subclass deficiencies associated with bronchiectasis. Am J Respir Crit Care Med 1996;153:650-5.
18. Sheikh S, Madiraju K, Steiner P, Rao M. Bronchiectasis in pediatric AIDS. Chest 1997;112:1202-7.
19. Quinzii C, Castellani C. The cystic fibrosis transmembrane regulator gene and male infertility. J Endocrinol Invest 2000;23:684-9.
20. Cohn JA, Friedman KJ, Noone PG, et al. Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med 1998;339:653-8.
21. Sharer N, Schwarz M, Malone G, et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med 1998;339:645-52.
22. Pignatti PF, Bombieri C, Marigo C, et al. Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis. Hum Mol Genet 1995;4:635-9.
23. Pignatti PF, Bombieri C, Benetazzo MG, et al. CFTR gene variant IVS8-5T in disseminated bronchiectasis. Am J Hum Genet 1996;58:889-92.
24. Miller PW, Hamosh A, Macek M, et al. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis. Am J Hum Genet 1996;59:45-51.
25. Castellani C, Benetazzo MG, Tamanini A, et al. Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrysineamia with normal sweat test. J Med Genet 2001;38:202-5.
26. Rosenstein BJ, Cutting GR for the Cystic Fibrosis Foundation Consensus Panel. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998;132:589-95.
27. Allain J, Saraux A, Guedes C, et al. Prevalence of symptomatic bronchiectasis in patients with rheumatoid arthritis. Rev Rheum Engl Ed 1997;64: 531-7.
28. Camus P, Colby TV. The lung in inflammatory bowel disease. Eur Respir J 2000;15:5- 10.
29. Nikolaizik WH, Warner JO. Aetiology of chronic suppurative lung disease. Arch Dis Child 1994;70:141-2.
30. Scala R, Aronne D, Palumbo U, et al. Prevalence, age distribution and aetiology of bronchiectasis: a retrospective study on 144 symptomatic patients. Monaldi Arch Chest Dis 2000;55:101-5.
31. Canciani M, Barlocco EG, Mastella G, et al. The saccharin method for testing mucociliary function in patients suspected of having primary ciliary dyskinesia. Pediatr Pulmonol 1988;5: 210-4.
32. Narang I, Ersu R, Wilson NM, Bush A. Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance. Thorax 2002;57(7):586-9.
33. Delmarco A, Pradal U, Cabrini G, et al. Nasal potential difference in cystic fibrosis patients presenting borderline sweat test. Eur Respir J 1997;10:1145-9.
34. Pradal U, Castellani C, Delmarco A, Mastella G. Nasal potential difference in congenital bilateral absence of the vas deferens. Am J Respir Crit Care Med 1998;158:896-901.
35. de Iongh RU, Rutland J. Ciliary defects in healthy subjects, bronchiectasis, and primary ciliary dyskinesia. Am J Respir Crit Care Med 1995;151:1559-67.
36. Rayner CF, Rutman A, Dewar A, et al. Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome. Am J Respir Crit care Med 1996;153:1123-9.
37. Angrill J, Augusti C, de Celis R, et al. Bacterial colonisation in patients with bronchiectasis: microbiological pattern and risk factors. Thorax 2002;57:15-9.
38. Shah PL, Mawdsley S, Nash K, et al. Determinants of chronic infection with Staphylococcus aureus in patients with bronchiectasis. Eur Respir J 1999;14:1340-4.
39. Roberts HR, Wells AU, Milne DG, et al. Airflow obstruction in bronchiectasis: correlation between computed tomography features and pulmonary function tests. Thorax 2000;55:198-204.
40. Jones AP, Rowe BH. Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis. Cochrane Database Syst Rev 2000; (2):CD000045.
41. Kolbe J, Wells A, Ram FS. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev 2000;(2):CD000996.
42. Lasserson T, Holt K, Greenstone M. Oral steroids for bronchiectasis (stable and acute exacerbations). Cochrane Database Syst Rev 2001;(4):CD002162.
43. Corless JA, Warburton CJ. Leukotriene receptor antagonists for non-cystic fibrosis bronchiectasis. Cochrane Database Syst Rev 2000;(4):CD002174.
44. Steele K, Greenstone M, Lasserson JA. Oral methyl-xanthines for bronchiectasis. Cochrane Database Syst Rev 2001;(1): CD00 2734.
45. Sheikh A, Nolan D, Greenstone M. Longacting beta-2-agonists for bronchiectasis. Cochrane Database Syst Rev 2001;(4):CD002155.
46. Lasserson T, Holt K, Evans D, Greenstone M. Anticholinergic therapy for bronchiectasis. Cochrane Database Syst Rev 2001;(4): CD002163.
47. Crockett AJ, Cranston JM, Latimer KM, Alpers JH. Mucolytics for bronchiectasis. Cochrane Database Syst Rev 2001;(1): CD001289.
48. Wills P, Greenstone M. Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev 2002;(1):CD002996.
49. Thomas J, Cook DJ, Brooks D. Chest physical therapy management of patients with cystic fibrosis: a meta-analysis. Am J Respir Crit Care Med 1995;151:846-50.
50. Barker AF, Couch L, Fiel SB, et al. Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med 2000; 162:481-5.
51. Koh YY, Lee MH, Sun YH, et al. Effect of roxithromycin on airway responsiveness in children with bronchiectasis: a double-blind, placebo-controlled study. Eur Respir J 1997; 10(5):994-9.
52. Jaffé A, Bush A. Anti-inflammatory effects of macrolides in lung disease. State of the art. Pediatr Pulmonol 2001;31:464-73.
53. Agasthian T, Deschamps C, Trastek VF, et al. Surgical management of bronchiectasis. Ann Thorac Surg 1996;62:976-80.
2. Callahan CW, Redding GJ. Bronchiectasis in children: orphan disease or persistent problem? Pediatr Pulmonol 2002;33:492-6.
3. Davis PB, Drumm M, Konstan MW. Cystic fibrosis. State of the art. Am J Respir Crit Care Med 1996;154:1229-56.
4. Padoan R, Pardo F, Giglio L, Bossi A. Regional differences in the incidence of cystic fibrosis in Italy. Ital J Pediatr 2001; 27:876-886.
5. Cavaillon OE, Chadelat K, Boule M, et al. Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease. Eur Respir J 1999;14:339-46.
6. Angrill J, Augusti C, De Celis R, et al. Bronchial inflammation and colonization in patients with clinically stable bronchiectasis. Am J Respir Crit Care Med 2001;164:1628-32.
7. Zheng L, Tipoe G, Lam WK, et al. Up-regulation of circulating adhesion molecules in bronchiectasis. Eur Respir J 2000;16:691-6.
8. Shum DKY, Chan SCH, Ip MSM. Neutrophil- mediated degradation of lung proteoglycans: stimulation by tumor necrosis factoralpha in sputum of patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1925- 31.
9. Zach MS. Lung disease in cystic fibrosis. An updated concept. State of the art review. Pediatr Pulmonol 1990;8:188-202.
10. Godfrey S, Springer C, Novinski N, et al. Exercise but not methacoline differentiates asthma from chronic lung disease in children. Thorax 1991;46:488-92.
11. Kim CK, Chung CY, Sook J, et al. Late abnormal findings on high-resolution computed tomography after Mycoplasma pneumoniae. Pediatrics 2000;105:372-8.
12. Pasteur MC, Helliwell SM, Houghton SJ, et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1277-84.
13. Kauffman HF, Tomee JFC, van der Werf TS, et al. Review of fungus-induced asthmatic reactions. Am J Respir Crit Care Med 1995; 151:2109-16.
14. Bush A, Cole P, Hariri M, et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J 1998;12:982-8.
15. Jones VF, Eid NS, Franco SM, et al. Familial congenital bronchiectasis: Williams-Campbell syndrome. Pediatr Pulmonol 1993;16:263- 7.
16. Rosen FS, Cooper MD, Wedgwood RJP. The primary immunodeficiencies. N Engl J Med 1995;333:431-40.
17. De Gracia J, Rodrigo MJ, Morell F, et al. IgG subclass deficiencies associated with bronchiectasis. Am J Respir Crit Care Med 1996;153:650-5.
18. Sheikh S, Madiraju K, Steiner P, Rao M. Bronchiectasis in pediatric AIDS. Chest 1997;112:1202-7.
19. Quinzii C, Castellani C. The cystic fibrosis transmembrane regulator gene and male infertility. J Endocrinol Invest 2000;23:684-9.
20. Cohn JA, Friedman KJ, Noone PG, et al. Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med 1998;339:653-8.
21. Sharer N, Schwarz M, Malone G, et al. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med 1998;339:645-52.
22. Pignatti PF, Bombieri C, Marigo C, et al. Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis. Hum Mol Genet 1995;4:635-9.
23. Pignatti PF, Bombieri C, Benetazzo MG, et al. CFTR gene variant IVS8-5T in disseminated bronchiectasis. Am J Hum Genet 1996;58:889-92.
24. Miller PW, Hamosh A, Macek M, et al. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis. Am J Hum Genet 1996;59:45-51.
25. Castellani C, Benetazzo MG, Tamanini A, et al. Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrysineamia with normal sweat test. J Med Genet 2001;38:202-5.
26. Rosenstein BJ, Cutting GR for the Cystic Fibrosis Foundation Consensus Panel. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998;132:589-95.
27. Allain J, Saraux A, Guedes C, et al. Prevalence of symptomatic bronchiectasis in patients with rheumatoid arthritis. Rev Rheum Engl Ed 1997;64: 531-7.
28. Camus P, Colby TV. The lung in inflammatory bowel disease. Eur Respir J 2000;15:5- 10.
29. Nikolaizik WH, Warner JO. Aetiology of chronic suppurative lung disease. Arch Dis Child 1994;70:141-2.
30. Scala R, Aronne D, Palumbo U, et al. Prevalence, age distribution and aetiology of bronchiectasis: a retrospective study on 144 symptomatic patients. Monaldi Arch Chest Dis 2000;55:101-5.
31. Canciani M, Barlocco EG, Mastella G, et al. The saccharin method for testing mucociliary function in patients suspected of having primary ciliary dyskinesia. Pediatr Pulmonol 1988;5: 210-4.
32. Narang I, Ersu R, Wilson NM, Bush A. Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance. Thorax 2002;57(7):586-9.
33. Delmarco A, Pradal U, Cabrini G, et al. Nasal potential difference in cystic fibrosis patients presenting borderline sweat test. Eur Respir J 1997;10:1145-9.
34. Pradal U, Castellani C, Delmarco A, Mastella G. Nasal potential difference in congenital bilateral absence of the vas deferens. Am J Respir Crit Care Med 1998;158:896-901.
35. de Iongh RU, Rutland J. Ciliary defects in healthy subjects, bronchiectasis, and primary ciliary dyskinesia. Am J Respir Crit Care Med 1995;151:1559-67.
36. Rayner CF, Rutman A, Dewar A, et al. Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome. Am J Respir Crit care Med 1996;153:1123-9.
37. Angrill J, Augusti C, de Celis R, et al. Bacterial colonisation in patients with bronchiectasis: microbiological pattern and risk factors. Thorax 2002;57:15-9.
38. Shah PL, Mawdsley S, Nash K, et al. Determinants of chronic infection with Staphylococcus aureus in patients with bronchiectasis. Eur Respir J 1999;14:1340-4.
39. Roberts HR, Wells AU, Milne DG, et al. Airflow obstruction in bronchiectasis: correlation between computed tomography features and pulmonary function tests. Thorax 2000;55:198-204.
40. Jones AP, Rowe BH. Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis. Cochrane Database Syst Rev 2000; (2):CD000045.
41. Kolbe J, Wells A, Ram FS. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev 2000;(2):CD000996.
42. Lasserson T, Holt K, Greenstone M. Oral steroids for bronchiectasis (stable and acute exacerbations). Cochrane Database Syst Rev 2001;(4):CD002162.
43. Corless JA, Warburton CJ. Leukotriene receptor antagonists for non-cystic fibrosis bronchiectasis. Cochrane Database Syst Rev 2000;(4):CD002174.
44. Steele K, Greenstone M, Lasserson JA. Oral methyl-xanthines for bronchiectasis. Cochrane Database Syst Rev 2001;(1): CD00 2734.
45. Sheikh A, Nolan D, Greenstone M. Longacting beta-2-agonists for bronchiectasis. Cochrane Database Syst Rev 2001;(4):CD002155.
46. Lasserson T, Holt K, Evans D, Greenstone M. Anticholinergic therapy for bronchiectasis. Cochrane Database Syst Rev 2001;(4): CD002163.
47. Crockett AJ, Cranston JM, Latimer KM, Alpers JH. Mucolytics for bronchiectasis. Cochrane Database Syst Rev 2001;(1): CD001289.
48. Wills P, Greenstone M. Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev 2002;(1):CD002996.
49. Thomas J, Cook DJ, Brooks D. Chest physical therapy management of patients with cystic fibrosis: a meta-analysis. Am J Respir Crit Care Med 1995;151:846-50.
50. Barker AF, Couch L, Fiel SB, et al. Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med 2000; 162:481-5.
51. Koh YY, Lee MH, Sun YH, et al. Effect of roxithromycin on airway responsiveness in children with bronchiectasis: a double-blind, placebo-controlled study. Eur Respir J 1997; 10(5):994-9.
52. Jaffé A, Bush A. Anti-inflammatory effects of macrolides in lung disease. State of the art. Pediatr Pulmonol 2001;31:464-73.
53. Agasthian T, Deschamps C, Trastek VF, et al. Surgical management of bronchiectasis. Ann Thorac Surg 1996;62:976-80.