The Zivert-Kartagener syndrome in children
A comprehensive clinicomorphological examination of 24 children with Zivert-Kartagener syndrome ascertained the complete triad (bronchiectasis, maldevelopment of the sinuses and transposition of the viscera) in all of them. Initial signs of the respiratory affection are shown to arise early. The disease progression depended primarily on the scope of pulmonary damage. Electron-microscopic findings at examination of the patients' tracheal mucosa obtained at bronchoscopy revealed reduced number of ciliary cells and abnormal inner structure of the cilia. The impairment in some patients was focal. The attention is drawn to the benefit of early diagnosis and follow-up for such patients who should be treated surgically after careful consideration of potential risks.