Cyanosis Due to Methemoglobinemia as the Presenting Sign of Glucose-6-Phosphate Dehydrogenase Deficiency in a Child: Diagnostic and Clinical Implications

Abstract

A previously healthy 3-year-old boy presented with pallor, jaundice, cyanosis, and a 24-hour history of vomiting and anorexia following fava bean ingestion. Clinical examination and laboratory findings were consistent with severe nonimmune hemolytic anemia with methemoglobinemia. Given the patient’s history, a previously unrecognized glucose-6-phosphate dehydrogenase deficiency was suspected and diagnosed. The aim of this article is to delineate the possible coexistence of methemoglobinemia and glucose-6-phosphate dehydrogenase deficiency in children presented with acute hemolysis and discuss its management while reviewing the existing literature.

Recommendations for diagnosis and treatment of methemoglobinemia

“…In case of suspicion of continuous exposure, such as to a pesticide, the medical personnel should wear adequate protective equipment and the patient should be decontaminated. Additionally, it is essential to search for a history of G6PD deficiency 79 . Indeed, methylene blue cannot be used in patients with G6PD as it is ineffective in such patients and it can worsen G6PD deficiency hemolysis.…”

“…Additionally, it is essential to search for a history of G6PD deficiency. 79 Indeed, methylene blue cannot be used in patients with G6PD as it is ineffective in such patients and it can worsen G6PD deficiency hemolysis. However, there are no sufficient evidence to recommend routine functional assay for G6PD activity.…”