Abnormal Axonemal Organization Of Respiratory Motile Cilia C4022987
Structural problems with hair-like cilia in the respiratory tract that help clear mucus. Often genetic, affecting children and adults. Treatment includes airway clearance techniques and managing infections.
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
Difficulty breathing shortly after birth due to underdeveloped ciliary clearance mechanisms.
Seek immediate emergency care if newborn shows rapid breathing, bluish skin, or retractions.
Chronic nasal congestion
(respiratory)Persistent blockage of nasal passages due to impaired mucociliary clearance leading to mucus buildup.
Frequency: 80-90% of patients
Chronic productive cough
(respiratory)Persistent cough with sputum production due to inability to clear mucus from airways.
Frequency: 75-85% of patients
Recurrent sinusitis
(respiratory)Repeated inflammation and infection of the sinus cavities due to poor mucus drainage.
Frequency: 70-80% of patients
Recurrent otitis media
(respiratory)Repeated middle ear infections due to eustachian tube dysfunction from impaired ciliary function.
Frequency: 60-70% of patients
Recurrent lower respiratory infections
(respiratory)Repeated pneumonia or bronchitis episodes due to inability to clear pathogens from lungs.
Frequency: 65-75% of patients
Bronchiectasis
(respiratory)Permanent widening of airways due to chronic inflammation and infection, leading to persistent cough and mucus production.
Frequency: 30-40% of patients
Nasal polyps
(respiratory)Noncancerous growths in the nasal passages caused by chronic inflammation.
Frequency: 20-30% of patients
Conductive hearing loss
(ear_nose_throat)Reduced hearing ability due to fluid accumulation in the middle ear from chronic otitis media.
Frequency: 25-35% of patients
Infertility
(reproductive)Reduced fertility due to dysfunction of motile cilia in reproductive organs, affecting sperm motility or fallopian tube function.
Frequency: 40-50% of adult patients
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
Regular pulmonary function and imaging surveillance
Periodic spirometry, chest CT imaging, and microbiological screening help detect early bronchiectasis, monitor disease progression, and identify infections requiring intervention.
Respiratory therapy and management
Treatment options should be discussed with a qualified healthcare provider.
Airway clearance techniques
Daily chest physiotherapy using percussion, postural drainage, or high-frequency chest wall oscillation helps mobilize secretions and reduce infection risk in ciliary dysfunction.
Additional treatment options
Targeted antibiotic therapy for respiratory infections
Prompt antibiotic treatment guided by culture and sensitivity for Pseudomonas or other pathogens helps control chronic infections and prevent bronchiectasis progression.
medicationSinus surgery for chronic sinusitis
Functional endoscopic sinus surgery may be indicated for severe chronic rhinosinusitis unresponsive to medical therapy to improve mucociliary clearance and quality of life.
surgicalBronchoscopy for airway assessment
Diagnostic bronchoscopy allows direct visualization of airways, collection of samples for culture, and assessment of ciliary structure and function through biopsy.
procedureWhen 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 Abnormal Axonemal Organization Of Respiratory Motile Cilia C4022987?
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.
Barbato A, Frischer T, Kuehni CE, et al. Primary ciliary dyskinesia: a consensus statement on diagnosis and management. Eur Respir J. 2010;35(5):1076-1083.
Goutaki M, Maurer E, Halbeisen FS, et al. The European Respiratory Society clinical guidelines for primary ciliary dyskinesia. Eur Respir J. 2023;62(1):2202143.
Werner C, Onnebrink JG, Omran H. Diagnosis and management of primary ciliary dyskinesia. Arch Dis Child. 2015;100(5):457-465.
Lucas JS, Burgess A, Mitchison HM, et al. Diagnosis and management of primary ciliary dyskinesia. Arch Dis Child. 2014;99(9):850-858.
Horani A, Ferkol TW. Advances in the understanding of the genetic basis of primary ciliary dyskinesia. Nat Rev Dis Primers. 2022;8(1):45.
Sagliocco C, Cohen-Cymberknoh M, Shteinberg M, et al. Longitudinal European registry of primary ciliary dyskinesia (PCD1). Eur Respir J. 2021;58(3):2002442.
Goutaki M, Boyer O, Riethmacher J, et al. Effects of upper respiratory infections in early life on lung function in children and adults: a systematic review. Eur Respir Rev. 2020;29(156):190144.
Shapiro AJ, Davis SD, Polineni D, et al. Diagnosis of primary ciliary dyskinesia. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2018;197(12):e24-e39.
Kuehni CE, Lucas JS. Diagnosis of primary ciliary dyskinesia: summary of the ERS task force report. Breathe (Sheff). 2017;13(3):166-177.
Leigh MW, Pittman JE, Carson JL, et al. Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome. Genet Med. 2009;11(7):473-487.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy