View source for Glucose-6-phosphate deficiency

==Background==
[[File:Pathology of G6PD deficiency 2.png|thumb|G6PD pathway]]
*Abbreviation: G6PD
*X-Linked recessive; protects against [[Malaria]]
*Mild form exists, which predominantly affects men of African decent. It is typically self-limited because it affects older RBCs (with less G6PD left)
*More severe form occurs predominantly in men of Mediterranean descent (specifically Greeks and Italians). Causes a more severe hemolytic anemia because RBCs of any age are involved
*Nonimmune mediated [[hemolytic anemia]]
*Stress or drugs can cause hemoglobin precipitation within the RBC
**Leads to removal of the cell from circulation via the spleen

===Precipitants===
*[[Infection]]
*Fava beans
*Medications
**[[Nitrofurantoin]]
**[[Phenazopyridine]]
**[[Dapsone]]
**[[Chloramphenicol]]
**[[Antimalarials]] (e.g. [[quinine]], [[mefloquine]])
**[[Sulfonamides]]
**[[Ciprofloxacin]], norfloxacin
**[[Methylene blue]]
**Vitamin K analogues (e.g. [[warfarin]])

==Clinical Features==
[[File:Jaundice.jpg|thumb|Pediatric jaundice with icterus of sclera.]]
*[[Hemolytic anemia]]
*[[Fatigue]]
*[[Jaundice]]
*Splenomegaly

==Complications==
*Severe hemolysis and [[anemia]]
*[[shock|Cardiovascular collapse]]

==Differential Diagnosis==
{{Anemia DDX}}

==Evaluation==
===Workup===
*CBC
**Heinz Bodies and Bite Cells on peripheral smear
*Retic Count
**Retic count high
***Coombs negative: G6PD, SCD, spherocytosis, microangiopathic hemolysis

==Management<ref>Schick P et al. eMedicine. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management. Sep 29, 2015. http://emedicine.medscape.com/article/200390-treatment#showall</ref>==
*Identify and discontinue precipitating agent
*Supportive care for anemia, with [[pRBCs|transfusions]] rarely needed
*Hemolysis usually self-limited, resolving within 8-14 days
*Infants
**Prolonged neonatal jaundice due to G6PD deficiency may require phototherapy
**[[Exchange transfusion]] for severe neonatal jaundice

===Alternative Antibiotics===
*[[Cephalexin]] (Keflex)

==Disposition==

==Also See==
*[[Anemia]]
*[[Malaria]]

==References==
<references/>

[[Category:Heme/Onc]]