Long-term outcome in Serbian patients with Wilson disease.
OBJECTIVE
To investigate survival rates, prognostic factors, and causes of death in Wilson disease (WD).


METHODS
In the years 1980-2007, a cohort of 142 patients with WD was prospectively registered (54 presented with neurologic symptoms, 49 with hepatic symptoms, 33 had mixed form, and data were missing for six patients). The duration of follow-up for patients alive was 11.1 +/- 8.8 years.


RESULTS
After initiation of treatment (d-penicillamine and zinc salts), 79% of patients had a stable or improved course of disease. Despite early diagnosis and appropriate therapy, 15 patients still had a relentlessly progressive course. Thirty patients died. The cumulative probability of survival in a 15-year period for the whole group was 76.7 +/- 4.9%. Better prognosis of WD was associated with male sex, younger age at onset, neurologic form of the disease, and treatment continuity. Causes of death were predominantly related to hepatic failure (16 patients), but also suicide (four patients) and cancer (three patients).


CONCLUSIONS
Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high.
Svetel
M
M
Institute of Neurology, School of Medicine, Belgrade, Serbia.
Pekmezović
T
T
Petrović
I
I
Tomić
A
A
Kresojević
N
N
Jesić
R
R
Kazić
S
S
Raicević
R
R
Stefanović
D
D
Delibasić
N
N
Zivanović
D
D
Dordević
M
M
Kostić
V S
VS
D017668:Age of Onset; D002423:Cause of Death; D002614:Chelating Agents / Q000627:therapeutic use; D015331:Cohort Studies; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D055771:Serbia
D002614:Chelating Agents; D010396:Penicillamine