Aspecte etiopatogenetice și de diagnostic al fibrozei hepatice la copii.

Introduction. Hepatic fibrosis (HF) is a complication of a congenital or acquired pathological process caused by an excess of non-functional liver tissue. The study aim was to assess the etiopathogenetic and diagnostic aspects of HF in children to evaluate surgical treatment. Materials and methods. The observational study was conducted within the CNSPCPP "Natalia Gheorghiu" of the MPHI Institute of Mother and Child, during 2018-2023, and reflects the results of the examination of 26 children aged 0-18 years diagnosed with HF caused by some congenital and acquired diseases. Paraclinical examinations included: liver ultrasound (USG) / liver elastography, MRI and CT imaging of the liver, molecular genetic testing (whole genome sequencing, next generation sequencing (NGS)), and liver biopsy. Statistical processing: through the Med Star (Medical Student Addiction Research) program. Results. In the 26 children diagnosed with HF, the following congenital pathologies were found Wilson's disease - 3 (11.53%) cases, biliary atresia - 6 (23.08%), autoimmune hepatitis - 2 (7.69%), sclerosing cholangitis - 2 (7.69%), glycogenosis - 2 (7.69%), congenital liver fibrosis - 2 (7.69%), hereditary hemochromatosis - 2 (7.69%) cases, as well as acquired pathologies such as: hepatitis B - 4 (15. 38%), hepatitis C - 3 (11.53%). In all children suspected of HF, changes in the biochemical examination of blood were detected, by increasing the values of liver enzymes, direct and indirect bilirubin, GGTP, alkaline phosphatase, LCTG, cholesterol, as well as increased levels of amylase, beta-lipoproteins, phospholipids, 5-nucleotidase, lipoprotein. There was a decrease in serum levels of total protein, albumin, and disorders of the coagulation system. USG/hepatic elastography revealed hepatosplenomegaly, increased echogenicity of liver tissue, decreased transhepatic perfusion with portal hypertension (HTP), and collaterals in the splanchnic bed. MRI of the liver was performed in 8 (30.78%) children in the cholangiographic regime and by CT with venous and arterial phase angiography - in 10 (38.46%) children. Characteristic changes for HF were assessed. The severity of HF was eloquent by the method of liver biopsy performed pre-and post-surgery. Histological changes of HF with varying degrees of impairment were found in all patients examined. Molecular genetic methods confirmed genetic diseases in 11 children with HF. Conclusions. HF occurs in many genetic and acquired pathologies and is associated with complex pathological mechanisms, with severe consequences and poor prognosis for the child's health. Early recognition of the symptoms of HF categorizes the investigations to identify the etiology of liver damage and suggests the type of treatment recommended individually. [ABSTRACT FROM AUTHOR]