Hamman–Rich syndrome in a young child
https://doi.org/10.21508/1027-4065-2019-64-4-83-87
Abstract
Hamman–Rich syndrome (idiopathic fibrosing alveolitis) is a lung disease of unknown nature, characterized by non-infectious inflammation of the interstitium, alveoli and terminal bronchioles with an outcome in progressive pulmonary fibrosis. Idiopathic fibrosing alveolitis in young children, despite the timely verification of the diagnosis and therapy, is characterized by an unfavorable prognosis and rapidly progressive course. The article describes the clinical case of Hamman–Rich syndrome in a child of 8 months, its main clinical manifestations and diagnostic features.
About the Authors
T. A. Minenkova
Kursk State Medical University
Russian Federation
Kursk
Russian Federation
Kursk
Yu. L. Mizernitsky
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
N. S. Razinkova
Kursk State Medical University
Russian Federation
Kursk
Russian Federation
Kursk
A. V. Serezhkina
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
M. V. Kostyuchenko
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
References
1. Lev N.S., Shmelev Е.I. Idiopathic fibrosing alveolitis. In the book: Chronic lung diseases in children. N.N. Rozinova., Yu.L. Mizernitskiy (eds). Moscow: Praktika, 2011; 140–144. (in Russ.).
2. Lev N.S., Rozinova N.N., Shmelev Е.I. Interstitial lung disease in children. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 2014; 59(1): 15–22. (in Russ.).
3. Shmelev Е.I. Idiopathic fibrosing alveolitis. Atmosfera. Pul’monologiya i allergologiya 2004; 1: 3–8. (in Russ.).
4. Collard H.R., Moore B.B., Flaherty K.R., Brown K.K., Kaner R.J., King T.E. Jr. et al. Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 636–643. DOI: 10.1055/s-2006-957337
5. Raghu G., Collard H.R., Egan J.J., Martinez F.J., Behr J., Brown K.K. et al. ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis; evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788–824. DOI:10.1164/rccm.201506- 1063ST
6. Behr J., Kreuter M., Hoeper M.M., Wirtz H., Klotsche J., Koschel D. et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS IPF registry. Eur Respir J 2015; 46: 186–196. DOI: 10.1183/09031936.00217614
7. Oldham J.M., Ma S-F., Martinez F.J., Anstrom K.J., Raghu G., Schwartz D.A. et al. TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idio-pathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2015; 192: 1475–1482. DOI: 10.1164/rccm.201505-1010OC
8. Fingerlin T.E., Murphy E., Zhang W., Peljto A.L., Brown K.K., Steele M.P. et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet 2015; 45(6): 613–620. DOI: 10.1038/ng.2609.
9. Noth I., Zhang Y., Ma S.F., Carlos F., Mathew B., Yong H.M.S. et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genomewide association study. Lancet Respir Med 2013;1(4):309–317. DOI: 10.1016/S2213-2600(13)70045-6
10. Ferreira A., Garvey C., Connors G.L., Hilling L., Rigler J., Farrell S., Cayou C., Shariat C., Collard H.R. Rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009; 135: 442–447. DOI: 10.1378/chest.08-1458
11. Rosas I.O., Richards T.J., Konishi K., Zhang Y., Gibson K., Lokshin A.E. et al. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008; 5: 93. DOI: 10.1371/journal.pmed.0050093
12. King T.E.Jr., Bradford W.Z., Castro-Bernardini S., Fagan E.A., Glaspole I., Glassberg M.K. et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083–2092. DOI: 10.1136/thoraxjnl-2015-207011
13. Ryu J.H., Moua T., Daniels C.E., Hartman T.E., Yi E.S., Utz J.P., Limper A.H. Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clin Proc 2014; 89: 1130–1142. DOI: 10.1016/j.mayocp.2014.03.016
14. Avdeeva O.Е., Avdeev S.N. Idiopathic fibrosing alveolitis: modern approaches to diagnosis and therapy. Consilium medicum 2002; 4(4): 195–201. (in Russ.).
Review
For citations:
Minenkova T.A., Mizernitsky Yu.L., Razinkova N.S., Serezhkina A.V., Kostyuchenko M.V. Hamman–Rich syndrome in a young child. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2019;64(4):83-87. (In Russ.) https://doi.org/10.21508/1027-4065-2019-64-4-83-87
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