Pierwotna dyskineza rzęsek z perspektywy otolaryngologa dziecięcego

Karolina Raczkowska-Łabuda, Elżbieta Niemczyk, *Lidia Zawadzka-Głos

Summary
Primary ciliary dyskinesia (PCD), and in particular the Kartagener’s Syndrome, is rare disease of genetic origin, the most common autosomal recessive type of inheritance. Highly diversed manifestation of the disease depends in particular on the age of the patient. Symptoms are mostly related to the respiratory tract (nasal cavity, paranasal sinuses, Eustachian tube, middle ear, throat, trachea, bronchi and bronchioles), that results from impaired ciliary clearance mechanism. Kartagener’s Syndrome triad (chronic sinusitis, bronchiectasis and situs inversus totalis) origin of the cilia’s construction disorder. It was found that the mobility of these organelles determines the rotation of viscera during fetal development. Location of organs among children with defective cilia structure is random. Dextrocardia and situs inversus totalis occur among 50 percent of patients (probability of occurrence is equal to 0.5). Rapid diagnosis and appropriate management of the patient requires the cooperation of many specialists: pediatricians, allergists, pulmonologists, ENT or physiotherapists. No universal treatment algorithm for the PCD affected patients was developed yet. Many dissonant information regarding the PCD occur in the literature including not only the incidence but also recommended therapy. The treatment of chronic otitis with effusion is highly controversial. The article is based on a history of the disease of the 4.5-year-old boy with a Kartagener’s Syndrome. Case report includes the period up to the diagnosis, leading specialists and the OME treatment attitude in the Department of Pediatric Otolaryngology, Medical University of Warsaw.

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