ARNOLD-Australasian Registry Network for Orphan Lung Disease

References

Northway WH, Jr., Moss RB, Carlisle KB, Parker BR, Popp RL, Pitlick PT, Eichler I, Lamm RL, Brown BW, Jr. Late pulmonary sequelae of bronchopulmonary dysplasia. N Engl J Med 1990;323(26):1793-9.

Wong PM, Lees AN, Louw J, Lee FY, French N, Gain K, Murray CP, Wilson A & Chambers DC. Emphysema in young adult survivors of moderate and severe bronchopulmonary dysplasia. Eur Respir J. 2008 Aug;32(2):321-8.

Vrijlandt EJ, Gerritsen J, Boezen HM, Grevink RG, Duiverman EJ. Lung function and exercise capacity in young adults born prematurely. Am J Respir Crit Care Med 2006;173(8):890-6.

Narang I, Rosenthal M, Cremonesini D, Silverman M, Bush A. Longitudinal evaluation of airway function 21 years after preterm birth. Am J Respir Crit Care Med. 2008 Jul 1;178(1):74-80

Doyle LW, Olinsky A, Faber B, Callanan C. Adverse effects of smoking on respiratory function in young adults born weighing less than 1000 grams. Pediatrics 2003;112(3 Pt 1):565-9.

Aukland SM, Halvorsen T, Fosse KR, Daltveit AK, Rosendahl K. High-resolution CT of the chest in children and young adults who were born prematurely: findings in a population-based study. Am J Roentgenol 2006;187(4):1012-8.

Aquino SL, Schechter MS, Chiles C, Ablin DS, Chipps B, Webb WR. High-resolution inspiratory and expiratory CT in older children and adults with bronchopulmonary dysplasia. AJR Am J Roentgenol 1999;173(4):963-7.

Halvorsen T, Skadberg BT, Eide GE, Roksund OD, Carlsen KH, Bakke P. Pulmonary outcome in adolescents of extreme preterm birth: a regional cohort study. Acta Paediatr 2004;93(10):1294-300.

Doyle LW, Faber B, Callanan C, Freezer N, Ford GW, Davis NM. Bronchopulmonary dysplasia in very low birth weight subjects and lung function in late adolescence. Pediatrics. 2006;118(1):108-13.

Howling SJ, Northway WH, Jr., Hansell DM, Moss RB, Ward S, Muller NL. Pulmonary sequelae of bronchopulmonary dysplasia survivors: high-resolution CT findings. Am J Roentgenol 2000;174(5):1323-6.

Doyle LW, Cheung MM, Ford GW, Olinsky A, Davis NM, Callanan C. Birth weight <1501 g and respiratory health at age 14. Arch Dis Child 2001;84(1):40-4.

Doyle LW, Ford GW, Olinsky A, Knoches AM, Callanan C. Bronchopulmonary dysplasia and very low birthweight: lung function at 11 years of age. J Paediatr Child Health 1996;32(4):339-43.

Jobe AH, Bancalari E. Bronchopulmonary dysplasia. Am J Respir Crit Care Med 2001;163:1723-1729.

Eber E, Zach MS. Long term sequelae of bronchopulmonary dysplasia (chronic lung disease of infancy). Thorax 2001;56(4):317-23.

Koumbourlis AC, Motoyama EK, Mutich RL, Mallory GB, Walczak SA, Fertal K. Longitudinal follow-up of lung function from childhood to adolescence in prematurely born patients with neonatal chronic lung disease. Pediatr Pulmonol 1996;21(1):28-34.

Grown up (>16 years) chronic neonatal lung disease

 

Dr. Dan Chambers, Queensland Centre for Pulmonary Transplantation & Vascular Disease, The Prince Charles Hospital, Brisbane

Definition: Lung disease associated with a history of preterm birth (usually < 32 weeks) in a patient >18 years

Causes: Preterm birth (<32 weeks or <1500g) is a strong risk factor, but occasionally babies born near term will have persistent respiratory difficulties and lung disease in adulthood. While there is limited information on other risk factors at this stage, bronchopulmonary dysplasia is probably a risk factor and disease severity in adulthood appears associated with time requiring supplemental oxygen as an infant. It is possible that the use of surfactant therapy in neonatal intensive care units from the early 1990s may modify the adult phenotype.

Clinical Presentations: Most patients present with breathlessness on exertion and exercise limitation, cough which may be productive, and wheezing. Patients may be misdiagnosed with asthma. Importantly the history of preterm birth may not come to light immediately as many infants recover significantly during early childhood. In a young adult presenting with respiratory symptoms it is important to enquire about the perinatal history.

Investigations:Full lung function usually reveals evidence of airflow limitation and gas trapping, although the total lung capacity is often normal. The diffusing capacity may be low. Chest X-ray may reveal evidence of hyperinflation. The CT-chest features are relatively specific in a young adult and include areas of hypo-attenuation representing gas trapping &/or emphysema, bullae, linear and triangular opacities which may be subpleural and, less commonly, bronchial wall thickening and areas of localized fibrosis.

Treatment: Anecdotally treatment has been similar to that prescribed for patients with asthma and/or cigarette smoking related COPD. There is no information on treatment efficacy in this disease. Transplantation has been indicated in children and adolescents with a history of chronic neonatal lung disease, but thus far there are no reports in the literature of transplantation for Grown Up Chronic Neonatal Lung Disease.