Korean J Perinatol.
2006 Mar;17(1):89-93.
Jaundice and Hemolytic Anemia Appearing within the First 24 Hour of Life due to Glucose-6-Phosphate Dehydrogenase Deficiency
- Affiliations
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- 1Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. ljhped@catholic.ac.kr
Abstract
- The most devastating clinical consequence of G-6-PD deficiency is neonatal hyperbilirubinemia which can be severe and result in kernicterus or even death, although glucose-6-phosphate dehydrogenase deficiency is responsible for two clinical syndromes, an episodic hemolytic anemia induced by infections or certain drugs and spontaneous chronic nonspherocytic hemolytic anemia. In the pathogenesis of neonatal hyperbilirubinemia associated with G-6-PD deficiency, decreased elimination of bilirubin has been suspected to be a key factor, because these neonates usually do not develop frank anemia even in the presence of severe hyperbilirubinemia. But, we experienced a glucose-6-phosphate dehydrogenase deficient male patient who showed jaundice and severe hemolytic anemia appearing within the first 24 hour of life. The patient had resolution of symptoms after phototherapy and transfusion. We report this case with a brief review of the related literatures.