Test Overview
Clinical Utility
Evaluation of patients with cyanosis
Confirming cases of suspected cytochrome b5 reductase (methemoglobin reductase) deficiency
Functional studies in families with cytochrome b5 reductase deficiency
Method
Spectrophotometry - kinetic
Method Description
Cytochrome b5 reductase (methemoglobin reductase) catalyzes the 1,4-dihydronicotinamide adenine dinucleotide (NADH)-linked reduction of several substrates, including ferricyanide. The activity at 37 degrees C is measured spectrophotometrically by measuring the oxidation of NADH to NAD(+) at 340 nm on an automated chemistry analyzer.
Result Included
Cytochrome b5 Reductase
Aliases/Synonyms
Diaphorase; Ferrihemoglobin Reductase; Methemoglobin Reductase; Methemoglobin Diaphorase
Specimen
Specimen Type
BloodContainers
Preferred Containers
Yellow top (ACD)
Acceptable Containers
Lavender top (EDTA)
Volume
Sample Volume
6.0 mL
Minimum Volume
1.0 mL
Collection & Handling
Handling Information
Send whole blood specimen in original tube. Do not aliquot.
Stability
| Refrigerated |
|---|
| 18 days |
Rejection Criteria
| Criteria | Specification |
|---|---|
| Hemolysis | Gross |
Performance & Interpretation
Turnaround Time
10 days
Results
-
Cytochrome b5 ReductaseU/g Hb≥12 months: 7.8 - 13.1
Comment
Reference values have not been established for patients who are younger than 12 months.
Cytochrome b5 reductase (methemoglobin reductase) activity in neonates (0-6 weeks of age) is normally 60% of the normal adult value. Adult values are attained by 2 to 3 months of age.
Heterozygotes have results slightly lower than the reference range. Homozygotes demonstrate little to no cytochrome b5 reductase activity and increased levels of methemoglobin.
Individuals who are glucose-6-phosphate dehydrogenase deficient are not candidates for methylene blue therapy. Administration of methylene blue to such persons will cause hemolysis or methemoglobin formation.
Referral Location
Out-of-Country
Interface & Setup
HL7 Interface Codes
| Order Code | Result Name | Result Codes | Units |
|---|---|---|---|
| CYB5RED WB | Cytochrome b5 Reductase, B | 65501 | U/g Hb |
Test Version
Last Updated
2025-11-11