Ciliary Dyskinesia Primary 48 Without Situs Inversus C5774214
A genetic disorder affecting tiny hair-like structures called cilia. It causes chronic respiratory and ear infections. Treatment involves managing infections and airway clearance.
Key facts at a glance
- ICD-10
- J98.9
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Neonatal respiratory distress
Chronic cough
(respiratory)Shortness of breath or cough
(respiratory)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent sinusitis
(respiratory)Bronchiectasis
(respiratory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Positive Expiratory Pressure Therapy
Devices providing resistance during exhalation to maintain open airways and promote mucus movement toward larger bronchi for expectoration.
Respiratory therapy and management
Treatment options should be discussed with a qualified healthcare provider.
Chronic Suppressive Antibiotic Therapy
Rotating antibiotic courses to suppress bacterial colonization and prevent acute exacerbations in chronic respiratory disease.
Additional treatment options
Imaging and Spirometry Monitoring
Periodic CT imaging and pulmonary function testing to detect structural changes and track functional decline over time.
monitoringNasal Saline Irrigation
Daily nasal rinsing with isotonic or hypertonic saline solutions to improve mucociliary function and reduce sinonasal symptoms.
complementaryBronchiectasis Resection
Targeted surgical removal of severely damaged lung segments in cases of localized bronchiectasis with recurrent infections unresponsive to medical therapy.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Ciliary Dyskinesia Primary 48 Without Situs Inversus C5774214?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Werner C, Bush A, Robinson P, et al. European Respiratory Society guidelines for the diagnosis and management of primary ciliary dyskinesia. Eur Respir J. 2023.
Leigh MW, Horani A, Kinghorn B, et al. Genetics and biology of primary ciliary dyskinesia. Paediatr Respir Rev. 2021.
Kobbernagel HE, Nielsen KG, Pressler T, et al. Efficacy and safety of azithromycin maintenance therapy in primary ciliary dyskinesia: a randomised controlled trial. Thorax. 2022.
Santharam V, Fenton SE, McManus R, et al. Airway clearance techniques in patients with primary ciliary dyskinesia: a systematic review and meta-analysis. Respir Med. 2020.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy