Methemoglobinemia is a blood disorder in which an abnormal amount of hemoglobin builds up in the blood. Hemoglobin is the oxygen-carrying molecule found in red blood cells. In some cases of methemoglobinemia, the hemoglobin is unable to carry oxygen effectively to body tissues.
Alternative NamesHemoglobin M disease; Erythrocyte reductase deficiency; Generalized reductase deficiency
Causes, incidence, and risk factorsMethemoglobinemia may be passed down through families (inherited). Or, it may result from exposure to certain drugs, chemicals, or foods (acquired).
There are two forms of inherited methemoglobinemia. The first form is passed on by both parents. The parents usually do not have the condition themselves, but they carry the gene that causes the condition. It occurs when there is a problem with an enzyme called cytochrome b5 reductase.
There are two types of this form of methemoglobinemia:
The second form of inherited methemoglobinemia, called hemoglobin M disease, is caused by defects in the hemoglobin molecule itself. Only one parent needs to pass on the abnormal gene in order for the child to inherit the disease.
Acquired methemoglobinemia is more common than the inherited forms. It occurs after exposure to certain chemicals and drugs, including:
The condition may also occur in infants who are very ill or who are fed too many vegetables containing nitrates (such as beets).
SymptomsSymptoms of type 1 methemoglobinemia (erythrocyte reductase deficiency) include:
Symptoms of type 2 methemoglobinemia (generalized reductase deficiency) include:
Symptoms of hemoglobin M disease include:
Symptoms of acquired methemoglobinemia include:
Methemoglobinemia can be diagnosed with a blood test.
A baby with this condition will have bluish skin color (cyanosis) at birth or shortly thereafter. Arterial blood gases and pulse oximetry results will be normal, making the condition challenging to diagnose.
TreatmentA medicine called methylene blue is used to treat persons with severe methemoglobinemia. Note: Methylene blue may be dangerous in patients who have or may be at risk for a blood disease called G6PD deficiency, and should not be used. If you or your child has G6PD deficiency, always tell your health care provider before receiving treatment.
Ascorbic acidmay also be used to reduce the level of methemoglobin.
Alternative treatments include hyperbaric oxygen therapyand exchange transfusions.
In most cases of mild acquired methemoglobinemia, no treatment is required. However, you should avoid the medicine or chemical that caused the problem. Severe cases may need treatment, such as a blood transfusion.
Expectations (prognosis)People with Type 1 methemoglobinemia and hemoglobin M disease usually do well. Type 2 methemoglobinemia is much more serious, and usually causes death within the first few years of life.
People with acquired methemoglobinemia usually do very well once the drug, food, or chemical that caused the problem is identified and avoided.
ComplicationsCall your health care provider if you have a family history of methemoglobinemia and you develop symptoms of this disorder.
Call your health care provider or emergency services immediately if you have severe shortness of breath.
PreventionGenetic counseling is recommended for couples with a family history of methemoglobinemia who are considering having children.
ReferencesDeBaun MR, Vichinsky E. Hemoglobinopathies. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th Ed. Philadelphia, PA: Saunders Elsevier; 2007: chap 462.
Gregg XT, Prchal JT. Red blood cell enzymopathies. In: Hoffman R, Benz EJ Jr., Shattil SS, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, PA: Elsevier Churchill Livingston; 2008: chap 45.
Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin -- a form of hemoglobin -- is produced. Hemoglobin is the molecule in red blood cells that distributes oxygen to the body. Methemoglobin cannot release oxygen.
In methemoglobinemia, the hemoglobin is unable to release oxygen effectively to body tissues.
Alternative NamesHemoglobin M disease; Erythrocyte reductase deficiency; Generalized reductase deficiency
Causes, incidence, and risk factorsMethemoglobinemia may be passed down through families (inherited or congenital). Or, it may be caused by exposure to certain drugs, chemicals, or foods (acquired).
There are two forms of inherited methemoglobinemia. The first form is passed on by both parents. The parents usually do not have the condition themselves, but they carry the gene that causes the condition. It occurs when there is a problem with an enzyme called cytochrome b5 reductase.
There are two types of this form of methemoglobinemia:
The second form of inherited methemoglobinemia is called hemoglobin M disease. It is caused by defects in the hemoglobin molecule itself. Only one parent needs to pass on the abnormal gene for the child to inherit the disease.
Acquired methemoglobinemia is more common than the inherited forms. It occurs in some people after they are exposed to certain chemicals and drugs, including:
The condition may also occur in infants who are very ill or who are fed too many vegetables containing nitrates (such as beets).
SymptomsSymptoms of type 1 methemoglobinemia (erythrocyte reductase deficiency) include:
Symptoms of type 2 methemoglobinemia (generalized reductase deficiency) include:
Symptoms of hemoglobin M disease include:
Symptoms of acquired methemoglobinemia include:
Methemoglobinemia can be diagnosed with a blood test.
A baby with this condition will have a bluish skin color (cyanosis) at birth or shortly afterward. Arterial blood gases and pulse oximetry tests may be done.
TreatmentA medicine called methylene blue is used to treat severe methemoglobinemia. Note: Methylene blue may be dangerous in patients who have or may be at risk for a blood disease called G6PD deficiency, and should not be used. If you or your child has G6PD deficiency, always tell your health care provider before receiving treatment.
Ascorbic acidmay also be used to reduce the level of methemoglobin.
Alternative treatments include hyperbaric oxygen therapyand exchange transfusions.
In most cases of mild acquired methemoglobinemia, no treatment is needed. However, you should avoid the medicine or chemical that caused the problem. Severe cases may need treatment, which may include a blood transfusion.
Expectations (prognosis)People with type 1 methemoglobinemia and hemoglobin M disease usually do well. Type 2 methemoglobinemia is much more serious, and usually causes death within the first few years of life.
People with acquired methemoglobinemia usually do very well once the drug, food, or chemical that caused the problem is identified and avoided.
ComplicationsCall your health care provider if you have a family history of methemoglobinemia and you develop symptoms of this disorder.
Call your health care provider or emergency services (911) immediately if you have severe shortness of breath.
PreventionGenetic counseling is recommended for couples with a family history of methemoglobinemia who are considering having children.
ReferencesDeBaun MR, Frei-Jones M, Vichinsky E. Hereditary methemoglobinemia. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 456.7.
Fernandez-Frackelton M, Bocock J. Cyanosis. In: Marx JA, Hockberger RS, Walls RM, et al, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 7th ed. Philadelphia, Pa: Mosby Elsevier; 2009:chap 29.
Reviewed ByReview Date: 08/24/2011
Linda Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
Congenital methemoglobinemia is typically benign and should be observed. If methemoglobinemia symptoms occur the person should be taken to the hospital for treatment.
If a person gets methemoglobinemia from a certain medication that medication should be avoided at all costs in the future. For people with congenital methemoglobinemia medications or other things that are known to oxidize hemoglobin should be avoided.
If found early, acquired methemoglobinemia can be easily treated with no side effects. After treatment with methylene blue the patient can expect a full recovery.
The day that nobody really cares about.
For acquired methemoglobinemia the typical treatment is with methylene blue. This is administered with an IV over a five-minute period and results are typically seen within 20 minutes.
Methanoglobnemia is mispelled, it's actually methemoglobinemia Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin -- a form of hemoglobin -- is produced. Hemoglobin is the molecule in red blood cells that distributes oxygen to the body. Methemoglobin cannot release oxygen. In methemoglobinemia, the hemoglobin is unable to release oxygen effectively to body tissues.
Acquired methemoglobinemia can be caused by exposure to certain medications, chemicals, or substances that can oxidize hemoglobin, converting it to methemoglobin. Common causes include nitrate or nitrite medications, certain anesthetics, certain antibiotic drugs (such as dapsone and sulfonamides), and certain chemicals used in industries like aniline dyes or certain pesticides.
Bluish
This is a congenital hemoglobinopathy where the hemoglobin has an increased affinity for oxygen, and therefore, it does not releases it to the tissues. The consequence is hypoxia, and clinically, the baby has a bluish to grey color. It is incompatible with life.
Water conataining nitrates can cause methemoglobinemia blue baby syndrome). The condition can progress from a blue-grey skin colour to coma and death if not treated..
DefinitionMethemoglobinemia is a blood disorder in which the body cannot reuse hemoglobinafter it is damaged. Hemoglobin is the oxygen-carrying molecule found in red blood cells. In some cases of methemoglobinemia, the hemoglobin is unable to effectively carry oxygen to body tissues.Acquired methemoglobinemia results from exposure to certain drugs, chemicals, or foods.The condition may also be passed down through families (inherited). See: MethemoglobinemiaReferencesSteinberg MH. Sickle cell disease and associated hemoglobinopathies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 167.
Nitrates on drinking water. A sort of "blue baby syndrome" can also be caused by methemoglobinemia. It is widely believed to be caused by nitrate contamination in groundwater resulting in decreased oxygen carrying capacity of hemoglobin in babies leading to death.