Bilirubin Induced Encephalopathy

Parvaneh Karimzadeh, Minoo Fallahi, Mohammad Kazemian, Naeeme Taslimi Taleghani, Shamsollah Nouripour, Mitra Radfar, Parvaneh Karimzadeh, Minoo Fallahi, Mohammad Kazemian, Naeeme Taslimi Taleghani, Shamsollah Nouripour, Mitra Radfar

Abstract

Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy (BIE) (2). The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with the risk factors for increased the blood level of indirect bilirubin are at risk for BIE, especially preterm neonates which are prone to low bilirubin kernicterus . BIE can be transient and acute (with early, intermediate and advanced phases)or be permanent, chronic and lifelong ( with tetrad of symptoms including visual (upward gaze palsy), auditory (sensory neural hearing loss), dental dysplasia abnormalities, and extrapyramidal disturbances (choreoathetosis cerebral palsy).Beside the abnormal neurologic manifestations of the jaundiced neonates ,brain MRI is the best imaging modality for the confirmation of the diagnosis. Although early treatment of extreme hyperbilirubinemia by phototherapy and exchange transfusion can prevent the BIE, unfortunately the chronic bilirubin encephalopathy does not have definitive treatment.

Keywords: Bilirubin Induced Encephalopathy; Kernicterus; Neonatal Jaundice.

Conflict of interest statement

The authors declare that there is no conflict of interests.

Figures

Figure-1
Figure-1
ABE. Coronal section through posterolateral lobes.(From:Zangen S, et al,fatal kernicterus in a girl deficient in G6OD,a paradigm of synergistic heterozygosity.J Pediatri.2009;154:616-619) 1 Hippocampus,2- Basal ganglia ,3-Substantia Nigra ,4-Thalamus
Figure-2
Figure-2
Back arching and opisthotonos in a 1-month-old neonate with kernicterus. The kernicterus was secondary to Crigler-Najjar syndrome
Figure -3)
Figure -3)
kernicterus face, Sixth Gree
Figure-4
Figure-4
Enamel dysplasia as a complication of BIE, Barberio GS
Figure -5
Figure -5
A: Coronal T2-weighted MRI sequence showing a bilateral, symmetrical hyperintense signal in the subthalamic nuclei (arrows), without a mass effect. B: Axial FLAIR MRI sequence showing a bilateral, symmetrical hyperintense signal in the globus pallidus (arrows). C: Axial diffusion-weighted MRI sequence showing no diffusion restriction. D: Axial T1-weighted MRI sequence showing no evidence of gadolinium enhancement

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