Methemoglobinemia Health Article

Definition

When excessive hemoglobin in the blood is converted to another chemical that cannot deliver oxygen to tissues, called methemoglobin.

Description

The molecule hemoglobin in the blood is responsible for binding oxygen to give to the body. When hemoglobin is oxidized to methemoglobin its structure changes and it is no longer able to bind oxygen. Hemoglobin is constantly under oxidizing stresses: however, normally less than 1% of a person's hemoglobin is in the methemoglobin state. This is due to the body's systems that reduce methemoglobin back to hemoglobin. Infants have a higher risk of acquiring methemoglobinemia because infant hemoglobin is more prone to be oxidized to methemoglobin.

Causes and symptoms

Methemoglobinemia can either be congenital or acquired.

There are two causes of the congenital form. One cause is a defect in the body's systems to reduce methemoglobin to hemoglobin. The other cause is a mutant form of hemoglobin called hemoglobin M that cannot bind to oxygen. Both of these forms are typically benign.

Acquired methemoglobinemia is caused by an external source, usually a drug or medication. Some of these medications include benzocaine, lidocaine and prilo-caine. These medications can inhibit the body's systems of reducing methemoglobin to hemoglobin resulting in methemoglobinemia.

With a methemoglobin level of 3-15% skin can turn to a pale gray or blue (cyanosis). With levels above 25% the following symptoms may be present:

• cyanosis unaffected by oxygen administration
• blood that is dark or chocolate in color that won't change to red in the presence of oxygen
• headache
• weakness
• confusion
• chest pain

When methemoglobin levels are above 70% death may result if not treated immediately.

Diagnosis

Diagnosis is based on the symptoms and history. If these are indicative of methemoglobinemia blood tests are performed to confirm the presence and level of methemoglobin.