We evaluated the excursion of the diaphragm in five patients with Duchenne muscular dystrophy maintained on a chest respirator. The excursion was determined by chest X-ray in all cases and by fluroscopy in two. Overnight monitoring using a pulse oximeter was performed on every patient. Two patients who demonstrated recurrent desaturation were examined by chest X-ray during sleep. The excursion of the diaphragm was very small in all cases. Furthermore, in the desaturation period during sleep, excursion was almost absent. In one of the two patients who underwent fluroscopy, paradoxical movement of diaphragm was noticed. In the other patient the diaphragm stopped momentarily at the early inspiratory phase after movement in a posterior direction. These findings show there is a limit to the effectiveness of a chest respirator in Duchenne muscular dystrophy, where respiratory muscle function declines progressively. Therefore regular respiratory function tests, especially sleep monitoring by pulse oximetry, must be done for patients maintained with a chest respirator.

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Peak expiratory flow rate (PEFR) measured by means of 3 different apparatus, a rollingsealed type spirometer (Box-spiro), a pneumotachometer type peak flow meter (Peakman 8) and a spring type mini-Wright peak flow meter (mini-Wright), were compared each other and evaluated for between-test varialility. Each of 40 asthmatic and 12 healthy children and youth aged 7 to 21 years performed maximum forced expiration 3 times each for 3 different apparatus in an alternating manner. Mean PEFR value differed considerably between the apparatus used, and was highest in mini-Wright. Although, correlation between 3 different PEFRs were very high, absolute values of PEFR measured with different apparatus were not comparable. On the other hand, FEV₁ values measured with Box-spiro and Peakman 8 were in good accord and showed even higher correlation. Between-test variability within 3 consecutive measurements calculated by the equation, (MAX-MIN) /MAX (%), were significantly higher in PEFR (by Peakman 8 and mini-Wright), compared to FEV₁ (by Peakman 8). Thus, we concluded that the accuracy and reproducibility of the measurement of PEFR is lower than FEV₁. PEFR monitoring may play an important role in the management of asthmatic children, but we think it should not be used alone without appropriate checking with spirometry.

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In Department of Pneumology, Children's Hospital Armand-Trousseau, bronchoscopy and bronchoalveolar lavage (BAL) are performed for the cases of resiratory diseases which are difficult to be diagnosed. They usually used the rigid bronchoscope orally in patients under 9 kg of body weight and the flexible bronchoscope in patients over 9 kg of body weight. Pediatric bronchoscopy and BAL are useful procedure for the cases of persistent or recurrent respiratory diseases and could be performed under local anaesthesia without severe complication. When BAL has to be performed, the tip of the flexible bronchoscope is wedged at an orifice of lobar or segmental bronchus. Total volume of injected physiological saline is 10% of functional residual capacity (FRC) divided into six aliquots. Each aliquot is injected and recollected through the channel of bronchoscope by handled syringes. BAL-fluid (BALF) is analysed for microbiology, biochemistry, cytology, cytokines. It is expected that the method of BAL would be unified among the pediatric pneumologists.

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We have a rare case of tracheobronchial stenosis and calcification together. Because our case has the calcification of claviculo-sternal and other joints, we diagnose as chondrodysplasia punctata. It is an interesting case concerned with the relation between stenosis and calcification of trachea.

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