O.V. KISELNIKOVA¹, L.I. MOZZHUKHINA¹, E.A. PANOVA², V.F. TEYF², E.A. KISELNIKOVA¹, A.A. SNIGIREV¹

 ¹Yaroslavl State Medical University, Yaroslavl

²Regional Children’s Clinical Hospital, Yaroslavl

 Contact details:

Kiselnikova O.V. — PhD (medicine), Associate Professor of the Pediatrics Department

Address: 5 Revolyutsionnaya St., Yaroslavl, tel.: +7-910-975-00-04, e-mail: kiselnikova1@yandex.ru

One of the early manifestations of a wide range of the hepatobiliary system diseases in pediatric practice is hyperbilirubinemia syndrome. The greatest concern is caused by conditions accompanied by «direct» hyperbilirubinemia, in which the level of conjugated bilirubin is more than 15% of the total level. Such hyperbilirubinemia is commonly called «cholestatic». Cholestatic liver diseases are very diverse, while underestimation of the pathological process dynamics and delayed therapeutic intervention can lead to death or severe disability. The article presents the clinical and anamnestic features of newborns and young children with the syndrome of «direct» hyperbilirubinemia to optimize the diagnosis and tactics of management, and a clinical case of late diagnosis of biliary atresia with a hyperbilirubinemia syndrome.

Key words: young children, conjugated hyperbilirubinemia, cholestasis, biliary atresia, gamma-glutamyl transpeptidase.

 REFERENCES

1. Degtyareva A.V., Degtyarev D.N. Modern principles of diagnosis and treatment of non-hemolytic hyperbilirubinemia in full-term and «late» premature children in the early neonatal period. Neonatologiya: novosti, mneniya, obuchenie, 2014, no. 3, pp. 67–76 (in Russ.).
2. Goryaynova A.N., Antsupova M.A., Zakharova I.N. Jaundice in a healthy newborn: causes, course, prognosis. Meditsinskiy sovet, 2017, no. 19, pp. 120–125 (in Russ.).
3. Zakharova I.N. Gepatomegalii u detey: posobie dlya vrachey-pediatrov [Hepatomegaly in children: a guide for pediatricians]. Moscow: Forte Print, 2012. 136 p.
4. Degtyareva A.V., Mukhina Yu.G., Volodin N.N. Differential diagnosis and treatment of cholestasis syndrome in newborns. (recommendations of RASPM, project). Voprosy prakticheskoy pediatrii, 2007, vol. 2, no. 1, pp. 55–63 (in Russ.).
5. Moyer V., Freese D.K., Whitington P.F., Olson A.D. et al. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr, 2004, vol. 39 (2), pp. 115–128.
6. Korovina N.A., Zakharova I.N., Pykov M.I., Mukhina Yu.G., Degtyareva A.V., Tumanova E.L., Malova N.E. Sindrom kholestaza u detey [Cholestasis syndrome in children]. Moscow: «Adamant», 2006. 91 p.
7. Ling S.C. Songenital cholestatic syndromes: what happens when children grow up. Can J Gastroenterol, 2007, vol. 21 (11), pp. 743–751.
8. Mukhina Yu.G., Degtyareva A.V. Kholestaz u novorozhdennykh i detey pervykh mesyatsev zhizni [Cholestasis in newborns and children of the first months of life]. Detskaya gastroenterologiya, part 11. Moscow, 2002. Pp. 306–351.
9. Degtyareva A.V. Atresia of the extrahepatic bile ducts. Rossiyskiy zhurnal gastroenterologii, gepatologii, kolonoproktologii, 2005, vol. 15, no. 6, pp. 8–16 (in Russ.).
10. Wang W., Zheng S. Studies of virus and immune system responses in biliary atresia. Int. J. Pediatr, 2006, vol. 33, pp. 270–271.