Abstract
A case of desquamative fibrosing alveolitis beginning in early infancy is described. The disease was characterized by tachypnoea, hypoxia relieved by O2, absence of signs in the chest or clubbing, and radiological and physiological evidence of hyperinflation. The diagnosis was made by needle biopsy of the lung. Treatment with steroids and immunosuppression was without effect and the infant died at 9 months. The disease has a high mortality in infancy, only 2 out of 9 reported cases having survived. The difference from the usual course in adults and older children is noted.
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- Brown C. H., Turner-Warwick M. The treatment of cryptogenic fibrosing alveolitis with immunosuppressant drugs. Q J Med. 1971 Apr;40(158):289–302. doi: 10.1093/oxfordjournals.qjmed.a067271. [DOI] [PubMed] [Google Scholar]
- FEINERMAN B., HARRIS L. E. Unusual interstitial pneumonitis; report of two cases occurring in children. Proc Staff Meet Mayo Clin. 1957 Oct 30;32(22):637–640. [PubMed] [Google Scholar]
- HILTON H. B., RENDLE-SHORT J. Diffuse progressive interstitial fibrosis of the lungs in childhood (Hamman-Rich syndrome). Arch Dis Child. 1961 Feb;36:102–106. doi: 10.1136/adc.36.185.102. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Howatt W. F., Heidelberger K. P., LeGlovan D. P., Schnitzer B. Desquamative interstitial pneumonia. Case report of an infant unresponsive to treatment. Am J Dis Child. 1973 Sep;126(3):346–348. doi: 10.1001/archpedi.1973.02110190304011. [DOI] [PubMed] [Google Scholar]
- LIEBOW A. A., STEER A., BILLINGSLEY J. G. DESQUAMATIVE INTERSTITIAL PNEUMONIA. Am J Med. 1965 Sep;39:369–404. doi: 10.1016/0002-9343(65)90206-8. [DOI] [PubMed] [Google Scholar]
- MANN T. P. Diffuse progressive interstitial fibrosis of lungs in infancy. Proc R Soc Med. 1959 Aug;52:638–639. [PubMed] [Google Scholar]
- Patchefsky A. S., Fraimow W., Hoch W. S. Desquamative interstitial pneumonia. Pathological findings and follow-up in 13 patients. Arch Intern Med. 1973 Aug;132(2):222–225. doi: 10.1001/archinte.132.2.222. [DOI] [PubMed] [Google Scholar]
- Scadding J. G., Hinson K. F. Diffuse fibrosing alveolitis (diffuse interstitial fibrosis of the lungs). Correlation of histology at biopsy with prognosis. Thorax. 1967 Jul;22(4):291–304. doi: 10.1136/thx.22.4.291. [DOI] [PMC free article] [PubMed] [Google Scholar]