01-06-2014 | Clinical Quiz
Cyanosis in a male Nigerian infant with acute kidney injury: Answers
Published in: Pediatric Nephrology | Issue 6/2014
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1.
Oxygen-resistant cyanosis in the absence of cardiorespiratory causes indicates methemoglobinemia. This patient developed methemoglobinemia as a consequence of rasburicase therapy in the setting of glucose-6-phosphate dehydrogenase (G6PD) deficiency.
2.
A blood gas analyzer can reliably measure the hemoglobin (Hb) subfractions in an arterial blood sample, including methemoglobin (metHb), with the spectrophotometric method. G6PD deficiency can be diagnosed biochemically or by molecular analysis of the G6PD gene.
3.
Patients of African, Asian, Mediterranean and Middle Eastern descent, as well as patients with a history and/or family history of unexplained cyanosis, hemolytic anemia, or unexplained jaundice are particularly at risk for G6PD deficiency and should be screened for this enzyme deficiency prior to rasburicase administration.