Fibrosing alveolitis and desquamative interstitial pneumonitis.
Identifieur interne : 000527 ( PubMed/Corpus ); précédent : 000526; suivant : 000528Fibrosing alveolitis and desquamative interstitial pneumonitis.
Auteurs : N. Sharief ; O F Crawford ; R. DinwiddieSource :
- Pediatric pulmonology [ 8755-6863 ] ; 1994.
English descriptors
- KwdEn :
- MESH :
- chemical , pharmacology : Chloroquine.
- chemical , therapeutic use : Chloroquine, Prednisolone.
- drug therapy : Pulmonary Fibrosis.
- genetics : Pulmonary Fibrosis.
- pathology : Pulmonary Fibrosis.
- Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Prognosis.
Abstract
We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) 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). Presently 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 FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.
DOI: 10.1002/ppul.1950170604
PubMed: 8090606
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pubmed:8090606Le document en format XML
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Dinwiddie</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1994">1994</date><idno type="RBID">pubmed:8090606</idno><idno type="pmid">8090606</idno><idno type="doi">10.1002/ppul.1950170604</idno><idno type="wicri:Area/PubMed/Corpus">000527</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000527</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Fibrosing alveolitis and desquamative interstitial pneumonitis.</title><author><name sortKey="Sharief, N" sort="Sharief, N" uniqKey="Sharief N" first="N" last="Sharief">N. Sharief</name><affiliation><nlm:affiliation>Respiratory Unit, Hospital for Sick Children, London, UK.</nlm:affiliation></affiliation></author><author><name sortKey="Crawford, O F" sort="Crawford, O F" uniqKey="Crawford O" first="O F" last="Crawford">O F Crawford</name></author><author><name sortKey="Dinwiddie, R" sort="Dinwiddie, R" uniqKey="Dinwiddie R" first="R" last="Dinwiddie">R. Dinwiddie</name></author></analytic><series><title level="j">Pediatric pulmonology</title><idno type="ISSN">8755-6863</idno><imprint><date when="1994" type="published">1994</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Child</term><term>Child, Preschool</term><term>Chloroquine (pharmacology)</term><term>Chloroquine (therapeutic use)</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Infant</term><term>Infant, Newborn</term><term>Male</term><term>Prednisolone (therapeutic use)</term><term>Prognosis</term><term>Pulmonary Fibrosis (drug therapy)</term><term>Pulmonary Fibrosis (genetics)</term><term>Pulmonary Fibrosis (pathology)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="pharmacology" xml:lang="en"><term>Chloroquine</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Chloroquine</term><term>Prednisolone</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Pulmonary Fibrosis</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Pulmonary Fibrosis</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Pulmonary Fibrosis</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Infant</term><term>Infant, Newborn</term><term>Male</term><term>Prognosis</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) 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). Presently 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 FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">8090606</PMID><DateCompleted><Year>1994</Year><Month>10</Month><Day>18</Day></DateCompleted><DateRevised><Year>2019</Year><Month>09</Month><Day>14</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">8755-6863</ISSN><JournalIssue CitedMedium="Print"><Volume>17</Volume><Issue>6</Issue><PubDate><Year>1994</Year><Month>Jun</Month></PubDate></JournalIssue><Title>Pediatric pulmonology</Title><ISOAbbreviation>Pediatr. Pulmonol.</ISOAbbreviation></Journal><ArticleTitle>Fibrosing alveolitis and desquamative interstitial pneumonitis.</ArticleTitle><Pagination><MedlinePgn>359-65</MedlinePgn></Pagination><Abstract><AbstractText>We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) 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). Presently 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 FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Sharief</LastName><ForeName>N</ForeName><Initials>N</Initials><AffiliationInfo><Affiliation>Respiratory Unit, Hospital for Sick Children, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Crawford</LastName><ForeName>O F</ForeName><Initials>OF</Initials></Author><Author ValidYN="Y"><LastName>Dinwiddie</LastName><ForeName>R</ForeName><Initials>R</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Pediatr Pulmonol</MedlineTA><NlmUniqueID>8510590</NlmUniqueID><ISSNLinking>1099-0496</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>886U3H6UFF</RegistryNumber><NameOfSubstance UI="D002738">Chloroquine</NameOfSubstance></Chemical><Chemical><RegistryNumber>9PHQ9Y1OLM</RegistryNumber><NameOfSubstance UI="D011239">Prednisolone</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D002648" MajorTopicYN="N">Child</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002675" MajorTopicYN="N">Child, Preschool</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002738" MajorTopicYN="N">Chloroquine</DescriptorName><QualifierName UI="Q000494" MajorTopicYN="N">pharmacology</QualifierName><QualifierName UI="Q000627" MajorTopicYN="Y">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005500" MajorTopicYN="N">Follow-Up Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007223" MajorTopicYN="N">Infant</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007231" MajorTopicYN="N">Infant, Newborn</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011239" MajorTopicYN="N">Prednisolone</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="Y">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D011379" MajorTopicYN="N">Prognosis</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011658" MajorTopicYN="N">Pulmonary Fibrosis</DescriptorName><QualifierName UI="Q000188" MajorTopicYN="Y">drug therapy</QualifierName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1994</Year><Month>6</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1994</Year><Month>6</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1994</Year><Month>6</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">8090606</ArticleId><ArticleId IdType="doi">10.1002/ppul.1950170604</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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