Methemoglobin Reductase, Blood
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
Confirming cases of heterozygous or homozygous methemoglobin reductase deficiency
Genetic studies in families with methemoglobin reductase deficiency
Kinetic Spectrophotometry (KS)
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Methemoglobin Reductase, B
Cytochrome B5 Reductase
Methemoglobin Diaphorase (Methemoglobin Reductase)
Methemoglobin Diaphorase (Methemoglobin Reductase)
Specimen Type Describes the specimen type needed for testing
Whole Blood ACD-B
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
Preferred: Yellow top (ACD)
Acceptable: Lavender top (EDTA)
Specimen Volume: 6 mL
Collection Instructions: Do not transfer blood to other containers.
Additional Information: Patient's age is required.
Forms: If not ordering electronically, please submit a Hematopathology/Molecular Oncology Request Form (Supply T241) with the specimen.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Mild OK; Gross reject
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
|Whole Blood ACD-B||Refrigerated||22 days|
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Methemoglobin reductase, also called "diaphorase," and more properly called cytochrome B5 reductase, is the only enzyme within the erythrocyte that maintains hemoglobin in the reduced (non-methemoglobin) state.
Persons who are heterozygous for methemoglobin reductase deficiency have no clinical or laboratory abnormalities, are not cyanotic, and have normal methemoglobin concentrations in their blood.
Persons who are homozygous for methemoglobin reductase deficiency have normal arterial oxygen saturation but have varying quantities of methemoglobin in their blood, generally 15% to 20%, and are quite cyanotic. Paradoxically, homozygotes have normal blood counts; the condition does not cause polycythemia. The reason for this apparent paradox seems to be that the presence of methemoglobin shifts the hemoglobin-O2 dissociation curve to the right, so that although the transport of oxygen is diminished, the delivery of oxygen to tissues is normal. The condition is quite benign, but may cause concern to parents of affected children, be a cosmetic embarrassment to the children, and alarm the attending physician. The cyanosis may be treated with methylene blue.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
6.6-13.3 U/g hemoglobin
Methemoglobin reductase activity in neonates (0-6 weeks) 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 methemoglobin reductase activity and increased levels of methemoglobin.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Individuals who are glucose-6-phosphate-dehydrogenase (G-6-PD) deficient are not candidates for methylene blue therapy. Administration of methylene blue to such persons will cause hemolysis or methemoglobin formation.
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
Beutler E: Methemoglobinemia and other causes of cyanosis. In Williams Hematology. Sixth edition. Edited by E Beutler, M Lichtman, WJ Williams, TJ Kipps. New York, McGraw-Hill Book Company, 2001, p 611
Method Description Describes how the test is performed and provides a method-specific reference
Methemoglobin reductase (cytochrome B5 reductase) catalyzes the NADH-linked reduction of several substrates, including ferricyanide. The activity at 30 degrees C is followed spectrophotometrically by measuring the oxidation of NADH at 340 nm.(Fairbank VF, Klee GG: Biochemical aspects of hematology. In Tietz Textbook of Clinical Chemistry. Third edition. Edited by CA Burtis, ER Ashwood. Philadelphia, WB Saunders Company, 1999, pp 1647-1648)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday through Friday
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
1 day (not reported Saturday or Sunday)
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
Test Classification Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.
CPT Code Information Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
|Result ID||Reporting Name||LOINC Code|
|9322||Methemoglobin Reductase, B||32703-1|