[A case of immotile-dyskinetic cilia syndrome responding to clenbuterol hydrochloride and azithromycin].
In this case, a 30-year-old man had been treated for chronic sinusitis and bronchiectasis since 2000, and presented at our outpatient clinic in May 2001 with chief complaints of massive yellow sputum expectoration and dyspnea. After he was admitted by our hospital, Pseudomonas aeruginosa bacteria were isolated at the rate of 10(8)/ml from his sputum culture. In electron-microscopic observation, the cilia of the bronchial epithelium were found to lack dynein arms. Semen examination revealed decreased sperm motility. Thus, the following diagnosis was made: diffuse bronchiectasis associated with the immotile-dyskinetic cilia syndrome, complicated with a P. aeruginosa infection. After the airway infection was ameliorated, 40 mg/day of clenbuterol hydrochloride was administered in combination with 250 mg of azithromycin, which was given twice a week, and which led to a markedly decreased frequency of exacerbation of airway infection. Moreover, chest CT scanning and respiratory function testing also indicated improvements. It was hypothesized that the decreased cilia motility due to P. aeruginosa-produced pyocyanin would be ameliorated with a b2 stimulant, and the inhibitory effect of a macrolide on the P. aeruginosa biofilm and production of pyocyanin would also be involved in the improvement of this case.
Itoh
Masayuki
M
Fifth Department of Internal Medicine, Tokyo Medical University, 3-20-1 Ami-cho, Inashiki-gun, Ibaraki 300-0395.
Kishi
Koji
K
Nakamura
Hiroyuki
H
Hatao
Hidekazu
H
Kioi
Kiwamu
K
Sudou
Akihiko
A
Kobayasi
Katsuyuki
K
Tuchida
Fumihiro
F
Adachi
Hideki
H
Yagyuu
Hisanaga
H
Oh-ishi
Shuji
S
Matsuoka
Takeshi
T
D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage*; D017963:Azithromycin / Q000008:administration & dosage*; D001993:Bronchodilator Agents / Q000008:administration & dosage*; D002925:Ciliary Motility Disorders / Q000188:drug therapy* / Q000209:etiology; D002976:Clenbuterol / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D006801:Humans; D008297:Male; D011552:Pseudomonas Infections / Q000150:complications*; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification
D000900:Anti-Bacterial Agents; D001993:Bronchodilator Agents; D017963:Azithromycin; D002976:Clenbuterol