Primary ciliary dyskinesia (PSD) is a disease characterized by a disorder in the cilia (hairs that are too visible to the eye) in some organs, resulting in the inability of these cilia to perform their functions. The disease is genetically innate. Since the cilia cannot function, there is a disorder in the excretion of secretions and, most commonly, phlegm accumulation may occur in the bronchi in the lungs. In approximately half of the patients, the internal organs in the chest and abdomen are located upside down. Due to impaired mucociliary clearance in PCD, recurrent chronic sinusitis, ear infection, and bronchial dilatation-bronchiectasis (BE) and hearing loss may occur in early childhood.
Those who are resistant to treatment and have recurrent upper and lower respiratory tract infections may occur. and the possibility of PCD should be considered in all patients in whom the underlying cause cannot be found. Patients often have a cough with phlegm, an inflammatory nasal discharge that persists in summer and winter, and recurrent ear infections. Especially in the neonatal period, complaints or the reverse placement of internal organs in the chest and abdomen (the heart is located on the right and the stomach is on the left) should be a warning.
PSD is a disease that is very difficult to diagnose. There is no single gold standard method to make a definitive diagnosis. Definitive diagnosis is made by carefully reviewing the clinical history and evaluating the screening test results in a specialized center and then applying definitive diagnostic methods.
In the diagnosis of PSD, taking a cilia sample from the nose or bronchus and examining the functions of the cilia using microscopic and more advanced methods. diagnosis is made.
Treatment of primary ciliary dyskinesia should aim to prevent progression of the disease and worsening of lung functions.
Respiratory treatment:
Monitoring of lung functions
Pulmonary rehabilitation and physical Airway cleaning with exercise combinations
It includes the treatment of upper and lower airway infections.
Patients should be followed up regularly every 3 months at the pediatric pulmonology center. Respiratory, ear, nose and throat and general evaluation should be performed every time the patient comes for a check-up. Additionally, oxygen status and respiratory function tests should be checked, sputum samples should be taken. culture should be taken. A chest X-ray does not need to be taken at every check-up, but it can be taken when infection or bronchial dilation is suspected. If there are clinical and chest radiography findings suggestive of bronchiectasis, tomography to confirm the diagnosis of bronchiectasis.
Airway clearance is essential in the treatment of PSD. Patients should receive regular chest physiotherapy and use devices that help expectoration and are recommended by their doctors. It is recommended that patients receive an annual flu vaccine in addition to the Ministry of Health vaccination program. In our country, pneumococcal vaccine has been included in the national vaccination program since 2008. It is recommended that children born before this date who have not received the pneumococcal vaccine be vaccinated with the 23-component polysaccharide pneumococcal vaccine. To maintain protection, the high-risk group can be vaccinated once more after 5 years.
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