Glucose-6-Phosphate Dehydrogenase


Sunquest Code:G6PDH  
Epic Code:LAB571Epic Name:Glucose 6 Phosphate Dehydrogenase
Synonyms:G-6-PDH; G6PD
Methodology:Enzymatic
CPT Code:82955
Turnaround Time:Specimens are sent to reference laboratory Mon-Sat; results are reported in 1-2 days.


Collection Instructions

Specimen:Blood
Optimal Volume:3 mL
Minimum\Peds Volume:1.5 mL
Container:Yellow (ACD, Solution A) tube available from laboratory (Green (lithium heparin, no gel), Green (sodium heparin, no gel), Purple (EDTA))
Causes for Rejection:Hemolyzed or frozen specimens.


Processing and Shipping

Specimen Processing:Whole blood. Do not process. Store in refrigerator. Do not freeze.
Shipping Instructions:Ship at refrigerated temperature.
Test Performed at or Referral Lab Lab Sendouts  (ARUP)
Referral Lab number:80135 (G6PD)


Interpretive

Reference Range:Effective Nov 17, 2014: 9.9-16.6 U/g Hg Note: Patients who have recently received transfusions have normal donor cells that may mask G-6-PD deficient erythrocytes.
Use:

Glucose-6-phosphate dehydrogenase (G-6-PD) catalyzes the first step in the pentose phosphate pathway, oxidizing glucose-6-phosphate to 6-phosphogluconate and reducing NADP to NADPH. G-6-PD deficiency in erythrocytes is a major cause of congenital non-spherocytic hemolytic anemia.

 

This deficiency affects approximately 200 million people worldwide, especially those individuals originating from Southeast Asia, Africa, the Mediterranean and India. In this disorder of RBC metabolism, a decreased enzyme level is found in older erythrocytes, resulting in RBC lysis in response to certain drugs (primaquine, chloramphenicol, acetanilide, phenacetin, vitamin K, sulfonamides and nitrofurans). Variants of G-6-PD-deficiency differ in the degree of residual enzyme activity (severe to mild). Severe deficiency decreases the RBC life span to such an extent that a chronic hemolytic anemia with jaundice is found even in the absence of a drug challenge, Females with G-6-PD deficiency may have difficulty in carrying pregnancy to term, possibly because of the effect of changing hormones on the sensitive RBCs.

 



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