Geographic variations in Wilson's disease
Certain features of Wilson's disease (WD) in Asia have been found to be different from those in other continents. The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblast...
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| Veröffentlicht in: | Journal of the Neurological Sciences 1993-07, Vol.117 (1), p.1-7 |
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| creator | Chu, Nai-Shin Hung, Tsu-Pei |
| description | Certain features of Wilson's disease (WD) in Asia have been found to be different from those in other continents. The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblastoma in the long arm of chromosome 13. The high proportion of patients with hepatic presentation accounts for early onset of WD in the Japanese and Chines series. Skeletal involvement, leg hyperpigmentation, dark complexion, amenorrhea, epileptic seizures, and cerebral white matter degeneration are relatively more common among WD patients in Asia. Excessive copper in the liver appears to have a protective effect against hepatocellular carcinoma and type B hepatitis. Electrophysiological studies suggest widespread functional disturbances of the CNS in WD. Side-effects from penicillamine are rather frequent and often lead to interruption of the therapy. Trien is found to be effective without adverse reactions. Oral zinc therapy may be a suitable alternative for long-term management of WD patients in developing Asian countries. |
| doi_str_mv | 10.1016/0022-510X(93)90145-O |
| format | Article |
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The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblastoma in the long arm of chromosome 13. The high proportion of patients with hepatic presentation accounts for early onset of WD in the Japanese and Chines series. Skeletal involvement, leg hyperpigmentation, dark complexion, amenorrhea, epileptic seizures, and cerebral white matter degeneration are relatively more common among WD patients in Asia. Excessive copper in the liver appears to have a protective effect against hepatocellular carcinoma and type B hepatitis. Electrophysiological studies suggest widespread functional disturbances of the CNS in WD. Side-effects from penicillamine are rather frequent and often lead to interruption of the therapy. Trien is found to be effective without adverse reactions. Oral zinc therapy may be a suitable alternative for long-term management of WD patients in developing Asian countries.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/0022-510X(93)90145-O</identifier><identifier>PMID: 8410043</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Shannon: Elsevier B.V</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Brain Diseases, Metabolic - etiology ; Brain Diseases, Metabolic - physiopathology ; Carcinoma, Hepatocellular - epidemiology ; China - epidemiology ; Comorbidity ; Consanguinity ; Copper - metabolism ; Disease Susceptibility ; Electroencephalography ; Electrophysiological investigation ; Ethnic Groups - genetics ; Europe - epidemiology ; Female ; Gene Frequency ; Genes, Recessive ; Genetic pattern ; Geographic variation ; Hepatitis B - epidemiology ; Hepatolenticular Degeneration - complications ; Hepatolenticular Degeneration - drug therapy ; Hepatolenticular Degeneration - epidemiology ; Hepatolenticular Degeneration - ethnology ; Hepatolenticular Degeneration - genetics ; Humans ; Incidence ; India - epidemiology ; Japan - epidemiology ; Liver Neoplasms - epidemiology ; Male ; Medical sciences ; Metabolic diseases ; Metals (hemochromatosis...) ; Other metabolic disorders ; Penicillamine - therapeutic use ; Phenotype ; Pregnancy ; Pregnancy Complications - drug therapy ; Prevalence ; Socio-economic problems ; Socioeconomic Factors ; White matter degeneration ; Wilson's disease</subject><ispartof>Journal of the Neurological Sciences, 1993-07, Vol.117 (1), p.1-7</ispartof><rights>1993</rights><rights>1993 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c386t-382e1cf11ebcdc647b0c8b48cdce65a83612364022dabb28ea47aa8023e312b83</citedby><cites>FETCH-LOGICAL-c386t-382e1cf11ebcdc647b0c8b48cdce65a83612364022dabb28ea47aa8023e312b83</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/0022510X9390145O$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>313,314,776,780,788,3537,27899,27901,27902,65306</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=4849458$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/8410043$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Chu, Nai-Shin</creatorcontrib><creatorcontrib>Hung, Tsu-Pei</creatorcontrib><title>Geographic variations in Wilson's disease</title><title>Journal of the Neurological Sciences</title><addtitle>J Neurol Sci</addtitle><description>Certain features of Wilson's disease (WD) in Asia have been found to be different from those in other continents. The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblastoma in the long arm of chromosome 13. The high proportion of patients with hepatic presentation accounts for early onset of WD in the Japanese and Chines series. Skeletal involvement, leg hyperpigmentation, dark complexion, amenorrhea, epileptic seizures, and cerebral white matter degeneration are relatively more common among WD patients in Asia. Excessive copper in the liver appears to have a protective effect against hepatocellular carcinoma and type B hepatitis. Electrophysiological studies suggest widespread functional disturbances of the CNS in WD. Side-effects from penicillamine are rather frequent and often lead to interruption of the therapy. Trien is found to be effective without adverse reactions. Oral zinc therapy may be a suitable alternative for long-term management of WD patients in developing Asian countries.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Brain Diseases, Metabolic - etiology</subject><subject>Brain Diseases, Metabolic - physiopathology</subject><subject>Carcinoma, Hepatocellular - epidemiology</subject><subject>China - epidemiology</subject><subject>Comorbidity</subject><subject>Consanguinity</subject><subject>Copper - metabolism</subject><subject>Disease Susceptibility</subject><subject>Electroencephalography</subject><subject>Electrophysiological investigation</subject><subject>Ethnic Groups - genetics</subject><subject>Europe - epidemiology</subject><subject>Female</subject><subject>Gene Frequency</subject><subject>Genes, Recessive</subject><subject>Genetic pattern</subject><subject>Geographic variation</subject><subject>Hepatitis B - epidemiology</subject><subject>Hepatolenticular Degeneration - complications</subject><subject>Hepatolenticular Degeneration - drug therapy</subject><subject>Hepatolenticular Degeneration - epidemiology</subject><subject>Hepatolenticular Degeneration - ethnology</subject><subject>Hepatolenticular Degeneration - genetics</subject><subject>Humans</subject><subject>Incidence</subject><subject>India - epidemiology</subject><subject>Japan - epidemiology</subject><subject>Liver Neoplasms - epidemiology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Metals (hemochromatosis...)</subject><subject>Other metabolic disorders</subject><subject>Penicillamine - therapeutic use</subject><subject>Phenotype</subject><subject>Pregnancy</subject><subject>Pregnancy Complications - drug therapy</subject><subject>Prevalence</subject><subject>Socio-economic problems</subject><subject>Socioeconomic Factors</subject><subject>White matter degeneration</subject><subject>Wilson's disease</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1993</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE9Lw0AQxRdRaq1-A4UcRO0hupvdJJOLIMV_UOhF0duy2Ux0JU3qTlrw25vY0KOnYXi_ebx5jJ0Kfi24SG44j6IwFvz9KpPTjAsVh4s9NhaQQhgDyH023iGH7Ijoi3OeAGQjNgIlOFdyzKaP2Hx4s_p0NtgY70zrmpoCVwdvrqKmvqSgcISG8JgdlKYiPBnmhL0-3L_MnsL54vF5djcPrYSkDSVEKGwpBOa2sIlKc24hV9AtmMQGZCIimaguWGHyPAI0KjUGeCRRiigHOWEXW9-Vb77XSK1eOrJYVabGZk06jbNMpVHagWoLWt8QeSz1yrul8T9acN03pPv3df--zqT-a0gvurOzwX-dL7HYHQ2VdPr5oBuypiq9qa2jHaZAZSruY95uMey62Dj0mqzD2mLhPNpWF437P8cvVAOA_A</recordid><startdate>19930701</startdate><enddate>19930701</enddate><creator>Chu, Nai-Shin</creator><creator>Hung, Tsu-Pei</creator><general>Elsevier B.V</general><general>Elsevier Science</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>19930701</creationdate><title>Geographic variations in Wilson's disease</title><author>Chu, Nai-Shin ; Hung, Tsu-Pei</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c386t-382e1cf11ebcdc647b0c8b48cdce65a83612364022dabb28ea47aa8023e312b83</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1993</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Brain Diseases, Metabolic - etiology</topic><topic>Brain Diseases, Metabolic - physiopathology</topic><topic>Carcinoma, Hepatocellular - epidemiology</topic><topic>China - epidemiology</topic><topic>Comorbidity</topic><topic>Consanguinity</topic><topic>Copper - metabolism</topic><topic>Disease Susceptibility</topic><topic>Electroencephalography</topic><topic>Electrophysiological investigation</topic><topic>Ethnic Groups - genetics</topic><topic>Europe - epidemiology</topic><topic>Female</topic><topic>Gene Frequency</topic><topic>Genes, Recessive</topic><topic>Genetic pattern</topic><topic>Geographic variation</topic><topic>Hepatitis B - epidemiology</topic><topic>Hepatolenticular Degeneration - complications</topic><topic>Hepatolenticular Degeneration - drug therapy</topic><topic>Hepatolenticular Degeneration - epidemiology</topic><topic>Hepatolenticular Degeneration - ethnology</topic><topic>Hepatolenticular Degeneration - genetics</topic><topic>Humans</topic><topic>Incidence</topic><topic>India - epidemiology</topic><topic>Japan - epidemiology</topic><topic>Liver Neoplasms - epidemiology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Metals (hemochromatosis...)</topic><topic>Other metabolic disorders</topic><topic>Penicillamine - therapeutic use</topic><topic>Phenotype</topic><topic>Pregnancy</topic><topic>Pregnancy Complications - drug therapy</topic><topic>Prevalence</topic><topic>Socio-economic problems</topic><topic>Socioeconomic Factors</topic><topic>White matter degeneration</topic><topic>Wilson's disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Chu, Nai-Shin</creatorcontrib><creatorcontrib>Hung, Tsu-Pei</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 the Neurological Sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Chu, Nai-Shin</au><au>Hung, Tsu-Pei</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Geographic variations in Wilson's disease</atitle><jtitle>Journal of the Neurological Sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>1993-07-01</date><risdate>1993</risdate><volume>117</volume><issue>1</issue><spage>1</spage><epage>7</epage><pages>1-7</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Certain features of Wilson's disease (WD) in Asia have been found to be different from those in other continents. The higher prevalence rate in Japan is presumably due to a higher consanguinity rate. In Chinese there is a tight linkage between WD and two gene loci for esterase D and retinoblastoma in the long arm of chromosome 13. The high proportion of patients with hepatic presentation accounts for early onset of WD in the Japanese and Chines series. Skeletal involvement, leg hyperpigmentation, dark complexion, amenorrhea, epileptic seizures, and cerebral white matter degeneration are relatively more common among WD patients in Asia. Excessive copper in the liver appears to have a protective effect against hepatocellular carcinoma and type B hepatitis. Electrophysiological studies suggest widespread functional disturbances of the CNS in WD. Side-effects from penicillamine are rather frequent and often lead to interruption of the therapy. Trien is found to be effective without adverse reactions. Oral zinc therapy may be a suitable alternative for long-term management of WD patients in developing Asian countries.</abstract><cop>Shannon</cop><pub>Elsevier B.V</pub><pmid>8410043</pmid><doi>10.1016/0022-510X(93)90145-O</doi><tpages>7</tpages></addata></record> |
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| subjects | Adolescent Adult Biological and medical sciences Brain Diseases, Metabolic - etiology Brain Diseases, Metabolic - physiopathology Carcinoma, Hepatocellular - epidemiology China - epidemiology Comorbidity Consanguinity Copper - metabolism Disease Susceptibility Electroencephalography Electrophysiological investigation Ethnic Groups - genetics Europe - epidemiology Female Gene Frequency Genes, Recessive Genetic pattern Geographic variation Hepatitis B - epidemiology Hepatolenticular Degeneration - complications Hepatolenticular Degeneration - drug therapy Hepatolenticular Degeneration - epidemiology Hepatolenticular Degeneration - ethnology Hepatolenticular Degeneration - genetics Humans Incidence India - epidemiology Japan - epidemiology Liver Neoplasms - epidemiology Male Medical sciences Metabolic diseases Metals (hemochromatosis...) Other metabolic disorders Penicillamine - therapeutic use Phenotype Pregnancy Pregnancy Complications - drug therapy Prevalence Socio-economic problems Socioeconomic Factors White matter degeneration Wilson's disease |
| title | Geographic variations in Wilson's disease |
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