Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience

Orphanet Journal of Rare Diseases - Tập 12 - Trang 1-9 - 2017
Vishal Saddi1, Sean Beggs2, Bruce Bennetts3,4, Joanne Harrison5, Neil Hime4,6, Nitin Kapur7, Jill Lipsett8, Lawrence M. Nogee9, Amy Phu4,6, Sadasivam Suresh7, André Schultz10, Hiran Selvadurai4,11, Stephanie Sherrard10, Roxanne Strachan1, Julian Vyas12, Yvonne Zurynski6, Adam Jaffé13
1Department of Respiratory Medicine, Discipline of Paediatrics, Sydney Children’s Hospital, Sydney, Australia
2Department of Paediatrics, Royal Hobart Hospital, Hobart, Australia
3Department of Molecular Genetics, The Children’s Hospital at Westmead, Sydney, Australia
4Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia
5Department of Respiratory Medicine, The Children’s Hospital, Melbourne, Australia
6Australian Paediatric Surveillance Unit, Kids Research Institute, Sydney, Australia
7Department of Respiratory Medicine, Lady Cilento Children’s Hospital, Brisbane, Australia
8Anatomical Pathology, S.A. Pathology, Women’s and Children’s Hospital, Adelaide, Australia
9Eudowood Neonatal Pulmonary Division, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, USA
10Department of Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Australia
11Department of Respiratory Medicine, The Children’s Hospital at Westmead, Sydney, Australia
12Department of Respiratory Paediatrics, Starship Children’s Hospital, Auckland, New Zealand
13School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, Australia

Tóm tắt

Childhood interstitial lung disease (chILD) represents a rare heterogeneous group of respiratory disorders. In the absence of randomized controlled clinical trials, global collaborations have utilized case series with an aim to standardising approaches to diagnosis and management. Australasian data are lacking. The aim of this study was to calculate prevalence and report the experience of chILD in Australasia over a decade. Paediatric pulmonologists in Australia and New Zealand involved in the care of patients aged 0–18 years with chILD completed a questionnaire on demographics, clinical features and outcomes, over a 10 year period. These data, together with data from the 2 reference genetics laboratories, were used to calculate prevalence. One hundred fifteen cases were identified equating to a period prevalence (range) of 1.5 (0.8–2.1) cases/million for children aged 0–18years. Clinical data were provided on 106 patients: the <2 year group comprised 66 children, median age (range) 0.50 years (0.01–1.92); the ≥2 year group comprised 40 children, median age 8.2 years (2.0–18.0). Management approach was heterogeneous. Overall, 79% of patients had a good clinical outcome. Mortality rate was 7% in the study population. chILD is rare in Australasia. This study demonstrates variation in the investigations and management of chILD cases across Australasia, however the general outcome is favorable. Further international collaboration will help finesse the understanding of these disorders.

Tài liệu tham khảo

Hime NJ, Zurynski Y, Fitzgerald D, Selvadurai H, Phu A, Deverell M, Elliott EJ, Jaffe A. Childhood interstitial lung disease: A systematic review. Pediatr Pulmonol. 2015;50:1383–92. Clement A, Nathan N, Epaud R, Fauroux B, Corvol H. Interstitial lung diseases in children. Orphanet J Rare Dis. 2010;5:22. Deterding RR, Brody AS, Hagood JS, Young LR. Round table discussion: children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 2010;23:91–6. Guillerman RP. Imaging of childhood interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 2010;23:43–68. Kurland G, Deterding RR, Hagood JS, Young LR, Brody AS, Castile RG, Dell S, Fan LL, Hamvas A, Hilman BC, Langston C, Nogee LM, Redding GJ. An Official American Thoracic Society Clinical Practice Guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188(3):376–94. Jaffe A, Zurynski Y, Beville L, Elliott E. Call for a national plan for rare diseases. J Paediatr Child Health. 2010;46:2–4. Hime NJ, Fitzgerald D, Robinson P, Selvadurai H, Asperen PV, Jaffé A, Zurynski Y. Childhood interstitial lung disease due to surfactant protein C deficiency: frequent use and costs of hospital services for a single case in Australia. Orphanet J Rare Dis. 2014;9:36. Dinwiddie R, Sharief N, Crawford O. Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and Ireland. Pediatr Pulmonol. 2002;34:23–9. Clement A, Allen J, Corrin B, Dinwiddie R, Ducou le Pointe H, Eber E, Laurent G, Marshall R, Midulla F, Nicholson AG, Pohunek P, Ratijen F, Spiteri M, de Blic J. Task force on chronic interstitial lung disease in immunocompetent children. Eur Respir J. 2004;24(4):686–97. Australian Bureau of Statistics, Australia, accessed 12 Nov 2016, <http://www.abs.gov.au/Ausstats/[email protected]/mf/3235.0> New Zealand Bureau of Statistics, New Zealand, accessed 12 Nov 2016, <http://m.stats.govt.nz/browse_for_stats/population.aspx>. Griese M, Haug M, Brasch F, Freihorst A, Lohse P, Kries RV, Zimmermann T, Hartl D. Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis. 2009;4:26. Fan LL, Kozinetz CA, Deterding RR, Brugman SM. Evaluation of a diagnostic approach to pediatric interstitial lung disease. Pediatrics. 1998;101:82–5. Andersen C, Ramsay JA, Nogee LM, Shah J, Wert SE, Paes B, Nowaczyk MJ. Recurrent familial neonatal deaths: hereditary surfactant protein B deficiency. Am J Perinatol. 2000;17(04):219–24. Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Bean JA, Brody AS, Nogee LM, Trapnell BC, Langston C. Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. 2007;176(11):1120–8. Fan LL, Kozinetz CA. Factors influencing survival in children with chronic interstitial lung disease. Am J Respir Crit Care Med. 1997;156:939–42. Kerby GS, Wagner BD, Popler J, Hay TC, Kopecky C, Wilcox SL, Quinones RR, Giller RH, Accurso FJ, Deterding RR. Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy. Pediatr Pulmonol. 2012;48:1008–15. Copley S, Bush A. Series: imaging. Paediatr Respir Rev. 2000;1:141–7. Long FR, Castile RG. Technique and clinical applications of full-inflation and end-exhalation controlled-ventilation chest CT in infants and young children. Pediatr Radiol. 2001;31:413–22. Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R, Hengst M, Kiper N, Nicholson AG, Wetzke M, Snijders D, Schwerk N, Griese M. European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax. 2015;70(11):1078–84. Nogee LM. Genetic basis of children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 2010;23(1):15–24. Soares JJ, Deutsch GH, Moore PE, Fazili MF, Austin ED, Brown RF, Sokolow AG, Hilmes MA, Young LR. Childhood interstitial lung diseases: an 18-year retrospective analysis. Pediatrics. 2013;132(4):684–91. Ayed AK, Raghunathan R. Thoracoscopy versus open lung biopsy in the diagnosis of interstitial lung disease: a randomised controlled trial. J R Coll Surg Edinb. 2000;45:159–63. O’reilly R, Kilner D, Ashworth M, Aurora P. Diffuse lung disease in infants less than 1 year of age: Histopathological diagnoses and clinical outcome. Pediatr Pulmonol. 2015;50:1000–8. Rauch D, Wetzke M, Reu S, Wesselak W, Schams A, Hengst M, Kammer B, Ley-Zaporozhan J, Kappler M, Proesmans M, Lange J, Escribano A, Kerem E, Ahrens F, Brasch F, Schwerk N, Griese M. Persistent tachypnea of infancy. Usual and aberrant. Am J Respir Crit Care Med. 2016;193:438–47. Griese M, Haug M, Hartl D, Teusch V, Glöckner-Pagel J, Brasch F. Hypersensitivity pneumonitis: lessons for diagnosis and treatment of a rare entity in children. Orphanet J Rare Dis. 2013;8:121. Buchvald F, Petersen BL, Damgaard K, Deterding R, Langston C, Fan LL, Deutsch GH, Dishop MK, Kristensen LA, Nielsen KG. Frequency, treatment, and functional outcome in children with hypersensitivity pneumonitis. Pediatr Pulmonol. 2011;46:1098–107. Fan LL. Hypersensitivity pneumonitis in children. Curr Opin Pediatr. 2002;14(3):323–6. doi:10.1097/00008480-200206000-00008. Dishop MK, Askin FB, Galambos C, White FV, Deterding RR, Young LR, Langston C. Children’s Interstitial Lung Disease (chILD) Network: classification of diffuse lung disease in older children and adolescents: a multi-institutional study of the Children’s Interstitial Lung Disease Network. Mod Pathol. 2007;20:287–8. Abstract.
Thông tin
Thông tin xuất bản

Orphanet Journal of Rare Diseases

Tập 12

1-9

Thông tin tác giả