Plasma Krebs von den Lungen glycoprotein, lung injury, and noninvasive ventilation in Duchenne muscular dystrophy.

OBJECTIVE: There have been few reports of ventilator-induced lung injury associated with noninvasive ventilation (NIV), but many with invasive mechanical ventilation. The purpose of this study was to detect subclinical NIV-associated lung injury by monitoring Krebs von den Lungen glycoprotein plasma levels. DESIGN: Forty-one Duchenne muscular dystrophy patients were divided into three categories: group 1, asymptomatic and not using ventilators; group 2, NIV use less than 24 hrs/day at full ventilatory support settings; and group 3, continuous NIV dependence. Plasma Krebs von den Lungen glycoprotein level was measured by electrochemical luminescent immunoassay using Krebs von den Lungen glycoprotein antibodies. One-way analysis of variance, followed by the Tukey-Kramer test, was used as appropriate to compare intergroup differences. RESULTS: Extent of ventilator dependence correlated with age (P < 0.05). Intergroup plasma Krebs von den Lungen glycoprotein levels were not significantly different. CONCLUSIONS: NIV used at volumes and pressures of full (invasive) ventilatory support may not induce the alveolar septal barrier injury commonly seen with invasive mechanical ventilation.

Indicators for ventilator use in Duchenne muscular dystrophy.

BACKGROUND: Noninvasive mechanical ventilation is being used up to continuously by patients with Duchenne muscular dystrophy (DMD). Invasive and noninvasive tests are used to assess ventilatory function but there are few reports relating them to extent of ventilator dependence for which simple and cost effective parameters are needed. OBJECTIVE: To investigate the relative efficacy of noninvasive lung function parameters for determining extent of need for ventilator use. MATERIALS AND METHODS: 83 DMD patients were divided into three groups: no ventilator use (asymptomatic) (n = 26) [Group 1], nocturnal ventilator use (symptomatic) (n = 20) [Group 2], and full-time ventilator dependence (n = 37) [Group 3]. Tidal volume (TV), vital capacity (VC), respiratory rate (RR), inspiratory time (Ti), respiratory cycle time (Ttot), rapid shallow breathing index (RSBI [RR/TV]), breathing intolerance index (BITI), ventilator requirement index (VRI) and a new parameter RR/VC were monitored and compared. Data were analyzed with receiver-operating-characteristic curves (ROC) and the area under the curve (AUC) was calculated. RESULTS: In group 2 and 3, patients used NIV for 3.3 ± 2.1 and 11.2 ± 4.7 years, respectively. By ROC comparison, RR/VC (RR/VC ≥ 0.024 [AUC, 0.921] and ≥0.071 [AUC, 0.935]), RR/TV (RR/TV ≥ 0.024 [AUC, 0.905] and ≥0.153 [AUC, 0.905]), and VC (VC ≤ 770 ml [AUC, 0.896] and ≤370 ml [AUC, 0.898]) represented to introduce nocturnal and continuous ventilator use, respectively. TV/VC, BITI, and VRI were either less sensitive or less specific. CONCLUSIONS: Lung function parameters including RR/VC, RR/TV, and VC are useful and inexpensive in predicting the extent of need for ventilator use. Overall, RR/VC is the most appropriate predictor for determining extent of need for ventilator use.

Duchenne muscular dystrophy: survival by cardio-respiratory interventions.

We describe survival in Duchenne dystrophy by invasive and noninvasive ventilation vs. untreated. Patients were untreated prior to 1984 (Group 1), underwent tracheotomy from 1984 until 1991 (Group 2), and were managed by noninvasive mechanical ventilation and cardioprotective medications subsequently (Group 3). Symptoms, vital capacity, and blood gases were monitored for all and spirometry, cough peak flows, carbon dioxide tension, and oximetry for Group 3. Sleep nasal ventilation was initiated for symptomatic hypoventilation. An oximeter and mechanical cough assistance were prescribed for maximum assisted cough peak flow <300 L/m. Patients used continuous noninvasive ventilation and mechanically assisted coughing as needed to maintain pulse oxyhemoglobin saturation ≥95%. Survival was compared by Kaplan-Meier analysis. The 56 of Group 1 died at 18.6±2.9, the 21 Group 2 at 28.1±8.3 years of age with three still alive, and the 88 using noninvasive ventilation had 50% survival to 39.6 years, p<0.001, respectively. We conclude that noninvasive mechanical ventilation and assisted coughing provided by specifically trained physicians and therapists, and cardioprotective medication can result in more favorable outcomes and better survival by comparison with invasive treatment.