Pediatric Wilson's disease: findings in different presentations. A cross-sectional study

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dc.authoridVarol, İlknur fatma/0000-0001-5212-218X
dc.authoridGUNGOR, SERDAL/0000-0003-3875-6770
dc.authorwosidgungor, sukru/JNR-9592-2023
dc.authorwosidVarol, İlknur fatma/ABH-6387-2020
dc.contributor.authorGungor, Sukru
dc.contributor.authorSelimoglu, Mukadder Ayse
dc.contributor.authorVarol, Fatma Ilknur
dc.contributor.authorGungor, Serdal
dc.date.accessioned2024-08-04T20:45:27Z
dc.date.available2024-08-04T20:45:27Z
dc.date.issued2018
dc.departmentİnönü Üniversitesien_US
dc.description.abstractBACKGROUND: Wilson's disease (WD) may present with different manifestations: from an asymptomatic state to liver cirrhosis. Here, we aimed to evaluate clinical presentations and laboratory findings and prognoses among WD cases. DESIGN AND SETTING: Cross-sectional study based on patients' records from the university hospital, Inonu University, Malatya, Turkey. METHODS: The medical records of 64 children with WD were evaluated focusing on the clinical, laboratory and liver biopsy findings in different clinical presentations. RESULTS: The mean age at diagnosis was 8.6 +/- 3.26 years (range 3.5-17) and mean length of follow-up was 2.49 years (range 0-9). There were 18 cases (28.1%), 12 (18.8%), 9 (14.1%) and 6 (9.4%) of chronic liver disease, fulminant liver failure, neurological WD and acute hepatitis, respectively. Nineteen (29.7%) were asymptomatic. The most common sign and laboratory finding were jaundice (45.3%) and hypertransaminasemia (85.9%), respectively. The lowest serum zinc level was found in the fulminant liver failure group (P = 0.035). Hepatosteatosis was detected in 35% of the 20 patients who underwent liver biopsy. Among those with hepatosteatosis, 57.1% were asymptomatic. While 35% had copper staining, 25% presented iron accumulation in liver biopsies. Nine cases underwent liver transplantation and seven of these presented fulminant liver failure (77.8%). CONCLUSION: The presentation, symptoms and signs of our cases were similar to those in previously reported series, except for the high proportion of fulminant WD cases. Further studies are needed to clarify the relationship between zinc levels and development of a fulminant course and between iron status and WD.en_US
dc.identifier.doi10.1590/1516-3180.2018.0210230718
dc.identifier.endpage309en_US
dc.identifier.issn1516-3180
dc.identifier.issue4en_US
dc.identifier.pmid30304203en_US
dc.identifier.scopus2-s2.0-85054772771en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage304en_US
dc.identifier.urihttps://doi.org/10.1590/1516-3180.2018.0210230718
dc.identifier.urihttps://hdl.handle.net/11616/98492
dc.identifier.volume136en_US
dc.identifier.wosWOS:000446303100006en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAssociacao Paulista Medicinaen_US
dc.relation.ispartofSao Paulo Medical Journalen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectHepatolenticular degenerationen_US
dc.subjectChilden_US
dc.subjectZincen_US
dc.subjectIronen_US
dc.titlePediatric Wilson's disease: findings in different presentations. A cross-sectional studyen_US
dc.typeArticleen_US

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