Abstract
Our aim was to on the basis of determining the degree of violation of the structure and function of the liver establish their relationships and to assess the dynamics of liver disease in its chronic illnesses in children. With the help of the developed scoring systems were used to assess the degree of liver dysfunction and the degree of disruption of the structure of the liver and the severity of portal hypertension. The results of the diagnostic methods 252 children aged 1 to 17 years (mean age of 11.8±3,5) with Wilson disease (WD), autoimmune hepatitis (AIH), chronic hepatitis C (CHC) were analyzed; 48 patients underwent liver transplantation. In children with WD, AIHand CHC liverfunction reduced by 41.3±12.9% to 28.8±12.5% and 19.1±7.8% respectively. Structure of the liver in children with WD, AIH and CHC was disturbed by 25.0±8.1% to 20.4±9.2% and 6.8±4.4% respectively. Thefunction and structure violations of the liver more pronounced in liver cirrhosis. The use of the developed scoring systems to monitor the severity of liver damage in the dynamics and evaluation of the effectiveness of the therapy is demonstrated. The degree of liver dysfunction is directly dependent on the degree of its structure. Abnormal liverfunction ≥40% and ≥40% of its structure with treatment failure can be used as a criterion of indicationsfor elective liver transplantation with its chronic diseases in children. Developed a point system to determine liver function and a point system to determine disruption of the structure of the liver and the severity of portal hypertension in children can serve as an objective criterion for assessing the severity of liver disease, monitoring their changes in the dynamics with the assessment of the effectiveness of the therapy and making decisions about the need for routine liver transplantation in its chronic illnesses in children.
Highlights
Developed a point system to determine liver function and a point system to determine disruption of the structure of the liver and the severity of portal hypertension in children can serve as an objective criterion for assessing the severity of liver disease, monitoring their changes in the dynamics with the assessment of the effectiveness of the therapy and making decisions about the need for routine liver transplantation in its chronic illnesses in children
Squires J.E. 50 years ago in the Journal of Pediatrics: needle biopsy of the liver in infants and children
Summary
Цель исследования: установить влияние нарушений структуры печени на степень расстройства функции и динамику их изменений при хронических болезнях печени у детей. У детей с БВ, АИГ и ХГС функция печени была снижена на 41,3±12,9, 28,8±12,5 и 19,1±7,8%, соответственно. Нарушение функции печени и ее структуры ≥40% при неэффективности терапии могут послужить критерием показаний к плановой трансплантации печени при ее хронических болезнях у детей. Заключение: разработанные балльные системы определения степени нарушения структуры и функции печени и выраженности портальной гипертензии у детей могут служить объективным критерием оценки степени тяжести поражения печени, наблюдения их изменений в динамике с оценкой эффективности проводимой терапии, определения прогноза заболевания и принятия решения о необходимости плановой трансплантации печени при ее хронических болезнях у детей. Структура и функция печени и их нарушения при хронических болезнях гепатобилиарной системы у детей.
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