Long-term outcome in Serbian patients with Wilson disease.

BACKGROUND AND PURPOSE: 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). CONCLUSION: Despite the relatively early diagnosis and treatment of our patients with WD, mortality was still considerably high.

[Multivascular atherosclerosis, the Sarajevo Vascular Study]. / Polivaskularna aterosklerotska bolest, sarajevska vaskularna studija.

INTRODUCTION: We started with Sarajevo Vascular Study (SVS) in 1994 with basic aim to evaluate arterial occlusive disease (AOD) of lower extremities and investigate possible effect of amelioration of risk factors to atherosclerosis regression. In 1996 we expanded SVS to polyvascular atherosclerotic disease, asymptomatic or symptomatic atherosclerotic disease on various vascular beds i.e. lower extremity arterial occlusive disease (AOD), cerebrovascular disease (CVD), coronary artery disease (CAD). We enrolled a total of 1680 pts. in the study but this number has changed depending on war migrations, comorbidity, or paramedical reasons. Follow-up of 10 yrs. had 645 pts, and follow-up of 8 yrs. had 1035 pts. METHOD: We enrolled a total of 1680 pts, 954 pts with symptomatic disease, and 246 pts without atherosclerotic disease but with at least 3/8 multiple risk factors (MRF), and as controls 400 pts without vascular disease and less than 3 MRF. STUDY DESIGN: one center, prospective, consecutive, with evaluation of epidemiological data--gender, age, comorbidity, antropometrical data, hemodynamic data--systolic and dystolic velocities, pulsatility and resistive indices, and morphologic data. Clinical variables--gender, age, hypertension, smoking, hyperlipoproteinemia, diabetes mellitus, obesity and fibrinogen. Score of MRF was calculated as x/8. RESULTS: Out of total of 645 pts (10 yrs. follow-up) 399 pts (62%) had AOD, out of them single AOD had 295 pts (74%), and polyvascular disease 104 pts (26%). 63 (61%) pts of symptomatic group had combination of AOD + CAD, and 41 pts (39%) had AOD + CVD + CAD. In the pts with 8 yrs. follow-up (n-1035) AOD had 548 pts. (53%), single AOD had 334 pts (57%) and polyvascular had 122 pts (39%). Out of symptomatic pts. 71 (58.2%) had AOD + CAD, and 51 (41.8%) had AOD + CAD + CVD. Asymptomatic disease on the very entry period of the study was significant for both groups, p < 0.01. CONCLUSIONS: (i) we found a significant number of asymptomatic atherosclerotic changes on other vascular beds, (ii) score of MRF has correlated with polyvascular disease and with overall outcome (iii) antropomethric, haemodynamic, and morphological parameters of human blood vessels have been measured and systematically documented in Bosnia and Herzegovina.