Childhood Interstitial Lung Disease: Review on Diagnosis and Management
International Journal of Clinical and Experimental Medical Sciences
Volume 2, Issue 5, September 2016, Pages: 78-89
Received: Jul. 11, 2016; Accepted: Aug. 8, 2016; Published: Aug. 26, 2016
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Enas Ahmed Hamed, Physiology Department, Faculty of Medicine, Assiut University, Assiut, Egypt
Moustafa M. Elsaeid, Pediatric Department, Faculty of Medicine, Assiut University, Assiut, Egypt
Khaled Saad, Pediatric Department, Faculty of Medicine, Assiut University, Assiut, Egypt
Hazem Abo-Zeed, Department of Radiology, Faculty of Medicine, Assiut University, Assiut, Egypt
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Within the past few years, there is a rapid expansion in our understanding of childhood interstitial lung disease (chILD). chILD refers to a diverse group of rare chronic and complex respiratory disorders in children, mainly in infants under two years of age, which includes immunological and developmental abnormalities. These disorders involve the interstitium as well as the distal airspaces that result in restrictive lung physiology and significant impairment of gas exchange. chILD is clinically complex and associated with high morbidity and mortality. This review aimed to describe chILD classification, epidemiology, diagnostic approaches, morbidity, treatments, and the outcomes of chILD.
Childhood Interstitial Lung Disease, chILD Syndrome, Diffuse Lung Disease
To cite this article
Enas Ahmed Hamed, Moustafa M. Elsaeid, Khaled Saad, Hazem Abo-Zeed, Childhood Interstitial Lung Disease: Review on Diagnosis and Management, International Journal of Clinical and Experimental Medical Sciences. Vol. 2, No. 5, 2016, pp. 78-89. doi: 10.11648/j.ijcems.20160205.12
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Hime NJ, Zurynski Y, Fitzgerald D, et al. Childhood interstitial lung disease: A systematic review. Pediatr Pulmonol. 2015; 50:1383-1392.
Kurland G, Deterding RR, Hagood JS, et al. Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy. Am J Respir Crit Care Med. 2013; 188: 376–394.
Deutsch GH, Young LR, Deterding RR, et al. Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. 2007; 176:1120–1128.
Griese M, Haug M, Brasch F, et al. Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis. 2009; 4:26.
Nathan N, Taam RA, Epaud R, et al. A national internet–linked based database for pediatric interstitial lung diseases: the French network. Orphanet journal of rare diseases. 2012; 7:40.
Dinwiddie R, Sharief N, Crawford O. Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland. Pediatr Pulmonol. 2002; 34: 23–29.
Kornum JB, Christensen S, Grijota M, et al. The incidence of interstitial lung disease 1995–2005: a Danish nationwide population–based study. BMC Pulm Med. 2008; 8:24.
Clement A. ERS Task Force. Task Force on Chronic Interstitial Lung Disease in Immunocompetent Children. Eur Respir J. 2004; 24:686–697.
Clement A, Nathan N, Epaud R, et al. Interstitial lung diseases in children. Orphanet J Rare Dis. 2010; 5:22.
Lee E, Seo JH, Kim HY, et al. Two series of familial cases with unclassified interstitial pneumonia with fibrosis. Allergy Asthma Immunol Res. 2012; 4:240–244.
Clement A, Henrion–Caude A, Fauroux B. The pathogenesis of interstitial lung diseases in children. Paediatr Respir Rev. 2004; 5: 94–97.
Clement A, Eber E. Interstitial lung diseases in infants and children. Eur Respir J. 2008; 31:658–666.
Guillot L, Nathan N, Tabary O, et al. Alveolar epithelial cells: master regulators of lung homeostasis. Int J Biochem Cell Biol. 2013; 45:2568–2573.
Wuyts WA, Dooms C, Verleden GM. The clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2013; 187:777.
Camelo A, Dunmore R, Sleeman MA, et al. The epithelium in idiopathic pulmonary fibrosis: breaking the barrier. Front Pharmacol. 2014; 4:173.
Steele MP, Schwartz DA. Molecular mechanisms in progressive idiopathic pulmonary fibrosis. Annu Rev Med. 2013; 64:265–276.
Kropski JA, Lawson WE, Young LR, et al. Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. Dis Model Mech. 2013; 6:9–17.
Avital A, Hevroni A, Godfrey S, et al. Natural history of five children with surfactant protein C mutations and interstitial lung disease. Pediatr Pulmonol. 2014; 49:1097–1105.
Wambach JA, Casey AM, Fishman MP, et al. Genotype–phenotype correlations for infants and children with ABCA3 deficiency. Am J Respir Crit Care Med. 2014; 189:1538-1543.
El-Saied MM, Saad K, Hamed EA, et al. Plasma levels of transforming growth factor-B1 connective tissue growth factor; soluble factor related apoptosis and urinary levels of desmosine in childhood interstitial lung diseases. Assiut Med J. 2016:40:89-102.
Fan L, Langston C. Pediatric interstitial lung disease. Am J Respir Crit Care Med. 2002; 165:1466-1467.
Kuo CS, Young LR. Interstitial lung disease in children. Current opinion. 2014; 26:320–.327.
Nogee LM. Genetic Basis of Children’s Interstitial Lung Disease. Pediatr Allergy Immunol Pulmonol. 2010; 23:15-24.
Fauroux B, Epaud R, Clement A. Clinical presentation of interstitial lung disease in children. Paediatr Respir Rev. 2004; 5:98-100.
Fan LL, Kozinetz CA. Factors influencing survival in children with chronic interstitial lung disease. Am J Respir Crit Care Med. 1997; 156:939-942.
Thomeer M, Grutters JC, Wuyts WA, et al. Clinical use of biomarkers of survival in pulmonary fibrosis. Respir Res. 2010; 11:89.
Brody AS, Guillerman RP. Don‘t let radiation scare trump patient care: 10 ways you can harm your patients by fear of radiation-induced cancer from diagnostic imaging. Thorax. 2014; 69:782–784.
Bush A, Cunningham S, de Blic J, et al; chILD-EU collaboration EU collaboration. European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax. 2015; 70:1078–1084.
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: 413-422.
Long FR. High-resolution CT of the lungs in infants and young children. J Thorac Imaging. 2001;16: 251-258.
Brody AS. Imaging considerations: interstitial lung disease in children. Radiol Clin North Am. 2005;43:391–403.
De Santis M, Bosello S, La Torre G, et al. Functional, radiological and biological markers of alveolitis and infections of the lower respiratory tract in patients with systemic sclerosis. Respir Res. 2005; 6:96.
British Thoracic Society recommendation. The diagnosis, assessment and treatment of diffuse parenchymal lung disease in adults Thorax. 1999; 54:S1–14.
Wells AU, Hogaboam CM. Update in diffuse parenchymal lung disease 2007. Am J Respir Crit Care Med. 2008; 177:580-584.
Gay SE, Kazerooni EA, Toews GB, et al. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am J Respir Crit Care Med. 1998; 157: 1063–1072.
Thomeer MJ, Dehaes B, Mortelmans L, et al. Pertechnegas lung clearance in different forms of interstitial lung disease. Eur Respir J. 2002; 19: 31–36.
Meyer KC, Raghu G, Baughman RP, et al; American Thoracic Society Committee on BAL in Interstitial Lung Disease. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2012; 185:1004-1014.
Dishop MK. Diagnostic Pathology of Diffuse Lung Disease in Children. Pediatric Allergy. Immunology and Pulmonology. 2010; 23:69–85.
Nathan SD, Shlobin OA, Ahmad S, et al. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest. 2007; 131:657–663.
Soares JJ, Deutsch GH, Moore PE, et al. Childhood interstitial lung diseases: an 18–year retrospective analysis. Pediatrics. 2013; 132:684–691.
Bush A, Anthony G, Barbato A, et al, chILD collaborators. Research in progress: Put the orphanage out of business. Thorax. 2013; 68:971–973.
Lenney W, Boner AL, Bont L, et al. Medicines used in respiratory diseases only seen in children. Eur Respir J. 2009; 34:531-551.
Fan LL, Deterding RR, Langston C. Pediatric interstitial lung disease revisited. Pediatr Pulmonol. 2004; 38:369–378.
Dinwiddie R. The lung in multi–system disease. Paediatr Respir Rev. 2000; 1:58–63.
Florescu DF, Murphy PJ, Kalil AC. Effects of prolonged use of azithromycin in patients with cystic fibrosis: a meta-analysis. Pulm Pharmacol Ther. 2009; 22:467–472.
Knyazhitskiy A, Masson RG, Corkey R, et al. Benefi cial response to macrolide antibiotic in a patient with desquamative interstitial pneumonia refractory to corticosteroid therapy. Chest. 2008; 134:185–187.
Zimmermann GS, von Wulffen W, Huppmann P, et al. Haemodynamic changes in pulmonary hypertension in patients with interstitial lung disease treated with PDE–5 inhibitors. Respirology. 2014; 19:700–706.
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