Prem Menon, M.D.     Vimla Menon, M.D.
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Asthma, Allergy and Immunology Center

Asthma, Allergy and Immunology Center
5217 Flanders Dr.
Baton Rouge, LA 70808
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Prem Menon, M.D.
Telephone: (225) 766-6931
1-800-Dr-Menon (1-800-376-3666)
Fax: (225) 766-9413

Vimla Menon, M.D.
Telephone: (225) 766-6931
Fax: (225) 766-9413

Home | Immunology - Primary Immune Deficiency Diseases (PIDDs)

Primary Immune Deficiency Diseases (PIDDs)

The immune system is a collection of cells and proteins that works to protect the body from potentially harmful infections caused by microorganisms such as bacteria, viruses and fungi. The immune system plays a role in the control of cancers and other diseases and also is responsible for causing allergies, hypersensitivity and rejection of transplanted organs, tissues and implants.

Primary immunodeficiency diseases are a group of chronic disorders that affect the bodyís immune system and prevent it from functioning properly.

PIDD would result in:

  • Recurrent and unusually severe infections, infections with unusual or resistant bacteria with greater risk of complications.
  • Inflammation.
  • Autoimmune diseases
  • Allergies.

General guidelines for determining if a patient is experiencing too many infections, per the American Academy of Allergy, Asthma and Immunology are:

  1. Need for more than four courses of antibiotics per year in children or more than two times in adults.
  2. The occurrence of more than four new ear infections in one year after four years of age.
  3. The development of pneumonia twice over any time.
  4. The occurrence of more than three episodes of bacterial sinusitis in one year or occurrence of chronic sinusitis.
  5. The need for preventive antibiotics to decrease the number of infections.
  6. Any unusually severe infection or infections caused by bacteria that do not usually cause infections in most people at the patientís age.
  7. During the first year of life having persistent fungal infections of the mouth or skin, prolonged diarrhea or persistent cough.

Common symptoms of PIDDs include:

  • Frequent and recurring bouts of pneumonia, bronchitis, sinus or ear infections, meningitis, skin infections or blood infections
  • Inflammation and infection of internal organs
  • Blood disorders (such as anemia)
  • Digestive problems (diarrhea or nausea)
  • Delayed growth
  • Malabsorption of food
  • Autoimmune disorders (lupus or rheumatoid arthritis)

Primary Immune Deficiency Diseases may affect the
B- Cells (antibodies), T- cells, T and B cells in combination, Complements or Phagocytes (white blood cells).

The most common immunodeficiencies are caused by defects in your ability to produce blood proteins called antibodies. Antibodies are proteins that attach to the germs and help the body to eliminate them.

Depending on which limb of the immune system is involved, patients may develop recurrent bacterial, viral, fungal, mycobacterial or parasitic infections often with unusual and antibiotic resistant bugs. High index of suspicion is necessary for early and accurate diagnosis.

The PIDDs are diagnosed with appropriate blood tests, i.e. immune evaluation. This involves tests designed to measure the numbers (quantity) and functions (quality) of the various aspects (limbs) of the immune system. Measurement of antibodies, response to immunizations, determination of white cell counts and measuring the T and B lymphocytes (types of blood cells) numbers and functions are important tests for diagnosing PIDDs.

Allergy skin tests are done because allergies often contribute to infection susceptibility, and many patients with immunodeficiency also suffer from allergies.

Management

Treatment involves preventing and treating infections, boosting the immune system with appropriate immunizations, and identifying and treating the underlying cause.
Treatment plan for PIDDs would include:

  • Treat all infections.
  • Consider antibiotic prophylaxis.
  • Appropriate immunizations.
  • Appropriate subspecialty referrals.
  • Monthly Intravenous Immunoglobulin (IVIG) infusions, administered at an outpatient clinic or by home infusions.
  • Weekly, fortnightly or monthly Subcutaneous Immunoglobulin (SCIG) infusions. SCIG could be self- administered.
  • Monitor for short and long term complications related to PIDDs.
  • Gamma Interferon therapy, is the more definite way of treating certain forms of PIDDs.
  • Partnering with the patient and educating them about their PIDD is extremely important. Networking with patients with similar diagnosis and joining PIDDs support group would be beneficial.

American Academy of Allergy, Asthma and Immunology recommends that patients should consult an allergist\ immunologisit if they:

  1. Have chronic or recurrent infectious rhinosinusitis.
  2. Have any of the following warning signs:
    • Eight or more infections within one year.
    • Two or more serious sinus infections within one year.
    • Two or more months on antibiotic with little or no effect.
    • Two or more pneumonia within one year.
    • Failure of an infant to gain weight or grow normally.
    • Recurrent, deep skin or organ abscesses.
    • Persistent thrush in mouth or elsewhere on skin after age one year.
    • Need for intravenous antibiotics to clear infections.
    • Two or more deep seated infections.
    • A family history of immunodeficiency

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