The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.
Identifieur interne : 000C37 ( PubMed/Curation ); précédent : 000C36; suivant : 000C38The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.
Auteurs : Christian J. Hendriksz [Royaume-Uni] ; Mathieu Anheim [France] ; Peter Bauer [Allemagne] ; Olivier Bonnot [France] ; Anupam Chakrapani [Royaume-Uni] ; Jean-Christophe Corvol [France] ; Tom J. De Koning [Pays-Bas] ; Anna Degtyareva [Russie] ; Carlo Dionisi-Vici [Italie] ; Sarah Doss [Allemagne] ; Thomas Duning [Allemagne] ; Paola Giunti [Royaume-Uni] ; Rosa Iodice [Italie] ; Tracy Johnston [Royaume-Uni] ; Dierdre Kelly [Royaume-Uni] ; Hans-Hermann Klünemann [Allemagne] ; Stefan Lorenzl [Allemagne] ; Alessandro Padovani [Italie] ; Miguel Pocovi [Espagne] ; Matthis Synofzik [Allemagne] ; Alta Terblanche [Afrique du Sud] ; Florian Then Bergh [Allemagne] ; Meral Topçu [Turquie] ; Christine Tranchant [France] ; Mark Walterfang [Australie] ; Christian Velten [Suisse] ; Stefan A. Kolb [Suisse]Source :
- Current medical research and opinion [ 1473-4877 ] ; 2017.
Descripteurs français
- KwdFr :
- MESH :
- épidémiologie : Maladie de Niemann-Pick de type C, Maladies rares.
- Humains, Prévalence.
English descriptors
- KwdEn :
- MESH :
- epidemiology : Niemann-Pick Disease, Type C, Rare Diseases.
- Humans, Prevalence.
Abstract
Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C. Patients in these at-risk groups ("clinical niches") have symptoms that are potentially related to NP-C, but go unrecognized due to other, more prevalent clinical features, and lack of awareness regarding underlying metabolic causes.
DOI: 10.1080/03007995.2017.1294054
PubMed: 28276873
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<sourceDesc><biblStruct><analytic><title xml:lang="en">The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.</title>
<author><name sortKey="Hendriksz, Christian J" sort="Hendriksz, Christian J" uniqKey="Hendriksz C" first="Christian J" last="Hendriksz">Christian J. Hendriksz</name>
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<country xml:lang="fr">Royaume-Uni</country>
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<author><name sortKey="Anheim, Mathieu" sort="Anheim, Mathieu" uniqKey="Anheim M" first="Mathieu" last="Anheim">Mathieu Anheim</name>
<affiliation wicri:level="1"><nlm:affiliation>c University of Strasbourg , Hautepierre Hospital , Strasbourg , France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>c University of Strasbourg , Hautepierre Hospital , Strasbourg </wicri:regionArea>
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<author><name sortKey="Bauer, Peter" sort="Bauer, Peter" uniqKey="Bauer P" first="Peter" last="Bauer">Peter Bauer</name>
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<author><name sortKey="Bonnot, Olivier" sort="Bonnot, Olivier" uniqKey="Bonnot O" first="Olivier" last="Bonnot">Olivier Bonnot</name>
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<author><name sortKey="Degtyareva, Anna" sort="Degtyareva, Anna" uniqKey="Degtyareva A" first="Anna" last="Degtyareva">Anna Degtyareva</name>
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<author><name sortKey="Dionisi Vici, Carlo" sort="Dionisi Vici, Carlo" uniqKey="Dionisi Vici C" first="Carlo" last="Dionisi-Vici">Carlo Dionisi-Vici</name>
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<author><name sortKey="Doss, Sarah" sort="Doss, Sarah" uniqKey="Doss S" first="Sarah" last="Doss">Sarah Doss</name>
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<author><name sortKey="Giunti, Paola" sort="Giunti, Paola" uniqKey="Giunti P" first="Paola" last="Giunti">Paola Giunti</name>
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<author><name sortKey="Iodice, Rosa" sort="Iodice, Rosa" uniqKey="Iodice R" first="Rosa" last="Iodice">Rosa Iodice</name>
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<author><name sortKey="Johnston, Tracy" sort="Johnston, Tracy" uniqKey="Johnston T" first="Tracy" last="Johnston">Tracy Johnston</name>
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<wicri:regionArea>p Birmingham Women's Hospital , Birmingham </wicri:regionArea>
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<author><name sortKey="Kelly, Dierdre" sort="Kelly, Dierdre" uniqKey="Kelly D" first="Dierdre" last="Kelly">Dierdre Kelly</name>
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<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>q Birmingham Children's Hospital , Birmingham </wicri:regionArea>
</affiliation>
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<author><name sortKey="Klunemann, Hans Hermann" sort="Klunemann, Hans Hermann" uniqKey="Klunemann H" first="Hans-Hermann" last="Klünemann">Hans-Hermann Klünemann</name>
<affiliation wicri:level="1"><nlm:affiliation>r University Clinic for Psychiatry and Psychotherapy, Regensburg University , Regensburg , Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>r University Clinic for Psychiatry and Psychotherapy, Regensburg University , Regensburg </wicri:regionArea>
</affiliation>
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<author><name sortKey="Lorenzl, Stefan" sort="Lorenzl, Stefan" uniqKey="Lorenzl S" first="Stefan" last="Lorenzl">Stefan Lorenzl</name>
<affiliation wicri:level="1"><nlm:affiliation>s Ludwig Maximillian University , Munich , Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>s Ludwig Maximillian University , Munich </wicri:regionArea>
</affiliation>
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<author><name sortKey="Padovani, Alessandro" sort="Padovani, Alessandro" uniqKey="Padovani A" first="Alessandro" last="Padovani">Alessandro Padovani</name>
<affiliation wicri:level="1"><nlm:affiliation>u Neurology Unit, Department of Clinical and Experimental Sciences , University of Brescia , Brescia , Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>u Neurology Unit, Department of Clinical and Experimental Sciences , University of Brescia , Brescia </wicri:regionArea>
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<author><name sortKey="Pocovi, Miguel" sort="Pocovi, Miguel" uniqKey="Pocovi M" first="Miguel" last="Pocovi">Miguel Pocovi</name>
<affiliation wicri:level="1"><nlm:affiliation>v University of Zaragoza , IISA, Zaragoza , Spain.</nlm:affiliation>
<country xml:lang="fr">Espagne</country>
<wicri:regionArea>v University of Zaragoza , IISA, Zaragoza </wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Synofzik, Matthis" sort="Synofzik, Matthis" uniqKey="Synofzik M" first="Matthis" last="Synofzik">Matthis Synofzik</name>
<affiliation wicri:level="1"><nlm:affiliation>w Department of Neurodegenerative Diseases , Hertie Institute for Clinical Brain Research , Tübingen, Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>w Department of Neurodegenerative Diseases , Hertie Institute for Clinical Brain Research , Tübingen</wicri:regionArea>
</affiliation>
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<author><name sortKey="Terblanche, Alta" sort="Terblanche, Alta" uniqKey="Terblanche A" first="Alta" last="Terblanche">Alta Terblanche</name>
<affiliation wicri:level="1"><nlm:affiliation>b University of Pretoria , Pretoria , South Africa.</nlm:affiliation>
<country xml:lang="fr">Afrique du Sud</country>
<wicri:regionArea>b University of Pretoria , Pretoria </wicri:regionArea>
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</author>
<author><name sortKey="Then Bergh, Florian" sort="Then Bergh, Florian" uniqKey="Then Bergh F" first="Florian" last="Then Bergh">Florian Then Bergh</name>
<affiliation wicri:level="1"><nlm:affiliation>y University of Leipzig , Department of Neurology , Leipzig , Germany.</nlm:affiliation>
<country xml:lang="fr">Allemagne</country>
<wicri:regionArea>y University of Leipzig , Department of Neurology , Leipzig </wicri:regionArea>
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</author>
<author><name sortKey="Topcu, Meral" sort="Topcu, Meral" uniqKey="Topcu M" first="Meral" last="Topçu">Meral Topçu</name>
<affiliation wicri:level="1"><nlm:affiliation>z Hacettepe University Children's Hospital , Ankara , Turkey.</nlm:affiliation>
<country xml:lang="fr">Turquie</country>
<wicri:regionArea>z Hacettepe University Children's Hospital , Ankara </wicri:regionArea>
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<author><name sortKey="Tranchant, Christine" sort="Tranchant, Christine" uniqKey="Tranchant C" first="Christine" last="Tranchant">Christine Tranchant</name>
<affiliation wicri:level="1"><nlm:affiliation>aa CHU Strasburg , Strasburg , France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>aa CHU Strasburg , Strasburg </wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Walterfang, Mark" sort="Walterfang, Mark" uniqKey="Walterfang M" first="Mark" last="Walterfang">Mark Walterfang</name>
<affiliation wicri:level="1"><nlm:affiliation>ab Royal Melbourne Hospital , Melbourne , Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>ab Royal Melbourne Hospital , Melbourne </wicri:regionArea>
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</author>
<author><name sortKey="Velten, Christian" sort="Velten, Christian" uniqKey="Velten C" first="Christian" last="Velten">Christian Velten</name>
<affiliation wicri:level="1"><nlm:affiliation>ac Actelion Pharmaceuticals Ltd , Allschwil , Switzerland.</nlm:affiliation>
<country xml:lang="fr">Suisse</country>
<wicri:regionArea>ac Actelion Pharmaceuticals Ltd , Allschwil </wicri:regionArea>
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<author><name sortKey="Kolb, Stefan A" sort="Kolb, Stefan A" uniqKey="Kolb S" first="Stefan A" last="Kolb">Stefan A. Kolb</name>
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<series><title level="j">Current medical research and opinion</title>
<idno type="eISSN">1473-4877</idno>
<imprint><date when="2017" type="published">2017</date>
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<term>Niemann-Pick Disease, Type C (epidemiology)</term>
<term>Prevalence</term>
<term>Rare Diseases (epidemiology)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Humains</term>
<term>Maladie de Niemann-Pick de type C (épidémiologie)</term>
<term>Maladies rares (épidémiologie)</term>
<term>Prévalence</term>
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<term>Rare Diseases</term>
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<keywords scheme="MESH" qualifier="épidémiologie" xml:lang="fr"><term>Maladie de Niemann-Pick de type C</term>
<term>Maladies rares</term>
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<term>Prevalence</term>
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<front><div type="abstract" xml:lang="en">Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C. Patients in these at-risk groups ("clinical niches") have symptoms that are potentially related to NP-C, but go unrecognized due to other, more prevalent clinical features, and lack of awareness regarding underlying metabolic causes.</div>
</front>
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<DateCreated><Year>2017</Year>
<Month>03</Month>
<Day>09</Day>
</DateCreated>
<DateCompleted><Year>2017</Year>
<Month>05</Month>
<Day>29</Day>
</DateCompleted>
<DateRevised><Year>2017</Year>
<Month>05</Month>
<Day>29</Day>
</DateRevised>
<Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1473-4877</ISSN>
<JournalIssue CitedMedium="Internet"><Volume>33</Volume>
<Issue>5</Issue>
<PubDate><Year>2017</Year>
<Month>May</Month>
</PubDate>
</JournalIssue>
<Title>Current medical research and opinion</Title>
<ISOAbbreviation>Curr Med Res Opin</ISOAbbreviation>
</Journal>
<ArticleTitle>The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.</ArticleTitle>
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<Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C. Patients in these at-risk groups ("clinical niches") have symptoms that are potentially related to NP-C, but go unrecognized due to other, more prevalent clinical features, and lack of awareness regarding underlying metabolic causes.</AbstractText>
<AbstractText Label="METHODS" NlmCategory="METHODS">Twelve potential clinical niches identified by clinical experts were evaluated based on a comprehensive, non-systematic review of literature published to date. Relevant publications were identified by targeted literature searches of EMBASE and PubMed using key search terms specific to each niche. Articles published in English or other European languages up to 2016 were included.</AbstractText>
<AbstractText Label="FINDINGS" NlmCategory="RESULTS">Several niches were found to be relevant based on available data: movement disorders (early-onset ataxia and dystonia), organic psychosis, early-onset cholestasis/(hepato)splenomegaly, cases with relevant antenatal findings or fetal abnormalities, and patients affected by family history, consanguinity, and endogamy. Potentially relevant niches requiring further supportive data included: early-onset cognitive decline, frontotemporal dementia, parkinsonism, and chronic inflammatory CNS disease. There was relatively weak evidence to suggest amyotrophic lateral sclerosis or progressive supranuclear gaze palsy as potential niches.</AbstractText>
<AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">Several clinical niches have been identified that harbor patients at increased risk of NP-C.</AbstractText>
</Abstract>
<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Hendriksz</LastName>
<ForeName>Christian J</ForeName>
<Initials>CJ</Initials>
<AffiliationInfo><Affiliation>a Salford Royal NHS Foundation Trust , Manchester , UK.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>b University of Pretoria , Pretoria , South Africa.</Affiliation>
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<Author ValidYN="Y"><LastName>Anheim</LastName>
<ForeName>Mathieu</ForeName>
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<AffiliationInfo><Affiliation>c University of Strasbourg , Hautepierre Hospital , Strasbourg , France.</Affiliation>
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<AffiliationInfo><Affiliation>d Institute of Medical Genetics and Applied Genomics, Tübingen University , Tübingen, Germany.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>e CENTOGENE AG , Rostock , Germany.</Affiliation>
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<AffiliationInfo><Affiliation>g Great Ormond St Hospital for Children , London , UK.</Affiliation>
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<AffiliationInfo><Affiliation>h Sorbonne University , UPMC and Hôpital Pitié-Salpêtrière, Department of Nervous System Diseases , Paris , France.</Affiliation>
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<AffiliationInfo><Affiliation>i University of Groningen , Groningen , the Netherlands.</Affiliation>
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<ForeName>Anna</ForeName>
<Initials>A</Initials>
<AffiliationInfo><Affiliation>j Federal State Budget Institution, Research Center for Obstetrics , Gynecology and Perinatology , Moscow , Russia.</Affiliation>
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<AffiliationInfo><Affiliation>k Bambino Gesù Children's Hospital , Rome , Italy.</Affiliation>
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<Author ValidYN="Y"><LastName>Doss</LastName>
<ForeName>Sarah</ForeName>
<Initials>S</Initials>
<AffiliationInfo><Affiliation>l Charite University Medicine Berlin , Department of Neurology , Berlin , Germany.</Affiliation>
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<AffiliationInfo><Affiliation>m Münster University Hospital , Münster, Germany.</Affiliation>
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<ForeName>Paola</ForeName>
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<AffiliationInfo><Affiliation>n University College London, Institute of Neurology , London , UK.</Affiliation>
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<Author ValidYN="Y"><LastName>Iodice</LastName>
<ForeName>Rosa</ForeName>
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<AffiliationInfo><Affiliation>o University Federico II Naples , Naples , Italy.</Affiliation>
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</Author>
<Author ValidYN="Y"><LastName>Johnston</LastName>
<ForeName>Tracy</ForeName>
<Initials>T</Initials>
<AffiliationInfo><Affiliation>p Birmingham Women's Hospital , Birmingham , UK.</Affiliation>
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<Author ValidYN="Y"><LastName>Kelly</LastName>
<ForeName>Dierdre</ForeName>
<Initials>D</Initials>
<AffiliationInfo><Affiliation>q Birmingham Children's Hospital , Birmingham , UK.</Affiliation>
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</Author>
<Author ValidYN="Y"><LastName>Klünemann</LastName>
<ForeName>Hans-Hermann</ForeName>
<Initials>HH</Initials>
<AffiliationInfo><Affiliation>r University Clinic for Psychiatry and Psychotherapy, Regensburg University , Regensburg , Germany.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Lorenzl</LastName>
<ForeName>Stefan</ForeName>
<Initials>S</Initials>
<AffiliationInfo><Affiliation>s Ludwig Maximillian University , Munich , Germany.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>t Paracelus Medical University , Salzburg , Austria.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Padovani</LastName>
<ForeName>Alessandro</ForeName>
<Initials>A</Initials>
<AffiliationInfo><Affiliation>u Neurology Unit, Department of Clinical and Experimental Sciences , University of Brescia , Brescia , Italy.</Affiliation>
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</Author>
<Author ValidYN="Y"><LastName>Pocovi</LastName>
<ForeName>Miguel</ForeName>
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<AffiliationInfo><Affiliation>v University of Zaragoza , IISA, Zaragoza , Spain.</Affiliation>
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<Author ValidYN="Y"><LastName>Synofzik</LastName>
<ForeName>Matthis</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>w Department of Neurodegenerative Diseases , Hertie Institute for Clinical Brain Research , Tübingen, Germany.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>x German Center for Neurodegenerative Diseases (DZNE) , Tübingen, Germany.</Affiliation>
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<Author ValidYN="Y"><LastName>Terblanche</LastName>
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<Initials>A</Initials>
<AffiliationInfo><Affiliation>b University of Pretoria , Pretoria , South Africa.</Affiliation>
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<Author ValidYN="Y"><LastName>Then Bergh</LastName>
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<AffiliationInfo><Affiliation>y University of Leipzig , Department of Neurology , Leipzig , Germany.</Affiliation>
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<AffiliationInfo><Affiliation>z Hacettepe University Children's Hospital , Ankara , Turkey.</Affiliation>
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<ForeName>Christine</ForeName>
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<AffiliationInfo><Affiliation>aa CHU Strasburg , Strasburg , France.</Affiliation>
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<Author ValidYN="Y"><LastName>Velten</LastName>
<ForeName>Christian</ForeName>
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<ForeName>Stefan A</ForeName>
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<AffiliationInfo><Affiliation>ac Actelion Pharmaceuticals Ltd , Allschwil , Switzerland.</Affiliation>
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<Keyword MajorTopicYN="Y">Epidemiology</Keyword>
<Keyword MajorTopicYN="Y">Inborn errors of metabolism (IEM)</Keyword>
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