摘要
Aim: The problem of kernicterus in infants with bronze baby syndrome (BBS) has been reviewed on the basis of cases reported in the literature. In addition, a new case concerning an infant with severe Rh haemolytic disease, who presented with BBS and who has developed neurological manifestations of kernicterus with magnetic resonance images showing basal ganglia abnormalities, is presented. In this patient, the total serum bilirubin (TSB) concentration ranged from 18.0 to 22.8 mg/dl (306 to 388 μmol/l) and the bilirubin/albumin (B/A) ratio was 6.0 (mg/g) (6.8 is the value at which an exchange transfusion should be considered). The case presented is important due to the fact that kernicterus appeared after an exchange transfusion was performed when the TSB level reached 22.8mg/dl (388 μmol/l) on 6th day of life while the haematocrit was 30%. From this case and fromother cases reported in the literature, we must stress that, even if the level at which hyperbilirubinemia poses a threat remains undefined, BBS may constitute an additional risk of developing kernicterus. Conclusion: The possible strategies for implementing an approach to the management of hyperbilirubinemia (especially the haemolytic kind) in the presence of BBS may include an exchange transfusion carried out at lower TSB concentration than previously recommended or an early administration of Sn-mesoporphyrin.
Aim: The problem of kernicterus in infants with bronze baby syndrome (BBS) has been reviewed on the basis of cases reported in the literature. In addition, a new case concerning an infant with severe Rh haemolytic disease, who presented with BBS and who has developed neurological manifestations of kernicterus with magnetic resonance images showing basal ganglia abnormalities, is presented. In this patient, the total serum bilirubin (TSB) concentration ranged from 18.0 to 22.8 mg/dl (306 to 388 μmol/l) and the bilirubin/albumin (B/A) ratio was 6.0 (mg/g) (6.8 is the value at which an exchange transfusion should be considered). The case presented is important due to the fact that kernicterus appeared after an exchange transfusion was performed when the TSB level reached 22.8mg/dl (388 μmol/l) on 6th day of life while the haematocrit was 30%. From this case and fromother cases reported in the literature, we must stress that, even if the level at which hyperbilirubinemia poses a threat remains undefined, BBS may constitute an additional risk of developing kernicterus. Conclusion: The possible strategies for implementing an approach to the management of hyperbilirubinemia (especially the haemolytic kind) in the presence of BBS may include an exchange transfusion carried out at lower TSB concentration than previously recommended or an early administration of Sn-mesoporphyrin.
