Nutritional assessment of children with Wilson's disease: single center experience

Nutritional status was accepted as a prognostic marker in children with chronic liver disease. In the literature, we aimed to retrospectively investigate 94 Wilson patients followed in our center due to the lack of studies investigating the frequency and prognostic effects of malnutrition and micronutrient deficiency in Wilson's patient.

Our studies included 94 Wilson's disease children in the Department of Child Gastroenterology, Hepatology and Nutrition of Inonu University Faculty of Medicine between 2006-2017. Presentation patterns, anthropometric measurements, laboratory findings and prognostic factors of these patients were analyzed retrospectively.

The mean age of the patients was 9.11±3.2 (3.5-17) and the female/male ratio was 40/54. Mean age was lower in asymptomatic patients (p=0.000). According to all parameters, malnutrition was detected in 43 patients (45.7%). Fulminant Wilson's disease had higher height and weight z scores than nerowilson patients (p=0.045, p=0.019, respectively). Hypocalcemia, hypophosphatemia, hypouricemia, hypoalbuminemia and anemia were more common in patients with cholestasis than without cholestasis (p<0.001). Vitamin A and E are lower in patients with cholestasis than without cholestasis (p<0.05). Hypocalcemia, hypophosphatemia and hypo-uricemia were found higher in the fulminant group (p<0.001). According to mortality scores (Dhawan, Model for end-stage liver disease and Child-Pugh). In patients with high mortality, height Z score was found to be high (p<0.05).

In Wilson's disease assessment of growth, detailed anthropometric measurements as well as vitamin, trace elements and electrolytes should be closely monitored.