ESPE Abstracts (2016) 86 P-P1-232

Extrahepatic Biliary Atresia in Combination with Toxic Cholestasis Due to Glibenclamide in a Case of Neonatal Diabetes

Thomas Kapellena, Gunter Flemminga, Heike Bartelta, Robin Wachowiakb & Wieland Kiessa

aHospital for Children and Adolescents, Leipzig, Germany; bHospital for Pediatric Surgery, Leipzig, Germany

Background: More than 20 gene loci are known to cause monogenic neonatal diabetes today. A definite mutation can be found in 65–70% of all cases. Mutations in the ATP sensitive potassium channel can frequently be treated by sulfonylurea. Glibenclamide is on of the drugs known to inhibit the bile salt export pump (BSEP). However most drug induced cholestasis cases are reported in adults.

Objective and hypotheses: Glibenclamide is used frequently to treat neonates with monogenic diabetes. To date there is no case of drug induced cholestasis reported in neonates. Extrahepatic biliary atresia seems to develop either intrauterine but in some cases even after birth. Causing factors are not known exactly. We hypothesize that the coincidence of extrahepatic biliary atresia in a patient treated with glibenclamide because of neonatal diabetes could be partially explained by drug induced cholestasis.

Method: We report about a boy with neonatal diabetes due to a KCNJ11 missense mutation diagnosed in the age of 2 weeks.

Results: Glibenclamide was started with very low doses (0.0125 mg/kgKG) immediately after diagnosis. Cholestatic icterus developed with age of 9 weeks. Glibenclamide was stopped immediately. Liver biopsy showed signs of extrahepatic cholestasis but also possible toxic signs. There was no improvement with conservative treatment. Intraoperative exploration and cholangiography showed an extrahepatic billary atresia. Biliodigestive anastomosis with Y-Roux (Kasai) was established by the pediatric surgeons. Diabetes is in remission at the moment. Cholestasis has completely recovered.

Conclusion: Cholestasis due to glibenclamide has to be taken into account when treatment is initiated in neonates with monogenic diabetes. Therefore we would recommend to monitor cholestatic parameters in a certain algorithm.

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