Results
The probands, a 7-year-old girl and a 10-month-boy, were both born full term. Their family histories were not remarkable. The elder sister presented with wet cough and running nose for 2 months and had been diagnosed with pneumonia and treated for 15 days in a local hospital without improvement. Her computed tomography (CT) (Fig.1A, C) scanning of lungs and sinus showed atelectasis and potential bronchiectasis in the right middle lobe and sinusitis with hypertrophy of tonsils and adenoids. Before transferred to the respiratory medicine department of our hospital, she had suffered from recurrent respiratory tract infection for almost 5 years. On physical examination, pharyngeal congestion and swelling of tonsil were found, with normal growth and development. Her nasal nitric oxide concentration (nNO) was much lower (38.5ppb) than the PCD-specific nNO cutoff value (287ppb) [4]. While the pulmonary function tests were normal. During bronchoscopy, massive mucilage secretions were found in segments of lingula, left upper and right middle lobes (Fig.1B). The culture of bronchoalveolar lavage (BAL) showed positive for Haemophilus infuenzae (Hin). The pure-tone audiometry proved conductive hearing loss. The air-bone gaps were 10dB to 25dB for the right ear and 15dB to 35dB for the left one. The otoscope and inner ear CT indicated otitis media in both of her ears, which was responsible for the hearing loss (Fig.1D, E, F).