Long-term outcome in Serbian patients with Wilson disease
Background and purpose: To investigate survival rates, prognostic factors, and causes of death in Wilson disease (WD). Methods: In the years 1980–2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form,...
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| Veröffentlicht in: | European journal of neurology 2009-07, Vol.16 (7), p.852-857 |
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| creator | Svetel, M. Pekmezović, T. Petrović, I. Tomić, A. Kresojević, N. Ješić, R. Kažić, S. Raičević, R. Stefanović, D. Delibašić, N. Živanović, D. ?or?ević, M. Kostić, V. S. |
| description | Background and purpose: To investigate survival rates, prognostic factors, and causes of death in Wilson disease (WD).
Methods: In the years 1980–2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form, and data were missing for six patients). The duration of follow‐up for patients alive was 11.1 ± 8.8 years.
Results: After initiation of treatment (d‐penicillamine and zinc salts), 79% of patients had a stable or improved course of disease. Despite early diagnosis and appropriate therapy, 15 patients still had a relentlessly progressive course. Thirty patients died. The cumulative probability of survival in a 15‐year period for the whole group was 76.7 ± 4.9%. Better prognosis of WD was associated with male sex, younger age at onset, neurologic form of the disease, and treatment continuity. Causes of death were predominantly related to hepatic failure (16 patients), but also suicide (four patients) and cancer (three patients).
Conclusion: Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high. |
| doi_str_mv | 10.1111/j.1468-1331.2009.02607.x |
| format | Article |
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Methods: In the years 1980–2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form, and data were missing for six patients). The duration of follow‐up for patients alive was 11.1 ± 8.8 years.
Results: After initiation of treatment (d‐penicillamine and zinc salts), 79% of patients had a stable or improved course of disease. Despite early diagnosis and appropriate therapy, 15 patients still had a relentlessly progressive course. Thirty patients died. The cumulative probability of survival in a 15‐year period for the whole group was 76.7 ± 4.9%. Better prognosis of WD was associated with male sex, younger age at onset, neurologic form of the disease, and treatment continuity. Causes of death were predominantly related to hepatic failure (16 patients), but also suicide (four patients) and cancer (three patients).
Conclusion: Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high.</description><identifier>ISSN: 1351-5101</identifier><identifier>EISSN: 1468-1331</identifier><identifier>EISSN: 1471-0552</identifier><identifier>DOI: 10.1111/j.1468-1331.2009.02607.x</identifier><identifier>PMID: 19473354</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Age of Onset ; Cause of Death ; causes of death ; Chelating Agents - therapeutic use ; Cohort Studies ; Female ; Hepatolenticular Degeneration - diagnosis ; Hepatolenticular Degeneration - drug therapy ; Hepatolenticular Degeneration - mortality ; Hepatolenticular Degeneration - physiopathology ; Humans ; Male ; mortality ; Penicillamine - therapeutic use ; Prognosis ; Retrospective Studies ; Serbia ; Wilson disease</subject><ispartof>European journal of neurology, 2009-07, Vol.16 (7), p.852-857</ispartof><rights>2009 The Author(s). Journal compilation © 2009 EFNS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3877-3be84e2618ddfc96a3d2bedd11e55b25253673bee8a23b0a5c729c89230079a73</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1468-1331.2009.02607.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1468-1331.2009.02607.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19473354$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Svetel, M.</creatorcontrib><creatorcontrib>Pekmezović, T.</creatorcontrib><creatorcontrib>Petrović, I.</creatorcontrib><creatorcontrib>Tomić, A.</creatorcontrib><creatorcontrib>Kresojević, N.</creatorcontrib><creatorcontrib>Ješić, R.</creatorcontrib><creatorcontrib>Kažić, S.</creatorcontrib><creatorcontrib>Raičević, R.</creatorcontrib><creatorcontrib>Stefanović, D.</creatorcontrib><creatorcontrib>Delibašić, N.</creatorcontrib><creatorcontrib>Živanović, D.</creatorcontrib><creatorcontrib>?or?ević, M.</creatorcontrib><creatorcontrib>Kostić, V. S.</creatorcontrib><title>Long-term outcome in Serbian patients with Wilson disease</title><title>European journal of neurology</title><addtitle>Eur J Neurol</addtitle><description>Background and purpose: To investigate survival rates, prognostic factors, and causes of death in Wilson disease (WD).
Methods: In the years 1980–2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form, and data were missing for six patients). The duration of follow‐up for patients alive was 11.1 ± 8.8 years.
Results: After initiation of treatment (d‐penicillamine and zinc salts), 79% of patients had a stable or improved course of disease. Despite early diagnosis and appropriate therapy, 15 patients still had a relentlessly progressive course. Thirty patients died. The cumulative probability of survival in a 15‐year period for the whole group was 76.7 ± 4.9%. Better prognosis of WD was associated with male sex, younger age at onset, neurologic form of the disease, and treatment continuity. Causes of death were predominantly related to hepatic failure (16 patients), but also suicide (four patients) and cancer (three patients).
Conclusion: Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high.</description><subject>Age of Onset</subject><subject>Cause of Death</subject><subject>causes of death</subject><subject>Chelating Agents - therapeutic use</subject><subject>Cohort Studies</subject><subject>Female</subject><subject>Hepatolenticular Degeneration - diagnosis</subject><subject>Hepatolenticular Degeneration - drug therapy</subject><subject>Hepatolenticular Degeneration - mortality</subject><subject>Hepatolenticular Degeneration - physiopathology</subject><subject>Humans</subject><subject>Male</subject><subject>mortality</subject><subject>Penicillamine - therapeutic use</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Serbia</subject><subject>Wilson disease</subject><issn>1351-5101</issn><issn>1468-1331</issn><issn>1471-0552</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkMtOwzAQRS0EolD4BZQVuwQ_4thesICqFKSqSDxUdpaTTMEljxKnavv3OLSUJbOZkebc0eggFBAcEV9X84jEiQwJYySiGKsI0wSLaH2ATvaLQz8zTkJOMOmhU-fmGGMqKD5GPaJiwRiPT5Aa19V72EJTBvWyzeoSAlsFz9Ck1lTBwrQWqtYFK9t-BFNbuLoKcuvAODhDRzNTODjf9T56vRu-DO7D8ePoYXAzDjMmhQhZCjIGmhCZ57NMJYblNIU8JwQ4TymnnCXCQyANZSk2PBNUZVJRhrFQRrA-utzeXTT11xJcq0vrMigKU0G9dDoRPE4oxf-CXlQiYy49eLEDl2kJuV40tjTNRv9a8cD1FljZAjZ_e6w7-3quO8m6k9wdVfrHvl7r4WTYTT4fbvPWtbDe503z6b9lguvpZKRv356UnIy4HrBvJNiFiw</recordid><startdate>200907</startdate><enddate>200907</enddate><creator>Svetel, M.</creator><creator>Pekmezović, T.</creator><creator>Petrović, I.</creator><creator>Tomić, A.</creator><creator>Kresojević, N.</creator><creator>Ješić, R.</creator><creator>Kažić, S.</creator><creator>Raičević, R.</creator><creator>Stefanović, D.</creator><creator>Delibašić, N.</creator><creator>Živanović, D.</creator><creator>?or?ević, M.</creator><creator>Kostić, V. S.</creator><general>Blackwell Publishing Ltd</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7TK</scope><scope>7X8</scope></search><sort><creationdate>200907</creationdate><title>Long-term outcome in Serbian patients with Wilson disease</title><author>Svetel, M. ; Pekmezović, T. ; Petrović, I. ; Tomić, A. ; Kresojević, N. ; Ješić, R. ; Kažić, S. ; Raičević, R. ; Stefanović, D. ; Delibašić, N. ; Živanović, D. ; ?or?ević, M. ; Kostić, V. S.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3877-3be84e2618ddfc96a3d2bedd11e55b25253673bee8a23b0a5c729c89230079a73</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Age of Onset</topic><topic>Cause of Death</topic><topic>causes of death</topic><topic>Chelating Agents - therapeutic use</topic><topic>Cohort Studies</topic><topic>Female</topic><topic>Hepatolenticular Degeneration - diagnosis</topic><topic>Hepatolenticular Degeneration - drug therapy</topic><topic>Hepatolenticular Degeneration - mortality</topic><topic>Hepatolenticular Degeneration - physiopathology</topic><topic>Humans</topic><topic>Male</topic><topic>mortality</topic><topic>Penicillamine - therapeutic use</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><topic>Serbia</topic><topic>Wilson disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Svetel, M.</creatorcontrib><creatorcontrib>Pekmezović, T.</creatorcontrib><creatorcontrib>Petrović, I.</creatorcontrib><creatorcontrib>Tomić, A.</creatorcontrib><creatorcontrib>Kresojević, N.</creatorcontrib><creatorcontrib>Ješić, R.</creatorcontrib><creatorcontrib>Kažić, S.</creatorcontrib><creatorcontrib>Raičević, R.</creatorcontrib><creatorcontrib>Stefanović, D.</creatorcontrib><creatorcontrib>Delibašić, N.</creatorcontrib><creatorcontrib>Živanović, D.</creatorcontrib><creatorcontrib>?or?ević, M.</creatorcontrib><creatorcontrib>Kostić, V. S.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>European journal of neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Svetel, M.</au><au>Pekmezović, T.</au><au>Petrović, I.</au><au>Tomić, A.</au><au>Kresojević, N.</au><au>Ješić, R.</au><au>Kažić, S.</au><au>Raičević, R.</au><au>Stefanović, D.</au><au>Delibašić, N.</au><au>Živanović, D.</au><au>?or?ević, M.</au><au>Kostić, V. S.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Long-term outcome in Serbian patients with Wilson disease</atitle><jtitle>European journal of neurology</jtitle><addtitle>Eur J Neurol</addtitle><date>2009-07</date><risdate>2009</risdate><volume>16</volume><issue>7</issue><spage>852</spage><epage>857</epage><pages>852-857</pages><issn>1351-5101</issn><eissn>1468-1331</eissn><eissn>1471-0552</eissn><abstract>Background and purpose: To investigate survival rates, prognostic factors, and causes of death in Wilson disease (WD).
Methods: In the years 1980–2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form, and data were missing for six patients). The duration of follow‐up for patients alive was 11.1 ± 8.8 years.
Results: After initiation of treatment (d‐penicillamine and zinc salts), 79% of patients had a stable or improved course of disease. Despite early diagnosis and appropriate therapy, 15 patients still had a relentlessly progressive course. Thirty patients died. The cumulative probability of survival in a 15‐year period for the whole group was 76.7 ± 4.9%. Better prognosis of WD was associated with male sex, younger age at onset, neurologic form of the disease, and treatment continuity. Causes of death were predominantly related to hepatic failure (16 patients), but also suicide (four patients) and cancer (three patients).
Conclusion: Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>19473354</pmid><doi>10.1111/j.1468-1331.2009.02607.x</doi><tpages>6</tpages></addata></record> |
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| subjects | Age of Onset Cause of Death causes of death Chelating Agents - therapeutic use Cohort Studies Female Hepatolenticular Degeneration - diagnosis Hepatolenticular Degeneration - drug therapy Hepatolenticular Degeneration - mortality Hepatolenticular Degeneration - physiopathology Humans Male mortality Penicillamine - therapeutic use Prognosis Retrospective Studies Serbia Wilson disease |
| title | Long-term outcome in Serbian patients with Wilson disease |
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