Fibrosing alveolitis and desquamative interstitial pneumonitis
Identifieur interne : 002C84 ( Main/Exploration ); précédent : 002C83; suivant : 002C85Fibrosing alveolitis and desquamative interstitial pneumonitis
Auteurs : N. Sharief [Royaume-Uni] ; O. F. Crawford [Royaume-Uni] ; R. Dinwiddie [Royaume-Uni]Source :
- Pediatric Pulmonology [ 8755-6863 ] ; 1994-06.
Descripteurs français
- KwdFr :
- Chloroquine (pharmacologie), Chloroquine (usage thérapeutique), Enfant, Enfant d'âge préscolaire, Femelle, Fibrose pulmonaire (anatomopathologie), Fibrose pulmonaire (génétique), Fibrose pulmonaire (traitement médicamenteux), Humains, Mâle, Nourrisson, Nouveau-né, Prednisolone (usage thérapeutique), Pronostic, Études de suivi.
- MESH :
- anatomopathologie : Fibrose pulmonaire.
- génétique : Fibrose pulmonaire.
- pharmacologie : Chloroquine.
- traitement médicamenteux : Fibrose pulmonaire.
- usage thérapeutique : Chloroquine, Prednisolone.
- Enfant, Enfant d'âge préscolaire, Femelle, Humains, Mâle, Nourrisson, Nouveau-né, Pronostic, Études de suivi.
English descriptors
- KwdEn :
- MESH :
- chemical , pharmacology : Chloroquine.
- chemical , therapeutic use : Chloroquine, Prednisolone.
- drug therapy : Pulmonary Fibrosis.
- genetics : Pulmonary Fibrosis.
- pathology : Pulmonary Fibrosis.
- Teeft :
- Alveolitis, Antiinflammatory agents, Better prognosis, Better response, Biopsy, Chest radiograph, Chest radiography, Chest radiology, Child, Child, Preschool, Chloroquine, Congenital rubella, Cryptogenic fibrosing alveolitis, Desquamative, Dramatic response, Family history, Female, Fibrosing, Fibrosing alveolitis, Fifth patient, Follow-Up Studies, Good response, Goodman gilman, Ground glass appearance, Humans, Infant, Infant, Newborn, Interstitial, Interstitial fibrosis, Lung biopsy, Lung disease, Lung fields, Lung function, Lung function tests, Male, Natural history, Neutrophil mobility, Open lung biopsy, Other child, Other investigators, Pathological changes, Pediatr, Pediatr pulmonol, Pergamon press, Pharmacological basis, Pneumonitis, Poor progress, Poor response, Prednisolone, Prognosis, Pulmonary fibrosis, Rubella, Sick children, Small dose, Specific treatment, Sputum culture, Steroid, Steroid therapy, Viral immunofluorescence, Worse prognosis, Year period.
Abstract
We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FNDIP) over the last 16 years by a review of all cases referred to a tertiary referral center. There were 25 cases, 16 boys and 9 girls (mean age at onset, 2.3 years; range, 7 days to 11.6 years). In each case the diagnosis was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7 weeks to 15.1 years). Presenting features were tachypnea (19), cyanosis (15), cough (12), exertional dyspnea (7). recurrent chest infections ± wheezing (9), and clubbing (8). Four patients recovered without antiinflammatory medication. The others received specific treatment. Of 11 patients given only prednisolone, six improved, two did not, and three died despite treatment. Of five patients receiving only chloroquine, four responded. Five patients received both prednisolone and chloroquine; one died, two responded well. There was poor progress in the remaining two. Of the 10 patients receiving chloroquine six (60%) showed a good response. A younger presentation carried a worse prognosis, but chest radiology at presentation and outcome were not interrelated. Those with mild histological changes all survived, but severe desquamation or fibrosis at biopsy was not related to outcome. In four cases there was a family history (16%). Patients with FNDIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment. Pediatr Pulmonol. 1994; 17:359–365. © 1994 Wiley‐Liss, Inc.
Url:
DOI: 10.1002/ppul.1950170604
Affiliations:
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Le document en format XML
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<term>Female</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
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<term>Prednisolone (therapeutic use)</term>
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<term>Chloroquine (usage thérapeutique)</term>
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<term>Enfant d'âge préscolaire</term>
<term>Femelle</term>
<term>Fibrose pulmonaire (anatomopathologie)</term>
<term>Fibrose pulmonaire (génétique)</term>
<term>Fibrose pulmonaire (traitement médicamenteux)</term>
<term>Humains</term>
<term>Mâle</term>
<term>Nourrisson</term>
<term>Nouveau-né</term>
<term>Prednisolone (usage thérapeutique)</term>
<term>Pronostic</term>
<term>Études de suivi</term>
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<term>Prednisolone</term>
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<term>Better prognosis</term>
<term>Better response</term>
<term>Biopsy</term>
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<term>Chest radiography</term>
<term>Chest radiology</term>
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<term>Child, Preschool</term>
<term>Chloroquine</term>
<term>Congenital rubella</term>
<term>Cryptogenic fibrosing alveolitis</term>
<term>Desquamative</term>
<term>Dramatic response</term>
<term>Family history</term>
<term>Female</term>
<term>Fibrosing</term>
<term>Fibrosing alveolitis</term>
<term>Fifth patient</term>
<term>Follow-Up Studies</term>
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<term>Goodman gilman</term>
<term>Ground glass appearance</term>
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<term>Infant</term>
<term>Infant, Newborn</term>
<term>Interstitial</term>
<term>Interstitial fibrosis</term>
<term>Lung biopsy</term>
<term>Lung disease</term>
<term>Lung fields</term>
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<term>Lung function tests</term>
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<term>Natural history</term>
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<front><div type="abstract" xml:lang="en">We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FNDIP) over the last 16 years by a review of all cases referred to a tertiary referral center. There were 25 cases, 16 boys and 9 girls (mean age at onset, 2.3 years; range, 7 days to 11.6 years). In each case the diagnosis was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7 weeks to 15.1 years). Presenting features were tachypnea (19), cyanosis (15), cough (12), exertional dyspnea (7). recurrent chest infections ± wheezing (9), and clubbing (8). Four patients recovered without antiinflammatory medication. The others received specific treatment. Of 11 patients given only prednisolone, six improved, two did not, and three died despite treatment. Of five patients receiving only chloroquine, four responded. Five patients received both prednisolone and chloroquine; one died, two responded well. There was poor progress in the remaining two. Of the 10 patients receiving chloroquine six (60%) showed a good response. A younger presentation carried a worse prognosis, but chest radiology at presentation and outcome were not interrelated. Those with mild histological changes all survived, but severe desquamation or fibrosis at biopsy was not related to outcome. In four cases there was a family history (16%). Patients with FNDIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment. Pediatr Pulmonol. 1994; 17:359–365. © 1994 Wiley‐Liss, Inc.</div>
</front>
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