Kayser–Fleischer ring in Wilson's disease: A cohort study

Abstract Aims To evaluate Wilson's disease (WD) features in Sardinian patients with Kayser–Fleischer (KF) ring and to evaluate correlations between modifications in KF and anti-copper therapy and systemic WD evolution. Patients and methods Sixty-seven WD patients (35 m/32 f; mean age 41 years)...

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Veröffentlicht in:European journal of internal medicine 2012-09, Vol.23 (6), p.e150-e156
Hauptverfasser: Fenu, M, Liggi, M, Demelia, E, Sorbello, O, Civolani, A, Demelia, L
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container_end_page e156
container_issue 6
container_start_page e150
container_title European journal of internal medicine
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creator Fenu, M
Liggi, M
Demelia, E
Sorbello, O
Civolani, A
Demelia, L
description Abstract Aims To evaluate Wilson's disease (WD) features in Sardinian patients with Kayser–Fleischer (KF) ring and to evaluate correlations between modifications in KF and anti-copper therapy and systemic WD evolution. Patients and methods Sixty-seven WD patients (35 m/32 f; mean age 41 years) were retrospectively studied. At diagnosis and during follow up comprehensive ophthalmologic and neurologic examinations, brain RMN and ECD SPECT, detailed objective laboratory studies and hepatic histological examination were performed on all patients for analysis. All patients were given anti-copper therapy with d -Penicillamine in mono-therapy or in combination with Zinc Salts. Results At diagnosis, KF was observed in 27% of patients with equal distribution in all age groups. Significant correlations between KF at diagnosis, neuro-psychiatric manifestations and pathologic features in brain RMN and in brain ECD SPECT were found at diagnosis. During follow up, a decrease in, or regression of KF was seen in 14% of patients. Anti-copper therapy leads to KF regression and prevents the appearance of KF. No significant correlations were observed between KF regression and clinical neurological or neuro-imaging improvement nor between KF modifications and clinical hepatic improvement. Conclusions Our study highlights the peculiar features of Sardinian WD patients: low representation of KF, its equal distribution in all age groups, significant correlation between KF at diagnosis and clinical neurological manifestations, pathologic brain RMN and brain ECD-SPECT are highlighted by our study. Anti-copper therapy induces KF regression and prevents its onset. Therefore, KF ring does appear to be a predictive factor in the neurological and hepatic evolution of WD.
doi_str_mv 10.1016/j.ejim.2012.04.005
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Patients and methods Sixty-seven WD patients (35 m/32 f; mean age 41 years) were retrospectively studied. At diagnosis and during follow up comprehensive ophthalmologic and neurologic examinations, brain RMN and ECD SPECT, detailed objective laboratory studies and hepatic histological examination were performed on all patients for analysis. All patients were given anti-copper therapy with d -Penicillamine in mono-therapy or in combination with Zinc Salts. Results At diagnosis, KF was observed in 27% of patients with equal distribution in all age groups. Significant correlations between KF at diagnosis, neuro-psychiatric manifestations and pathologic features in brain RMN and in brain ECD SPECT were found at diagnosis. During follow up, a decrease in, or regression of KF was seen in 14% of patients. Anti-copper therapy leads to KF regression and prevents the appearance of KF. No significant correlations were observed between KF regression and clinical neurological or neuro-imaging improvement nor between KF modifications and clinical hepatic improvement. Conclusions Our study highlights the peculiar features of Sardinian WD patients: low representation of KF, its equal distribution in all age groups, significant correlation between KF at diagnosis and clinical neurological manifestations, pathologic brain RMN and brain ECD-SPECT are highlighted by our study. Anti-copper therapy induces KF regression and prevents its onset. Therefore, KF ring does appear to be a predictive factor in the neurological and hepatic evolution of WD.</description><identifier>ISSN: 0953-6205</identifier><identifier>EISSN: 1879-0828</identifier><identifier>DOI: 10.1016/j.ejim.2012.04.005</identifier><identifier>PMID: 22863441</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Adolescent ; Adult ; Brain - diagnostic imaging ; Brain - pathology ; Chelating Agents - therapeutic use ; Cohort Studies ; Disease Progression ; Female ; Hepatolenticular Degeneration - drug therapy ; Hepatolenticular Degeneration - pathology ; Hepatolenticular Degeneration - physiopathology ; Humans ; Internal Medicine ; Italy ; Kayser–Fleischer ring ; Limbus Corneae - pathology ; Liver ; Liver - pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurological involvement ; Outcome ; Penicillamine - therapeutic use ; Retrospective Studies ; Therapy ; Tomography, Emission-Computed, Single-Photon ; Treatment Outcome ; Wilson's disease</subject><ispartof>European journal of internal medicine, 2012-09, Vol.23 (6), p.e150-e156</ispartof><rights>European Federation of Internal Medicine.</rights><rights>2012 European Federation of Internal Medicine.</rights><rights>Copyright © 2012 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c411t-bdbedcfe6affa898d5bc68714838e8ac94910bb23da967f2c1929fe6cb1a182b3</citedby><cites>FETCH-LOGICAL-c411t-bdbedcfe6affa898d5bc68714838e8ac94910bb23da967f2c1929fe6cb1a182b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.ejim.2012.04.005$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,778,782,3539,27907,27908,45978</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22863441$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Fenu, M</creatorcontrib><creatorcontrib>Liggi, M</creatorcontrib><creatorcontrib>Demelia, E</creatorcontrib><creatorcontrib>Sorbello, O</creatorcontrib><creatorcontrib>Civolani, A</creatorcontrib><creatorcontrib>Demelia, L</creatorcontrib><title>Kayser–Fleischer ring in Wilson's disease: A cohort study</title><title>European journal of internal medicine</title><addtitle>Eur J Intern Med</addtitle><description>Abstract Aims To evaluate Wilson's disease (WD) features in Sardinian patients with Kayser–Fleischer (KF) ring and to evaluate correlations between modifications in KF and anti-copper therapy and systemic WD evolution. Patients and methods Sixty-seven WD patients (35 m/32 f; mean age 41 years) were retrospectively studied. At diagnosis and during follow up comprehensive ophthalmologic and neurologic examinations, brain RMN and ECD SPECT, detailed objective laboratory studies and hepatic histological examination were performed on all patients for analysis. All patients were given anti-copper therapy with d -Penicillamine in mono-therapy or in combination with Zinc Salts. Results At diagnosis, KF was observed in 27% of patients with equal distribution in all age groups. Significant correlations between KF at diagnosis, neuro-psychiatric manifestations and pathologic features in brain RMN and in brain ECD SPECT were found at diagnosis. During follow up, a decrease in, or regression of KF was seen in 14% of patients. Anti-copper therapy leads to KF regression and prevents the appearance of KF. No significant correlations were observed between KF regression and clinical neurological or neuro-imaging improvement nor between KF modifications and clinical hepatic improvement. Conclusions Our study highlights the peculiar features of Sardinian WD patients: low representation of KF, its equal distribution in all age groups, significant correlation between KF at diagnosis and clinical neurological manifestations, pathologic brain RMN and brain ECD-SPECT are highlighted by our study. Anti-copper therapy induces KF regression and prevents its onset. Therefore, KF ring does appear to be a predictive factor in the neurological and hepatic evolution of WD.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Brain - diagnostic imaging</subject><subject>Brain - pathology</subject><subject>Chelating Agents - therapeutic use</subject><subject>Cohort Studies</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Hepatolenticular Degeneration - drug therapy</subject><subject>Hepatolenticular Degeneration - pathology</subject><subject>Hepatolenticular Degeneration - physiopathology</subject><subject>Humans</subject><subject>Internal Medicine</subject><subject>Italy</subject><subject>Kayser–Fleischer ring</subject><subject>Limbus Corneae - pathology</subject><subject>Liver</subject><subject>Liver - pathology</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Neurological involvement</subject><subject>Outcome</subject><subject>Penicillamine - therapeutic use</subject><subject>Retrospective Studies</subject><subject>Therapy</subject><subject>Tomography, Emission-Computed, Single-Photon</subject><subject>Treatment Outcome</subject><subject>Wilson's disease</subject><issn>0953-6205</issn><issn>1879-0828</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kcFu1DAQhi1E1S6lL8AB5QaXpDNONmsDQqqqllZU4gCIo-XYE-qQTYonQdob78Ab9klwtIUDB05z-f5fM98I8QyhQMD6tCuoC9tCAsoCqgJg_UisUG10Dkqqx2IFel3mtYT1kXjC3AHgBqA8FEdSqrqsKlyJ1-_tjine__x12VNgd0sxi2H4moUh-xJ6HocXnPnAZJleZWeZG2_HOGU8zX73VBy0tmc6eZjH4vPlxafzq_zmw7vr87Ob3FWIU974hrxrqbZta5VWft24Wm2wUqUiZZ2uNELTyNJbXW9a6VBLnXDXoEUlm_JYvNz33sXx-0w8mW3alPreDjTObBBKWSNCLRMq96iLI3Ok1tzFsLVxlyCzSDOdWaSZRZqByiRpKfT8oX9utuT_Rv5YSsCbPUDpyh-BomEXaHDkQyQ3GT-G__e__Sfu-jAEZ_tvtCPuxjkOyZ9BwyljPi5vW76GEgC0xPI3iiSSyg</recordid><startdate>20120901</startdate><enddate>20120901</enddate><creator>Fenu, M</creator><creator>Liggi, M</creator><creator>Demelia, E</creator><creator>Sorbello, O</creator><creator>Civolani, A</creator><creator>Demelia, L</creator><general>Elsevier B.V</general><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>20120901</creationdate><title>Kayser–Fleischer ring in Wilson's disease: A cohort study</title><author>Fenu, M ; Liggi, M ; Demelia, E ; Sorbello, O ; Civolani, A ; Demelia, L</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c411t-bdbedcfe6affa898d5bc68714838e8ac94910bb23da967f2c1929fe6cb1a182b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Brain - diagnostic imaging</topic><topic>Brain - pathology</topic><topic>Chelating Agents - therapeutic use</topic><topic>Cohort Studies</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Hepatolenticular Degeneration - drug therapy</topic><topic>Hepatolenticular Degeneration - pathology</topic><topic>Hepatolenticular Degeneration - physiopathology</topic><topic>Humans</topic><topic>Internal Medicine</topic><topic>Italy</topic><topic>Kayser–Fleischer ring</topic><topic>Limbus Corneae - pathology</topic><topic>Liver</topic><topic>Liver - pathology</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Neurological involvement</topic><topic>Outcome</topic><topic>Penicillamine - therapeutic use</topic><topic>Retrospective Studies</topic><topic>Therapy</topic><topic>Tomography, Emission-Computed, Single-Photon</topic><topic>Treatment Outcome</topic><topic>Wilson's disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Fenu, M</creatorcontrib><creatorcontrib>Liggi, M</creatorcontrib><creatorcontrib>Demelia, E</creatorcontrib><creatorcontrib>Sorbello, O</creatorcontrib><creatorcontrib>Civolani, A</creatorcontrib><creatorcontrib>Demelia, L</creatorcontrib><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>European journal of internal medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Fenu, M</au><au>Liggi, M</au><au>Demelia, E</au><au>Sorbello, O</au><au>Civolani, A</au><au>Demelia, L</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Kayser–Fleischer ring in Wilson's disease: A cohort study</atitle><jtitle>European journal of internal medicine</jtitle><addtitle>Eur J Intern Med</addtitle><date>2012-09-01</date><risdate>2012</risdate><volume>23</volume><issue>6</issue><spage>e150</spage><epage>e156</epage><pages>e150-e156</pages><issn>0953-6205</issn><eissn>1879-0828</eissn><abstract>Abstract Aims To evaluate Wilson's disease (WD) features in Sardinian patients with Kayser–Fleischer (KF) ring and to evaluate correlations between modifications in KF and anti-copper therapy and systemic WD evolution. Patients and methods Sixty-seven WD patients (35 m/32 f; mean age 41 years) were retrospectively studied. At diagnosis and during follow up comprehensive ophthalmologic and neurologic examinations, brain RMN and ECD SPECT, detailed objective laboratory studies and hepatic histological examination were performed on all patients for analysis. All patients were given anti-copper therapy with d -Penicillamine in mono-therapy or in combination with Zinc Salts. Results At diagnosis, KF was observed in 27% of patients with equal distribution in all age groups. Significant correlations between KF at diagnosis, neuro-psychiatric manifestations and pathologic features in brain RMN and in brain ECD SPECT were found at diagnosis. During follow up, a decrease in, or regression of KF was seen in 14% of patients. Anti-copper therapy leads to KF regression and prevents the appearance of KF. No significant correlations were observed between KF regression and clinical neurological or neuro-imaging improvement nor between KF modifications and clinical hepatic improvement. Conclusions Our study highlights the peculiar features of Sardinian WD patients: low representation of KF, its equal distribution in all age groups, significant correlation between KF at diagnosis and clinical neurological manifestations, pathologic brain RMN and brain ECD-SPECT are highlighted by our study. Anti-copper therapy induces KF regression and prevents its onset. Therefore, KF ring does appear to be a predictive factor in the neurological and hepatic evolution of WD.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>22863441</pmid><doi>10.1016/j.ejim.2012.04.005</doi></addata></record>
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subjects Adolescent
Adult
Brain - diagnostic imaging
Brain - pathology
Chelating Agents - therapeutic use
Cohort Studies
Disease Progression
Female
Hepatolenticular Degeneration - drug therapy
Hepatolenticular Degeneration - pathology
Hepatolenticular Degeneration - physiopathology
Humans
Internal Medicine
Italy
Kayser–Fleischer ring
Limbus Corneae - pathology
Liver
Liver - pathology
Magnetic Resonance Imaging
Male
Middle Aged
Neurological involvement
Outcome
Penicillamine - therapeutic use
Retrospective Studies
Therapy
Tomography, Emission-Computed, Single-Photon
Treatment Outcome
Wilson's disease
title Kayser–Fleischer ring in Wilson's disease: A cohort study
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