Symptoms and treatment of deficiency of Pseudocholinesterase

Pseudocholinesterase deficiency

Description

Pseudocholinesterase (soo-doe-koh-lines-tur-r) deficiency is a rare disorder that makes them sensitive to certain muscle relaxants — succinylcholine or mivacurium — used during general anesthesia. Mivacurium is no longer available in the united States, but is used sometimes in other countries.

Succinylcholine is a drug designed to briefly relax the muscles during a medical procedure, such as surgery. With pseudocholinesterase deficiency, the body's muscles to stay relaxed during a longer time than expected.

This temporary loss of the ability to move the muscles (paralysis) that makes you unable to breathe or move on his own account. This can last for several hours. You may need help breathing with a ventilator until they start breathing on their own.

Pseudocholinesterase deficiency can be caused by a gene change (mutation) that is inherited. This condition can also be caused by an illness, injury, or certain medications.

There is No cure for the pseudocholinesterase deficiency. But if you are diagnosed with the disorder, your health care provider can use other types of muscle relaxers that do not cause muscle paralysis that lasts for longer than expected.

Symptoms

To the majority of people with pseudocholinesterase deficiency, there are no signs or symptoms of the disease occur until it reaches the muscle relaxant succinylcholine. This medication is used as part of anesthesia.

The signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can't move or breathe on his own. The length of time that can vary widely among people with this disorder.

When to see a doctor

If you have a family history of pseudocholinesterase deficiency or a family member who had a problem with the anesthesia, tell your health care provider before you undergo any medical procedure that requires anesthesia.

Causes

If you have pseudocholinesterase deficiency, your body does not have pseudocholinesterase or a very low amount. This enzyme is needed to break down (metabolize) medicines known as esters of the hill. Succinylcholine is used as part of anesthesia to relax the muscles during medical procedures.

Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after arriving succinylcholine. This prevents you from moving or breathing on your own for a couple of hours longer than expected. How long it takes your body to metabolize the drug depends on how much pseudocholinesterase enzyme that produces and how well it works.

Pseudocholinesterase deficiency may be inherited or acquired.

Inherited pseudocholinesterase deficiency

Inherited pseudocholinesterase deficiency is caused by a change in the butyrylcholinesterase (BCHE) gene. This gene provides instructions for making the pseudocholinesterase enzyme needed to break down the esters of the hill. If you have this gene is altered, as they do not produce this enzyme or the enzyme does not work well.

Genetic tests can show if you have this change in the gene. Have pseudocholinesterase deficiency, must inherit two affected genes — one from each parent (autosomal recessive inheritance). If you inherit only one affected gene, which do not have the disorder. But the process of esters of the hill at a slower pace than people who do not have an affected gene.

With an affected gene, you are a carrier and can pass the change in the gene on to their children. But I'm not going to develop the disorder unless they are also inheriting an affected gene from the other parent.

Acquired pseudocholinesterase deficiency

You can develop pseudocholinesterase deficiency as a result of the conditions that cause you to make less of the pseudocholinesterase enzyme. These conditions include chronic infections, kidney or liver disease, malnutrition, severe burns, cancer, or pregnancy. Certain medications can also reduce the production of the enzyme. Acquired pseudocholinesterase deficiency is not inherited and may not be passed on to their children.

Risk factors

Your risk of pseudocholinesterase deficiency is higher if you or a first-degree relative, like a parent, child or sibling, has:

• A change in the gene that causes the disorder
• A story of a problem during the anesthesia that is suspected to be caused by deficiency of pseudocholinesterase

Prevention

If you have a family history of pseudocholinesterase deficiency or a family member who had a problem with the anesthesia, tell your health care provider before a medical procedure that requires anesthesia.

If you have a family history of pseudocholinesterase deficiency, you can avoid problems during anesthesia to have the evidence before the procedure.

The assessment of its risk of pseudocholinesterase deficiency allows your health care provider to avoid certain muscle relaxers, if necessary.

Pseudocholinesterase deficiency

Diagnosis

Pseudocholinesterase deficiency may be suspected when you have problems to regain control of the muscles and breathing after you get the muscle relaxant succinylcholine as part of the anesthesia. A blood test can determine if you have enough of the pseudocholinesterase enzyme.

To diagnose inherited pseudocholinesterase deficiency, the change in the gene that causes the disorder is identified by the use of genetic testing. A blood sample is collected and sent to a laboratory for analysis. Ask your health care provider if the members of the family should be tested before the surgery.

Treatment

If you have pseudocholinesterase deficiency, the health care provider that gives you the anesthesia (anesthesiologist) can prevent succinylcholine that can trigger prolonged muscle relaxation. The anesthesiologist can choose other muscle relaxants in his place.

There is No cure for the pseudocholinesterase deficiency. If you have the disorder and receives a muscle relaxant that prolongs the effect of the anesthesia recovery, it is likely that you will need medical assistance. If necessary, a machine that takes over the work of breathing (mechanical ventilation), and sedation are provided while you recover and start to breathe on their own. This can take several hours.

With pseudocholinesterase deficiency, which may also be sensitive to other drugs. These may include the local anesthetic drug, also called local anesthetics. Examples are procaine, tetracaine, benzocaine, and cocaine.

If you have been diagnosed with pseudocholinesterase deficiency, wear a medical alert bracelet or necklace and carry a card in your wallet. This allows health professionals are aware of their risk, especially in case of emergency.