Sestre Milosrdnice University hospital and Institute of Clinical Medical Research
Abstract
Wilson’s disease is a rare autosomal recessive disorder of copper metabolism. It causes cirrhosis of the liver, consequently followed by disorder of the menstrual cycle and infertility. Successful decopperizing may lead to restoration of the ovulatory cycle and enable pregnancy. Increased copper levels may cause preeclampsia, intrauterine growth restriction and neurologic damages in the fetus. Pregnant women with decompensated liver cirrhosis face more complications, including bleeding from esophageal varices, liver failure, encephalopathy, and rupture of the splenic artery. We present a case of Wilson’s disease in a patient who had spontaneously conceived three times. The first pregnancy ended with delivery of a healthy baby at term. In second pregnancy, medically induced abortion was performed in the 12th week because of deterioration of the underlying disease, liver cirrhosis with portal hypertension. In the same year, the patient underwent liver transplantation. Two years after the transplantation, the patient spontaneously conceived and delivered vaginally a healthy child.Wilsonova bolest je rijedak autosomno recesivni poremećaj metabolizma bakra. Uzrokuje cirozu jetre te posljedično poremećaj menstruacijskog ciklusa i infertilitet. Uspješna dekuprinizacija može dovesti do ponovne pojave ovulacijskih ciklusa i omogućiti trudnoću. Povećane vrijednosti bakra mogu uzrokovati preeklampsiju, intrauterini zastoj u rastu te neurološka oštećenja ploda. Trudnoća kod trudnica s dekompenziranom cirozom jetre povećava komplikacije kod majke, uključujući krvarenje iz varikoziteta jednjaka, zatajenje jetre, encefalopatije i rupture lijenalne arterije. Prikazuje se bolesnica s Wilsonovom bolešću koja je tri puta spontano zanijela. Prva trudnoća okončana je porodom zdravog djeteta u terminu. Druga trudnoća prekinuta je u 12. tjednu medicinski induciranim pobačajem zbog pogoršanja osnovne bolesti, ciroze jetre s portalnom hipertenzijom. Iste godine u bolesnice je učinjena transplantacija jetre, a dvije godine nakon transplantacije spontano je zanijela i vaginalno rodila zdravo dijete
