PT  - JOURNAL ARTICLE
AU  - A Whitelaw
AU  - A Evans
AU  - B Corrin
TI  - Immotile cilia syndrome: a new cause of neonatal respiratory distress.
AID  - 10.1136/adc.56.6.432
DP  - 1981 Jun 01
TA  - Archives of Disease in Childhood
PG  - 432--435
VI  - 56
IP  - 6
4099  - http://adc.bmj.com/content/56/6/432.short
4100  - http://adc.bmj.com/content/56/6/432.full
SO  - Arch Dis Child1981 Jun 01; 56
AB  - Kartagener's syndrome is a condition that consists of situs inversus, bronchiectasis, and sinusitis. Some patients have respiratory symptoms that date from early infancy, and electron microscopical examination has shown that adults with this condition lack dynein arms in ciliary microtubules. It has been suggested that an inherited defect in ciliary ultrastructure, the immotile cilia, is the basis for the syndrome. We report 6 patients who presented within the first 24 hours of life with tachypnoea, retraction, rales, and dextrocardia. Five of them had situs inversus. They all responded to physiotherapy after antibiotics had failed to improve their condition. Electron microscopical examination of the cilia in 2 of the infants confirmed deficiency of dynein arms. All 6 infants had recurrent respiratory infections. Four went on to develop recurrent otitis media, 2 had sinusitis, and 1 now has bronchiectasis. The immotile cilia syndrome should be added to the list of causes of respiratory distress in newborn infants. The condition is worth diagnosing because of the good response to physiotherapy, and because of the likelihood of recurrent respiratory problems.