Evaluation of neurologically symptomatic and asymptomatic patients in childhood Wilson’s disease with central nervous system involvement: A retrospective observational study

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dc.contributor.authorYücel, Gül
dc.contributor.authorGungor, Sukru
dc.date.accessioned2024-08-04T19:42:43Z
dc.date.available2024-08-04T19:42:43Z
dc.date.issued2023
dc.departmentİnönü Üniversitesien_US
dc.description.abstractAim: The aim was to evaluate the clinical and laboratory characteristics of children with Wilson’s disease (WD) with and without neurological presentation with abnormalities on brain magnetic resonance imaging (MRI) and to describe the relationship of these observations with disease severity and functional outcome and their impact on prognosis. Materials and Methods: Demographic, neuropsychiatric findings, laboratory, disease severity and functional results of 48 children with neurologically symptomatic and asymptomatic WD were evaluated retrospectively. Results: A significant positive correlation was found between the neurologic symptom score and functional outcome in WD with neurological presentation (p<0.001). A significant positive correlation was found between disease severity scores (PELD, MELD, Child Pugh and Dhwin score) and modified Rankin scores (mRS) in WD without neurological presentation (p <0.001, 0.004, <0.001, 0.001, respectively). In addition, a significant positive correlation was found between total bilirubin, direct bilirubin and International Normalized Ratio (INR) values and mRS scores (p= 0.006, 0.012, 0.004, respectively). Kayser-Fleischer Ring sign in the eye was higher in the group with neurological symtomatic WD (p<0.001). The number of patients presenting with clinic of fulminant hepatitis and hepatic encephalopathy was significantly higher in the neurological asymptomatic group (p<0.001). Conclusion: Brain MRI changes may occur even in hepatic WD and presymptomatic cases, although infrequently, regardless of the presence of neurological symptoms. It should be known that neurological symptoms in children and adolescents may occur without significant liver disease. Even without neurological symptoms, all children with WD should have a brain MRI before treatment.en_US
dc.identifier.doi10.5455/annalsmedres.2023.07.169
dc.identifier.endpage975en_US
dc.identifier.issn2636-7688
dc.identifier.issue8en_US
dc.identifier.startpage970en_US
dc.identifier.trdizinid1205887en_US
dc.identifier.urihttps://doi.org/10.5455/annalsmedres.2023.07.169
dc.identifier.urihttps://search.trdizin.gov.tr/yayin/detay/1205887
dc.identifier.urihttps://hdl.handle.net/11616/88624
dc.identifier.volume30en_US
dc.indekslendigikaynakTR-Dizinen_US
dc.language.isoenen_US
dc.relation.ispartofAnnals of Medical Researchen_US
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleEvaluation of neurologically symptomatic and asymptomatic patients in childhood Wilson’s disease with central nervous system involvement: A retrospective observational studyen_US
dc.typeArticleen_US

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