Primary ciliary dyskinesia diagnosis management in low-resource setting, a practical approach
, , , , and | May 02, 2024
About this article
Article Category: Case Report
Published Online: May 02, 2024
Page range: 122 - 130
DOI: https://doi.org/10.2478/pneum-2023-0034
Keywords
© 2022 Andreea Dumitroae et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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PICADAR score, reproduced after “PICADAR: a diagnostic predictive tool for primary ciliary dyskinesia” [17]
| Does the patient have a daily wet cough that started in early childhood? | Yes - complete PICADAR No - STOP. PICADAR is not designed for patients without a wet cough | |
|---|---|---|
| 1. Was the patient born pre-term or full term? | Term | 2 |
| 2. Did the patient experience chest symptoms in the neonatal period (e.g., tachypnoea, cough, pneumonia)? | Yes | 2 |
| 3. Was the patient admitted to a neonatal unit? | Yes | 2 |
| 4. Does the patient have a situs abnormality (situs inversus or heterotaxy)? | Yes | 4 |
| 5. Does the patient have a congenital heart defect? | Yes | 2 |
| 6. Does the patient have persistent perennial rhinitis? | Yes | 1 |
| 7. Does the patient experience chronic ear or hearing symptoms (e.g., glue year, serous otitis media, hearing loss, ear perforation)? | Yes | 1 |
NA-CDCF schematic diagram, reproduced after “Clinical Features and Associated Likelihood of Primary Ciliary Dyskinesia in Children and Adolescents”. Early onset is defined as onset before 6 months of age; year-round is defined as occurring in all 12 months of the year; wet cough is defined as sounds productive even if unable to expectorate sputum [18].
| Unexplained neonatal respiratory distress (sensitivity 81%, specificity 68%) | Term gestation, Supplemental O2 requirement ≥ 1day and no meconium aspiration (sensitivity 57%, specificity 89%) |
| Chronic cough (sensitivity 97%, specificity 17%) | Year-round wet cough with early onset ≤ 6 months of age (sensitivity 62%, specificity 74%) |
| Chronic nasal congestion (sensitivity 97%, specificity 19%) | Year-round with early onset ≤ 6 months of age (sensitivity 74%, specificity 60%) |
| Situs inversus totalis (sensitivity 46%, specificity 92%) | Other laterality defects (sensitivity 53%, specificity 85%) |
Clinical Index, a seven-item questionnaire, reproduced after “Evaluation of a Clinical Index as a Predictive Tool for Primary Ciliary Dyskinesia”[l9]
| Did the child manifest with significant respiratory difficulties with breathing after birth? | Yes=1 point |
| Did the child have rhinitis or excessive mucus production in the first 2 months of life? | Yes=1 point |
| Did the child suffer from pneumonia? | Yes=1 point |
| Did the child present with 3 or more episodes of bronchitis? | Yes=1 point |
| Was the child treated for chronic secretory otitis or suffered from >3 episodes of acute otitis? | Yes=1 point |
| Does the child have a year-round nasal discharge or nasal obstruction? | Yes=1 point |
| Was the child treated with antibiotics for acute upper respiratory tract infection >3 times? | Yes=1 point |