Description

X-linked agammaglobulinemia (a-gam-uh-glob-u-lih-NEE-me-uh), also called XLA, is a disorder of the immune system transmitted through families, called legacy. XLA makes it difficult to fight infections. People with XLA may get infections of the inner ear, sinuses, respiratory tract, bloodstream and internal organs.

XLA almost always affects males. But females can carry the genes linked to the condition. The majority of people with XLA was diagnosed in infancy or early childhood, after they have had repeated infections. Some people are not diagnosed until they are adults.

Symptoms

The majority of babies with XLA appear healthy for the first few months. They are protected by proteins called antibodies they received from their mothers before birth.

When these antibodies out of their systems, the babies begin to reach repetition of bacterial infections. The infections can be fatal. Infections can involve the ears, lungs, sinuses and skin.

Male infants are born with XLA have:

  • Very small tonsils.
  • Small or not to the lymph nodes.

Causes

X-linked agammaglobulinemia is caused by a change in a gene. People with the condition do not produce proteins called antibodies that fight infection. Approximately 40% of people with the condition have a family member who has it.

Complications

People with XLA can live in its most typical of the life. You should try to take part in regular activities for their ages. But the repetition of infections linked to XLA likely that require careful observation and treatment. It may cause damage to organs and be life-threatening.

Possible complications include:

  • Long-term, so-called chronic lung disease.
  • Increased risk of certain types of cancer.
  • Infectious arthritis.
  • Increased risk of infections of the central nervous system of live vaccines.

Diagnosis

The diagnosis involves a clinical history of repeated infections, and a physical examination. Blood tests and perhaps genetic testing can confirm the diagnosis.

Treatment

There is No cure for XLA . The goal of treatment is to stimulate the immune system to prevent infections. There is also a quick treatment for infections that occur.

Drugs

Medications to treat XLA include:

  • Gamma globulin.This is a type of protein that is found in the blood that contains antibodies against the infections. He is put in a vein, it is called infusion, every 2 to 4 weeks, or given with weekly injections. The reactions to the gamma globulin may include headache, chills, back pain, and nausea. The reactions are more likely to occur during a viral infection, like a cold.
  • Antibiotics. Some people with XLA to take antibiotics to prevent infection. Others take antibiotics for bacterial infections more than people with XLA .

Gamma globulin. This is a type of protein that is found in the blood that contains antibodies against the infections. He is put in a vein, it is called infusion, every 2 to 4 weeks, or given with weekly injections.

The reactions to the gamma globulin may include headache, chills, back pain, and nausea. The reactions are more likely to occur during a viral infection, like a cold.

Your healthcare provider will probably suggest that you have follow-up visits every 6 to 12 months to detect complications of the XLA . Also likely is that it was said to not get live vaccines, such as live polio, measles-mumps-rubella, or varicella vaccine.

Symptoms and treatment of X-linked agammaglobulinemia