Cause of death in Wilson disease
Identifieur interne : 002C23 ( Istex/Corpus ); précédent : 002C22; suivant : 002C24Cause of death in Wilson disease
Auteurs : John M. WalsheSource :
- Movement Disorders [ 0885-3185 ] ; 2007-11-15.
English descriptors
- KwdEn :
Abstract
Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21693
Links to Exploration step
ISTEX:53D3E948962FB32A64EAB696CB48519EC165FC9BLe document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Cause of death in Wilson disease</title>
<author><name sortKey="Walshe, John M" sort="Walshe, John M" uniqKey="Walshe J" first="John M." last="Walshe">John M. Walshe</name>
<affiliation><mods:affiliation>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</mods:affiliation>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:53D3E948962FB32A64EAB696CB48519EC165FC9B</idno>
<date when="2007" year="2007">2007</date>
<idno type="doi">10.1002/mds.21693</idno>
<idno type="url">https://api.istex.fr/document/53D3E948962FB32A64EAB696CB48519EC165FC9B/fulltext/pdf</idno>
<idno type="wicri:Area/Istex/Corpus">002C23</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Cause of death in Wilson disease</title>
<author><name sortKey="Walshe, John M" sort="Walshe, John M" uniqKey="Walshe J" first="John M." last="Walshe">John M. Walshe</name>
<affiliation><mods:affiliation>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</mods:affiliation>
</affiliation>
</author>
</analytic>
<monogr></monogr>
<series><title level="j">Movement Disorders</title>
<title level="j" type="sub">Official Journal of the Movement Disorder Society</title>
<title level="j" type="abbrev">Mov. Disord.</title>
<idno type="ISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<date type="published" when="2007-11-15">2007-11-15</date>
<biblScope unit="vol">22</biblScope>
<biblScope unit="issue">15</biblScope>
<biblScope unit="page" from="2216">2216</biblScope>
<biblScope unit="page" to="2220">2220</biblScope>
</imprint>
<idno type="ISSN">0885-3185</idno>
</series>
<idno type="istex">53D3E948962FB32A64EAB696CB48519EC165FC9B</idno>
<idno type="DOI">10.1002/mds.21693</idno>
<idno type="ArticleID">MDS21693</idno>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Caeruloplasmin</term>
<term>late diagnosis</term>
<term>malignancy</term>
<term>other causes</term>
<term>serum copper</term>
</keywords>
</textClass>
<langUsage><language ident="en">en</language>
</langUsage>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society</div>
</front>
</TEI>
<istex><corpusName>wiley</corpusName>
<author><json:item><name>John M. Walshe MD, ScD, FRCP</name>
<affiliations><json:string>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</json:string>
</affiliations>
</json:item>
</author>
<subject><json:item><lang><json:string>eng</json:string>
</lang>
<value>Caeruloplasmin</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>serum copper</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>late diagnosis</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>malignancy</value>
</json:item>
<json:item><lang><json:string>eng</json:string>
</lang>
<value>other causes</value>
</json:item>
</subject>
<language><json:string>eng</json:string>
</language>
<abstract>Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society</abstract>
<qualityIndicators><score>4.489</score>
<pdfVersion>1.3</pdfVersion>
<pdfPageSize>594 x 792 pts</pdfPageSize>
<refBibsNative>true</refBibsNative>
<abstractCharCount>986</abstractCharCount>
<pdfWordCount>2689</pdfWordCount>
<pdfCharCount>16027</pdfCharCount>
<pdfPageCount>5</pdfPageCount>
<abstractWordCount>150</abstractWordCount>
</qualityIndicators>
<title>Cause of death in Wilson disease</title>
<genre><json:string>Serial article</json:string>
</genre>
<host><volume>22</volume>
<pages><total>5</total>
<last>2220</last>
<first>2216</first>
</pages>
<issn><json:string>0885-3185</json:string>
</issn>
<issue>15</issue>
<subject><json:item><value>Research Article</value>
</json:item>
</subject>
<genre></genre>
<language><json:string>unknown</json:string>
</language>
<title>Movement Disorders</title>
<doi><json:string>10.1002/(ISSN)1531-8257</json:string>
</doi>
</host>
<publicationDate>2007</publicationDate>
<copyrightDate>2007</copyrightDate>
<doi><json:string>10.1002/mds.21693</json:string>
</doi>
<id>53D3E948962FB32A64EAB696CB48519EC165FC9B</id>
<fulltext><json:item><original>true</original>
<mimetype>application/pdf</mimetype>
<extension>pdf</extension>
<uri>https://api.istex.fr/document/53D3E948962FB32A64EAB696CB48519EC165FC9B/fulltext/pdf</uri>
</json:item>
<json:item><original>false</original>
<mimetype>application/zip</mimetype>
<extension>zip</extension>
<uri>https://api.istex.fr/document/53D3E948962FB32A64EAB696CB48519EC165FC9B/fulltext/zip</uri>
</json:item>
<istex:fulltextTEI uri="https://api.istex.fr/document/53D3E948962FB32A64EAB696CB48519EC165FC9B/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Cause of death in Wilson disease</title>
</titleStmt>
<publicationStmt><authority>ISTEX</authority>
<publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<availability><p>Wiley Subscription Services, Inc., A Wiley Company</p>
</availability>
<date>2007</date>
</publicationStmt>
<sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Cause of death in Wilson disease</title>
<author><persName><forename type="first">John M.</forename>
<surname>Walshe</surname>
<roleName type="degree">MD, ScD, FRCP</roleName>
</persName>
<note type="correspondence"><p>Correspondence: Broom Lodge, 58 High Street, Hemingford Grey, Huntingdon, PE28 9BN, United Kingdom</p>
</note>
<affiliation>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</affiliation>
</author>
</analytic>
<monogr><title level="j">Movement Disorders</title>
<title level="j" type="sub">Official Journal of the Movement Disorder Society</title>
<title level="j" type="abbrev">Mov. Disord.</title>
<idno type="pISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<idno type="DOI">10.1002/(ISSN)1531-8257</idno>
<imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<pubPlace>Hoboken</pubPlace>
<date type="published" when="2007-11-15"></date>
<biblScope unit="vol">22</biblScope>
<biblScope unit="issue">15</biblScope>
<biblScope unit="page" from="2216">2216</biblScope>
<biblScope unit="page" to="2220">2220</biblScope>
</imprint>
</monogr>
<idno type="istex">53D3E948962FB32A64EAB696CB48519EC165FC9B</idno>
<idno type="DOI">10.1002/mds.21693</idno>
<idno type="ArticleID">MDS21693</idno>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><creation><date>2007</date>
</creation>
<langUsage><language ident="en">en</language>
</langUsage>
<abstract xml:lang="en"><p>Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society</p>
</abstract>
<textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head>
<item><term>Caeruloplasmin</term>
</item>
<item><term>serum copper</term>
</item>
<item><term>late diagnosis</term>
</item>
<item><term>malignancy</term>
</item>
<item><term>other causes</term>
</item>
</list>
</keywords>
</textClass>
<textClass><keywords scheme="Journal Subject"><list><head>Article category</head>
<item><term>Research Article</term>
</item>
</list>
</keywords>
</textClass>
</profileDesc>
<revisionDesc><change when="2007-04-05">Received</change>
<change when="2007-07-07">Registration</change>
<change when="2007-11-15">Published</change>
</revisionDesc>
</teiHeader>
</istex:fulltextTEI>
<json:item><original>false</original>
<mimetype>text/plain</mimetype>
<extension>txt</extension>
<uri>https://api.istex.fr/document/53D3E948962FB32A64EAB696CB48519EC165FC9B/fulltext/txt</uri>
</json:item>
</fulltext>
<metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration>
<istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName>
<publisherLoc>Hoboken</publisherLoc>
</publisherInfo>
<doi registered="yes">10.1002/(ISSN)1531-8257</doi>
<issn type="print">0885-3185</issn>
<issn type="electronic">1531-8257</issn>
<idGroup><id type="product" value="MDS"></id>
</idGroup>
<titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title>
<title type="subtitle">Official Journal of the Movement Disorder Society</title>
<title type="short">Mov. Disord.</title>
</titleGroup>
</publicationMeta>
<publicationMeta level="part" position="150"><doi origin="wiley" registered="yes">10.1002/mds.v22:15</doi>
<numberingGroup><numbering type="journalVolume" number="22">22</numbering>
<numbering type="journalIssue">15</numbering>
</numberingGroup>
<coverDate startDate="2007-11-15">15 November 2007</coverDate>
</publicationMeta>
<publicationMeta level="unit" type="article" position="120" status="forIssue"><doi origin="wiley" registered="yes">10.1002/mds.21693</doi>
<idGroup><id type="unit" value="MDS21693"></id>
</idGroup>
<countGroup><count type="pageTotal" number="5"></count>
</countGroup>
<titleGroup><title type="articleCategory">Research Article</title>
<title type="tocHeading1">Research Articles</title>
</titleGroup>
<copyright ownership="thirdParty">Copyright © 2007 Movement Disorder Society</copyright>
<eventGroup><event type="manuscriptReceived" date="2007-04-05"></event>
<event type="manuscriptRevised" date="2007-06-20"></event>
<event type="manuscriptAccepted" date="2007-07-07"></event>
<event type="publishedOnlineEarlyUnpaginated" date="2007-08-21"></event>
<event type="firstOnline" date="2007-08-21"></event>
<event type="publishedOnlineFinalForm" date="2007-11-27"></event>
<event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.3.6 mode:FullText source:FullText result:FullText" date="2010-04-21"></event>
<event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-02-02"></event>
<event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-31"></event>
</eventGroup>
<numberingGroup><numbering type="pageFirst">2216</numbering>
<numbering type="pageLast">2220</numbering>
</numberingGroup>
<correspondenceTo>Broom Lodge, 58 High Street, Hemingford Grey, Huntingdon, PE28 9BN, United Kingdom</correspondenceTo>
<linkGroup><link type="toTypesetVersion" href="file:MDS.MDS21693.pdf"></link>
</linkGroup>
</publicationMeta>
<contentMeta><countGroup><count type="figureTotal" number="0"></count>
<count type="tableTotal" number="2"></count>
<count type="referenceTotal" number="13"></count>
<count type="wordTotal" number="3174"></count>
</countGroup>
<titleGroup><title type="main" xml:lang="en">Cause of death in Wilson disease</title>
<title type="short" xml:lang="en">Cause of Death in Wilson Disease</title>
</titleGroup>
<creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>John M.</givenNames>
<familyName>Walshe</familyName>
<degrees>MD, ScD, FRCP</degrees>
</personName>
<contactDetails><email>penicillamine@waitrose.com</email>
</contactDetails>
</creator>
</creators>
<affiliationGroup><affiliation xml:id="af1" countryCode="GB" type="organization"><unparsedAffiliation>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</unparsedAffiliation>
</affiliation>
</affiliationGroup>
<keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">Caeruloplasmin</keyword>
<keyword xml:id="kwd2">serum copper</keyword>
<keyword xml:id="kwd3">late diagnosis</keyword>
<keyword xml:id="kwd4">malignancy</keyword>
<keyword xml:id="kwd5">other causes</keyword>
</keywordGroup>
<abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title>
<p>Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society</p>
</abstract>
</abstractGroup>
</contentMeta>
</header>
</component>
</istex:document>
</istex:metadataXml>
<!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Cause of death in Wilson disease</title>
</titleInfo>
<titleInfo type="abbreviated" lang="en"><title>Cause of Death in Wilson Disease</title>
</titleInfo>
<titleInfo type="alternative" contentType="CDATA" lang="en"><title>Cause of death in Wilson disease</title>
</titleInfo>
<name type="personal"><namePart type="given">John M.</namePart>
<namePart type="family">Walshe</namePart>
<namePart type="termsOfAddress">MD, ScD, FRCP</namePart>
<affiliation>Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London</affiliation>
<description>Correspondence: Broom Lodge, 58 High Street, Hemingford Grey, Huntingdon, PE28 9BN, United Kingdom</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<typeOfResource>text</typeOfResource>
<genre authority="originalCategForm">article</genre>
<originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher>
<place><placeTerm type="text">Hoboken</placeTerm>
</place>
<dateIssued encoding="w3cdtf">2007-11-15</dateIssued>
<dateCaptured encoding="w3cdtf">2007-04-05</dateCaptured>
<dateValid encoding="w3cdtf">2007-07-07</dateValid>
<copyrightDate encoding="w3cdtf">2007</copyrightDate>
</originInfo>
<language><languageTerm type="code" authority="rfc3066">en</languageTerm>
<languageTerm type="code" authority="iso639-2b">eng</languageTerm>
</language>
<physicalDescription><internetMediaType>text/html</internetMediaType>
<extent unit="tables">2</extent>
<extent unit="references">13</extent>
<extent unit="words">3174</extent>
</physicalDescription>
<abstract lang="en">Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society</abstract>
<subject lang="en"><genre>Keywords</genre>
<topic>Caeruloplasmin</topic>
<topic>serum copper</topic>
<topic>late diagnosis</topic>
<topic>malignancy</topic>
<topic>other causes</topic>
</subject>
<relatedItem type="host"><titleInfo><title>Movement Disorders</title>
<subTitle>Official Journal of the Movement Disorder Society</subTitle>
</titleInfo>
<titleInfo type="abbreviated"><title>Mov. Disord.</title>
</titleInfo>
<subject><genre>article category</genre>
<topic>Research Article</topic>
</subject>
<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part><date>2007</date>
<detail type="volume"><caption>vol.</caption>
<number>22</number>
</detail>
<detail type="issue"><caption>no.</caption>
<number>15</number>
</detail>
<extent unit="pages"><start>2216</start>
<end>2220</end>
<total>5</total>
</extent>
</part>
</relatedItem>
<identifier type="istex">53D3E948962FB32A64EAB696CB48519EC165FC9B</identifier>
<identifier type="DOI">10.1002/mds.21693</identifier>
<identifier type="ArticleID">MDS21693</identifier>
<accessCondition type="use and reproduction" contentType="copyright">Copyright © 2007 Movement Disorder Society</accessCondition>
<recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin>
<recordContentSource>WILEY</recordContentSource>
</recordInfo>
</mods>
</metadata>
<serie></serie>
</istex>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/Istex/Corpus
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 002C23 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Istex/Corpus/biblio.hfd -nk 002C23 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Santé |area= MovDisordV3 |flux= Istex |étape= Corpus |type= RBID |clé= ISTEX:53D3E948962FB32A64EAB696CB48519EC165FC9B |texte= Cause of death in Wilson disease }}
![]() | This area was generated with Dilib version V0.6.23. | ![]() |