Ultrasound in Alagille syndrome diagnosis

50 patients with Alagille syndrome (first group) and 80 patients with biliary atresia (second group) were investigated in the 2000-2016 period. A retrospective analysis of hepatobiliary system, spleen, cardiovascular, and urinary systems ultrasound results was done. The age of the children at admission was 1 month of life. Ultrasound examinations of hepatobiliary system and spleen were carried out at the age of 1, 2 and 3 months of life; cardiovascular system - 1-3 months; urinary system - 1, 2, 3, 6 and 12 months. When carrying out ROC-analysis the ultrasound changes of hepatobiliary system, identified within three months of life, were taken into account. Diagnostic value of an isolated hepatobiliary system ultrasound pathologic signs in Alagille syndrome diagnosis was low. However, the sensitivity of ultrasound “triangular cord" sign in biliary atresia diagnosis was 50%, specificity - 100%, AUC - 0.750. The sensitivity of gallbladder absence or fibrous cord gallbladder form sign in biliary atresia diagnosis was 88%, specificity - 72%, AUC - 0.797. The sensitivity of diffuse periportal echogenicity sign in the biliary atresia diagnosis was 100%, specificity - 88% and AUC - 0.940. It should be noted that above mentioned signs were found on background of cholestasis, hepatomegaly and splenomegaly (100% patients with biliary atresia at the age of 3 months old). Pathologic changes of kidneys, manifested by 3 months of life, were significantly increasing in occurrence frequency by 12 months (P from 0.0295 to 0. 0001). In biliary atresia ultrasound changes in the kidneys were absent. Iincreased renal echogenicity, decreased corticomedullary differentiation on background of cholestasis have a relatively high sensitivity (70% and 64% respectively) and 100% specificity in the diagnosis of Alagille syndrome (ultrasound signs identified at 12 months of life). Ultrasound findings in hepatobiliary system organs and spleen, cardiovascular and urinary systems provide useful information for differential diagnosis and adequate treatment of infants with cholestasis.

ультразвуковая диагностика, холестаз, синдром Алажилля, билиарная атрезия, симптом “треугольного рубца”, симптом “перипортального фиброза”, повышение эхогенности паренхимы почек, нарушение кортико-медуллярной дифференцировки, дети раннего возраста, ultrasound diagnostics, cholestasis, Alagille syndrome, biliary atresia, “triangular cord", diffuse periportal echogenicity, increased renal echogenicity, reduced corticomedullary differentiation, infants

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