title: Are irreversible morphological [corrected] signs of portal hypertension in neurological form of Wilson's disease associated with treatment delay? A pilot study.
abstract: Aim of this study was to evaluate the rate of morphological liver and spleen abnormalities in patients with neurological clinical presentation of Wilson's disease (WD). Fourteen patients with neurological presentation of WD divided into group A (5 patients who initiated chelating therapy <24 months from the first symptoms) and group B (9 patients whose therapy started ≥24 months after the initial symptoms) underwent abdominal MRI examination. Abnormal findings on abdominal MRI were present in 28% of patients with neurological form of WD. Significant hepatosplenomegaly was present in none of the patients from group A and in 4 (44%) patients from group B. In addition, macronodular liver cirrhosis and peritoneal effusion were evident in two and one patient from group B, respectively, and in none of the patients from group A. Our results suggest that severe portal hypertension and liver damage in patients with neurological presentation of WD might be reversible or do not even develop if chelating treatment is initiated <2 years after the onset of symptoms.
authors.affiliation: Institute of Oncology, Institutski put 4, 21204 Sremska Kamenica, Serbia. dusko.b.kozic@gmail.com
authors.forename: D B
authors.initials: DB
authors.lastname: Kozic
authors.forename: R
authors.initials: R
authors.lastname: Semnic
authors.forename: I
authors.initials: I
authors.lastname: Petrovic
authors.forename: M
authors.initials: M
authors.lastname: Svetel
authors.forename: J
authors.initials: J
authors.lastname: Ostojic
authors.forename: V S
authors.initials: VS
authors.lastname: Kostic
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000473:pathology*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D010865:Pilot Projects; D055815:Young Adult