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Biomedical subjects

A Delaubier

Publications and source records attributed to A Delaubier.

9 recordsLinked to original sources
PubMed1997

Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy.

OBJECTIVE: To analyze the relationship between disease progression, pulmonary volumes, respiratory muscle strength (maximum inspiratory [MIP] and expiratory [MEP] pressure), and arterial blood gases for patients with Duchenne muscular dystrophy (DMD). DESIGN: An inception cohort study of pulmonary volumes, MIPs, and MEPs, correlated with age and PaCO2 levels and with each other using linear and nonlinear regression analyses. SETTING: Outpatient clinic. PATIENTS: Fifty-two consecutive DMD patients who presented for regular evaluations at a regional DMD center. RESULTS: Maximum expiratory pressures were 47.7% +/- 10.9% of normal in the 167- to 14-year-old patients and decreased linearly thereafter (MEP% = -2.7 x age +73.8; p < .001). Declines in MEP also correlated linearly with expiratory reserve volume (p < .001) and inversely with residual volume (p < .001). By contrast, MIP was 66.3% +/- 19.0% in the 357- to 14-year-old patients and then declined to 30.2% +/- 19.5% after age 14. No linear relationships were found with age but declines did correlate linearly with inspiratory reserve volume (p < .001) and total lung capacity (p < .001). PaCO2 elevations correlated best with decreases in MIP (p < .0001) and appeared when MIP was below 30cmH2O. CONCLUSIONS: Lung volume changes in DMD patients correlate with respiratory muscle weakness, and although inspiratory muscle dysfunction plays a key role in the development of chronic ventilatory insufficiency, reductions in expiratory muscle strength are the first signs of dysfunction and lead to the first episodes of respiratory failure.

Adolescent↗
PubMed1995

Treatment of respiratory insufficiency in Duchenne's muscular dystrophy: nasal ventilation in the initial stages.

Fourteen patients with Duchenne's muscular dystrophy (DMD) received nasal ventilation at the beginning of the declining phase of their vital capacity (VC). At that point, the mean value (SD) of their vital capacity was 1,756 (363) mL notably inferior to the predicted values. Nasal ventilation was performed according to a precise protocol, with an average nocturnal duration of 8 h. The annual decline of vital capacity was about 70 ml, showing a significant reduction compared with the value of 200 mL.yr-1 of untreated patients. Follow-up of treated patients (3.8 yrs; SD 1.9 yrs) has enabled us to distinguish different groups of results with useful practical consequences. In 29% of cases, the initial progression of decline of vital capacity was poorly modified by the treatment. In 71% of patients, the decline of VC was significantly decreased. In 35% of cases tracheal ventilation followed nasal ventilation. The normal mortality rate of DMD has greatly changed as a result of this method.

Adolescent↗
PubMed1987

Intermittent positive pressure ventilation via nasal access in the management of respiratory insufficiency.

These are preliminary observations of the introduction of a new technique of noninvasive positive pressure respiratory support for patients with subacute or chronic respiratory failure. Clinical situations where intubation or tracheostomy may have been performed were managed by intermittent positive pressure ventilation via nasal access (NIPPV) with a CPAP mask, or a custom constructed Vel-Foam nose piece. Four patients were managed at home with the use of portable volume ventilators. One patient employed the technique while hospitalized with subacute respiratory failure. Two patients, otherwise dependent on mouth intermittent positive pressure ventilation (MIPPV) 24 hours a day, received necessary dental care with NIPPV support. In a large population with a decade or more follow-up, MIPPV was shown to be an effective noninvasive technique to support respiration in patients with the most severe paralytic respiratory failure. Preliminary observations suggest that NIPPV may compare favorably with MIPPV and deserves more widespread study and application.

Adult↗
PubMed1984

The treatment of scoliosis in Duchenne muscular dystrophy.

There are not, as yet, clear indications for the surgical management of scoliosis in Duchenne muscular dystrophy (DMD), taking into account the varying severity of the clinical course. Monitoring the vital capacity can be most important for the indication and timing of surgery. In some cases, delaying surgical intervention with conservative management using spinal braces and wheelchair inserts can permit the restrictive lung syndrome to advance to the point that surgery will be contraindicated. Ten such patients conservatively treated for an average of 5 years exhibited perhaps a slower progression but ultimately an advanced deformity. From a second group of five carefully selected and surgically treated patients, indications for spinal surgery were reviewed. Surgical intervention should be prophylactically undertaken when there is high risk of a rapidly evolving curve with a severe restrictive lung syndrome.

Braces↗