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grantDate 2018-11-23-04:00^^<http://www.w3.org/2001/XMLSchema#date>
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publicationDate 2018-11-23-04:00^^<http://www.w3.org/2001/XMLSchema#date>
publicationNumber RU-2670619-C9
titleOfInvention Method of differential diagnosis of congenital cholestatic diseases in young children
abstract FIELD: medicine. n SUBSTANCE: invention relates to medicine, namely to pediatrics, pediatric gastroenterology and hepatology, and can be used for early and accurate diagnosis of liver damage manifested by cholestasis in children of the first months of life. For differential diagnosis of congenital cholestatic diseases in infants with prolonged jaundice in the neonatal period, which is accompanied by hypo- or achiolia of the stool, and / or hepato- or hepatosplenomegaly, perform hepatobiliary scintigraphy with the determination of the time of maximum accumulation (Tmax) of the radiopharmaceutical in the liver, the elimination half-life (T1/2) of RFP from the liver and the time of RFP entering the intestine. Additionally, biochemical blood test is performed to determine the level of gamma-glutamyltranspeptidase (GGTP), total bilirubin and its fractions and alanine aminotransferase (ALT). Depending on the results of the conducted studies, based on the set of established diagnostic criteria, differential diagnosis of progressive family intrahepatic cholestasis of type 1 or 2, biliary atresia, Alagill syndrome, type 1 hereditary tyrosinemia. n EFFECT: invention provides expansion of possibilities of differential diagnosis by increasing the number of detected nosologies related to congenital cholestatic diseases in young children, simplification, ensuring the availability of research and early diagnosis of diseases, allowing timely selection of the necessary therapeutic tactics. n 1 cl, 1 dwg, 3 ex
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