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Abstract
Background: Crigler-Najjar syndrome (CNS) is a rare genetic disorder characterized by an inability to conjugate bilirubin, untreated babies with CNS type I rapidly develop high plasma level of UCB (20-50 mg/dl) which result in serious neurological damage and ultimately lead to death. MRS is a valuable non-invasive MR technique for monitoring brain metabolism in vivo that may shed new insights into the pathogenesis of bilirubin-induced brain injury and the neural basis of long term disability in infants and children with chronic bilirubin encephalopathy. Objective: To document neurologic, neurodevelopmental finding of cases suffering from CNS type I and MRS findings then result were described and correlated. Study design and methodology: A cross sectional study was conducted at Cairo university pediatric hospital in the period between April 2018 to January 2019. The participants of the study were 35 children aged 1-12 years old, divided into 2 groups, the first group consist of 23 children with CNS type I & the other group consist of 12 children as control group. All children had thorough physical, neurologic and there developmental status was tested, MRI and MRS were performed on all patients