Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.
Identifieur interne : 000A10 ( Main/Exploration ); précédent : 000A09; suivant : 000A11Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.
Auteurs : William R. Wilcox [États-Unis] ; Ulla Feldt-Rasmussen [Danemark] ; Ana Maria Martins [Brésil] ; Alberto Ortiz [Espagne] ; Roberta M. Lemay [États-Unis] ; Ana Jovanovic [Royaume-Uni] ; Dominique P. Germain [France] ; Carmen Varas [Chili] ; Katherine Nicholls [Australie] ; Frank Weidemann [Allemagne] ; Robert J. Hopkin [États-Unis]Source :
- JIMD reports [ 2192-8304 ] ; 2017.
Abstract
Fabry disease, an X-linked inherited lysosomal storage disorder, is caused by mutations in the gene encoding α-galactosidase, GLA. In patients with Fabry disease, glycosphingolipids accumulate in various cell types, triggering a range of cellular and tissue responses that result in a wide spectrum of organ involvement. Although variable, gastrointestinal symptoms are among the most common and significant early clinical manifestations; they tend to persist into adulthood if left untreated. To further understand the effects of sustained enzyme replacement therapy (ERT) with agalsidase beta on gastrointestinal symptoms in heterozygotes, a data analysis of female patients enrolled in the Fabry Registry was conducted. To be included, females of any age must have received agalsidase beta (average dose 1.0 mg/kg every 2 weeks) for at least 2.5 years. Measured outcomes were self-reported gastrointestinal symptoms (abdominal pain, diarrhea). Outcomes at baseline and last follow-up, and their change from baseline to last follow-up, were assessed. Relevant data were available for 168 female patients. Mean age at the start of ERT was 43 years and mean treatment duration 5.7 years. Baseline pre-treatment abdominal pain was reported by 45% of females and diarrhea by 39%. At last follow-up, 31% reported abdominal pain (p < 0.01) and 27% diarrhea (p < 0.01). The results of this Fabry Registry analysis suggest that while on sustained treatment with agalsidase beta (1.0 mg/kg every 2 weeks), both abdominal pain and diarrhea improved in many female patients with Fabry disease.
DOI: 10.1007/8904_2017_28
PubMed: 28510034
Affiliations:
- Allemagne, Australie, Brésil, Chili, Danemark, Espagne, France, Royaume-Uni, États-Unis
- Communauté de Madrid, Hovedstaden, Massachusetts, Ohio, État de São Paulo
- Copenhague, Madrid, São Paulo
- Université de São Paulo
Links toward previous steps (curation, corpus...)
- to stream PubMed, to step Corpus: 000B95
- to stream PubMed, to step Curation: 000B92
- to stream PubMed, to step Checkpoint: 000B92
- to stream Ncbi, to step Merge: 004821
- to stream Ncbi, to step Curation: 004821
- to stream Ncbi, to step Checkpoint: 004821
- to stream Main, to step Merge: 000A05
- to stream Main, to step Curation: 000A10
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.</title>
<author><name sortKey="Wilcox, William R" sort="Wilcox, William R" uniqKey="Wilcox W" first="William R" last="Wilcox">William R. Wilcox</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Room 305H, Atlanta, GA, 30322, USA. william.wilcox@emory.edu.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Room 305H, Atlanta, GA, 30322</wicri:regionArea>
<wicri:noRegion>30322</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Feldt Rasmussen, Ulla" sort="Feldt Rasmussen, Ulla" uniqKey="Feldt Rasmussen U" first="Ulla" last="Feldt-Rasmussen">Ulla Feldt-Rasmussen</name>
<affiliation wicri:level="3"><nlm:affiliation>Department of Medical Endocrinology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.</nlm:affiliation>
<country xml:lang="fr">Danemark</country>
<wicri:regionArea>Department of Medical Endocrinology, Rigshospitalet, Copenhagen University Hospital, Copenhagen</wicri:regionArea>
<placeName><settlement type="city">Copenhague</settlement>
<region type="région" nuts="2">Hovedstaden</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Martins, Ana Maria" sort="Martins, Ana Maria" uniqKey="Martins A" first="Ana Maria" last="Martins">Ana Maria Martins</name>
<affiliation wicri:level="4"><nlm:affiliation>Reference Center for Inborn Errors of Metabolism, Federal University of São Paulo, São Paulo, Brazil.</nlm:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Reference Center for Inborn Errors of Metabolism, Federal University of São Paulo, São Paulo</wicri:regionArea>
<placeName><settlement type="city">São Paulo</settlement>
<region type="state">État de São Paulo</region>
<settlement type="city">São Paulo</settlement>
</placeName>
<orgName type="university">Université de São Paulo</orgName>
</affiliation>
</author>
<author><name sortKey="Ortiz, Alberto" sort="Ortiz, Alberto" uniqKey="Ortiz A" first="Alberto" last="Ortiz">Alberto Ortiz</name>
<affiliation wicri:level="3"><nlm:affiliation>Unidad de Dialisis, IIS-Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Madrid, Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>Unidad de Dialisis, IIS-Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Madrid</wicri:regionArea>
<placeName><settlement type="city">Madrid</settlement>
<region nuts="2" type="region">Communauté de Madrid</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Lemay, Roberta M" sort="Lemay, Roberta M" uniqKey="Lemay R" first="Roberta M" last="Lemay">Roberta M. Lemay</name>
<affiliation wicri:level="2"><nlm:affiliation>Strategic Epidemiology and Biostatistics, Rare Diseases, Sanofi Genzyme, Cambridge, MA, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Strategic Epidemiology and Biostatistics, Rare Diseases, Sanofi Genzyme, Cambridge, MA</wicri:regionArea>
<placeName><region type="state">Massachusetts</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Jovanovic, Ana" sort="Jovanovic, Ana" uniqKey="Jovanovic A" first="Ana" last="Jovanovic">Ana Jovanovic</name>
<affiliation wicri:level="1"><nlm:affiliation>Mark Holland Metabolic Unit, Salford Royal NHS Foundation Trust, Salford, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Mark Holland Metabolic Unit, Salford Royal NHS Foundation Trust, Salford</wicri:regionArea>
<wicri:noRegion>Salford</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Germain, Dominique P" sort="Germain, Dominique P" uniqKey="Germain D" first="Dominique P" last="Germain">Dominique P. Germain</name>
<affiliation wicri:level="1"><nlm:affiliation>Division of Medical Genetics, University of Versailles - St Quentin en Yvelines, Paris-Saclay University, Montigny, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Division of Medical Genetics, University of Versailles - St Quentin en Yvelines, Paris-Saclay University, Montigny</wicri:regionArea>
<wicri:noRegion>Montigny</wicri:noRegion>
<wicri:noRegion>Montigny</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Varas, Carmen" sort="Varas, Carmen" uniqKey="Varas C" first="Carmen" last="Varas">Carmen Varas</name>
<affiliation wicri:level="1"><nlm:affiliation>Fabry Disease Multidisciplinary Team, Departamento Dermatología y ETS, Hospital San Pablo de Coquimbo, Coquimbo, Chile.</nlm:affiliation>
<country xml:lang="fr">Chili</country>
<wicri:regionArea>Fabry Disease Multidisciplinary Team, Departamento Dermatología y ETS, Hospital San Pablo de Coquimbo, Coquimbo</wicri:regionArea>
<wicri:noRegion>Coquimbo</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Nicholls, Katherine" sort="Nicholls, Katherine" uniqKey="Nicholls K" first="Katherine" last="Nicholls">Katherine Nicholls</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Nephrology, Royal Melbourne Hospital, Parkville, VIC, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Nephrology, Royal Melbourne Hospital, Parkville, VIC</wicri:regionArea>
<wicri:noRegion>VIC</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Weidemann, Frank" sort="Weidemann, Frank" uniqKey="Weidemann F" first="Frank" last="Weidemann">Frank Weidemann</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Internal Medicine II, Katharinen-Hospital Unna, Unna, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Internal Medicine II, Katharinen-Hospital Unna, Unna</wicri:regionArea>
<wicri:noRegion>Unna</wicri:noRegion>
<wicri:noRegion>Unna</wicri:noRegion>
<wicri:noRegion>Unna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hopkin, Robert J" sort="Hopkin, Robert J" uniqKey="Hopkin R" first="Robert J" last="Hopkin">Robert J. Hopkin</name>
<affiliation wicri:level="2"><nlm:affiliation>Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH</wicri:regionArea>
<placeName><region type="state">Ohio</region>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PubMed</idno>
<date when="2017">2017</date>
<idno type="RBID">pubmed:28510034</idno>
<idno type="pmid">28510034</idno>
<idno type="doi">10.1007/8904_2017_28</idno>
<idno type="wicri:Area/PubMed/Corpus">000B95</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000B95</idno>
<idno type="wicri:Area/PubMed/Curation">000B92</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">000B92</idno>
<idno type="wicri:Area/PubMed/Checkpoint">000B92</idno>
<idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">000B92</idno>
<idno type="wicri:Area/Ncbi/Merge">004821</idno>
<idno type="wicri:Area/Ncbi/Curation">004821</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">004821</idno>
<idno type="wicri:doubleKey">2192-8304:2017:Wilcox W:improvement:of:fabry</idno>
<idno type="wicri:Area/Main/Merge">000A05</idno>
<idno type="wicri:Area/Main/Curation">000A10</idno>
<idno type="wicri:Area/Main/Exploration">000A10</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en">Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.</title>
<author><name sortKey="Wilcox, William R" sort="Wilcox, William R" uniqKey="Wilcox W" first="William R" last="Wilcox">William R. Wilcox</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Room 305H, Atlanta, GA, 30322, USA. william.wilcox@emory.edu.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Room 305H, Atlanta, GA, 30322</wicri:regionArea>
<wicri:noRegion>30322</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Feldt Rasmussen, Ulla" sort="Feldt Rasmussen, Ulla" uniqKey="Feldt Rasmussen U" first="Ulla" last="Feldt-Rasmussen">Ulla Feldt-Rasmussen</name>
<affiliation wicri:level="3"><nlm:affiliation>Department of Medical Endocrinology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.</nlm:affiliation>
<country xml:lang="fr">Danemark</country>
<wicri:regionArea>Department of Medical Endocrinology, Rigshospitalet, Copenhagen University Hospital, Copenhagen</wicri:regionArea>
<placeName><settlement type="city">Copenhague</settlement>
<region type="région" nuts="2">Hovedstaden</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Martins, Ana Maria" sort="Martins, Ana Maria" uniqKey="Martins A" first="Ana Maria" last="Martins">Ana Maria Martins</name>
<affiliation wicri:level="4"><nlm:affiliation>Reference Center for Inborn Errors of Metabolism, Federal University of São Paulo, São Paulo, Brazil.</nlm:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Reference Center for Inborn Errors of Metabolism, Federal University of São Paulo, São Paulo</wicri:regionArea>
<placeName><settlement type="city">São Paulo</settlement>
<region type="state">État de São Paulo</region>
<settlement type="city">São Paulo</settlement>
</placeName>
<orgName type="university">Université de São Paulo</orgName>
</affiliation>
</author>
<author><name sortKey="Ortiz, Alberto" sort="Ortiz, Alberto" uniqKey="Ortiz A" first="Alberto" last="Ortiz">Alberto Ortiz</name>
<affiliation wicri:level="3"><nlm:affiliation>Unidad de Dialisis, IIS-Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Madrid, Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>Unidad de Dialisis, IIS-Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Madrid</wicri:regionArea>
<placeName><settlement type="city">Madrid</settlement>
<region nuts="2" type="region">Communauté de Madrid</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Lemay, Roberta M" sort="Lemay, Roberta M" uniqKey="Lemay R" first="Roberta M" last="Lemay">Roberta M. Lemay</name>
<affiliation wicri:level="2"><nlm:affiliation>Strategic Epidemiology and Biostatistics, Rare Diseases, Sanofi Genzyme, Cambridge, MA, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Strategic Epidemiology and Biostatistics, Rare Diseases, Sanofi Genzyme, Cambridge, MA</wicri:regionArea>
<placeName><region type="state">Massachusetts</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Jovanovic, Ana" sort="Jovanovic, Ana" uniqKey="Jovanovic A" first="Ana" last="Jovanovic">Ana Jovanovic</name>
<affiliation wicri:level="1"><nlm:affiliation>Mark Holland Metabolic Unit, Salford Royal NHS Foundation Trust, Salford, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Mark Holland Metabolic Unit, Salford Royal NHS Foundation Trust, Salford</wicri:regionArea>
<wicri:noRegion>Salford</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Germain, Dominique P" sort="Germain, Dominique P" uniqKey="Germain D" first="Dominique P" last="Germain">Dominique P. Germain</name>
<affiliation wicri:level="1"><nlm:affiliation>Division of Medical Genetics, University of Versailles - St Quentin en Yvelines, Paris-Saclay University, Montigny, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Division of Medical Genetics, University of Versailles - St Quentin en Yvelines, Paris-Saclay University, Montigny</wicri:regionArea>
<wicri:noRegion>Montigny</wicri:noRegion>
<wicri:noRegion>Montigny</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Varas, Carmen" sort="Varas, Carmen" uniqKey="Varas C" first="Carmen" last="Varas">Carmen Varas</name>
<affiliation wicri:level="1"><nlm:affiliation>Fabry Disease Multidisciplinary Team, Departamento Dermatología y ETS, Hospital San Pablo de Coquimbo, Coquimbo, Chile.</nlm:affiliation>
<country xml:lang="fr">Chili</country>
<wicri:regionArea>Fabry Disease Multidisciplinary Team, Departamento Dermatología y ETS, Hospital San Pablo de Coquimbo, Coquimbo</wicri:regionArea>
<wicri:noRegion>Coquimbo</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Nicholls, Katherine" sort="Nicholls, Katherine" uniqKey="Nicholls K" first="Katherine" last="Nicholls">Katherine Nicholls</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Nephrology, Royal Melbourne Hospital, Parkville, VIC, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Department of Nephrology, Royal Melbourne Hospital, Parkville, VIC</wicri:regionArea>
<wicri:noRegion>VIC</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Weidemann, Frank" sort="Weidemann, Frank" uniqKey="Weidemann F" first="Frank" last="Weidemann">Frank Weidemann</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Internal Medicine II, Katharinen-Hospital Unna, Unna, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>Department of Internal Medicine II, Katharinen-Hospital Unna, Unna</wicri:regionArea>
<wicri:noRegion>Unna</wicri:noRegion>
<wicri:noRegion>Unna</wicri:noRegion>
<wicri:noRegion>Unna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hopkin, Robert J" sort="Hopkin, Robert J" uniqKey="Hopkin R" first="Robert J" last="Hopkin">Robert J. Hopkin</name>
<affiliation wicri:level="2"><nlm:affiliation>Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH</wicri:regionArea>
<placeName><region type="state">Ohio</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">JIMD reports</title>
<idno type="ISSN">2192-8304</idno>
<imprint><date when="2017" type="published">2017</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Fabry disease, an X-linked inherited lysosomal storage disorder, is caused by mutations in the gene encoding α-galactosidase, GLA. In patients with Fabry disease, glycosphingolipids accumulate in various cell types, triggering a range of cellular and tissue responses that result in a wide spectrum of organ involvement. Although variable, gastrointestinal symptoms are among the most common and significant early clinical manifestations; they tend to persist into adulthood if left untreated. To further understand the effects of sustained enzyme replacement therapy (ERT) with agalsidase beta on gastrointestinal symptoms in heterozygotes, a data analysis of female patients enrolled in the Fabry Registry was conducted. To be included, females of any age must have received agalsidase beta (average dose 1.0 mg/kg every 2 weeks) for at least 2.5 years. Measured outcomes were self-reported gastrointestinal symptoms (abdominal pain, diarrhea). Outcomes at baseline and last follow-up, and their change from baseline to last follow-up, were assessed. Relevant data were available for 168 female patients. Mean age at the start of ERT was 43 years and mean treatment duration 5.7 years. Baseline pre-treatment abdominal pain was reported by 45% of females and diarrhea by 39%. At last follow-up, 31% reported abdominal pain (p < 0.01) and 27% diarrhea (p < 0.01). The results of this Fabry Registry analysis suggest that while on sustained treatment with agalsidase beta (1.0 mg/kg every 2 weeks), both abdominal pain and diarrhea improved in many female patients with Fabry disease.</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Australie</li>
<li>Brésil</li>
<li>Chili</li>
<li>Danemark</li>
<li>Espagne</li>
<li>France</li>
<li>Royaume-Uni</li>
<li>États-Unis</li>
</country>
<region><li>Communauté de Madrid</li>
<li>Hovedstaden</li>
<li>Massachusetts</li>
<li>Ohio</li>
<li>État de São Paulo</li>
</region>
<settlement><li>Copenhague</li>
<li>Madrid</li>
<li>São Paulo</li>
</settlement>
<orgName><li>Université de São Paulo</li>
</orgName>
</list>
<tree><country name="États-Unis"><noRegion><name sortKey="Wilcox, William R" sort="Wilcox, William R" uniqKey="Wilcox W" first="William R" last="Wilcox">William R. Wilcox</name>
</noRegion>
<name sortKey="Hopkin, Robert J" sort="Hopkin, Robert J" uniqKey="Hopkin R" first="Robert J" last="Hopkin">Robert J. Hopkin</name>
<name sortKey="Lemay, Roberta M" sort="Lemay, Roberta M" uniqKey="Lemay R" first="Roberta M" last="Lemay">Roberta M. Lemay</name>
</country>
<country name="Danemark"><region name="Hovedstaden"><name sortKey="Feldt Rasmussen, Ulla" sort="Feldt Rasmussen, Ulla" uniqKey="Feldt Rasmussen U" first="Ulla" last="Feldt-Rasmussen">Ulla Feldt-Rasmussen</name>
</region>
</country>
<country name="Brésil"><region name="État de São Paulo"><name sortKey="Martins, Ana Maria" sort="Martins, Ana Maria" uniqKey="Martins A" first="Ana Maria" last="Martins">Ana Maria Martins</name>
</region>
</country>
<country name="Espagne"><region name="Communauté de Madrid"><name sortKey="Ortiz, Alberto" sort="Ortiz, Alberto" uniqKey="Ortiz A" first="Alberto" last="Ortiz">Alberto Ortiz</name>
</region>
</country>
<country name="Royaume-Uni"><noRegion><name sortKey="Jovanovic, Ana" sort="Jovanovic, Ana" uniqKey="Jovanovic A" first="Ana" last="Jovanovic">Ana Jovanovic</name>
</noRegion>
</country>
<country name="France"><noRegion><name sortKey="Germain, Dominique P" sort="Germain, Dominique P" uniqKey="Germain D" first="Dominique P" last="Germain">Dominique P. Germain</name>
</noRegion>
</country>
<country name="Chili"><noRegion><name sortKey="Varas, Carmen" sort="Varas, Carmen" uniqKey="Varas C" first="Carmen" last="Varas">Carmen Varas</name>
</noRegion>
</country>
<country name="Australie"><noRegion><name sortKey="Nicholls, Katherine" sort="Nicholls, Katherine" uniqKey="Nicholls K" first="Katherine" last="Nicholls">Katherine Nicholls</name>
</noRegion>
</country>
<country name="Allemagne"><noRegion><name sortKey="Weidemann, Frank" sort="Weidemann, Frank" uniqKey="Weidemann F" first="Frank" last="Weidemann">Frank Weidemann</name>
</noRegion>
</country>
</tree>
</affiliations>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Asie/explor/AustralieFrV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000A10 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 000A10 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Asie |area= AustralieFrV1 |flux= Main |étape= Exploration |type= RBID |clé= pubmed:28510034 |texte= Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry. }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Main/Exploration/RBID.i -Sk "pubmed:28510034" \ | HfdSelect -Kh $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd \ | NlmPubMed2Wicri -a AustralieFrV1
This area was generated with Dilib version V0.6.33. |