Bile Porphyrin Analysis in the Evaluation of Variegate Porphyria
VARIEGATE porphyria is an autosomal dominant disorder characterized clinically by attacks of neurologic dysfunction, photocutaneous lesions, or both. The symptoms that occur during attacks, chiefly abdominal pain, are identical to those of acute intermittent porphyria. 1 2 3 4 5 Skin lesions may be...
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| Veröffentlicht in: | The New England journal of medicine 1991-05, Vol.324 (20), p.1408-1411 |
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| container_title | The New England journal of medicine |
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| creator | Logan, George M Weimer, Mary K Ellefson, Mark Pierach, Claus A Bloomer, Joseph R |
| description | VARIEGATE porphyria is an autosomal dominant disorder characterized clinically by attacks of neurologic dysfunction, photocutaneous lesions, or both. The symptoms that occur during attacks, chiefly abdominal pain, are identical to those of acute intermittent porphyria.
1
2
3
4
5
Skin lesions may be present when patients are not having attacks of neurologic dysfunction, but they were present in less than half of the affected persons in some studies.
2
Moreover, the skin lesions may be mild and may not be mentioned by the patient.
During acute attacks, the urinary abnormalities of variegate porphyria are indistinguishable from those of acute intermittent porphyria, with marked elevations in . . . |
| doi_str_mv | 10.1056/NEJM199105163242005 |
| format | Article |
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1
2
3
4
5
Skin lesions may be present when patients are not having attacks of neurologic dysfunction, but they were present in less than half of the affected persons in some studies.
2
Moreover, the skin lesions may be mild and may not be mentioned by the patient.
During acute attacks, the urinary abnormalities of variegate porphyria are indistinguishable from those of acute intermittent porphyria, with marked elevations in . . .</description><identifier>ISSN: 0028-4793</identifier><identifier>EISSN: 1533-4406</identifier><identifier>DOI: 10.1056/NEJM199105163242005</identifier><identifier>PMID: 2020296</identifier><identifier>CODEN: NEJMAG</identifier><language>eng</language><publisher>Boston, MA: Massachusetts Medical Society</publisher><subject>Abdomen ; Adolescent ; Adult ; Aged ; Bile ; Bile - chemistry ; Biological and medical sciences ; Chromatography ; Enzymes ; Excretion ; Feces ; Feces - chemistry ; Female ; Gastroenterology ; Genetic disorders ; Humans ; levels ; Liver diseases ; Male ; man ; Medical research ; Medical sciences ; Metabolic diseases ; Metabolism ; Middle Aged ; Oxidation ; Pain ; Patients ; Porphyria ; Porphyrias - diagnosis ; Porphyrias - metabolism ; porphyrin ; Porphyrins ; Porphyrins - analysis ; Porphyrins - metabolism ; Skin</subject><ispartof>The New England journal of medicine, 1991-05, Vol.324 (20), p.1408-1411</ispartof><rights>1992 INIST-CNRS</rights><rights>Copyright Massachusetts Medical Society May 16, 1991</rights><rights>Copyright Massachusetts Medical Society, Publishing Division May 16, 1991</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c535t-3da38a1b0e39c84b7f8d545f6594cee0db88e15f4ef6648360bfaa9b22000c583</citedby><cites>FETCH-LOGICAL-c535t-3da38a1b0e39c84b7f8d545f6594cee0db88e15f4ef6648360bfaa9b22000c583</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.nejm.org/doi/pdf/10.1056/NEJM199105163242005$$EPDF$$P50$$Gmms$$H</linktopdf><linktohtml>$$Uhttps://www.nejm.org/doi/full/10.1056/NEJM199105163242005$$EHTML$$P50$$Gmms$$H</linktohtml><link.rule.ids>314,776,780,2746,2747,26080,27901,27902,52357,54039</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=5314041$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/2020296$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Logan, George M</creatorcontrib><creatorcontrib>Weimer, Mary K</creatorcontrib><creatorcontrib>Ellefson, Mark</creatorcontrib><creatorcontrib>Pierach, Claus A</creatorcontrib><creatorcontrib>Bloomer, Joseph R</creatorcontrib><title>Bile Porphyrin Analysis in the Evaluation of Variegate Porphyria</title><title>The New England journal of medicine</title><addtitle>N Engl J Med</addtitle><description>VARIEGATE porphyria is an autosomal dominant disorder characterized clinically by attacks of neurologic dysfunction, photocutaneous lesions, or both. The symptoms that occur during attacks, chiefly abdominal pain, are identical to those of acute intermittent porphyria.
1
2
3
4
5
Skin lesions may be present when patients are not having attacks of neurologic dysfunction, but they were present in less than half of the affected persons in some studies.
2
Moreover, the skin lesions may be mild and may not be mentioned by the patient.
During acute attacks, the urinary abnormalities of variegate porphyria are indistinguishable from those of acute intermittent porphyria, with marked elevations in . . .</description><subject>Abdomen</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Bile</subject><subject>Bile - chemistry</subject><subject>Biological and medical sciences</subject><subject>Chromatography</subject><subject>Enzymes</subject><subject>Excretion</subject><subject>Feces</subject><subject>Feces - chemistry</subject><subject>Female</subject><subject>Gastroenterology</subject><subject>Genetic disorders</subject><subject>Humans</subject><subject>levels</subject><subject>Liver diseases</subject><subject>Male</subject><subject>man</subject><subject>Medical research</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Metabolism</subject><subject>Middle Aged</subject><subject>Oxidation</subject><subject>Pain</subject><subject>Patients</subject><subject>Porphyria</subject><subject>Porphyrias - diagnosis</subject><subject>Porphyrias - metabolism</subject><subject>porphyrin</subject><subject>Porphyrins</subject><subject>Porphyrins - analysis</subject><subject>Porphyrins - metabolism</subject><subject>Skin</subject><issn>0028-4793</issn><issn>1533-4406</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1991</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BEC</sourceid><sourceid>BENPR</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNqFkVtLAzEQhYMoWi-_QIRFxRdZTTJJmrxZpd7w9qC-LtltYrfspSa7Qv-9KV0qiJfMQwbmOzMcDkK7BJ8QzMXpw_D2nigVeiKAMooxX0E9wgFixrBYRT2MqYxZX8EG2vR-gsMjTK2jdYpDKdFDZ-d5YaKn2k3HM5dX0aDSxcznPgp9MzbR8EMXrW7yuopqG71ql5s33Xwp9DZas7rwZqf7t9DL5fD54jq-e7y6uRjcxRkH3sQw0iA1SbEBlUmW9q0cccat4IplxuBRKqUh3DJjhWASBE6t1iqlwRXOuIQtdLTYO3X1e2t8k5S5z0xR6MrUrU8k5jS45v-CRFDVB0oCuP8NnNStC_59QikoTgHmZw9-g4iSwEifURUoWFCZq713xiZTl5fazRKCk3lWyQ9ZBdVet7tNSzNaarpwwvywm2uf6cI6XWW5X2IcCMNs7uN4gZWlTyozKf88-gnCuqSw</recordid><startdate>19910516</startdate><enddate>19910516</enddate><creator>Logan, George M</creator><creator>Weimer, Mary K</creator><creator>Ellefson, Mark</creator><creator>Pierach, Claus A</creator><creator>Bloomer, Joseph R</creator><general>Massachusetts Medical Society</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>0TZ</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>8AO</scope><scope>8C1</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AN0</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BEC</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>HCIFZ</scope><scope>K0Y</scope><scope>LK8</scope><scope>M0R</scope><scope>M0T</scope><scope>M1P</scope><scope>M2M</scope><scope>M2O</scope><scope>M2P</scope><scope>M7P</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7T3</scope><scope>8FD</scope><scope>FR3</scope><scope>P64</scope><scope>7X8</scope></search><sort><creationdate>19910516</creationdate><title>Bile Porphyrin Analysis in the Evaluation of Variegate Porphyria</title><author>Logan, George M ; Weimer, Mary K ; Ellefson, Mark ; Pierach, Claus A ; Bloomer, Joseph R</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c535t-3da38a1b0e39c84b7f8d545f6594cee0db88e15f4ef6648360bfaa9b22000c583</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1991</creationdate><topic>Abdomen</topic><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Bile</topic><topic>Bile - chemistry</topic><topic>Biological and medical sciences</topic><topic>Chromatography</topic><topic>Enzymes</topic><topic>Excretion</topic><topic>Feces</topic><topic>Feces - chemistry</topic><topic>Female</topic><topic>Gastroenterology</topic><topic>Genetic disorders</topic><topic>Humans</topic><topic>levels</topic><topic>Liver diseases</topic><topic>Male</topic><topic>man</topic><topic>Medical research</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Metabolism</topic><topic>Middle Aged</topic><topic>Oxidation</topic><topic>Pain</topic><topic>Patients</topic><topic>Porphyria</topic><topic>Porphyrias - diagnosis</topic><topic>Porphyrias - metabolism</topic><topic>porphyrin</topic><topic>Porphyrins</topic><topic>Porphyrins - analysis</topic><topic>Porphyrins - metabolism</topic><topic>Skin</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Logan, George M</creatorcontrib><creatorcontrib>Weimer, Mary K</creatorcontrib><creatorcontrib>Ellefson, Mark</creatorcontrib><creatorcontrib>Pierach, Claus A</creatorcontrib><creatorcontrib>Bloomer, Joseph R</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Pharma and Biotech Premium PRO</collection><collection>Nursing & Allied Health Database</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central UK/Ireland</collection><collection>British Nursing Database</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>eLibrary</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>SciTech Premium Collection</collection><collection>New England Journal of Medicine</collection><collection>ProQuest Biological Science Collection</collection><collection>Consumer Health Database</collection><collection>Healthcare Administration Database</collection><collection>Medical Database</collection><collection>Psychology Database</collection><collection>ProQuest research library</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>Research Library (Corporate)</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><collection>Human Genome Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>The New England journal of medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Logan, George M</au><au>Weimer, Mary K</au><au>Ellefson, Mark</au><au>Pierach, Claus A</au><au>Bloomer, Joseph R</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Bile Porphyrin Analysis in the Evaluation of Variegate Porphyria</atitle><jtitle>The New England journal of medicine</jtitle><addtitle>N Engl J Med</addtitle><date>1991-05-16</date><risdate>1991</risdate><volume>324</volume><issue>20</issue><spage>1408</spage><epage>1411</epage><pages>1408-1411</pages><issn>0028-4793</issn><eissn>1533-4406</eissn><coden>NEJMAG</coden><abstract>VARIEGATE porphyria is an autosomal dominant disorder characterized clinically by attacks of neurologic dysfunction, photocutaneous lesions, or both. The symptoms that occur during attacks, chiefly abdominal pain, are identical to those of acute intermittent porphyria.
1
2
3
4
5
Skin lesions may be present when patients are not having attacks of neurologic dysfunction, but they were present in less than half of the affected persons in some studies.
2
Moreover, the skin lesions may be mild and may not be mentioned by the patient.
During acute attacks, the urinary abnormalities of variegate porphyria are indistinguishable from those of acute intermittent porphyria, with marked elevations in . . .</abstract><cop>Boston, MA</cop><pub>Massachusetts Medical Society</pub><pmid>2020296</pmid><doi>10.1056/NEJM199105163242005</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | The New England journal of medicine, 1991-05, Vol.324 (20), p.1408-1411 |
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| subjects | Abdomen Adolescent Adult Aged Bile Bile - chemistry Biological and medical sciences Chromatography Enzymes Excretion Feces Feces - chemistry Female Gastroenterology Genetic disorders Humans levels Liver diseases Male man Medical research Medical sciences Metabolic diseases Metabolism Middle Aged Oxidation Pain Patients Porphyria Porphyrias - diagnosis Porphyrias - metabolism porphyrin Porphyrins Porphyrins - analysis Porphyrins - metabolism Skin |
| title | Bile Porphyrin Analysis in the Evaluation of Variegate Porphyria |
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