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Biomedical subjects

F Lofaso

Publications and source records attributed to F Lofaso.

At least 19 recordsLinked to original sources

Effect of intrathecal baclofen on sleep and respiratory function in patients with spasticity.

OBJECTIVE: To prospectively evaluate the effect of pump-infused intrathecal baclofen infusion (ITB) in therapeutic doses on sleep quality and on daytime and nighttime respiratory function in patients with severe spasticity. METHODS: We prospectively evaluated 20 consecutive patients (mean +/- SD age 45 +/- 13 years). We assessed spasticity and obtained polysomnography, pulmonary function tests, and resting energy expenditure measurements 1 week before and at least 8 days after pump implantation. Patients stopped oral baclofen upon pump implantation but continued other medications unchanged. We report descriptive statistics as means +/- SD. RESULTS: Most of the patients had multiple sclerosis (n = 9) or spinal cord injury (n = 8); there was one case each of cerebral palsy, hereditary spastic paraplegia, and Friedreich ataxia. Before and after ITB initiation, mean Ashworth scores were 2.75 +/- 0.85 and 1.15 +/- 0.36, and mean spasm scores were 3.75 +/- 0.55 and 1.00 +/- 0.56. ITB improved total sleep time (p = 0.05) and sleep efficiency (p = 0.01) and reduced periodic leg movements (p = 0.02). ITB did not modify sleep-related respiratory events, lung function tests, CO2 rebreathing response, or resting energy expenditure. CONCLUSION: Compared with oral baclofen, intrathecal baclofen infusion did not affect respiratory function and improved sleep continuity. Intrathecal baclofen infusion in therapeutic doses may act at the spinal level rather than at the supraspinal level.

Adult↗

Inhibitory effects of repeated hyperoxia on breathing in newborn mice.

Brief oxygen therapy is commonly used for resuscitation at birth or prevention of hypoxaemia before procedures during the neonatal period. However, O(2) may severely depress breathing, especially when administered repeatedly. The aim of the present study was to test the effects of repeated hyperoxia on breathing control in newborn mice. A total of 97 Swiss mouse pups were assigned to O(2) or air on post-natal day 0, 1 or 2. Each pup in the O(2) group was subjected to four hyperoxic tests (100% O(2) for 3 min followed by 12 min normoxia), whereas pups in the air group were maintained in normoxia. Breathing variables were measured using flow-through barometric plethysmography. O(2) significantly decreased minute ventilation as seen in a decrease in respiratory rate. This decrease became significantly larger with repeated exposure and ranged -17- -26% for all ages combined. Furthermore, hyperoxia increased total apnoea duration, as compared with the baseline value. In newborn mice, repeated hyperoxia increasingly depressed breathing. This finding further supports a need for stringent control of oxygen therapy, most notably repeated oxygen administration in the neonatal period for premature newborn infants and those carried to term.

Age Factors↗

Sniff nasal inspiratory pressure: what is the optimal number of sniffs?

Sniff nasal inspiratory pressure (SNIP) measurement is a volitional noninvasive assessment of inspiratory muscle strength. A maximum of 10 sniffs is generally used. The purpose of the present study was to investigate whether the maximum SNIP improved after the tenth sniff. In total, 20 healthy volunteers and 305 patients with various neuromuscular and lung diseases were encouraged to perform 40 and 20 sniffs, respectively. The best SNIP among the first 10 sniffs was lower than the best SNIP among the next 10 sniffs in the healthy volunteers and patients. The SNIP improvement after the twentieth sniff was marginal. In conclusion, a learning effect persists after the tenth sniff. The current authors suggest using 10 additional sniffs when the best result of the first 10 sniffs is slightly below normal, or when sniff nasal inspiratory pressure is used to monitor a progressive decline in inspiratory muscle strength.

Adult↗

Characteristics of tracheostomy phonation valves.

Phonation valves are commonly used devices that allow the restoration of speech in tracheostomised patients. However, their use should not compromise the physiological benefit of tracheostomy. Six commercialised phonation valves were studied in a dynamic set-up simulating a respiratory frequency of 20 breaths.min(-1), a tidal volume of 0.5 L and a peak flow rate of 0.5 L.s(-1). Resistance and additional work of breathing (WOB) were calculated. In 10 tracheostomised patients, evaluations using no phonation valve (baseline), and the most and one of the least resistive valves were carried out. Respiratory patterns and gas exchanges were recorded. Inspiratory difficulty was evaluated using the modified Borg scale. Valves displayed a wide array of resistance ranging 1.3-5.9 cmH2O.L(-1).s(-1). Additional WOB varied with a ratio of 4.4 between the best and the worst valve. While the different clinical conditions did not modify respiratory patterns and gas exchanges, a significant effect on the Borg scale rating was observed using ANOVA and post hoc analysis of baseline versus worst valve and one of the best valves versus worst valve. In conclusion, the variety of aerodynamic characteristics of phonation valves should be considered when choosing the device, according to the underlying condition of the patients benefiting from their use.

Adult↗

Significance of phrenic nerve electrophysiological abnormalities in Guillain-Barré syndrome.

The authors investigated whether the amplitude and latency of diaphragm compound muscle action potential helped predict respiratory failure in Guillain-Barré syndrome. Both variables were significantly but weakly correlated with vital capacity (VC) and were similar in unventilated (n = 60) and ventilated (n = 10) patients. In ventilated patients, motor loss severity, progression, and VC reduction were significantly greater, and bulbar dysfunction was more common. Predicting respiratory failure must rely on clinical features and VC.

Action Potentials↗

[Domiciliary non-invasive ventilation in children].

INTRODUCTION: Non-invasive positive pressure ventilation (NPPV) represents a particularly interesting technique of ventilatory support in paediatrics. Indeed, a significant number of pathologies that may be responsible for chronic respiratory insufficiency in childhood, such as neuromuscular diseases, obstruction of the upper airways, disorders of chest wall and/or the lungs, and disorders of ventilatory control may all lead to alveolar hypoventilation that can be improved by ventilatory support. BACKGROUND: Few physiological studies have been performed on NPPV in children. The most appropriate modes and settings for each pathology have not been clearly defined, and the criteria for commencing NPPV are based essentially on consensus guidelines for the management of neuromuscular disorders. VIEWPOINT: All the health care professionals managing these children should combine their efforts to evaluate more precisely the medium and long-term physiological effects of NPPV on the respiratory muscles, the development of the respiratory system, inspiratory activity, the indications for starting treatment and, above all, the benefits in terms of psycho-neurological development and quality of life. CONCLUSIONS: A better evaluation of the medium and long-term physiological and psychological benefits together with technical improvements in ventilators and associated equipment should allow a rapid expansion in the use of domiciliary NPPV in children.

Child↗

Respiratory insufficiency and limb muscle weakness in adults with Pompe's disease.

The objective of the present study was to prospectively evaluate relationships linking age, respiratory function and locomotor function in 29 outpatients with late-onset Pompe's disease and to retrospectively determine clinical outcomes. Using univariate regression analysis, vital capacity (VC) was weakly, but significantly, correlated to shoulder motility, Walton score and lower-limb Modified Medical Research Council score. Six patients were able to walk without a walking aid and with only the help of a handrail on the stairs (Walton score=3), although VC was <50%. No parameters were significantly correlated with age. As assessed retrospectively, VC and locomotion deteriorated over time in most patients. In contrast, among the 16 patients started on invasive or noninvasive ventilation with VC monitoring, eight had a VC increase at the first measurement time-point. The absence of correlation with age and the presence, in some patients, of severe respiratory insufficiency without severe limb girdle muscle weakness indicate that respiratory function should be monitored independently from the degree of peripheral muscle weakness. Mechanical ventilation and tracheostomy may improve vital capacity and should, therefore, be taken into account when evaluating treatments for the adult form of Pompe's disease.

Age Distribution↗

Resting energy expenditure in Duchenne patients using home mechanical ventilation.

Nutritional status is both important and difficult to assess in patients with Duchenne muscular dystrophy (DMD), particularly in those requiring mechanical ventilation (MV). The current authors evaluated body composition (bio-impedancemetry), resting energy expenditure (REE; indirect calorimetry) and energy intake in 20 adult patients with DMD using home MV (nocturnal: n = 13; continuous: n = 7) and 12 age-matched healthy controls. The patients were smaller in height than the controls and had a lower body weight. Most of the reduction in body mass index was accounted for by a reduction in fat free mass (FFM). REE (kJ) was significantly reduced in the patients (4559+/-853 kJ x 24 h(-1) versus 7407+/-1312 kJ x 24 h(-1)), but the difference disappeared after correction for FFM. REE and FFM were correlated in both the controls and patients, but less strongly in the latter, the lower strength of the association being due to the patients using continuous MV (REE and FFM uncorrelated). The food intake of the patients was 1.2+/-0.4 greater than their REE. This study shows that patients with advanced forms of Duchenne muscular dystrophy have balanced energy intakes and resting energy expenditure.

Adult↗

Inspiratory muscle load and capacity in chronic heart failure.

BACKGROUND: Although breathlessness is common in chronic heart failure (CHF), the role of inspiratory muscle dysfunction remains unclear. We hypothesised that inspiratory muscle endurance, expressed as a function of endurance time (Tlim) adjusted for inspiratory muscle load and inspiratory muscle capacity, would be reduced in CHF. METHODS: Endurance was measured in 10 healthy controls and 10 patients with CHF using threshold loading at 40% maximal oesophageal pressure (Poes(max)). Oesophageal pressure-time product (PTPoes per cycle) and Poes(max) were used as indices of inspiratory muscle load and capacity, respectively. RESULTS: Although Poes(max) was slightly less in the CHF group (-117.7 (23.6) v -100.0 (18.3) cm H(2)O; 95% CI -37.5 to 2.2 cm H(2)O, p = 0.1), Tlim was greatly reduced (1800 v 306 (190) s; 95% CI 1368 to 1620 s, p<0.0001) and the observed PTPoes per cycle/Poes(max) was increased (0.13 (0.05) v 0.21 (0.04); 95% CI -0.11 to -0.03, p = 0.001). Most of this increased inspiratory muscle load was due to a maladaptive breathing pattern, with a reduction in expiratory time (3.0 (5.8) v 1.1 (0.3) s; 95% CI 0.3 to 3.5 s, p = 0.03) accompanied by an increased inspiratory time relative to total respiratory cycle (Ti/Ttot) (0.43 (0.14) v 0.62 (0.07); 95% CI -0.3 to -0.1, p = 0.001). However, log Tlim, which incorporates the higher inspiratory muscle load to capacity ratio caused by this altered breathing pattern, was >/=85% predicted in seven of 10 patients. CONCLUSIONS: Although a marked reduction in endurance time was observed in CHF, much of this reduction was explained by the increased inspiratory muscle load to capacity ratio, suggesting that the major contributor to task failure was a maladaptive breathing pattern rather than impaired inspiratory muscle endurance.

Aged↗

Setting of noninvasive pressure support in young patients with cystic fibrosis.

The aim of the current study was to compare a clinical noninvasive method of setting up noninvasive pressure support ventilation (PS-NI) in young patients with cystic fibrosis (CF), based on parameters such as breathing frequency, arterial oxygen saturation and comfort rating, with a more invasive method (PS-I) targeted at optimising unloading of the inspiratory muscles and enhancing patient-ventilator synchronisation. PS-NI and PS-I were compared in random order in 10 children with CF. PS-NI differed from PS-I with regard to the level of inspiratory pressure (n=5), rate of inspiratory pressurisation (n=1), inspiratory trigger sensitivity (n=2) and expiratory trigger sensitivity (n=5). Although both methods modified breathing pattern, improved oxygen saturation and reduced diaphragmatic pressure time product (450+/-91 cmH2O.s(-1).min(-1) during spontaneous breathing, and 129+/-125 and 104+/-75 cmH2O.s(-1).min(-1) during PS-NI and PS-I, respectively), patient-ventilator synchrony and patient comfort were enhanced more during PS-I. In young patients with cystic fibrosis, setting up pressure support using a clinical noninvasive approach based on easily measurable parameters, such as respiratory rate and comfort rating, is as effective as a more invasive technique based on unloading of the inspiratory muscles and optimising patient-ventilator synchronisation. However, whilst the standard clinical method is satisfactory in the majority of patients, more invasive measurements should be considered in patients who have difficulty synchronising with the ventilator to enhance patient tolerance and compliance.

Adolescent↗

Intermittent hypoxia induces transient arousal delay in newborn mice.

Previous studies suggested that defective arousal might be a major mechanism in sleep-disordered breathing such as sudden infant death syndrome and obstructive sleep apnea. In this study, we examined the effects of intermittent hypoxia (IH) on the arousal response to hypoxia in 4-day-old mice. We hypothesized that IH would increase arousal latency, as previously reported in other species, and we measured the concomitant changes in ventilation to shed light on the relationship between breathing and arousal. Arousal was scored according to behavioral criteria. Breathing variables were measured noninvasively by use of whole-body flow plethysmography. In the hypoxic group (n = 14), the pups were exposed to 5% O(2) in N(2) for 3 min and returned to air for 6 min. This test was repeated eight times. The normoxic mice (n = 14) were constantly exposed to normoxia. The hypoxic mice showed a 60% increase in arousal latency (P < 0.0001). Normoxic controls showed virtually no arousals. IH depressed normoxic ventilation below baseline prehypoxic levels, while preserving the ventilatory response to hypoxia. The breathing pattern and arousal responses recovered fully after 2 h of normoxia. We conclude that IH rapidly and reversibly depressed breathing and delayed arousal in newborn mice. Both effects may be due to hypoxia-induced release of inhibitory neurotransmitters acting concomitantly on both functions.

Animals↗

Electrophysiology to predict mechanical ventilation in Guillain-Barré syndrome.

To determine whether electrophysiological features predict endotracheal mechanical ventilation (ETMV) in Guillain-Barré syndrome (GBS). Non-ventilated GBS patients admitted to an ICU underwent standard electrophysiological testing. Endotracheal mechanical ventilation was decided by physicians who were unaware of electrophysiological results. Sixty consecutive patients underwent electrophysiological testing within 17 days of GBS onset; based on Hadden's criteria, 37 (62%) had primary demyelinating, 18 (30%) equivocal and five (8%) axonal disease. Time at electrophysiological testing and proportions of patients treated by plasma exchange and intravenous immunoglobulins were similar in the three groups, whereas primary demyelinating patients had worse results for disability grade and arm grade. The ETMV was required within 20 days of electrophysiological testing in 20 patients, 17 (46%) in the primary demyelinating group, three (17%) in equivocal group and none in the axonal group (P = 0.02). This prospective study suggests that electrophysiological demyelination may predict a need for ETMV in GBS.

Adolescent↗

Limitations of sniff nasal pressure in patients with severe neuromuscular weakness.

BACKGROUND: Inspiratory muscle strength in patients with neuromuscular disorders can be assessed using sniff inspiratory nasal pressure (Pn(sn)) and maximum inspiratory mouth pressure (PI(max)). However, the relative merits of Pn(sn) against PI(max) are not known in patients with severe neuromuscular disease. OBJECTIVE: To investigate whether severity of disease modifies the relation between Pn(sn) and PI(max). METHODS: Vital capacity (VC), Pn(sn), and PI(max) were measured in 258 patients with neuromuscular disorders. RESULTS: Data were analysed from 241 patients, 17 being unable to perform PI(max) or Pn(sn) manoeuvres. The correlation between Pn(sn) and PI(max) was +0.94 (p<0.0001), with a mean (SD) difference between Pn(sn) and PI(max) of -4.8 (21.2) cm H(2)O (the limits of agreement were 37.6 and -47.2 cm H(2)O). VC (% predicted) was positively correlated with Pn(sn)/PI(max) (r = +0.86; p<0.0001), with a lower Pn(sn)/PI(max) value in patients with a VC <40% of predicted than in those with a VC >40% (0.80 (0.35) v 1.04 (0.41); p<0.0001). CONCLUSIONS: PI(max) is greater than Pn(sn) in patients with a severe restrictive ventilatory defect caused by neuromuscular disease. Pn(sn) may not accurately reflect inspiratory muscle strength in such patients and it is thus advisable to use both tests.

Adult↗

MMP-9 correlates with electrophysiologic abnormalities in Guillain-Barré syndrome.

To investigate the role of MMP-9 in Guillain-Barré syndrome, the authors correlated electrophysiologic abnormalities and MMP-9 plasma levels in a series of 21 patients. MMP-9 plasma levels were higher in the demyelinating group than in the nondemyelinating group, and in patients with high CSF protein level. Increase of MMP-9 circulating levels correlated with the increase of F waves latencies, reduction of CMAP amplitude, and decrease of nerve conduction velocities. Circulating MMP-9 may contribute to the peripheral nerve dysfunction of demyelinating Guillain-Barré syndrome.

Action Potentials↗

Utility of actigraphy in the diagnosis of obstructive sleep apnea.

STUDY OBJECTIVES: To determine whether adding actimetry to simplified polygraphy (respiratory-parameter monitoring without neurophysiologic variable recording) improves apnea-hypopnea index (AHI) evaluation as compared to simplified polygraphy alone. DESIGN: Comparison of AHI values obtained by all-night polysomnography and by simplified polygraphy with and without actimetry. SETTING: A teaching-hospital sleep laboratory in Garches, France. PATIENTS: 20 adults with suspected obstructive sleep apnea syndrome (OSAS). MEASUREMENTS AND RESULTS: Data were analyzed by two scorers working independently. AHI was calculated as the number of apneas and hypopneas per hour of sleep time (polysomnography: AHI-pg), per hour of time in bed (simplified polygraphy: AHI-tib), and per hour of actimetry-estimated total sleep time (AHI-act). AHI-pg showed that 12 patients had OSAS (AHI>10), which was severe (AHI > or =30) in eight. AHI-act was more closely correlated to AHI-pg (r=0.976) than was AHI-tib (r=0.940). According to the Bland and Altman method, AHI-tib underestimated the AHI in two patients and AHI-act overestimated the AHI in one patient. For the diagnosis of severe OSAS, sensitivity and negative predictive value were 50% and 75% with AHI-tib as compared to 88% and 92.5% with AHI-act. CONCLUSIONS: Actimetry, when added to simplified polygraphy, may assist in the diagnosis of OSAS.

Acceleration↗