Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Identifieur interne : 002183 ( Main/Exploration ); précédent : 002182; suivant : 002184Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Auteurs : M. M. U. Chowdhury [Royaume-Uni] ; E. J. Williams ; J. S. Morris ; B. J. M. Ferguson ; A. D. Mcgregor [Royaume-Uni] ; A. R. Hedges ; J. D. Stamatakis ; F. M. Pope [Royaume-Uni]Source :
- British Journal of Dermatology [ 0007-0963 ] ; 2002-12.
English descriptors
- Teeft :
- A1at, Antitrypsin, Antitrypsin deficiency, Bayer, Bayer corporation, Bridgend, British association, British journal, Case report, Dermatologist, Dermatology, Functional activity, Human enzyme, Liver disease, Liver transplantation, Maintenance therapy, Panniculitis, Phenotype, Prolastin, Rapid progression, Septicaemia, Severe panniculitis, Skin necrosis, Trunk distribution, Upper thighs.
Abstract
Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin®, Bayer, Bridgend, U.K.) has been life‐saving.
Url:
DOI: 10.1046/j.1365-2133.2002.05095.x
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream Istex, to step Corpus: 001B82
- to stream Istex, to step Curation: 001B82
- to stream Istex, to step Checkpoint: 001010
- to stream Main, to step Merge: 002205
- to stream Main, to step Curation: 002183
Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)</title><author><name sortKey="Chowdhury, M M U" sort="Chowdhury, M M U" uniqKey="Chowdhury M" first="M. M. U." last="Chowdhury">M. M. U. Chowdhury</name></author><author><name sortKey="Williams, E J" sort="Williams, E J" uniqKey="Williams E" first="E. J." last="Williams">E. J. Williams</name></author><author><name sortKey="Morris, J S" sort="Morris, J S" uniqKey="Morris J" first="J. S." last="Morris">J. S. Morris</name></author><author><name sortKey="Ferguson, B J M" sort="Ferguson, B J M" uniqKey="Ferguson B" first="B. J. M." last="Ferguson">B. J. M. Ferguson</name></author><author><name sortKey="Mcgregor, A D" sort="Mcgregor, A D" uniqKey="Mcgregor A" first="A. D." last="Mcgregor">A. D. Mcgregor</name></author><author><name sortKey="Hedges, A R" sort="Hedges, A R" uniqKey="Hedges A" first="A. R." last="Hedges">A. R. Hedges</name></author><author><name sortKey="Stamatakis, J D" sort="Stamatakis, J D" uniqKey="Stamatakis J" first="J. D." last="Stamatakis">J. D. Stamatakis</name></author><author><name sortKey="Pope, F M" sort="Pope, F M" uniqKey="Pope F" first="F. M." last="Pope">F. M. Pope</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:320A14EECB86A0B293D0B68ADBE525E814EC4D08</idno><date when="2002" year="2002">2002</date><idno type="doi">10.1046/j.1365-2133.2002.05095.x</idno><idno type="url">https://api.istex.fr/ark:/67375/WNG-LNM9NQ39-D/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001B82</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001B82</idno><idno type="wicri:Area/Istex/Curation">001B82</idno><idno type="wicri:Area/Istex/Checkpoint">001010</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">001010</idno><idno type="wicri:doubleKey">0007-0963:2002:Chowdhury M:severe:panniculitis:caused</idno><idno type="wicri:Area/Main/Merge">002205</idno><idno type="wicri:Area/Main/Curation">002183</idno><idno type="wicri:Area/Main/Exploration">002183</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main">Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)</title><author><name sortKey="Chowdhury, M M U" sort="Chowdhury, M M U" uniqKey="Chowdhury M" first="M. M. U." last="Chowdhury">M. M. U. Chowdhury</name><affiliation wicri:level="1"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Dermatology, University Hospital of Wales, Cardiff CF14 4XW</wicri:regionArea><wicri:noRegion>Cardiff CF14 4XW</wicri:noRegion></affiliation></author><author><name sortKey="Williams, E J" sort="Williams, E J" uniqKey="Williams E" first="E. J." last="Williams">E. J. Williams</name><affiliation><wicri:noCountry code="subField"></wicri:noCountry></affiliation></author><author><name sortKey="Morris, J S" sort="Morris, J S" uniqKey="Morris J" first="J. S." last="Morris">J. S. Morris</name><affiliation><wicri:noCountry code="subField"></wicri:noCountry></affiliation></author><author><name sortKey="Ferguson, B J M" sort="Ferguson, B J M" uniqKey="Ferguson B" first="B. J. M." last="Ferguson">B. J. M. Ferguson</name><affiliation><wicri:noCountry code="subField"></wicri:noCountry></affiliation></author><author><name sortKey="Mcgregor, A D" sort="Mcgregor, A D" uniqKey="Mcgregor A" first="A. D." last="Mcgregor">A. D. Mcgregor</name><affiliation wicri:level="1"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Plastic Surgery, Morriston Hospital, Swansea</wicri:regionArea><wicri:noRegion>Swansea</wicri:noRegion></affiliation></author><author><name sortKey="Hedges, A R" sort="Hedges, A R" uniqKey="Hedges A" first="A. R." last="Hedges">A. R. Hedges</name><affiliation><wicri:noCountry code="no comma">Surgery and</wicri:noCountry></affiliation></author><author><name sortKey="Stamatakis, J D" sort="Stamatakis, J D" uniqKey="Stamatakis J" first="J. D." last="Stamatakis">J. D. Stamatakis</name><affiliation><wicri:noCountry code="no comma">Surgery and</wicri:noCountry></affiliation></author><author><name sortKey="Pope, F M" sort="Pope, F M" uniqKey="Pope F" first="F. M." last="Pope">F. M. Pope</name><affiliation wicri:level="1"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Dermatology, Princess of Wales Hospital, Bridgend CF31 1RQ</wicri:regionArea><wicri:noRegion>Bridgend CF31 1RQ</wicri:noRegion></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">British Journal of Dermatology</title><title level="j" type="alt">BRITISH JOURNAL OF DERMATOLOGY</title><idno type="ISSN">0007-0963</idno><idno type="eISSN">1365-2133</idno><imprint><biblScope unit="vol">147</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="1258">1258</biblScope><biblScope unit="page" to="1261">1261</biblScope><biblScope unit="page-count">4</biblScope><publisher>Blackwell Science Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2002-12">2002-12</date></imprint><idno type="ISSN">0007-0963</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0007-0963</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>A1at</term><term>Antitrypsin</term><term>Antitrypsin deficiency</term><term>Bayer</term><term>Bayer corporation</term><term>Bridgend</term><term>British association</term><term>British journal</term><term>Case report</term><term>Dermatologist</term><term>Dermatology</term><term>Functional activity</term><term>Human enzyme</term><term>Liver disease</term><term>Liver transplantation</term><term>Maintenance therapy</term><term>Panniculitis</term><term>Phenotype</term><term>Prolastin</term><term>Rapid progression</term><term>Septicaemia</term><term>Severe panniculitis</term><term>Skin necrosis</term><term>Trunk distribution</term><term>Upper thighs</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin®, Bayer, Bridgend, U.K.) has been life‐saving.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country></list><tree><noCountry><name sortKey="Ferguson, B J M" sort="Ferguson, B J M" uniqKey="Ferguson B" first="B. J. M." last="Ferguson">B. J. M. Ferguson</name><name sortKey="Hedges, A R" sort="Hedges, A R" uniqKey="Hedges A" first="A. R." last="Hedges">A. R. Hedges</name><name sortKey="Morris, J S" sort="Morris, J S" uniqKey="Morris J" first="J. S." last="Morris">J. S. Morris</name><name sortKey="Stamatakis, J D" sort="Stamatakis, J D" uniqKey="Stamatakis J" first="J. D." last="Stamatakis">J. D. Stamatakis</name><name sortKey="Williams, E J" sort="Williams, E J" uniqKey="Williams E" first="E. J." last="Williams">E. J. Williams</name></noCountry><country name="Royaume-Uni"><noRegion><name sortKey="Chowdhury, M M U" sort="Chowdhury, M M U" uniqKey="Chowdhury M" first="M. M. U." last="Chowdhury">M. M. U. Chowdhury</name></noRegion><name sortKey="Mcgregor, A D" sort="Mcgregor, A D" uniqKey="Mcgregor A" first="A. D." last="Mcgregor">A. D. Mcgregor</name><name sortKey="Pope, F M" sort="Pope, F M" uniqKey="Pope F" first="F. M." last="Pope">F. M. Pope</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Sante/explor/ChloroquineV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 002183 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 002183 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Sante
|area= ChloroquineV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= ISTEX:320A14EECB86A0B293D0B68ADBE525E814EC4D08
|texte= Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
}}
| This area was generated with Dilib version V0.6.33. |  |