Skip to main content

Glucose-6-Phosphate Dehydrogenase Deficiency, Pediatric

Kandace L. Gollomp, MD and Michele Puszkarczuk Lambert, MD, MSTR Reviewed 10/2018
 


BASICS

DESCRIPTION

Deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD) that leads to increased RBC vulnerability to oxidative stress and may cause hemolytic anemia. Several types of genet...

DIAGNOSIS

HISTORY

  • Symptoms of anemia include fatigue, irritability, and malaise.

  • Dark urine (cola or tea colored) may follow moderate to severe hemolysis; may develop jaundice (particularly scleral icte...

TREATMENT

GENERAL MEASURES

  • Removal of the oxidant stressor is of primary importance:

    • Discontinue the suspected drug and/or treat the infection.

      • In class 3 and 4 patients, essential drug therapy may be co...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Most deficient individuals remain asymptomatic.

  • When hemolysis does occur, it tends to be self-limited and resolves spontaneously, with a return to normal hemoglob...

ADDITIONAL READING

  • Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet.  2008;371(9606):64–74. [View Abstract on OvidInsights]

  • Frank JE. Diagnosis and management of G6P...

CODES

ICD9

282.2 Anemias due to disorders of glutathione metabolism 

ICD10

D55.0 Anemia due to glucose-6-phosphate dehydrogenase deficiency 

SNOMED

  • 62403005 Glucose-6-phosphate dehydrogenase deficiency ane...

FAQ

  • Q: Do I need to follow a special diet or avoid medications if I have G6PD deficiency?

  • A: Although most patients will have no symptoms of their disease, certain medications may cause transient hemol...

Subscribe to Access Full Content

Sign Up for a 10-Day Free Trial

Sign up for a 10-day FREE Trial now and receive full access to all content.

 
×