Abstract

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder in the world and more than 400 million people have a deficiency in this enzyme. G6PD deficiency is an X-linked disorder, and one of its important signs is the presence of hemolytic anemia. It is a worldwide important cause of neonatal jaundice and causes life threatening hemolytic crisis in childhood. Here a 2- year- old boy was admitted into department of pediatrics of Sylhet Women’s Medical College Hospital with fever for 2 days & passage of dark color urine for 1 day. There was history of accidental naphthalene ingestion present prior to develop symptoms. The boy was clinically febrile, severely pale, anicteric. No organomegaly was present. It was confirmed by G6PD level assay. Symptomatic & supportive treatment was given with packed cell volume transfusion and avoidance of triggering factors was advised. Genetic counseling was also done.

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