VIOLATION OF THE STRUCTURE AND FUNCTIONS IN CASES OF WILSON’S DISEASE IN CHILDREN: THE CURRENT STATE OF THE PROBLEM

In article we presented dynamic analysis of liver function and structure in children with Wilson’s disease based on the author’s system for determination the degree of liver function and structure disorder that was created on the basis of the International Classification of Functioning using the statistical analysis of the digital expression of a set of changes in indices reflecting major violations of liver function and structures and severity of portal hypertension. We found that that the first examination in children with Wilson’s disease in 96,6 % of cases showed a decrease in the liver function by 25,0-95,0 % corresponding to a moderate and severe degree of impairment, whereas after 6 and 12 months of treatment, similar changes registered significantly less often - in 87,5 %, and 86,6 % of patients, respectively. Moderate and severe disruption of liver structure and severity of portal hypertension according to the offered system at the first hospitalization was noted at 77,8 % of children with cirrhosis in the outcome of Wilson’s disease, after 6 months of therapy in 50,0 % of patients, and after 12 months - only in 36,0 % of children. The ROC analysis of the proposed scoring system established its moderate significance in determining indications for liver transplantation in terms of the extent of liver function impairment and high significance in determining the extent of liver structure disorder and the severity of portal hypertension in children with Wilson’s disease. Thus, the proposed scoring systems for assessing the degree of impairment of the function and structure of the liver can be an objective criterion for assessing the severity of liver damage, controlling its changes in dynamics, assessing the effectiveness of the therapy and indications for liver transplantation. The proposed method can be used to create of “the patient’s model” with Wilson’s disease, followed by the development of standards and protocols for the provision of medical care, and also used in the medical and social expertise of children to determine the degree of disruption of the structure and function of the liver when determining the category of “disabled child”.

Wilson’s disease, children, liver function disorder, liver structure disorder, portal hypertension, cirrhosis, international classification of functioning, medical and social expertise

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