Transplant surgeon’s strategies in cases of cholestatic jaundice in children of the first months of life
DOI:
https://doi.org/10.15574/SP.2025.3(147).6066Keywords:
jaundice, biliary atresia, Bayler's disease, Alagille syndrome, children, liver transplantation, chronic cholestasis, metabolic diseases, Caroli's diseaseAbstract
Aim. To study the features of differential diagnosis of cholestatic jaundice in children of the first months of life, the terms and methods of conservative and/or surgical treatment before the development of irreversible cirrhotic changes in the liver.
Materials and methods. The object of the study was 274 children aged 1 to 8.5 months with clinical, biochemical, instrumental signs of hepatobiliary pathology against the background of delayed physical and neuropsychiatric development. The research methods included, in addition to general clinical, instrumental, determination of markers of genetic diseases, viral hepatitis, TORCH infections; histological studies of liver biopsies, multidisciplinary consultations.
Results. During the examination of children, the following results were obtained: Bayler's disease and syndrome - 2, Alazhil syndrome - 4, tyrosinemia - 1, α1-antitrypsin deficiency - 1, Caroli's disease - 1, galactosemia - 1, extrahepatic choledochal cysts - 2, biliary atresia (BA) - 56, neonatal hepatitis - 186 children. Against the background of treatment (conservative and surgical), biochemical and clinical indicators improved or normalized. In some orphan diseases, liver transplantation (LT) in seven children ensured a full-fledged quality of life. The term of surgical interventions (Kasai operation, LT) in cases of BA was up to 60 days of life. The volume of surgical interventions depended on the form and type of BA, indications for LT. LT was performed in 37 children with BA. Three children with cholestatic hepatitis died due to concomitant multiple congenital malformations and critical congenital malformations of the heart and large vessels.
Conclusions. Diagnosis of metabolic hereditary orphan cholestatic diseases should begin with neonatal screening, but at present it is not available for all these diseases in Ukraine. Therefore, even before the age of 1 month, orphan diseases with cholestasis should be diagnosed. LT is indicated for patients in whom the cholestatic process is diagnosed late, in case of recurrent cholangitis after KO, and in the presence of a threat of multiorgan failure.
The study was performed in accordance with the principles of the Declaration of Helsinki. The study protocol was approved by the Local Ethics Committee. Informed consent was obtained from the children's parents for the study.
The author declares that there is no conflict of interest.
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