Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management

D. Hacking, R. Smyth, N J Shaw, G. Kokia, H. Carty, D. Heaf

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

BACKGROUND Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. METHODS In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. RESULTS In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. CONCLUSION The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.
Original languageEnglish
Pages (from-to)152-157
JournalArchives of Disease in Childhood
Volume83
Issue number2
DOIs
Publication statusPublished - 2000

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Idiopathic Pulmonary Fibrosis
Hydroxychloroquine
Pulmonary Fibrosis
Prednisolone
Dyspnea
Thorax
Steroids
Tomography
Conservative Treatment
Parturition
Exercise
Oxygen
Biopsy
Lung
Mortality

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Hacking, D. ; Smyth, R. ; Shaw, N J ; Kokia, G. ; Carty, H. ; Heaf, D. / Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management. In: Archives of Disease in Childhood. 2000 ; Vol. 83, No. 2. pp. 152-157.
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Hacking, D, Smyth, R, Shaw, NJ, Kokia, G, Carty, H & Heaf, D 2000, 'Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management', Archives of Disease in Childhood, vol. 83, no. 2, pp. 152-157. https://doi.org/10.1136/adc.83.2.152

Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management. / Hacking, D.; Smyth, R.; Shaw, N J; Kokia, G.; Carty, H.; Heaf, D.

In: Archives of Disease in Childhood, Vol. 83, No. 2, 2000, p. 152-157.

Research output: Contribution to journalArticle

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AB - BACKGROUND Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. METHODS In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. RESULTS In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. CONCLUSION The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.

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