[Immotile cilia syndrome. Presentation of 3 cases]. / Síndrome de los cilios inmóviles. Presentación de tres casos.

Immotile cilia syndrome is an uncommon disorder, with an autosomal recessive mode of inheritance; the incidence is about 1/25,000 and is a cause of chronic disease of both upper and lower airways, male infertility and, in 50%, situs inversus totalis. It's pathogenesis lies in a variety of genetically determined alteration of ciliary ultrastructure. We report three cases, two in siblings, who had a typical syndrome, all of them with situs inversus totalis (Kartagener syndrome), chronic nasal symptoms, sinusitis and serous otitis with hearing loss. Two had recurrent productive cough with chest X-ray changes suggestive of bronchiectasis. The diagnosis was established in two by bronchial biopsy, showing complete and partial dynein deficiency respectively. In the third case it was based an clinical grounds and familial history. All three children are doing well up to now.

[Bronchodilating effect of terbutaline inhaled with and without a tube spacer in asthmatic children]. / Efecto broncodilatador de la terbutalina inhalada con y sin extensor en niños asmáticos.

Eighteen children aged 10.3 +/- 2.4 years who needed regular use of antiasthmatic drugs and showed after abandoning them with the appropriate intervals a FEV1 below 85% of their highest previous value, received in a randomized, double-blind crossover fashion two puffs of terbutaline 1 hour apart on two consecutive days. The drug was administered either with the conventional inhaler or a 10.3 X 2 cm. Forced spirometry, peak flow, flow-volume loops, and whole body plethysmography were performed basally, and 5, 15 and 45 minutes after each puff. The peak increase of the FEV1 was greater with the spacer (41.7% vs 30.4%, p less than 0.05). The increase of FEV1 was higher with the spacer at 5 minutes following the first puff and at 15 and 45 minutes after the second puff. No difference was observed in the other variables measured. Sixteen inhaled the drug "coordinatedly". The spacer offers some advantage in asthmatic children who inhale terbutaline mostly in a coordinated way.