[The Crigler-Najjar syndrome, type I. Therapeutic dilemmas]

Crigler-Najjar syndrome type I is a rare hereditary disease that is manifested by high concentration of unconjugated bilirubin in serum, which may lead to neurologic disorders. Management consists of conservative therapy and partial liver transplantation and, since recently, hepatocyte transplantation. Both therapeutic procedures have been continually improved and are yielding better results. We present a girl, aged three and a half years, who has been living at the Neonatology Department of the University Childrens Hospital in Belgrade since birth due to the permanent need for phototherapy and other measures so as to keep her bilirubin at minimal level. We present numerous management dilemmas, having in mind the controversial attitudes of physicians from other world centres, starting from those who consider that surgery should be done before the age of six years to those who consider that the patient should be treated conservatively only. Our attempts to avoid neurological disorders developing in our little patient surpasses professional interest only, because this girl is an exceptionally lovely and smart child with IQ 128.