OP- 15 OUTCOME OF PREGNANCIES IN A SERIES OF WILSON DISEASE PATIENTS

Conflict of interest

No

Introduction and Objectives

Wilson disease (WD) is an inherited disease that mainly affects the liver and brain due to copper overload. Pregnancy in WD is a challenging situation. Our aim was to analyze the clinical aspects and outcomes of pregnancies in WD.

Patients / Materials and Methods

In a series of 289 WD cases (1963-2024), we reviewed the medical records of 123 women, 26 of whom became pregnant at least once (1-5). A total of 52 pregnancies were recorded, but 3 were excluded because data on the conceptus were missing. Pregnancy outcomes were correlated with disease severity and anti-copper medication. Hepatic and/or neuropsychiatric manifestations were categorized as 1) asymptomatic/mild or 2) moderate/severe. Pregnancy outcomes were considered 1) successful if the preterm/term infant survived the neonatal period or 2) unsuccessful if there was a miscarriage/fetal demise/perinatal death. Drug regimen during pregnancy were 1) penicillamine (DPA), 2) zinc (Zn) or 3) DPA/Zn if the patient had switched therapy for any reason. Statistical analysis was carried out using Fisher´s test, with significance level at p<0.05.

Results and Discussion

Of the 49 pregnancies analyzed, 2 (4.1%) ended in maternal death and 17/49 (34.7%) in miscarriage/fetal or neonatal death. The Table below presents the results. The analysis of medication and pregnancy outcomes combined deteriorating and stable cases. There was a correlation between successful births and asymptomatic patients who had given birth before the onset of WD (p<0.001). In stable patients, successful births were slightly above significance (p=0.062). There was no significant difference among the outcomes in relation to the medication taken.

Conclusions

Pregnancy in symptomatic WD is potentially harmful to mother and conceptus. Successful births were significantly associated with pregnancies before the onset of WD symptoms, but were also possible with stable disease under treatment. Anti-copper drug regimen was not associated with pregnancy outcome.