Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia
OBJECTIVESGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesishe...
Gespeichert in:
| Veröffentlicht in: | Journal of pediatric gastroenterology and nutrition 2003-11, Vol.37 (5), p.566-570 |
|---|---|
| Hauptverfasser: | , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 570 |
|---|---|
| container_issue | 5 |
| container_start_page | 566 |
| container_title | Journal of pediatric gastroenterology and nutrition |
| container_volume | 37 |
| creator | Marfaing-Koka, Anne Wolf, Martine Boyer-Neumann, Catherine Meyer, Dominique Odievre, Michel Labrune, Philippe |
| description | OBJECTIVESGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesishepatocyte, endothelial cell, or both. The role of inflammation was investigated by the measurement of tumor necrosis factor alpha (TNF-α) and interleukin-6 (IL-6) levels in plasma.
METHODSTwenty-seven patients with GSD-Ia were evaluated, as were 14 patients with other types of GSD and 30 healthy control subjects. Of the 41 patients with GSD, 15 also had hepatic adenoma (14 patients with GSD-Ia and 1 with GSD type III).
RESULTSIn patients with GSD-Ia, there was a two-fold increase in all hepatocyte-synthesized proteins (i.e., factor VII, protein C, C4b binding protein) compared with control subjects and patients with other types of GSD. The proteins with mixed endothelial and hepatocyte origin (i.e., antithrombin and protein S) also were significantly increased but to a lesser extent. In contrast, the mean concentration of von Willebrand factor, which is exclusively synthesized in endothelial cells, was normal, as was the concentration of TNF-α and IL-6.
CONCLUSIONSThese results suggest that the hyperproteinemia of GSD-Ia (including hemostatic proteins) is attributable to hepatocyte dysfunction and not related to an inflammatory process. |
| doi_str_mv | 10.1097/00005176-200311000-00011 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_71317205</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>71317205</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4361-6b9c304ce0565e06f51a6d72fd10e4a1afabdc3c36294619b5ee5705c78986173</originalsourceid><addsrcrecordid>eNp1kU1PGzEQhq2KqgToX6h8gdtSz_pr94hogUiRQCqcLcc7C1u8dmpvivLvcUgKp1qyRyM974z0mBAK7BxYq7-zciRoVdWMcYDSVeUCfCIzkFxVomFwQGas1rqqAdQhOcr5d0G0kOwLOQQhG9BtMyPP8-AS2owdXeBf9JnGnt7gGPNkp8HRuxQnHEKmNiGdhw5XWJ4wbbF56L0dx8LFQIdAr_3GxUcM9NcUk31E-mPI29H0frMqYXtCPvfWZ_y6r8fk4ern_eVNtbi9nl9eLConuIJKLVvHmXDIpJLIVC_Bqk7XfQcMhQXb22XnuOOqboWCdikRpWbS6aZtFGh-TM52c1cp_lljnsw4ZIfe24BxnY0GDrpmsoDNDnQp5pywN6s0jDZtDDCzFW3-iTbvos2b6BL9tt-xXo7YfQT3Zgtwugdsdtb3yQY35A9O1qIF1RZO7LiX6CdM-dmvXzCZJ7R-ejL_-2j-Ctf7lNc</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>71317205</pqid></control><display><type>article</type><title>Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia</title><source>MEDLINE</source><source>Journals@Ovid Complete</source><source>Wiley Online Library All Journals</source><creator>Marfaing-Koka, Anne ; Wolf, Martine ; Boyer-Neumann, Catherine ; Meyer, Dominique ; Odievre, Michel ; Labrune, Philippe</creator><creatorcontrib>Marfaing-Koka, Anne ; Wolf, Martine ; Boyer-Neumann, Catherine ; Meyer, Dominique ; Odievre, Michel ; Labrune, Philippe</creatorcontrib><description>OBJECTIVESGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesishepatocyte, endothelial cell, or both. The role of inflammation was investigated by the measurement of tumor necrosis factor alpha (TNF-α) and interleukin-6 (IL-6) levels in plasma.
METHODSTwenty-seven patients with GSD-Ia were evaluated, as were 14 patients with other types of GSD and 30 healthy control subjects. Of the 41 patients with GSD, 15 also had hepatic adenoma (14 patients with GSD-Ia and 1 with GSD type III).
RESULTSIn patients with GSD-Ia, there was a two-fold increase in all hepatocyte-synthesized proteins (i.e., factor VII, protein C, C4b binding protein) compared with control subjects and patients with other types of GSD. The proteins with mixed endothelial and hepatocyte origin (i.e., antithrombin and protein S) also were significantly increased but to a lesser extent. In contrast, the mean concentration of von Willebrand factor, which is exclusively synthesized in endothelial cells, was normal, as was the concentration of TNF-α and IL-6.
CONCLUSIONSThese results suggest that the hyperproteinemia of GSD-Ia (including hemostatic proteins) is attributable to hepatocyte dysfunction and not related to an inflammatory process.</description><identifier>ISSN: 0277-2116</identifier><identifier>EISSN: 1536-4801</identifier><identifier>DOI: 10.1097/00005176-200311000-00011</identifier><identifier>PMID: 14581798</identifier><identifier>CODEN: JPGND6</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott Williams & Wilkins, Inc</publisher><subject>Adolescent ; Adult ; Antithrombins - analysis ; Biological and medical sciences ; Blood Proteins - analysis ; Carbohydrates (enzymatic deficiencies). Glycogenosis ; Child ; Child, Preschool ; Complement Inactivator Proteins ; Endothelial Cells - metabolism ; Errors of metabolism ; Factor VII - analysis ; Glycogen Storage Disease Type I - blood ; Glycoproteins - blood ; Hemostasis ; Humans ; Inflammation - blood ; Interleukin-6 - analysis ; Liver - metabolism ; Medical sciences ; Metabolic diseases ; Plasminogen Activator Inhibitor 1 - blood ; Protein C - analysis ; Protein S - analysis ; Triglycerides - blood ; Tumor Necrosis Factor-alpha - analysis ; von Willebrand Factor - analysis</subject><ispartof>Journal of pediatric gastroenterology and nutrition, 2003-11, Vol.37 (5), p.566-570</ispartof><rights>2003 Lippincott Williams & Wilkins, Inc.</rights><rights>2004 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4361-6b9c304ce0565e06f51a6d72fd10e4a1afabdc3c36294619b5ee5705c78986173</citedby><cites>FETCH-LOGICAL-c4361-6b9c304ce0565e06f51a6d72fd10e4a1afabdc3c36294619b5ee5705c78986173</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=15249169$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/14581798$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marfaing-Koka, Anne</creatorcontrib><creatorcontrib>Wolf, Martine</creatorcontrib><creatorcontrib>Boyer-Neumann, Catherine</creatorcontrib><creatorcontrib>Meyer, Dominique</creatorcontrib><creatorcontrib>Odievre, Michel</creatorcontrib><creatorcontrib>Labrune, Philippe</creatorcontrib><title>Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia</title><title>Journal of pediatric gastroenterology and nutrition</title><addtitle>J Pediatr Gastroenterol Nutr</addtitle><description>OBJECTIVESGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesishepatocyte, endothelial cell, or both. The role of inflammation was investigated by the measurement of tumor necrosis factor alpha (TNF-α) and interleukin-6 (IL-6) levels in plasma.
METHODSTwenty-seven patients with GSD-Ia were evaluated, as were 14 patients with other types of GSD and 30 healthy control subjects. Of the 41 patients with GSD, 15 also had hepatic adenoma (14 patients with GSD-Ia and 1 with GSD type III).
RESULTSIn patients with GSD-Ia, there was a two-fold increase in all hepatocyte-synthesized proteins (i.e., factor VII, protein C, C4b binding protein) compared with control subjects and patients with other types of GSD. The proteins with mixed endothelial and hepatocyte origin (i.e., antithrombin and protein S) also were significantly increased but to a lesser extent. In contrast, the mean concentration of von Willebrand factor, which is exclusively synthesized in endothelial cells, was normal, as was the concentration of TNF-α and IL-6.
CONCLUSIONSThese results suggest that the hyperproteinemia of GSD-Ia (including hemostatic proteins) is attributable to hepatocyte dysfunction and not related to an inflammatory process.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Antithrombins - analysis</subject><subject>Biological and medical sciences</subject><subject>Blood Proteins - analysis</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Complement Inactivator Proteins</subject><subject>Endothelial Cells - metabolism</subject><subject>Errors of metabolism</subject><subject>Factor VII - analysis</subject><subject>Glycogen Storage Disease Type I - blood</subject><subject>Glycoproteins - blood</subject><subject>Hemostasis</subject><subject>Humans</subject><subject>Inflammation - blood</subject><subject>Interleukin-6 - analysis</subject><subject>Liver - metabolism</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Plasminogen Activator Inhibitor 1 - blood</subject><subject>Protein C - analysis</subject><subject>Protein S - analysis</subject><subject>Triglycerides - blood</subject><subject>Tumor Necrosis Factor-alpha - analysis</subject><subject>von Willebrand Factor - analysis</subject><issn>0277-2116</issn><issn>1536-4801</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kU1PGzEQhq2KqgToX6h8gdtSz_pr94hogUiRQCqcLcc7C1u8dmpvivLvcUgKp1qyRyM974z0mBAK7BxYq7-zciRoVdWMcYDSVeUCfCIzkFxVomFwQGas1rqqAdQhOcr5d0G0kOwLOQQhG9BtMyPP8-AS2owdXeBf9JnGnt7gGPNkp8HRuxQnHEKmNiGdhw5XWJ4wbbF56L0dx8LFQIdAr_3GxUcM9NcUk31E-mPI29H0frMqYXtCPvfWZ_y6r8fk4ern_eVNtbi9nl9eLConuIJKLVvHmXDIpJLIVC_Bqk7XfQcMhQXb22XnuOOqboWCdikRpWbS6aZtFGh-TM52c1cp_lljnsw4ZIfe24BxnY0GDrpmsoDNDnQp5pywN6s0jDZtDDCzFW3-iTbvos2b6BL9tt-xXo7YfQT3Zgtwugdsdtb3yQY35A9O1qIF1RZO7LiX6CdM-dmvXzCZJ7R-ejL_-2j-Ctf7lNc</recordid><startdate>200311</startdate><enddate>200311</enddate><creator>Marfaing-Koka, Anne</creator><creator>Wolf, Martine</creator><creator>Boyer-Neumann, Catherine</creator><creator>Meyer, Dominique</creator><creator>Odievre, Michel</creator><creator>Labrune, Philippe</creator><general>Lippincott Williams & Wilkins, Inc</general><general>Lippincott</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>7X8</scope></search><sort><creationdate>200311</creationdate><title>Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia</title><author>Marfaing-Koka, Anne ; Wolf, Martine ; Boyer-Neumann, Catherine ; Meyer, Dominique ; Odievre, Michel ; Labrune, Philippe</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4361-6b9c304ce0565e06f51a6d72fd10e4a1afabdc3c36294619b5ee5705c78986173</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Antithrombins - analysis</topic><topic>Biological and medical sciences</topic><topic>Blood Proteins - analysis</topic><topic>Carbohydrates (enzymatic deficiencies). Glycogenosis</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Complement Inactivator Proteins</topic><topic>Endothelial Cells - metabolism</topic><topic>Errors of metabolism</topic><topic>Factor VII - analysis</topic><topic>Glycogen Storage Disease Type I - blood</topic><topic>Glycoproteins - blood</topic><topic>Hemostasis</topic><topic>Humans</topic><topic>Inflammation - blood</topic><topic>Interleukin-6 - analysis</topic><topic>Liver - metabolism</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Plasminogen Activator Inhibitor 1 - blood</topic><topic>Protein C - analysis</topic><topic>Protein S - analysis</topic><topic>Triglycerides - blood</topic><topic>Tumor Necrosis Factor-alpha - analysis</topic><topic>von Willebrand Factor - analysis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marfaing-Koka, Anne</creatorcontrib><creatorcontrib>Wolf, Martine</creatorcontrib><creatorcontrib>Boyer-Neumann, Catherine</creatorcontrib><creatorcontrib>Meyer, Dominique</creatorcontrib><creatorcontrib>Odievre, Michel</creatorcontrib><creatorcontrib>Labrune, Philippe</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>MEDLINE - Academic</collection><jtitle>Journal of pediatric gastroenterology and nutrition</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marfaing-Koka, Anne</au><au>Wolf, Martine</au><au>Boyer-Neumann, Catherine</au><au>Meyer, Dominique</au><au>Odievre, Michel</au><au>Labrune, Philippe</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia</atitle><jtitle>Journal of pediatric gastroenterology and nutrition</jtitle><addtitle>J Pediatr Gastroenterol Nutr</addtitle><date>2003-11</date><risdate>2003</risdate><volume>37</volume><issue>5</issue><spage>566</spage><epage>570</epage><pages>566-570</pages><issn>0277-2116</issn><eissn>1536-4801</eissn><coden>JPGND6</coden><abstract>OBJECTIVESGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesishepatocyte, endothelial cell, or both. The role of inflammation was investigated by the measurement of tumor necrosis factor alpha (TNF-α) and interleukin-6 (IL-6) levels in plasma.
METHODSTwenty-seven patients with GSD-Ia were evaluated, as were 14 patients with other types of GSD and 30 healthy control subjects. Of the 41 patients with GSD, 15 also had hepatic adenoma (14 patients with GSD-Ia and 1 with GSD type III).
RESULTSIn patients with GSD-Ia, there was a two-fold increase in all hepatocyte-synthesized proteins (i.e., factor VII, protein C, C4b binding protein) compared with control subjects and patients with other types of GSD. The proteins with mixed endothelial and hepatocyte origin (i.e., antithrombin and protein S) also were significantly increased but to a lesser extent. In contrast, the mean concentration of von Willebrand factor, which is exclusively synthesized in endothelial cells, was normal, as was the concentration of TNF-α and IL-6.
CONCLUSIONSThese results suggest that the hyperproteinemia of GSD-Ia (including hemostatic proteins) is attributable to hepatocyte dysfunction and not related to an inflammatory process.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott Williams & Wilkins, Inc</pub><pmid>14581798</pmid><doi>10.1097/00005176-200311000-00011</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0277-2116 |
| ispartof | Journal of pediatric gastroenterology and nutrition, 2003-11, Vol.37 (5), p.566-570 |
| issn | 0277-2116 1536-4801 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_71317205 |
| source | MEDLINE; Journals@Ovid Complete; Wiley Online Library All Journals |
| subjects | Adolescent Adult Antithrombins - analysis Biological and medical sciences Blood Proteins - analysis Carbohydrates (enzymatic deficiencies). Glycogenosis Child Child, Preschool Complement Inactivator Proteins Endothelial Cells - metabolism Errors of metabolism Factor VII - analysis Glycogen Storage Disease Type I - blood Glycoproteins - blood Hemostasis Humans Inflammation - blood Interleukin-6 - analysis Liver - metabolism Medical sciences Metabolic diseases Plasminogen Activator Inhibitor 1 - blood Protein C - analysis Protein S - analysis Triglycerides - blood Tumor Necrosis Factor-alpha - analysis von Willebrand Factor - analysis |
| title | Increased Levels of Hemostatic Proteins are Independent of Inflammation in Glycogen Storage Disease Type Ia |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-28T10%3A25%3A30IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Increased%20Levels%20of%20Hemostatic%20Proteins%20are%20Independent%20of%20Inflammation%20in%20Glycogen%20Storage%20Disease%20Type%20Ia&rft.jtitle=Journal%20of%20pediatric%20gastroenterology%20and%20nutrition&rft.au=Marfaing-Koka,%20Anne&rft.date=2003-11&rft.volume=37&rft.issue=5&rft.spage=566&rft.epage=570&rft.pages=566-570&rft.issn=0277-2116&rft.eissn=1536-4801&rft.coden=JPGND6&rft_id=info:doi/10.1097/00005176-200311000-00011&rft_dat=%3Cproquest_cross%3E71317205%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=71317205&rft_id=info:pmid/14581798&rfr_iscdi=true |