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This pre-referral guideline provides information on the evaluation for Primary Immune Deficiency (PID) in children of all ages.

Initial work-up

Warning signs for PID include:

• 8 or more new infections within one year.
• 2 or more serious sinus infections within one year.
• 2 or more months on antibiotics with little or no effect.
• 2 or more pneumonias within one year.
• Failure of an infant to gain weight or grow normally.
• Recurrent deep skin or organ diseases.
• Persistent thrush in the mouth or elsewhere on skin after age one year.
• Need for intravenous antibiotics to clear infections.
• 2 or more deep-seated infections.
• A family history of immune deficiency or suspected immune deficiency.

Diagnostics

• Full blood count.
• Liver function tests.
• Immunoglobulins: IgG, IgA and IgM.
• Microbiology e.g. blood/CSF or sputum culture.
• Virology e.g.ÌýNasopharyngeal Aspirate.
• Chest x-ray (if history of chest infections).

Notes

PID fact sheets for parentsÌýcan be found at

Pre-referral assessment/treatment

• All suspected cases of PID should be referred to the RCH for diagnosis and treatment - this is a PRIORITY REFERRAL.
• GPs should treat infections in the usual manner, antibiotics being the first line of treatment.

When to refer

• All suspected cases of PID should be referred to the RCH for diagnosis and treatment.
• CLEARLY INDICATE that the child has a suspected IMMUNODEFICIENCY.
• If the child is < 1 year old, the referral will be triaged as urgent. If unsure, contact the department on 03 9345 5522.

Referral criteria are 1 or more of the following conditions:

• 8 or more new infections within one year.
• 2 or more serious sinus infections within one year.
• 2 or more months on antibiotics with little or no effect.
• 2 or more pneumonias within one year.
• Failure of an infant to gain weight or grow normally.
• Recurrent deep skin or organ diseases.
• Persistent thrush in the mouth or elsewhere on skin after age one year.
• Need for intravenous antibiotics to clear infections.
• 2 or more deep-seated infections.
• A family history of immune deficiency or suspected immune deficiency.

Information needed:

• History of infections - date, type of infection, severity, treatment trialed and response.Ìý
• Family history.
• Diagnostic test results - organisms identified.
• Current management.

Contact information

Resources

• Clinical practice guidelines
• Information booklet
• Parent information website Kids Health Info
• Kids Connect (RCH Primary Care Liaison) www.rch.org.au/kidsconnect
  Contains all referral forms, clinic information, other pre-referral guidelines and parent information.
• Feedback
  We welcome your feedback on this guideline: kids.connect@rch.org.au / tel (03) 9345 4645 / fax (03) 9345 4650.

References

Paediatric Handbook (2009).ÌýEighth Edition, Blackwell Publishing. By the staff of the Royal Children's Hospital, Melbourne, Australia.

These guidelines were developed by specialists at the Royal Children's Hospital and reviewed by a working group of metropolitan and rural general practitioners in Victoria. Last reviewed in December 2008.

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