Table 1. Comparison of the number and percentage of correct answers, given by the surveyed physicians taking part in the study.
| No. | Questions | 2016 n = 82 | 2019 n = 67 | P-value | ||
|---|---|---|---|---|---|---|
| Correct answers | ||||||
| n | % | n | % | |||
| 1. | PIDs occur only in children | 28 | 34.1 | 49 | 73.1a | <0.0001 |
| 2. | Telangiectasia may be specific to: | |||||
| a) hepatic insufficiency, | 50 | 61.0 | 56 | 83.6* | 0.0024 | |
| b) ataxia-telangiectasia syndrome (Louis-Bar syndrome) | 44 | 53.7 | 55 | 82.1* | 0.0003 | |
| 3. | The absence of thymus confirms Di George syndrome | 41 | 50.0 | 39 | 58.2 | 0.3175 |
| 4. | Common variable immunodeficiency (CVID) is most often diagnosed in children | 15 | 18.3 | 16 | 23.9 | 0.4032 |
| 5. | Oncological diseases can be a sign of PID | 50 | 61.0 | 53 | 79.1* | 0.0172 |
| 6. | AFP (alpha-fetoprotein) appears in high concentrations in A-T syndrome | 39 | 47.6 | 47 | 70.1* | 0.0055 |
| 7. | Four or more new ear infections within 1 year may be a warning sign of PID | 67 | 81.7 | 58 | 86.6 | 0.4221 |
| 8. | Failure of a child to gain weight normally may be a sign of PID | 47 | 57.3 | 65 | 97.0* | <0.0001 |
| 9. | Repeated abscesses of skin and organs (without damage to the tissue integrity caused by trauma) may be a sign of PID | 60 | 73.2 | 62 | 92.5* | 0.0023 |
| 10. | Numerous (6 and more) of ‘coffee-with-milk’ colored spots are specific to: | |||||
| a) Nijmegen breakage syndrome (NBS) | 28 | 34.1 | 59 | 88.1* | <0.0001 | |
| b) Louis-Bar syndrome | 15 | 18.3 | 58 | 86.6* | <0.0001 | |
| c) Bruton's agammaglobulinemia | 52 | 63.4 | 53 | 79.1* | 0.0368 | |
| 11. | Two or more cases of pneumonia in a year may be the only clinical manifestation of PID | 38 | 46.3 | 43 | 64.2* | 0.0297 |
| 12. | Four or more episodes of infection (otitis, bronchitis, pneumonia) in an adult patient may be a sign of PID | 56 | 68.3 | 56 | 83.6* | 0.0316 |
| 13. | In adults, two or more cases of pneumonia (radiographically confirmed) within three years may be a sign of PID | 58 | 70.7 | 45 | 67.2 | 0.6391 |
| 14. | Children diagnosed with microcephaly should undergo genetic testing | 30 | 36.6 | 25 | 37.3 | 0.9260 |
| 15. | Infections with atypical localization or caused by atypical pathogens may be a sign of PID | 67 | 81.7 | 58 | 86.6 | 0.4221 |
| 16. | Dysmorphic facial features are specific to: | |||||
| а) common variable immunodeficiency (CVID) | 64 | 78.0 | 55 | 82.1 | 0.5406 | |
| b) DiGeorge syndrome | 31 | 37.8 | 57 | 85.1* | <0.0001 | |
| c) Nijmegen breakage syndrome | 27 | 32.9 | 61 | 91.0* | <0.0001 | |
| 17. | The only method of treatment for PID with antibody deficiency is therapy with intravenous or subcutaneous immunoglobulin agents | 82 | 100 | 66 | 98.5 | 0.2670 |
| 18. | Normal levels of leukocytes (WBC), hemoglobin, platelets, HCT are sufficient to exclude neutropenia | 66 | 80.5 | 52 | 77.6 | 0.6670 |
| 19. | Live vaccines are contraindicated for patients with NBS | 59 | 72.0 | 57 | 85.1 | 0.0550 |
| 20. | Inflammation+ thrombocytopenia + eczema may be the signs of: | |||||
| a) Wiskott-Aldrich syndrome | 55 | 67.1 | 61 | 91.0* | 0.0005 | |
| b) atopic dermatitis | 58 | 70.7 | 59 | 88.1* | 0.0104 | |
| 21. | In cases of Nijmegen syndrome chest X-ray examination is allowed | 9 | 11.0 | 37 | 55.2* | <0.0001 |
| 22. | Live vaccines can be administered to children with severe PID | 82 | 100 | 64 | 95.5 | 0.0529 |
| 23. | Vaccination against pneumococcus should be given to children with PID that have retained the ability to synthesize antibodies (within the risk group) | 63 | 76.8 | 59 | 88.1 | 0,0767 |
| 24. | All adults with primary and secondary asplenia should be vaccinated against pneumococcus and meningococcus | 36 | 43.9 | 57 | 85.1* | <0.0001 |
| 25. | Autoimmune diseases are much more common in patients with PID | 64 | 78.0 | 58 | 86.6 | 0.1793 |
| Total | 1481 | 58.3 | 1640 | 79.0* | <0.0001 | |
* the difference is statistically significant