Wilson’s disease in children: Analysis of 41 cases

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dc.contributor.authorTursun, Serkan
dc.contributor.authorGülerman, Hacer Fulya
dc.date.accessioned2025-01-21T16:17:17Z
dc.date.available2025-01-21T16:17:17Z
dc.date.issued2019
dc.departmentKırıkkale Üniversitesi
dc.description.abstractAim: This study aimed to present clinical and laboratory features of 41 children with Wilson's disease.Material and Methods: The medical records of all of the patients who had got a diagnosis of Wilson's disease between 2001 June and 2005 March in Ankara Dr. Sami Ulus Training& Research Hospital, Turkey. Demographic, clinical and biochemical information was obtained from the patients' records. Findings were retrospectively analyzed by the SPSS Windows 16.0 (SPSS Inc. IL, USA) statistical software.Results: A total of 41 patients had got the diagnosis of Wilson's disease: 24 boys and 17 girls, between in the range of 3-14 years old. The mean age of patients was 9.05±2.84 years. Kayser-Fleischer rings were observed in 24 patients. Urinary copper excretion in 24-hours urine was high in 39 of 40 patients. Serum ceruloplasmin levels were found low in 35 of 40 patients. Thirteen of patients were diagnosed after the family screening. Pathologic brain MR findings were detected in 4 of 8 patients without neurological system complaints or physical examination findings. Conclusion: Especially in societies that consanguineous marriages are so common, Wilson's disease should be considered in differential diagnosis of chronic liver diseases, prolonged hypertransaminasemia, and degenerative brain disorders of unknown origin. In our study, it has been shown that Wilson's disease diagnosis and follow-up preserved the value of classical diagnostic methods and it has been shown that neuroimaging may be useful for early detection of neurological involvement even if neurological findings do not occur.
dc.identifier.doi10.18663/tjcl.493563
dc.identifier.endpage25
dc.identifier.issn2149-8296
dc.identifier.issue1
dc.identifier.startpage18
dc.identifier.trdizinid415621
dc.identifier.urihttps://doi.org/10.18663/tjcl.493563
dc.identifier.urihttps://search.trdizin.gov.tr/tr/yayin/detay/415621
dc.identifier.urihttps://hdl.handle.net/20.500.12587/22608
dc.identifier.volume10
dc.indekslendigikaynakTR-Dizin
dc.language.isoen
dc.relation.ispartofTurkish Journal of Clinics and Laboratory
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.snmzKA_20241229
dc.subjectGenel ve Dahili Tıp
dc.subjectPatoloji
dc.subjectPediatri
dc.titleWilson’s disease in children: Analysis of 41 cases
dc.typeArticle

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