Fabry disease and the skin : data from FOS, the Fabry outcome survey
Identifieur interne : 000473 ( PascalFrancis/Checkpoint ); précédent : 000472; suivant : 000474Fabry disease and the skin : data from FOS, the Fabry outcome survey
Auteurs : C. H. Orteu [Royaume-Uni] ; T. Jansen [Allemagne] ; O. Lidove [France] ; R. Jaussaud [France] ; D. A. Hushes [Royaume-Uni] ; G. Pintos-Morell [Espagne] ; U. Ramaswami [Royaume-Uni] ; R. Parini [Italie] ; G. Sunder-Plassman [Autriche] ; M. Beck [Allemagne] ; A. B. Mehta [Royaume-Uni]Source :
- British journal of dermatology : (1951) [ 0007-0963 ] ; 2007.
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Abstract
Background Fabry disease (also known as Anderson-Fabry disease) is a rare, X-linked lysosomal storage disorder that is characterized by accumulation of globotriaosylceramide throughout a range of tissues in the body. Objectives To ascertain the prevalence and nature of cutaneous manifestations in patients with Fabry disease and to relate these to the severity of systemic manifestations of the disease. Methods We have documented the dermatological features of this disease with reference to data from 714 patients (345 males, 369 females) registered on the Fabry Outcome Survey (FOS), a multicentre European database. Results We confirm that the commonest disease manifestation is angiokeratoma. Overall, 78% of males and 50% of females had one or more dermatological abnormality, the commonest being angiokeratoma (66% males, 36% females), hypohidrosis (53% males, 28% females), telangiectasia (23% males, 9% females) and lymphoedema (16% males, 6% females). We demonstrate for the first time that the presence of cutaneous vascular lesions correlates with the severity of the systemic manifestations of the disease (pain, renal failure, cardiac disease, premature cerebrovascular disease) as assessed by a severity scoring system. Although the condition is X linked, there is a surprisingly high prevalence of abnormalities in females. Conclusions The FOS database is a useful epidemiological tool in establishing the variety and relevance of cutaneous manifestations in Fabry disease. The present study confirms that the presence of dermatological manifestations appears to be a marker of greater severity of systemic disease, which emphasizes the importance of the dermatological assessment of these patients.
Affiliations:
- Allemagne, Autriche, Espagne, France, Italie, Royaume-Uni
- Champagne-Ardenne, Grand Est, Rhénanie-Palatinat, Vienne (Autriche), Île-de-France
- Mayence, Paris, Reims, Vienne (Autriche)
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Pintos-Morell</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Paediatrics, University Hospital Germans Trias i Pujol</s1><s2>Badalona</s2><s3>ESP</s3><sZ>6 aut.</sZ></inist:fA14><country>Espagne</country><wicri:noRegion>Badalona</wicri:noRegion></affiliation></author><author><name sortKey="Ramaswami, U" sort="Ramaswami, U" uniqKey="Ramaswami U" first="U." last="Ramaswami">U. Ramaswami</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Paediatrics, Addenbrooke's Hospital</s1><s2>Cambridge</s2><s3>GBR</s3><sZ>7 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Cambridge</wicri:noRegion></affiliation></author><author><name sortKey="Parini, R" sort="Parini, R" uniqKey="Parini R" first="R." last="Parini">R. Parini</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Paediatrics, University of Milan</s1><s2>Monza</s2><s3>ITA</s3><sZ>8 aut.</sZ></inist:fA14><country>Italie</country><wicri:noRegion>Monza</wicri:noRegion></affiliation></author><author><name sortKey="Sunder Plassman, G" sort="Sunder Plassman, G" uniqKey="Sunder Plassman G" first="G." last="Sunder-Plassman">G. Sunder-Plassman</name><affiliation wicri:level="3"><inist:fA14 i1="09"><s1>Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna</s1><s2>Vienna</s2><s3>AUT</s3><sZ>9 aut.</sZ></inist:fA14><country>Autriche</country><placeName><settlement type="city">Vienne (Autriche)</settlement><region nuts="2" type="province">Vienne (Autriche)</region></placeName></affiliation></author><author><name sortKey="Beck, M" sort="Beck, M" uniqKey="Beck M" first="M." last="Beck">M. Beck</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>University of Mainz</s1><s2>Mainz</s2><s3>DEU</s3><sZ>10 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Rhénanie-Palatinat</region><settlement type="city">Mayence</settlement></placeName></affiliation></author><author><name sortKey="Mehta, A B" sort="Mehta, A B" uniqKey="Mehta A" first="A. B." last="Mehta">A. B. Mehta</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Haematology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>5 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>London NW3 2QG</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">07-0370161</idno><date when="2007">2007</date><idno type="stanalyst">PASCAL 07-0370161 INIST</idno><idno type="RBID">Pascal:07-0370161</idno><idno type="wicri:Area/PascalFrancis/Corpus">000451</idno><idno type="wicri:Area/PascalFrancis/Curation">000509</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">000473</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">000473</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Fabry disease and the skin : data from FOS, the Fabry outcome survey</title><author><name sortKey="Orteu, C H" sort="Orteu, C H" uniqKey="Orteu C" first="C. H." last="Orteu">C. H. Orteu</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Dermatology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>1 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>London NW3 2QG</wicri:noRegion></affiliation></author><author><name sortKey="Jansen, T" sort="Jansen, T" uniqKey="Jansen T" first="T." last="Jansen">T. Jansen</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Dermatology, Ruhr University</s1><s2>Bochum</s2><s3>DEU</s3><sZ>2 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Bochum</wicri:noRegion><wicri:noRegion>Ruhr University</wicri:noRegion><wicri:noRegion>Bochum</wicri:noRegion></affiliation></author><author><name sortKey="Lidove, O" sort="Lidove, O" uniqKey="Lidove O" first="O." last="Lidove">O. Lidove</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Department of Internal Medicine, Bichat Hospital</s1><s2>Paris</s2><s3>FRA</s3><sZ>3 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Jaussaud, R" sort="Jaussaud, R" uniqKey="Jaussaud R" first="R." last="Jaussaud">R. Jaussaud</name><affiliation wicri:level="3"><inist:fA14 i1="04"><s1>Department of Internal Medicine and Infectious Diseases, Robert Debré Hospital</s1><s2>Reims</s2><s3>FRA</s3><sZ>4 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Grand Est</region><region type="old region">Champagne-Ardenne</region><settlement type="city">Reims</settlement></placeName></affiliation></author><author><name sortKey="Hushes, D A" sort="Hushes, D A" uniqKey="Hushes D" first="D. A." last="Hushes">D. A. Hushes</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Haematology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>5 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>London NW3 2QG</wicri:noRegion></affiliation></author><author><name sortKey="Pintos Morell, G" sort="Pintos Morell, G" uniqKey="Pintos Morell G" first="G." last="Pintos-Morell">G. Pintos-Morell</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Paediatrics, University Hospital Germans Trias i Pujol</s1><s2>Badalona</s2><s3>ESP</s3><sZ>6 aut.</sZ></inist:fA14><country>Espagne</country><wicri:noRegion>Badalona</wicri:noRegion></affiliation></author><author><name sortKey="Ramaswami, U" sort="Ramaswami, U" uniqKey="Ramaswami U" first="U." last="Ramaswami">U. Ramaswami</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Paediatrics, Addenbrooke's Hospital</s1><s2>Cambridge</s2><s3>GBR</s3><sZ>7 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Cambridge</wicri:noRegion></affiliation></author><author><name sortKey="Parini, R" sort="Parini, R" uniqKey="Parini R" first="R." last="Parini">R. Parini</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Department of Paediatrics, University of Milan</s1><s2>Monza</s2><s3>ITA</s3><sZ>8 aut.</sZ></inist:fA14><country>Italie</country><wicri:noRegion>Monza</wicri:noRegion></affiliation></author><author><name sortKey="Sunder Plassman, G" sort="Sunder Plassman, G" uniqKey="Sunder Plassman G" first="G." last="Sunder-Plassman">G. Sunder-Plassman</name><affiliation wicri:level="3"><inist:fA14 i1="09"><s1>Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna</s1><s2>Vienna</s2><s3>AUT</s3><sZ>9 aut.</sZ></inist:fA14><country>Autriche</country><placeName><settlement type="city">Vienne (Autriche)</settlement><region nuts="2" type="province">Vienne (Autriche)</region></placeName></affiliation></author><author><name sortKey="Beck, M" sort="Beck, M" uniqKey="Beck M" first="M." last="Beck">M. Beck</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>University of Mainz</s1><s2>Mainz</s2><s3>DEU</s3><sZ>10 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="2">Rhénanie-Palatinat</region><settlement type="city">Mayence</settlement></placeName></affiliation></author><author><name sortKey="Mehta, A B" sort="Mehta, A B" uniqKey="Mehta A" first="A. B." last="Mehta">A. B. Mehta</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Haematology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>5 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>London NW3 2QG</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">British journal of dermatology : (1951)</title><title level="j" type="abbreviated">Br. j. dermatol. : (1951)</title><idno type="ISSN">0007-0963</idno><imprint><date when="2007">2007</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">British journal of dermatology : (1951)</title><title level="j" type="abbreviated">Br. j. dermatol. : (1951)</title><idno type="ISSN">0007-0963</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Angiokeratoma</term><term>Dermatology</term><term>Dyshidrosis</term><term>Evolution</term><term>Fabry disease</term><term>Hyperhidrosis</term><term>Lipids</term><term>Lymphedema</term><term>Prognosis</term><term>Skin disease</term><term>Surveillance</term><term>Survey</term><term>Telangiectasia</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Sphingolipidose héréditaire Fabry</term><term>Peau pathologie</term><term>Angiokératome</term><term>Hyperhidrose</term><term>Dyshidrose</term><term>Lymphoedème</term><term>Télangiectasie</term><term>Pronostic</term><term>Evolution</term><term>Enquête</term><term>Surveillance</term><term>Dermatologie</term><term>Lipide</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background Fabry disease (also known as Anderson-Fabry disease) is a rare, X-linked lysosomal storage disorder that is characterized by accumulation of globotriaosylceramide throughout a range of tissues in the body. Objectives To ascertain the prevalence and nature of cutaneous manifestations in patients with Fabry disease and to relate these to the severity of systemic manifestations of the disease. Methods We have documented the dermatological features of this disease with reference to data from 714 patients (345 males, 369 females) registered on the Fabry Outcome Survey (FOS), a multicentre European database. Results We confirm that the commonest disease manifestation is angiokeratoma. Overall, 78% of males and 50% of females had one or more dermatological abnormality, the commonest being angiokeratoma (66% males, 36% females), hypohidrosis (53% males, 28% females), telangiectasia (23% males, 9% females) and lymphoedema (16% males, 6% females). We demonstrate for the first time that the presence of cutaneous vascular lesions correlates with the severity of the systemic manifestations of the disease (pain, renal failure, cardiac disease, premature cerebrovascular disease) as assessed by a severity scoring system. Although the condition is X linked, there is a surprisingly high prevalence of abnormalities in females. Conclusions The FOS database is a useful epidemiological tool in establishing the variety and relevance of cutaneous manifestations in Fabry disease. The present study confirms that the presence of dermatological manifestations appears to be a marker of greater severity of systemic disease, which emphasizes the importance of the dermatological assessment of these patients.</div></front></TEI><inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>0007-0963</s0></fA01><fA02 i1="01"><s0>BJDEAZ</s0></fA02><fA03 i2="1"><s0>Br. j. dermatol. : (1951)</s0></fA03><fA05><s2>157</s2></fA05><fA06><s2>2</s2></fA06><fA08 i1="01" i2="1" l="ENG"><s1>Fabry disease and the skin : data from FOS, the Fabry outcome survey</s1></fA08><fA11 i1="01" i2="1"><s1>ORTEU (C. H.)</s1></fA11><fA11 i1="02" i2="1"><s1>JANSEN (T.)</s1></fA11><fA11 i1="03" i2="1"><s1>LIDOVE (O.)</s1></fA11><fA11 i1="04" i2="1"><s1>JAUSSAUD (R.)</s1></fA11><fA11 i1="05" i2="1"><s1>HUSHES (D. A.)</s1></fA11><fA11 i1="06" i2="1"><s1>PINTOS-MORELL (G.)</s1></fA11><fA11 i1="07" i2="1"><s1>RAMASWAMI (U.)</s1></fA11><fA11 i1="08" i2="1"><s1>PARINI (R.)</s1></fA11><fA11 i1="09" i2="1"><s1>SUNDER-PLASSMAN (G.)</s1></fA11><fA11 i1="10" i2="1"><s1>BECK (M.)</s1></fA11><fA11 i1="11" i2="1"><s1>MEHTA (A. B.)</s1></fA11><fA14 i1="01"><s1>Department of Dermatology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>1 aut.</sZ></fA14><fA14 i1="02"><s1>Department of Dermatology, Ruhr University</s1><s2>Bochum</s2><s3>DEU</s3><sZ>2 aut.</sZ></fA14><fA14 i1="03"><s1>Department of Internal Medicine, Bichat Hospital</s1><s2>Paris</s2><s3>FRA</s3><sZ>3 aut.</sZ></fA14><fA14 i1="04"><s1>Department of Internal Medicine and Infectious Diseases, Robert Debré Hospital</s1><s2>Reims</s2><s3>FRA</s3><sZ>4 aut.</sZ></fA14><fA14 i1="05"><s1>Department of Haematology, Royal Free Hospital</s1><s2>London NW3 2QG</s2><s3>GBR</s3><sZ>5 aut.</sZ><sZ>11 aut.</sZ></fA14><fA14 i1="06"><s1>Department of Paediatrics, University Hospital Germans Trias i Pujol</s1><s2>Badalona</s2><s3>ESP</s3><sZ>6 aut.</sZ></fA14><fA14 i1="07"><s1>Department of Paediatrics, Addenbrooke's Hospital</s1><s2>Cambridge</s2><s3>GBR</s3><sZ>7 aut.</sZ></fA14><fA14 i1="08"><s1>Department of Paediatrics, University of Milan</s1><s2>Monza</s2><s3>ITA</s3><sZ>8 aut.</sZ></fA14><fA14 i1="09"><s1>Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna</s1><s2>Vienna</s2><s3>AUT</s3><sZ>9 aut.</sZ></fA14><fA14 i1="10"><s1>University of Mainz</s1><s2>Mainz</s2><s3>DEU</s3><sZ>10 aut.</sZ></fA14><fA17 i1="01" i2="1"><s1>FOS Investigators</s1><s3>INC</s3></fA17><fA20><s1>331-337</s1></fA20><fA21><s1>2007</s1></fA21><fA23 i1="01"><s0>ENG</s0></fA23><fA43 i1="01"><s1>INIST</s1><s2>1043</s2><s5>354000149951880170</s5></fA43><fA44><s0>0000</s0><s1>© 2007 INIST-CNRS. All rights reserved.</s1></fA44><fA45><s0>43 ref.</s0></fA45><fA47 i1="01" i2="1"><s0>07-0370161</s0></fA47><fA60><s1>P</s1></fA60><fA61><s0>A</s0></fA61><fA64 i1="01" i2="1"><s0>British journal of dermatology : (1951)</s0></fA64><fA66 i1="01"><s0>GBR</s0></fA66><fC01 i1="01" l="ENG"><s0>Background Fabry disease (also known as Anderson-Fabry disease) is a rare, X-linked lysosomal storage disorder that is characterized by accumulation of globotriaosylceramide throughout a range of tissues in the body. Objectives To ascertain the prevalence and nature of cutaneous manifestations in patients with Fabry disease and to relate these to the severity of systemic manifestations of the disease. Methods We have documented the dermatological features of this disease with reference to data from 714 patients (345 males, 369 females) registered on the Fabry Outcome Survey (FOS), a multicentre European database. Results We confirm that the commonest disease manifestation is angiokeratoma. Overall, 78% of males and 50% of females had one or more dermatological abnormality, the commonest being angiokeratoma (66% males, 36% females), hypohidrosis (53% males, 28% females), telangiectasia (23% males, 9% females) and lymphoedema (16% males, 6% females). We demonstrate for the first time that the presence of cutaneous vascular lesions correlates with the severity of the systemic manifestations of the disease (pain, renal failure, cardiac disease, premature cerebrovascular disease) as assessed by a severity scoring system. Although the condition is X linked, there is a surprisingly high prevalence of abnormalities in females. Conclusions The FOS database is a useful epidemiological tool in establishing the variety and relevance of cutaneous manifestations in Fabry disease. The present study confirms that the presence of dermatological manifestations appears to be a marker of greater severity of systemic disease, which emphasizes the importance of the dermatological assessment of these patients.</s0></fC01><fC02 i1="01" i2="X"><s0>002B08I</s0></fC02><fC02 i1="02" i2="X"><s0>002B22D02</s0></fC02><fC02 i1="03" i2="X"><s0>002B08J</s0></fC02><fC03 i1="01" i2="X" l="FRE"><s0>Sphingolipidose héréditaire Fabry</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="ENG"><s0>Fabry disease</s0><s5>01</s5></fC03><fC03 i1="01" i2="X" l="SPA"><s0>Esfingolipidosis hereditaria Fabry</s0><s5>01</s5></fC03><fC03 i1="02" i2="X" l="FRE"><s0>Peau pathologie</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="ENG"><s0>Skin disease</s0><s5>02</s5></fC03><fC03 i1="02" i2="X" l="SPA"><s0>Piel patología</s0><s5>02</s5></fC03><fC03 i1="03" i2="X" l="FRE"><s0>Angiokératome</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" l="ENG"><s0>Angiokeratoma</s0><s5>03</s5></fC03><fC03 i1="03" i2="X" l="SPA"><s0>Angioqueratoma</s0><s5>03</s5></fC03><fC03 i1="04" i2="X" l="FRE"><s0>Hyperhidrose</s0><s5>04</s5></fC03><fC03 i1="04" i2="X" l="ENG"><s0>Hyperhidrosis</s0><s5>04</s5></fC03><fC03 i1="04" i2="X" l="SPA"><s0>Hiperhidrosis</s0><s5>04</s5></fC03><fC03 i1="05" i2="X" l="FRE"><s0>Dyshidrose</s0><s5>05</s5></fC03><fC03 i1="05" i2="X" l="ENG"><s0>Dyshidrosis</s0><s5>05</s5></fC03><fC03 i1="05" i2="X" l="SPA"><s0>Dishidrosis</s0><s5>05</s5></fC03><fC03 i1="06" i2="X" l="FRE"><s0>Lymphoedème</s0><s5>06</s5></fC03><fC03 i1="06" i2="X" l="ENG"><s0>Lymphedema</s0><s5>06</s5></fC03><fC03 i1="06" i2="X" l="SPA"><s0>Linfedema</s0><s5>06</s5></fC03><fC03 i1="07" i2="X" l="FRE"><s0>Télangiectasie</s0><s5>07</s5></fC03><fC03 i1="07" i2="X" l="ENG"><s0>Telangiectasia</s0><s5>07</s5></fC03><fC03 i1="07" i2="X" l="SPA"><s0>Telangiectasia</s0><s5>07</s5></fC03><fC03 i1="08" i2="X" l="FRE"><s0>Pronostic</s0><s5>09</s5></fC03><fC03 i1="08" i2="X" l="ENG"><s0>Prognosis</s0><s5>09</s5></fC03><fC03 i1="08" i2="X" l="SPA"><s0>Pronóstico</s0><s5>09</s5></fC03><fC03 i1="09" i2="X" l="FRE"><s0>Evolution</s0><s5>10</s5></fC03><fC03 i1="09" i2="X" l="ENG"><s0>Evolution</s0><s5>10</s5></fC03><fC03 i1="09" i2="X" l="SPA"><s0>Evolución</s0><s5>10</s5></fC03><fC03 i1="10" i2="X" l="FRE"><s0>Enquête</s0><s5>11</s5></fC03><fC03 i1="10" i2="X" l="ENG"><s0>Survey</s0><s5>11</s5></fC03><fC03 i1="10" i2="X" l="SPA"><s0>Encuesta</s0><s5>11</s5></fC03><fC03 i1="11" i2="X" l="FRE"><s0>Surveillance</s0><s5>12</s5></fC03><fC03 i1="11" i2="X" l="ENG"><s0>Surveillance</s0><s5>12</s5></fC03><fC03 i1="11" i2="X" l="SPA"><s0>Vigilancia</s0><s5>12</s5></fC03><fC03 i1="12" i2="X" l="FRE"><s0>Dermatologie</s0><s5>13</s5></fC03><fC03 i1="12" i2="X" l="ENG"><s0>Dermatology</s0><s5>13</s5></fC03><fC03 i1="12" i2="X" l="SPA"><s0>Dermatología</s0><s5>13</s5></fC03><fC03 i1="13" i2="X" l="FRE"><s0>Lipide</s0><s5>25</s5></fC03><fC03 i1="13" i2="X" l="ENG"><s0>Lipids</s0><s5>25</s5></fC03><fC03 i1="13" i2="X" l="SPA"><s0>Lípido</s0><s5>25</s5></fC03><fC07 i1="01" i2="X" l="FRE"><s0>Appareil circulatoire pathologie</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="ENG"><s0>Cardiovascular disease</s0><s5>37</s5></fC07><fC07 i1="01" i2="X" l="SPA"><s0>Aparato circulatorio patología</s0><s5>37</s5></fC07><fC07 i1="02" i2="X" l="FRE"><s0>Enzymopathie</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="ENG"><s0>Enzymopathy</s0><s5>38</s5></fC07><fC07 i1="02" i2="X" l="SPA"><s0>Enzimopatía</s0><s5>38</s5></fC07><fC07 i1="03" i2="X" l="FRE"><s0>Lipoïdose</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="ENG"><s0>Lipoidosis</s0><s5>39</s5></fC07><fC07 i1="03" i2="X" l="SPA"><s0>Lipoidosis</s0><s5>39</s5></fC07><fC07 i1="04" i2="X" l="FRE"><s0>Maladie héréditaire</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="ENG"><s0>Genetic disease</s0><s5>40</s5></fC07><fC07 i1="04" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0><s5>40</s5></fC07><fC07 i1="05" i2="X" l="FRE"><s0>Métabolisme pathologie</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="ENG"><s0>Metabolic diseases</s0><s5>41</s5></fC07><fC07 i1="05" i2="X" l="SPA"><s0>Metabolismo patología</s0><s5>41</s5></fC07><fC07 i1="06" i2="X" l="FRE"><s0>Système nerveux pathologie</s0><s5>42</s5></fC07><fC07 i1="06" i2="X" l="ENG"><s0>Nervous system diseases</s0><s5>42</s5></fC07><fC07 i1="06" i2="X" l="SPA"><s0>Sistema nervioso patología</s0><s5>42</s5></fC07><fC07 i1="07" i2="X" l="FRE"><s0>Vaisseau sanguin pathologie</s0><s5>43</s5></fC07><fC07 i1="07" i2="X" l="ENG"><s0>Vascular disease</s0><s5>43</s5></fC07><fC07 i1="07" i2="X" l="SPA"><s0>Vaso sanguíneo patología</s0><s5>43</s5></fC07><fC07 i1="08" i2="X" l="FRE"><s0>Angiome</s0><s5>44</s5></fC07><fC07 i1="08" i2="X" l="ENG"><s0>Angioma</s0><s5>44</s5></fC07><fC07 i1="08" i2="X" l="SPA"><s0>Angioma</s0><s5>44</s5></fC07><fC07 i1="09" i2="X" l="FRE"><s0>Dyskératose</s0><s5>45</s5></fC07><fC07 i1="09" i2="X" l="ENG"><s0>Dyskeratosis</s0><s5>45</s5></fC07><fC07 i1="09" i2="X" l="SPA"><s0>Disqueratosis</s0><s5>45</s5></fC07><fC07 i1="10" i2="X" l="FRE"><s0>Hyperkératose</s0><s5>46</s5></fC07><fC07 i1="10" i2="X" l="ENG"><s0>Hyperkeratosis</s0><s5>46</s5></fC07><fC07 i1="10" i2="X" l="SPA"><s0>Hiperqueratosis</s0><s5>46</s5></fC07><fC07 i1="11" i2="X" l="FRE"><s0>Glande sudoripare pathologie</s0><s5>47</s5></fC07><fC07 i1="11" i2="X" l="ENG"><s0>Sweat gland disease</s0><s5>47</s5></fC07><fC07 i1="11" i2="X" l="SPA"><s0>Glándula sudorípara patología</s0><s5>47</s5></fC07><fC07 i1="12" i2="X" l="FRE"><s0>Dermatose bulleuse</s0><s5>48</s5></fC07><fC07 i1="12" i2="X" l="ENG"><s0>Bullous dermatosis</s0><s5>48</s5></fC07><fC07 i1="12" i2="X" l="SPA"><s0>Dermatosis bulosa</s0><s5>48</s5></fC07><fC07 i1="13" i2="X" l="FRE"><s0>Immunopathologie</s0><s5>49</s5></fC07><fC07 i1="13" i2="X" l="ENG"><s0>Immunopathology</s0><s5>49</s5></fC07><fC07 i1="13" i2="X" l="SPA"><s0>Inmunopatología</s0><s5>49</s5></fC07><fC07 i1="14" i2="X" l="FRE"><s0>Lymphatique pathologie</s0><s5>50</s5></fC07><fC07 i1="14" i2="X" l="ENG"><s0>Lymphatic vessel disease</s0><s5>50</s5></fC07><fC07 i1="14" i2="X" l="SPA"><s0>Linfático patología</s0><s5>50</s5></fC07><fN21><s1>239</s1></fN21><fN44 i1="01"><s1>OTO</s1></fN44><fN82><s1>OTO</s1></fN82></pA></standard></inist><affiliations><list><country><li>Allemagne</li><li>Autriche</li><li>Espagne</li><li>France</li><li>Italie</li><li>Royaume-Uni</li></country><region><li>Champagne-Ardenne</li><li>Grand Est</li><li>Rhénanie-Palatinat</li><li>Vienne (Autriche)</li><li>Île-de-France</li></region><settlement><li>Mayence</li><li>Paris</li><li>Reims</li><li>Vienne (Autriche)</li></settlement></list><tree><country name="Royaume-Uni"><noRegion><name sortKey="Orteu, C H" sort="Orteu, C H" uniqKey="Orteu C" first="C. H." last="Orteu">C. H. Orteu</name></noRegion><name sortKey="Hushes, D A" sort="Hushes, D A" uniqKey="Hushes D" first="D. A." last="Hushes">D. A. Hushes</name><name sortKey="Mehta, A B" sort="Mehta, A B" uniqKey="Mehta A" first="A. B." last="Mehta">A. B. Mehta</name><name sortKey="Ramaswami, U" sort="Ramaswami, U" uniqKey="Ramaswami U" first="U." last="Ramaswami">U. Ramaswami</name></country><country name="Allemagne"><noRegion><name sortKey="Jansen, T" sort="Jansen, T" uniqKey="Jansen T" first="T." last="Jansen">T. Jansen</name></noRegion><name sortKey="Beck, M" sort="Beck, M" uniqKey="Beck M" first="M." last="Beck">M. Beck</name></country><country name="France"><region name="Île-de-France"><name sortKey="Lidove, O" sort="Lidove, O" uniqKey="Lidove O" first="O." last="Lidove">O. Lidove</name></region><name sortKey="Jaussaud, R" sort="Jaussaud, R" uniqKey="Jaussaud R" first="R." last="Jaussaud">R. Jaussaud</name></country><country name="Espagne"><noRegion><name sortKey="Pintos Morell, G" sort="Pintos Morell, G" uniqKey="Pintos Morell G" first="G." last="Pintos-Morell">G. Pintos-Morell</name></noRegion></country><country name="Italie"><noRegion><name sortKey="Parini, R" sort="Parini, R" uniqKey="Parini R" first="R." last="Parini">R. Parini</name></noRegion></country><country name="Autriche"><region name="Vienne (Autriche)"><name sortKey="Sunder Plassman, G" sort="Sunder Plassman, G" uniqKey="Sunder Plassman G" first="G." last="Sunder-Plassman">G. Sunder-Plassman</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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|texte= Fabry disease and the skin : data from FOS, the Fabry outcome survey
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