Structural and functional changes in organs of the abdominal cavity in patients with Wilson’s disease

The paper presents the results of the ultrasound diagnosis of 76 patients with neurological forms of hepatocerebral dystrophy, or Wilson’s disease (WD), who were examined and treated at the clinic of the Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine. According to ultrasound diagnosis, all patients had pathological changes in the liver. In 58 % of patients, these changes corresponded to chronic hepatitis, in 42 % — to liver cirrhosis. 32 % of patients had evidence of portal hypertension. A Doppler test showed that background hepatic hemodynamics in patients with neurological forms of hepatocerebral dystrophy was within normal limits, but 82 % of patients had an impaired reciprocal autoregulation of liver microcirculation. It indicates a decrease in the compensatory and adaptive capabilities of the liver. This position is confirmed by the fact that 70 % of such patients have a decrease in the vasoactive function of the endothelium. In general, the indicator for the group was only 8.12 %, with a norm of 10 % or more. Despite the young average age of our patients (29.7 years), only 30 % of them had a normal vasoactive reaction. These were patients under the age of 25 with chronic hepatitis. The degree of endothelial dysfunction was significantly higher in patients with liver cirrhosis compared to those with chronic hepatitis. According to ultrasound elastography, most examined patients with WD (88 %) had increased stiffness of the liver parenchyma. On average, it was 10.62 kPa with a range from 4.74 to 20.69 kPa (norm 0.4–6.0 kPa). Thus, patients with neurological forms of WD who are observed by a neuropathologist should undergo an abdominal ultrasound before each course of treatment, but at least 1–2 times a year.