Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management
Identifieur interne : 001352 ( Istex/Checkpoint ); précédent : 001351; suivant : 001353Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management
Auteurs : Doug Hacking ; Rosalind Smyth ; Nigel Shaw ; George Kokia ; Helen Carty ; David HeafSource :
- Archives of Disease in Childhood [ 0003-9888 ] ; 2000-08-01.
English descriptors
- Teeft :
- Alveolar septa, Alveolitis, Avected children, Biopsy, Biopsy sample, Biopsy samples, Bronchoalveolar lavage, Brosing, Brosing alveolitis, Brosis, Case series, Centile, Centile height, Centile prednisolone, Centile prednisolone trial, Centile weight, Chest radiographs, Clinical presentation, Desquamative, European society, Family history, Fibrosing alveolitis, General anaesthesia, Good prognosis, Good response, Histological, Histological analysis, Home oxygen, Home oxygen exercise limitation, Idiopathic, Insuycient lung tissue, Interstitial, Interstitial disease, Interstitial lung, Interstitial lung disease, Interstitial shadowing, Lung biopsy, Lung disease, Lung function, Lymphocyte, Macrophage, Median, Median time, Months exercise limitation, Months years, Natural history, Nocturnal oxygen, Oral prednisolone, Oral steroids, Patient group, Pediatr, Pediatr pulmonol, Percutaneous, Percutaneous lung biopsy, Pneumonitis, Polymorphonuclear cells, Poor prognosis, Prednisolone, Previous reports, Prognosis, Pulmonary hypertension, Rapid responses, Respiratory symptoms, Royal liverpool, Small numbers, Steroid, Steroid response, Symptom, Tissue samples, Vital capacity, Weight gain, Year period, Years birth.
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.
Url:
DOI: 10.1136/adc.83.2.152
Affiliations:
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uniqKey="Heaf D" first="David" last="Heaf">David Heaf</name><affiliation><wicri:noCountry code="syntax">???</wicri:noCountry></affiliation></author></analytic><monogr></monogr><series><title level="j">Archives of Disease in Childhood</title><title level="j" type="abbrev">Arch Dis Child</title><idno type="ISSN">0003-9888</idno><idno type="eISSN">1468-2044</idno><imprint><publisher>BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health</publisher><date type="published" when="2000-08-01">2000-08-01</date><biblScope unit="volume">83</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="152">152</biblScope></imprint><idno type="ISSN">0003-9888</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0003-9888</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Alveolar septa</term><term>Alveolitis</term><term>Avected children</term><term>Biopsy</term><term>Biopsy sample</term><term>Biopsy samples</term><term>Bronchoalveolar lavage</term><term>Brosing</term><term>Brosing alveolitis</term><term>Brosis</term><term>Case series</term><term>Centile</term><term>Centile height</term><term>Centile prednisolone</term><term>Centile prednisolone trial</term><term>Centile weight</term><term>Chest radiographs</term><term>Clinical presentation</term><term>Desquamative</term><term>European society</term><term>Family history</term><term>Fibrosing alveolitis</term><term>General anaesthesia</term><term>Good prognosis</term><term>Good response</term><term>Histological</term><term>Histological analysis</term><term>Home oxygen</term><term>Home oxygen exercise limitation</term><term>Idiopathic</term><term>Insuycient lung tissue</term><term>Interstitial</term><term>Interstitial disease</term><term>Interstitial lung</term><term>Interstitial lung disease</term><term>Interstitial shadowing</term><term>Lung biopsy</term><term>Lung disease</term><term>Lung function</term><term>Lymphocyte</term><term>Macrophage</term><term>Median</term><term>Median time</term><term>Months exercise limitation</term><term>Months years</term><term>Natural history</term><term>Nocturnal oxygen</term><term>Oral prednisolone</term><term>Oral steroids</term><term>Patient group</term><term>Pediatr</term><term>Pediatr pulmonol</term><term>Percutaneous</term><term>Percutaneous lung biopsy</term><term>Pneumonitis</term><term>Polymorphonuclear cells</term><term>Poor prognosis</term><term>Prednisolone</term><term>Previous reports</term><term>Prognosis</term><term>Pulmonary hypertension</term><term>Rapid responses</term><term>Respiratory symptoms</term><term>Royal liverpool</term><term>Small numbers</term><term>Steroid</term><term>Steroid response</term><term>Symptom</term><term>Tissue samples</term><term>Vital capacity</term><term>Weight gain</term><term>Year period</term><term>Years birth</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="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.</div></front></TEI><affiliations><list></list><tree><noCountry><name sortKey="Carty, Helen" sort="Carty, Helen" uniqKey="Carty H" first="Helen" last="Carty">Helen Carty</name><name sortKey="Hacking, Doug" sort="Hacking, Doug" uniqKey="Hacking D" first="Doug" last="Hacking">Doug Hacking</name><name sortKey="Heaf, David" sort="Heaf, David" uniqKey="Heaf D" first="David" last="Heaf">David Heaf</name><name sortKey="Kokia, George" sort="Kokia, George" uniqKey="Kokia G" first="George" last="Kokia">George Kokia</name><name sortKey="Shaw, Nigel" sort="Shaw, Nigel" uniqKey="Shaw N" first="Nigel" last="Shaw">Nigel Shaw</name><name sortKey="Smyth, Rosalind" sort="Smyth, Rosalind" uniqKey="Smyth R" first="Rosalind" last="Smyth">Rosalind Smyth</name></noCountry></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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