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Acid maltase deficiency in adults presenting as respiratory failure.

During the past nine years 10 patients with the adult form of acid maltase deficiency have been observed at the Mayo Clinic. Three of the adults presented with respiratory failure. In all three the respiratory manifestations dominated the clinical picture and the cause of the respiratory failure (muscle weakness) and the underlying myopathy (glycogen storage disease) were initially unsuspected. Careful evaluation of the respiratory function tests, including the maximal static respiratory pressures, electromyographic examination and histochemical and biochemical studies of muscle biopsy specimens eventually led to the correct diagnosis.

Adult↗

Bronchiectasis: correlation of high-resolution CT findings with health-related quality of life.

AIM: To evaluate the relationship between the severity of bronchiectatic diseases, as evident on high-resolution computed tomography (HRCT) and the patient's quality of life measured using the St George's Respiratory Questionnaire (SGRQ). METHODS AND MATERIALS: Forty-six patients (25 women, 21 men, mean age: 63 years) with bronchiectatic disease as evident on recent HRCT examinations were recruited. Each patient completed the SGRQ and underwent respiratory function tests. HRCT findings were blindly and independently scored by two radiologists, using the modified Bhalla scoring system. The relationships between HRCT scores, SGRQ scores and pulmonary function tests were evaluated. RESULTS: The patients' total CT score did not correlate with the SGRQ scores. However, patients with more advanced disease on HRCT, significantly differed in their SGRQ scores from patients with milder bronchiectatic disease. A significant correlation was found between the CT scores for the middle and distal lung zones and the activity, impacts and total SGRQ scores. No correlation was found between CT scores and respiratory function test indices. However, a significant correlation was found between the SGRQ scores and most of the respiratory function test indices. CONCLUSION: A correlation between the severity of bronchiectatic disease as expressed in HRCT and the health-related quality of life exists in patients with a more severe bronchiectatic disease but not in patients with mild disease. Such correlation depends on the location of the bronchiectasis in the pulmonary tree.

Adult↗

[Heart arrhythmias in chronic respiratory failure due to chronic obstructive bronchopneumopathy: the incidence and correlations with nocturnal respiratory disorders].

The aim of this study was to assess: frequency and type of cardiac arrhythmias in patients with severe stable chronic obstructive lung disease (COLD) and chronic respiratory failure (CRF); diurnal or nocturnal predominance of the detected arrhythmias; prospective relationships between arrhythmias and nocturnal hypoxemic-hypercapnic episodes. All patients were examined with careful and complete medical history, chest roentgenogram, standard electrocardiogram, mono and bidimensional echocardiogram, respiratory function tests, arterial blood gases at rest, 24-hour dynamic electrocardiographic recording and, simultaneously, transcutaneous nocturnal monitoring of respiratory gases (TCNM). We studied 14 men: all complained of exercise-induced dyspnoea for 8 +/- 6 years; 10 of them reported usual nocturnal snoring. Respiratory function tests provided the following values (expressed in percentage compared with theoretical ones): vital capacity 58.6 +/- 15, forced expiratory volume/s 36 +/- 19, Tiffeneau index 60 +/- 19, Motley index 160 +/- 35, carbon monoxide diffusion capacity 48 +/- 26. Arterial blood gas analysis at rest resulted: PO2 47 +/- 4.5 mmHg, PCO2 49 +/- 7.9 mmHg, pH 7.38 +/- 0.3. Right ventricular diameter obtained with mono and bidimensional echocardiogram was 32 +/- 4.6 mm. Right pulmonary descending artery measured on chest roentgenogram was 23 +/- 3.8 mm. Nocturnal transcutaneous monitoring of respiratory gases showed mean PO2 of 40 +/- 9.7 mmHg and mean PCO2 of 75 +/- 19 mmHg. During night-time maximum percentage reductions of PO2 (36 +/- 17%) were measured.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Post-intubation pulmonary function test in Guillain-Barré syndrome.

The value of daily postintubation respiratory function tests in predicting duration of ventilation in 37 mechanically ventilated patients with Guillain-Barré syndrome (GBS) was studied. Patients ventilated for less than 3 weeks were compared with those ventilated more than 3 weeks. Daily vital capacity and maximal inspiratory and expiratory pressures were summed to an integrated pulmonary function (PF) score. We calculated the PF ratio, which represents the PF score at day 12 after intubation divided by the PF score on the day of intubation. The PF ratio was greater than 1 in all 10 patients ventilated less than 3 weeks and was less than 1 in 19 of 27 patients ventilated for longer (P = 0.0001, Fisher exact test). The sensitivity of a PF ratio less than 1 for predicting duration of ventilation greater than 3 weeks was 70%; the specificity and positive predictive value were 100%. This study thus suggests that serial postintubation respiratory tests can provide a measure of respiratory status in patients with GBS. These parameters may help predict duration of ventilation and need for tracheostomy. If, at day 12, the PF ratio is less than 1, it is highly unlikely that patients will be weaned within 3 weeks, and tracheostomy should be performed. If the ratio is greater than 1, tracheostomy should be deferred, because a substantial proportion of these patients may be successfully weaned from the ventilator within 3 weeks.

Guillain-Barre Syndrome↗

Changes in HRCT findings in patients with respiratory bronchiolitis-associated interstitial lung disease after smoking cessation.

High-resolution computed tomography (HRCT) findings in patients with respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) are varied and nonspecific. There is no known report of changes in HRCT findings and respiratory function test results for RB-ILD patients following the cessation of smoking. Five patients with RB-ILD, confirmed by surgical lung biopsy, were retrospectively studied. Each stopped cigarette smoking and did not receive corticosteroid therapy after diagnosis. The clinical symptoms, respiratory function test results and HRCT findings obtained at the final observation were compared with those from the time of diagnosis. Ground-glass opacity and centrilobular nodules corresponding to pathological respiratory bronchiolitis, as well as intralobular fine linear-reticular opacity corresponding to fibrosis involving the subpleural alveolar septa, showed computed tomography-pathological correlations. Both clinical symptoms and the diffusing capacity of the lungs for carbon monoxide improved significantly following smoking cessation, as did ground-glass opacity and centrilobular nodules seen during the initial HRCT examination. Centrilobular nodules and ground-glass opacity, which are the main features of high-resolution computed tomography of respiratory bronchiolitis-associated interstitial lung disease patients and represent pathological respiratory bronchiolitis, can be improved by smoking cessation. The diffusing capacity of the lung for carbon monoxide in respiratory function tests can be also improved.

Adult↗

Spiral CT evaluation of pulmonary emphysema using a low-dose technique.

PURPOSE: To evaluate the diagnostic accuracy and clinical acceptability of low-dose spiral CT for determining pulmonary volumes and emphysema extension in patients with pulmonary emphysema, in comparison with studies based on spiral CT at conventional dose. MATERIALS AND METHODS: We prospectively evaluated eighteen patients, current or former smokers, with a clinical diagnosis of chronic obstructive pulmonary disease. All the patients underwent: HRCT with three scans at predetermined levels; quantitative spiral CT, with two inspiratory scans, one conventional scan at 240 mA, and the second one a low-dose scan at 80 mA. We used the following parameters: 120 kV, rotation time 0.8", scan time less than 20" (single inspiratory breath-hold), layer thickness 7.5 mm, pitch 6 (high speed), interpolation algorithm at 180 degrees. A 3D reconstruction was performed, with segmentation of the lungs and automatic quantification of pulmonary volumes. We compared the volumes of absolute and percent emphysema and the ratings of the dose delivered to the patient (CTDIw and DLP) obtained with the two spiral CT scans with each other and with the respiratory function tests. RESULTS: The average total lung capacity (TLC) obtained by conventional-dose spiral CT (CTs1) was 6889.4 cc (SD +/-1813.2), and the capacity with low-dose spiral CT (CTs2) was 6929.4 cc (SD +/-1811.6). The percentage of emphysema was 39.7% (range: 2.2-63.5%; SD: +/-19.9) for the CTs1 and 41.1% (range: 2.1-66.4%; SD: +/-20). The CTDIw corresponding to CTs1 was 12.2 mGy (range: 11.9-16.4; SD: +/-1), the one corresponding to CTs2, 3.6 mGy (range: 3.6-4.9; SD: +/-0.3). The DLP corresponding to CTs1 was 391.7 mGy x cm (range: 333.3-518.9; SD: +/-46.7), the one corresponding to CTs2 was 117.8 mGy x cm (range: 100.3-156; SD: +/-14). As for the respiratory function tests, the total lung capacity (TLC) obtained by body plethysmography was 7061 cc (SD: +/-2029.7); the percent TLC was 115.9 (range: 66-165; SD: +/-27.6), the forced expiratory volume at one second (FEV1%, percentage of predicted value) was 46.7% (range: 17-123; SD: +/-27.3), residual volume (RV%) as a percentage of predicted value was 186.3 (range: 84-359; SD: +/-80.7), the Tiffeneau index (TI) was 46% (range: 25-71; SD: +/-15.7). We observed a very significant correlation between radiological and functional TLC for both CT methods. The percentage scores for emphysema obtained with the two methods correlated significantly with the functional indexes. The pixel index of CTs1 correlated with TLC% (r=0.87; p<0.0001), FEV1% (r=-0.53; p<0.02), RV% (r=0.76; p=0.004), TI (r=-0.79; p=0.0001). The pixel index of CTs2 correlated with TLC% (r=0.87; p<0.0001), FEV1% (r=-0.56; p=0.01), RV% (r=0.78; p=0.003), TI (r=-0.8; p=0.0001). The adoption of the method with low tube current entailed a highly significant reduction in the estimated dose delivered to patients (CTDIw and DLP) with r=0.9 and p < 0.0001. DISCUSSION AND CONCLUSIONS: Quantitative low-dose spiral CT is a very good method to quantify pulmonary volumes and calculate the extension of the anatomic emphysema. The reduction of mA from 240 to 80 lowers the estimated dose by 30%, without compromising the accuracy of the results. Our study achieved a highly significant correlation between the results obtained with the two spiral CT techniques and between these results and the respiratory function tests. In clinical practice, the easiest way to reduce the dose in spiral CT of the lung is to reduce the tube current. The low-dose method allows a significant reduction in radiation exposure. Further studies are required to establish to what extent the dose can be reduced without increasing in quantum noise and thereby compromising the quality of the study.

Adult↗