PID in children manifest differently, 10 warning signs help in diagnosis
We asked MUDr. Eva Hlaváčková from the Department of Clinical Immunology and Allergology, Faculty of Medicine, Masaryk University, and St. Anne's University Hospital in Brno about the essential details regarding the 10 warning signs of primary immunodeficiencies (PID) and in which cases the vaccination schedule needs to be adapted for sick children.
Which of the 10 warning signs or what combination of them most often leads doctors to diagnose primary immunodeficiency in children?
It is always a set of symptoms. Children who meet 2 or more criteria should be referred to an immunological center for examination. Generally, the statistically most common symptoms are the presence of disease in the family and the child's failure to thrive in combination with other warning signs.
Doctors should suspect an initial immune disorder in cases of severe infections, the need for repeated intravenous antibiotic administration, infections not responding to standard treatment, or infections caused by opportunistic pathogens, i.e., bacteria, viruses, or fungi that do not cause infection in healthy individuals but cannot be fought off by patients with immune disorders. Attention should also be drawn to the occurrence of abscesses or fungal infections.
The set of 10 warning signs of primary immunodeficiencies is very useful for doctors in preliminary diagnostics.
Can immune disorders manifest on the skin? Does this not make it difficult to distinguish them from other conditions?
Skin manifestations of immune disorders are diverse, and the patient's symptoms must always be assessed as a whole. Cooperation with pediatric dermatologists is also crucial. Skin manifestations are not a complication for diagnosis; on the contrary, they can aid in determining the diagnosis.
When is the diagnosis of immune disorders made in children? More in infancy, or later when there is increased illness after joining larger groups?
Diagnosis depends on the genetic basis and clinical manifestation of the disorder. Severe combined immunodeficiencies or those affecting the cellular component of the immune system manifest right after birth, within the first 6 months. Antibody immunodeficiencies manifest around 6 months of age. Phagocyte function disorders can also be diagnosed within the first weeks or months of life, but also later. Certain diagnoses manifest in preschool, school, or adult age. Joining a group (usually at 2 to 3 years old) is not a trigger for primary immunodeficiency.
How do you assess the awareness of primary immunodeficiencies among pediatric and adolescent doctors?
I think it is good at present. We continue to spread awareness of PID during professional meetings as part of the activities of the working group for primary immunodeficiencies of the Czech Society of Allergology and Clinical Immunology CLS JEP. Pediatricians refer a part of the patients to immunologists directly or consult with immunologists about their patients' conditions. Another group of patients comes to the care of immunologists through the hospital sector, where they are hospitalized and further examined for serious health complications.
If the diagnosis of primary immunodeficiency is confirmed, do you give the parents of child patients any recommendations regarding the vaccination schedule?
The exact procedure clearly depends on the specific diagnosis. Currently, according to the classification of the International Union of Immunological Societies (IUIS), there are 330 primary immunodeficiencies, and recommendations may vary based on the nature of the disease.
There are diagnoses where no adjustment to the vaccination schedule is needed, such as in the case of selective IgA deficiency. For antibody immune disorders, parents need to be explained that their child cannot produce antibodies even after vaccination. These children regularly receive products containing antibodies, which they would otherwise produce themselves if they were healthy. For some diagnoses, it makes sense to create an individual vaccination plan, so cooperation among the immunologist, family, and vaccination advisory center is beneficial. However, we also work with patients who must not be vaccinated with live vaccines. We always strive to fully explain everything to parents.
Test for patients: Are you at risk of immune disorders?
Petra Dulíková
editorial team MeDitorial
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