The aim of our thought was to evaluate the long-term tenor of nasal ventilation in patients with advanced Duchenne's muscular dystrophy (DMD) To this last we compared the clinical and pulmonary function course of five make subordinates affected with chronic ventilatory failure owing to DMD and treated with nasal intermittent positive squeezing ventilation (NIPPV) with that of an unventilated comparison group; the latter consisted of another five patients with DMD with a similar extent of clinical and respiratory functional impairment.

The aim of our thought was to evaluate the long-term tenor of nasal ventilation in patients with advanced Duchenne's muscular dystrophy (DMD) To this last we compared the clinical and pulmonary function course of five make subordinates affected with chronic ventilatory failure owing to DMD and treated with nasal intermittent positive squeezing ventilation (NIPPV) with that of an unventilated comparison group; the latter consisted of another five patients with DMD with a similar extent of clinical and respiratory functional impairment, who refused long-term mechanical ventilation. The duration of the follow-up was 24 month At the conclusion of the trial, all patients treated with NIPPV were still alive; in contrast, four of five patients who underwent simple conservative treatment had already died (mean survival, 97 [+ or -] 58 months) After 6 month of follow-up mean los of FVC and maximal voluntary ventilation was considerably higher in nonventilated controls (respectively: -0.23 L vs + 003 L and -5 L/min v -15 L/min). These are the first comparative outcomes confirming that long-term NIPPV helps to stabilize pulmonary function and to put off the expectancy of life of patients with DMD

Several studies have reported that chronic ventilatory failure, sometimes with a superimposed acute respiratory insufficiency to be paid to pneumonia or atelectasis, is the major cause of death in Duchenne's muscular dystrophy (DMD)[1-3]

In novel years, with the appearance of nasal ventilation, there has been a rapid increase in the application of noninvasive long-term mechanical ventilation for the treatment of chronic hypoventilation in patients with neuromuscular disorders.[4-6]

There is general agreement about the efficacy of long-term nasal intermittent positive press ventilation (NIPPV) in determining a sustained reversal of chronic hypercarbia in patients with DMD with normalization of [PaCO.sub.2], alleviation of symptoms related to [COsub2] retention, and prolongation of life.[7-10]

Nevertheless, as controll studies concerning the part of NIPPV in DMD have not to this time been performed, the real effectiveness of mechanical ventilation via nasal mask forward pulmonary function and the life expectancy of patients with DMD could not be completely evaluated.

In our article, we analyze the clinical and pulmonary function course of ten patients with advanced DMD affected with chronic respiratory failure; five were administered long-term NIPPV, while the other five refused this treatment. The pair groups of patients had similar features from a clinical and respiratory functional point of view, which made it possible to consider the comparison form into groups (not ventilated subjects), though not randomly selecteded as a control group, and enabled us to assess the efficacy of long-term NIPPV in patients with DMD in conditions similar to those of a controll trial.

MATERIALS AND METHODS

Between January 1987 and June 1990 ten unselect young male patients affected with advanced DMD were referr to the Respiratory Pathophysiology Department of the City Hospital of Padua, because of symptoms consistent with chronic hypercapnia (ie, hypersomnolence, insomnia, morning headaches, fatigue, enuresis).

The diagnosis of DMD was based upon standard clinical, enzymatic, electromyographic, and biopsy criteria.

At the beginning of the thought the mean age was 201 [+ or -] 52 years (range, 12 to 27 years) and all the patients belonged to the IX functional class of Inkley's classification (wheelchair-bound).[2] Moreover, they were released from respiratory tract infections. Prior to referral, unrelenting cardiomyopathy had been excluded through a two-dimensional echocardiography in eight patients and by way of clinical and radiographic findings in the remaining two

All the subdues were administered the following examinations: clinical evaluation; chest radiograph: the posteroanterior radiograph of the chest was taken for calculating the measure of scoliotic deformity, according to the [i]modus operandi[/i] of Cobb[11], and pulmonary function exhibitions including the following: (1) conventional spirometry, performed following standard criteria,[12] in seated position, with a 10-L closed-circuit automated spirometer (Baires 80 Biomedin, Padua); static lung convolutions were measured by the closed-circuit helium dilution technique; and (2) measurement of maximal voluntary ventilation (MVV)[13]

allusion values are those of the European Community[14] or of Polgar and Promadhar,[15] for make submissives older or younger than 18 years, respectively. Arm span was used for determining percent predicted values in patients with plain scoliosis.

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Pulmonary function proofs were not performed in undivided patient because of lack of cooperation.

All enthralls also underwent arterial blood gas analysis during spontaneous breathing onward room air (ABL 30, Radiometer, Copenhagen, Denmark).

Since all the patients evidenced stable daytime hypercapnia ([PaCO.sub.2] [greater than] 45 mm Hg) they were advised to be subjected to long-term mechanical ventilation via nasal mask, in agreement with other authors.[10,16]

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