Primary Immune Deficiency at Pediatric Clinic DRHC Dubai

Primary immune deficiency is a chronic disease caused by a genetic defect in the immune system. The immune system protects our bodies from germs such as bacteria, viruses, and fungi that cause infection. People with PID have an immune system that doesn’t work properly against these germs, so they have infections that last a long time and are hard to treat, or they suffer from frequent, severe infections.

PID can be mild or severe. If your child has 2 or more of these signs, you should ask for medical attention.

• 8 or more ear infections within 1 year.
• 2 or more sinus infections within 1 year.
• 2 or more lung infections within 1 year.
• Failure of a baby to gain weight.
• Oral thrush after age 1.
• Need for IV antibiotics to clear infections.
• 2 or more deep seated infections.
• Deep skin or organ abscesses that keep coming back.
• A family history of PID.
• Serious infections with unusual pathogens.

PID can be diagnosed by a blood test. It is best to find out as soon as possible. Early detection and treatment can save life for a child.

Blood Tests for PID Diagnosis

CBC should be obtained to screen for T-cell or phagocytic disorders. T cell disorders are characterized by lymphocytopenia. Lymphocyte count is age-dependent if the absolute lymphocyte count is 2 standard deviations below the mean. T cell disorder can be confirmed by a lack or delay of hypersensitivity skin test response to candida, mumps, or tetanus in children older than 1 year and by lymphocyte subset analysis at any age. This test will screen for the number and percentage of T cells, B cells, and natural killer cells.

Phagocytic disorders are characterized by neutropenia and abnormalities in granules in neutrophils if the count is less than 1500 per m^3. PID should be suspected if the Neutrophil count is normal; then granulocyte function tests can be performed.

Serum immunoglobulin: Patients with B cell disorders have low serum immunoglobulin levels and decreased production of IG to vaccination.

Complement testing: Complement disorders are screened by checking the components of the classic and alternative pathways. The classic pathway is checked with CH50 assay; if it's normal, then it means the child does not have a significant complement deficiency, but if the result is abnormal, then the alternative pathway should be checked with the AH50 or CH100 assay.

Newborn Screening for SCID in newborns is recommended as early identification and treatment with stem cell transplantation, which can prevent infant death. Screening involves the detection of T cell receptor excision circles by polymerase chain reaction using a newborn heel stick dried blood spot. T cell receptor excision circles are pieces of DNA produced only by T-cells.

Causes

• B-cell (antibody) deficiency
• T cell deficiencies
• Combined B and T cell deficiencies
• Defective phagocytes
• Complement deficiency

Managing Infections

Infections require rapid and aggressive treatment with antibiotics. Infections that don’t respond may require hospitalization and IV treatments. Some people need long-term antibiotics to prevent infections.

Treatment to Boost the Immune System

• Immunoglobulin therapy for IG deficiency
• Gamma interferon therapy to treat chronic granulomatous disease.
• Growth factor for a lack of white blood cells.

When the child has PID, there are certain vaccines that they should not have:

• Oral polio
• MMR vaccine
• BCG vaccine
• Varicella vaccine
• Rotavirus vaccine
• Yellow fever vaccine

Stem Cell Transplantation

Stem Cell transplantation offers a permanent cure for several forms of life-threatening immunodeficiency.

Dubai Pediatric Clinic at Dr. Rami Hamed Center provides one of the leading pediatricians in Dubai. Please call +97142798200 to book an appointment today with us!