Perinatal/Neonatal Case Presentation

Journal of Perinatology (2008) 28, 306–309; doi:10.1038/sj.jp.7211919

Severe hemolysis with normal blood count in a glucose-6-phosphate dehydrogenase deficient neonate

M Kaplan1,2, C Hammerman1,2, H J Vreman3, R J Wong3 and D K Stevenson3

  1. 1Department of Neonatology, Shaare Zedek Medical Center, Jerusalem, Israel
  2. 2Faculty of Medicine of the Hebrew University, Jerusalem, Israel
  3. 3Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA

Correspondence: Dr M Kaplan, Department of Neonatology, Shaare Zedek Medical Center, PO Box 3235, Jerusalem 91031, Israel. E-Mail: kaplan@cc.huji.ac.il

Received 4 September 2007; Revised 29 October 2007; Accepted 28 November 2007.

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Abstract

A premature glucose-6-phosphate dehydrogenase (G-6-PD) deficient neonate was readmitted for exponential rise in the plasma bilirubin concentration to 33.0 mg dl-1. Blood carboxyhemoglobin (2.8% of total hemoglobin, >threefold normal value) confirmed the presence of hemolysis; however, hematological indices were unchanged from the birth hospitalization. Serum unbound bilirubin, although present, was probably at a concentration insufficient to cause bilirubin encephalopathy. In G-6-PD deficient neonates, severe hemolysis may occur in the absence of hematological changes typical of a hemolytic process.

Keywords:

glucose-6-phosphate dehydrogenase deficiency, bilirubin, hemolysis, unbound bilirubin, hemoglobin, carboxyhemoglobin

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