ChloroquineV1, PubMed, Corpus, bibRecord, 000237

A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.

Identifieur interne : 000237 ( PubMed/Corpus ); précédent : 000236; suivant : 000238

A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.

Auteurs : Chana I C. Chin ; Shirleen Loloyan Kohn ; Thomas G. Keens ; Monique F. Margetis ; Roberta M. Kato

Source :

Orphanet journal of rare diseases [ 1750-1172 ] ; 2015.

RBID : pubmed:26289251

English descriptors

KwdEn :
- Hemosiderosis (therapy), Humans, Lung Diseases (therapy), Physicians, Practice Patterns, Physicians', Surveys and Questionnaires.
MESH :
- therapy : Hemosiderosis, Lung Diseases.
- Humans, Physicians, Practice Patterns, Physicians', Surveys and Questionnaires.

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death.

DOI: 10.1186/s13023-015-0319-5
PubMed: 26289251

Links to Exploration step

pubmed:26289251

Le document en format XML

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<author><name sortKey="Chin, Chana I C" sort="Chin, Chana I C" uniqKey="Chin C" first="Chana I C" last="Chin">Chana I C. Chin</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. chana.chantaw@gmail.com.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Kohn, Shirleen Loloyan" sort="Kohn, Shirleen Loloyan" uniqKey="Kohn S" first="Shirleen Loloyan" last="Kohn">Shirleen Loloyan Kohn</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. sloloyan@chla.usc.edu.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Keens, Thomas G" sort="Keens, Thomas G" uniqKey="Keens T" first="Thomas G" last="Keens">Thomas G. Keens</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Margetis, Monique F" sort="Margetis, Monique F" uniqKey="Margetis M" first="Monique F" last="Margetis">Monique F. Margetis</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Kato, Roberta M" sort="Kato, Roberta M" uniqKey="Kato R" first="Roberta M" last="Kato">Roberta M. Kato</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. rkato@chla.usc.edu.</nlm:affiliation>
</affiliation>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.</title>
<author><name sortKey="Chin, Chana I C" sort="Chin, Chana I C" uniqKey="Chin C" first="Chana I C" last="Chin">Chana I C. Chin</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. chana.chantaw@gmail.com.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Kohn, Shirleen Loloyan" sort="Kohn, Shirleen Loloyan" uniqKey="Kohn S" first="Shirleen Loloyan" last="Kohn">Shirleen Loloyan Kohn</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. sloloyan@chla.usc.edu.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Keens, Thomas G" sort="Keens, Thomas G" uniqKey="Keens T" first="Thomas G" last="Keens">Thomas G. Keens</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Margetis, Monique F" sort="Margetis, Monique F" uniqKey="Margetis M" first="Monique F" last="Margetis">Monique F. Margetis</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Kato, Roberta M" sort="Kato, Roberta M" uniqKey="Kato R" first="Roberta M" last="Kato">Roberta M. Kato</name>
<affiliation><nlm:affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. rkato@chla.usc.edu.</nlm:affiliation>
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<series><title level="j">Orphanet journal of rare diseases</title>
<idno type="eISSN">1750-1172</idno>
<imprint><date when="2015" type="published">2015</date>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Hemosiderosis (therapy)</term>
<term>Humans</term>
<term>Lung Diseases (therapy)</term>
<term>Physicians</term>
<term>Practice Patterns, Physicians'</term>
<term>Surveys and Questionnaires</term>
</keywords>
<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Hemosiderosis</term>
<term>Lung Diseases</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Humans</term>
<term>Physicians</term>
<term>Practice Patterns, Physicians'</term>
<term>Surveys and Questionnaires</term>
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<front><div type="abstract" xml:lang="en">Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death.</div>
</front>
</TEI>
<pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">26289251</PMID>
<DateCompleted><Year>2016</Year>
<Month>04</Month>
<Day>25</Day>
</DateCompleted>
<DateRevised><Year>2018</Year>
<Month>11</Month>
<Day>13</Day>
</DateRevised>
<Article PubModel="Electronic"><Journal><ISSN IssnType="Electronic">1750-1172</ISSN>
<JournalIssue CitedMedium="Internet"><Volume>10</Volume>
<PubDate><Year>2015</Year>
<Month>Aug</Month>
<Day>20</Day>
</PubDate>
</JournalIssue>
<Title>Orphanet journal of rare diseases</Title>
<ISOAbbreviation>Orphanet J Rare Dis</ISOAbbreviation>
</Journal>
<ArticleTitle>A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.</ArticleTitle>
<Pagination><MedlinePgn>98</MedlinePgn>
</Pagination>
<ELocationID EIdType="doi" ValidYN="Y">10.1186/s13023-015-0319-5</ELocationID>
<Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death.</AbstractText>
<AbstractText Label="METHODS" NlmCategory="METHODS">We surveyed physicians who care for patients with IPH via a web-based survey to assess the most common practices. 88 providers responded, caring for 274 IPH patients from five continents.</AbstractText>
<AbstractText Label="RESULTS" NlmCategory="RESULTS">63.3 % of respondents had patients that were initially misdiagnosed with anemia (60.0 %) or gastrointestinal bleed (18.2 %). Respondents varied in diagnostic tools used for evaluation. The key difference was in the use of lung biopsy (51.9 %) for diagnosis. Common medications respondents used for treatment at initial presentation and chronic maintenance therapy were corticosteroids (98.7 and 84.0 %, initial and chronic therapy respectively), hydroxychloroquine (33.3 and 64.0 %), azathioprine (8.0 and 37.3 %), and cyclophosphamide (4.0 and 16.0 %). There was agreement on the use of corticosteroids for exacerbation amongst all respondents. Reported deaths before adulthood occurred in 7.3 % of patients. We conclude that there were common features and specific variations in physician management of IPH. Respondents were divided on whether to perform lung biopsy for diagnosis.</AbstractText>
<AbstractText Label="CONCLUSION" NlmCategory="CONCLUSIONS">Despite the availability of various immunomodulators, corticosteroids remained the primary therapy. We speculate that the standardization of care for diffuse alveolar hemorrhage will improve patient outcomes.</AbstractText>
</Abstract>
<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Chin</LastName>
<ForeName>Chana I C</ForeName>
<Initials>CI</Initials>
<AffiliationInfo><Affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. chana.chantaw@gmail.com.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Kohn</LastName>
<ForeName>Shirleen Loloyan</ForeName>
<Initials>SL</Initials>
<AffiliationInfo><Affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. sloloyan@chla.usc.edu.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Keens</LastName>
<ForeName>Thomas G</ForeName>
<Initials>TG</Initials>
<AffiliationInfo><Affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Margetis</LastName>
<ForeName>Monique F</ForeName>
<Initials>MF</Initials>
<AffiliationInfo><Affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Kato</LastName>
<ForeName>Roberta M</ForeName>
<Initials>RM</Initials>
<AffiliationInfo><Affiliation>Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. rkato@chla.usc.edu.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California. rkato@chla.usc.edu.</Affiliation>
</AffiliationInfo>
</Author>
</AuthorList>
<Language>eng</Language>
<PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType>
</PublicationTypeList>
<ArticleDate DateType="Electronic"><Year>2015</Year>
<Month>08</Month>
<Day>20</Day>
</ArticleDate>
</Article>
<MedlineJournalInfo><Country>England</Country>
<MedlineTA>Orphanet J Rare Dis</MedlineTA>
<NlmUniqueID>101266602</NlmUniqueID>
<ISSNLinking>1750-1172</ISSNLinking>
</MedlineJournalInfo>
<SupplMeshList><SupplMeshName Type="Disease" UI="C536281">Idiopathic pulmonary hemosiderosis</SupplMeshName>
</SupplMeshList>
<CitationSubset>IM</CitationSubset>
<MeshHeadingList><MeshHeading><DescriptorName UI="D006486" MajorTopicYN="N">Hemosiderosis</DescriptorName>
<QualifierName UI="Q000628" MajorTopicYN="Y">therapy</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008171" MajorTopicYN="N">Lung Diseases</DescriptorName>
<QualifierName UI="Q000628" MajorTopicYN="Y">therapy</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D010820" MajorTopicYN="Y">Physicians</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D010818" MajorTopicYN="Y">Practice Patterns, Physicians'</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D011795" MajorTopicYN="Y">Surveys and Questionnaires</DescriptorName>
</MeshHeading>
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<Month>04</Month>
<Day>14</Day>
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A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.

A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.

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