Kartagener's syndrome: a re-visit with Chinese perspectives.
The objective of this study was to evaluate the clinical, radiological investigation profiles, and ciliary function and ultrastructure in Chinese patients with Kartagener's syndrome (presence of dextrocardia, sinusitis and bronchiectasis). All patients with dextrocardia were assessed for the presence of sinusitis and bronchiectasis in our hospital network. Patients identified with Kartagener's were assessed when they were at steady state for their bronchiectasis. Seven cases (4 males; mean age 34.9 years) were identified and systematically reviewed. The mean 24 h sputum volume was 26.6 +/- 32.77 mL/day and the patients suffered from a mean of 2.9 exacerbations/year. Nasal symptoms (anosmia in one, obstruction in six and persistent discharge in three patients) were common. Only two cases (1 M) were married and both had normal fertility. Lung function assessment showed a mean FEV1/FVC of 83.3 +/- 38.78/86.5 +/- 36.72 (% predicted) with little reversibility. High resolution computerized tomography (HRCT) revealed bronchiectactic involvement of the lower lobes in seven and middle lobe/lingula in four cases. Assessment of alpha-1-anti-trypsin, aspergillus precipitins, auto-antibodies and serology for Pseudomonas pseudomallei was normal. Sputum culture yielded Pseudomonas aeruginosa in three, Haemophilus influenzae in three and commensals in one case. Phase contrast microscopy assessment of respiratory cilia, obtained by brushing the inferior turbinate, revealed that most of the mucosa was unciliated. The mean ciliary beat frequency was 5.2 +/- 6.76 Hz (range 0-13.7; normal range 12-18 Hz). Four patients had immotile cilia whilst the rest had normal ciliary movement. Transmission electron microscopy showed the absence of dynein arms in four patients. The results of this study show that patients with Kartagener's syndrome may have normal ciliary ultrastructure and the absence of dynein arms is not necessarily associated with ciliary immotility. The presence of ciliary immotility might have prognostic value as these patients appear to have more active bronchiectasis. Our experience on this series should help clinicians in the investigation and management of these patients.
Tsang
K W
KW
University Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong.
Ip
M
M
Ooi
C G
CG
Wong
M P
MP
Ho
C S
CS
Sun
Z
Z
Tam
C Y
CY
Lam
W K
WK
D000293:Adolescent; D000328:Adult; D044466:Asian People; D002681:China; D002923:Cilia / Q000648:ultrastructure; D005260:Female; D005298:Fertility; D006801:Humans; D007619:Kartagener Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D008854:Microscopy, Electron; D008858:Microscopy, Phase-Contrast; D008875:Middle Aged; D013183:Sputum / Q000382:microbiology; D014057:Tomography, X-Ray Computed