LAD is an immune deficiency in which phagocytes are not able to migrate to the site of infection to fight off invading germs. This inability to fight germs results in recurrent, life-threatening infections and poor wound healing. LAD is an autosomal recessive disease (disease caused when two copies of the abnormal gene are present).
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Causes
LAD is caused by a lack of a molecule called CD18. These molecules are normally found on the outer surface of phagocytes. Without them, phagocytes cannot attach to blood vessel walls and enter infected tissues, where they help fight infection. Mutations in the gene that instructs, or codes for, the production of CD18 cause LAD.
Signs and symptoms
Children with LAD cannot fight off infection properly and may have any of the following:
- Severe infections of the soft tissue
- Eroding skin sores without pus
- Severe infections of the gums with tooth loss
- Infections of the digestive system
- Wounds that heal slowly or not at all
The severe form of LAD, known as type 1, commonly causes death in infancy or early childhood. Children with a more moderate form of the disease may survive into young adulthood.
Diagnosis
Blood tests can diagnose children with LAD; they will show a very high number of white blood cells and very low levels of CD18. A doctor may suspect LAD if wounds do not heal properly. Children who develop severe infections caused by bacteria and whose wounds are slow to heal also may have LAD.
Treatment
Doctors use antibiotics to prevent and treat infections. Some children with LAD have been treated successfully with bone marrow transplants.