phenylephrine-hydrochloride and Neuromuscular-Diseases

The upper airways are a complex structure with multiple functions; many of the muscles participate in the act of respiration. The neural control of upper airway muscles is distinct from that of chest wall muscles under a variety of circumstances. Coordinated activation of upper airway muscles, both regionally and inter-regionally, results in changes in upper airway size and resistance, alterations in the route of airflow, and increases in the ability of the airways to resist collapse. Several disorders have now been described in which neuromuscular drive abnormalities and/or mechanical dysfunction of the upper airways occur, resulting in clinical disease.

Topics: Afferent Pathways; Behavior; Blood Pressure; Bronchial Spasm; Humans; Hypercapnia; Hypoxia; Laryngeal Diseases; Larynx; Lip; Mechanoreceptors; Muscles; Neuromuscular Diseases; Nose; Palate, Soft; Pharynx; Pulmonary Stretch Receptors; Reflex; Respiration; Respiratory Physiological Phenomena; Respiratory System; Sleep Apnea Syndromes; Vagus Nerve; Vocal Cords

Evaluation of the effectiveness of chronic nightly mechanical ventilation through a nasal mask system in patients with neuromuscular diseases or patients with thorax malformations or COPD.. Centre for Home Mechanical Ventilation, University Hospital Utrecht.. Retrospective study.. During the period June 1988-May 1993, 64 patients (11-69 years old) were treated with nasal mask ventilation during the night: 37 men (most of whom had Duchenne's disease (18) and 27 women (most of whom had (post-polio) kyphoscoliosis (n = 13). The effects on arterial blood gases were analysed. Complications during use of the nasal mask systems were recorded.. The duration of the nasal mask ventilation was 2 to 56 months. Arterial PCO2 and PO2 improved during the night as well as by day during spontaneous respiration. The most common problems were necrosis of the skin of the bridge of the nose, leakage of air through the mouth or insufflation of air into the stomach. After the start of the mechanical ventilation quality of life improved considerably.. Nasal ventilation at night is sufficient and simple. The installation of a tracheostoma can be postponed or avoided with this non-invasive kind of ventilation.

Topics: Adolescent; Adult; Aged; Child; Female; Home Care Services; Humans; Lung Diseases, Obstructive; Male; Masks; Middle Aged; Neuromuscular Diseases; Nose; Positive-Pressure Respiration; Postpoliomyelitis Syndrome; Respiratory Insufficiency; Retrospective Studies

Respiratory failure related to neuromuscular weakness has commonly been managed with long-term ventilation by tracheostomy. Currently, nasal positive pressure ventilation is being used with success in this patient population. It has been suggested that initiation of nasal positive pressure ventilation is best done in an intensive care setting.. Nine patients were placed on nasal ventilation during a brief admission to a medical floor staffed by nurses trained in respiratory care. This process was facilitated by use of a standardized protocol for nursing staff that identified treatment objectives.. Following the initiation of nasal positive pressure ventilation, this group of patients experienced near normalization of daytime measurements of partial pressure of arterial carbon dioxide, reported increases in daytime energy levels, and relief of nighttime signs and symptoms. These objectives were accomplished with no significant complications. Use of this protocol may also decrease length and cost of hospitalization and improve patient acceptance of noninvasive ventilatory assistance.

Topics: Adult; Blood Gas Analysis; Clinical Nursing Research; Clinical Protocols; Critical Care; Fatigue; Fees and Charges; Humans; Length of Stay; Lung Volume Measurements; Masks; Middle Aged; Neuromuscular Diseases; Nose; Patient Care Planning; Patient Compliance; Positive-Pressure Respiration; Respiratory Insufficiency; Sleep Wake Disorders; Treatment Outcome

In patients with neuromuscular disorders, assessment of respiratory function relies on forced vital capacity (FVC) measurements. Providing complementary respiratory outcomes may be useful for clinical trials. Diaphragm sniff ultrasound (US) is a noninvasive technique that can assess diaphragm function that may be affected in patients with neuromuscular disorders.. We aimed to provide normal values of sniff diaphragm ultrasound, to assess the relationship between sniff diaphragm US, vital capacity (VC) and sniff nasal pressure. Additionally, we aimed to evaluate the diagnostic accuracy of sniff diaphragm US for predicting restrictive pulmonary insufficiency.. We included patients with neuromuscular disorders that had been tested with a sniff diaphragm US and functional respiratory tests. Healthy subjects were also included to obtain normal diaphragm sniff ultrasound. We performed diaphragm tissue Doppler imaging (TDI) and time movement (TM) diaphragm echography combined with sniff maneuver.. A total of 89 patients with neuromuscular diseases and 27 healthy subjects were included in our study. In patients, the median age was 32 years [25; 50] and the median FVC was 34% of predicted [18; 55]. Sniff diaphragm motion using TM ultrasound was significantly associated with sniff nasal pressure, both for the right hemidiaphragm (r = 0.6 p <0.0001) and the left hemidiaphragm (r = 0.63 p = 0.0008). Right sniff peak TDI velocity was also significantly associated with FVC (r = 0.72, p<0.0001) and with sniff nasal pressure (r = 0.66 p<0.0001). Sniff diaphragm ultrasound using either TM mode or TDI displayed significant accuracy for predicting FVC<60% with an area under curve (AUC) reaching 0.93 (p<0.0001) for the right sniff diaphragm ultrasound in TM mode and 0.86 (p<0.001) for right peak diaphragm TDI velocity.. Sniff diaphragm TM and TDI measures were significantly associated with sniff nasal pressure. Sniff diaphragm TM and TDI had a high level of accuracy to reveal respiratory involvement in patients with neuromuscular disorders. This technique is useful to assess and follow up diaphragm function in patients with neuromuscular disorders. It may be used as a respiratory outcome for clinical trials.

Topics: Adult; Case-Control Studies; Diaphragm; Female; Humans; Male; Middle Aged; Movement; Neuromuscular Diseases; Nose; Pressure; Respiration; ROC Curve; Tidal Volume; Time Factors; Ultrasonography, Doppler; Vital Capacity

The ability to accurately determine respiratory muscle strength is vitally important in patients with neuromuscular disorders (NMD). Sniff nasal inspiratory pressure (SNIP), a test of inspiratory muscle strength, is easier to perform for many NMD patients than the more commonly used determination of maximum inspiratory pressure measured at the mouth (MIP). However, due to an inconsistent approach in the literature, the optimal technique to perform the SNIP maneuver is unclear. Therefore, we systematically evaluated the impact of performing the maneuver with nostril contralateral to the pressure-sensing probe open (SNIPOP) versus closed (SNIPCL), on determination of inspiratory muscle strength in NMD patients as well as control subjects with normal respiratory muscle function.. NMD patients (n = 52) and control subjects without respiratory dysfunction (n = 52) were studied. SNIPOP, SNIPCL, and MIP were measured during the same session and compared using ANOVA. Agreement and bias were assessed with intraclass correlation coefficients (ICC) and Bland-Altman plots.. Mean MIP values were 58.2 and 94.0 cmH2O in NMD and control subjects, respectively (p<0.001). SNIPCL was greater than SNIPOP in NMD (51.9 ±31.0 vs. 36.9 ±25.4 cmH2O; p<0.001) as well as in controls (89.2 ±28.1 vs. 69.2 ±29.2 cmH2O; p<0.001). In both populations, the ICC between MIP and SNIPCL (NMD = 0.78, controls = 0.35) was higher than for MIP and SNIPOP (NMD = 0.53, controls = 0.06). In addition, SNIPCL was more often able to exclude inspiratory muscle weakness than SNIPOP.. SNIPCL values are systematically higher than SNIPOP in both normal subjects and NMD patients. Therefore, SNIPCL is a useful complementary test for ruling out inspiratory muscle weakness in individuals with low MIP values.

Topics: Adult; Aged; Female; Humans; Male; Maximal Respiratory Pressures; Middle Aged; Neuromuscular Diseases; Nose; Respiratory Muscles; Smell

The sniff nasal inspiratory pressure (SNIP) consists in the measurement of pressure through an occluded nostril during sniffs performed through the controlateral nostril. It is an accurate and noninvasive approximation of esophageal pressure swing during sniff maneuvers. However SNIP can underestimate esophageal pressure swing in subjects with nasal obstruction, patients with chronic obstructive pulmonary disease and severe neuromuscular patients. Nevertheless, since SNIP maneuver has predicted normal values, is noninvasive and is easier to perform than maximal inspiratory pressure (MIP) maneuver, it could be considered as the first simple test to use in order to assess inspiratory muscle weakness. In addition, because it is as reproducible as MIP, it can be suitable to follow inspiratory muscle function in chronic neuromuscular patients. Because, of the important limit of agreement between SNIP and MIP, these two methods are not interchangeable but complementary.

Topics: Forced Expiratory Volume; Humans; Inhalation; Neuromuscular Diseases; Nose; Predictive Value of Tests; Pulmonary Disease, Chronic Obstructive

This article evaluates the long-term clinical and physiologic effects of nocturnal nasal intermittent positive-pressure ventilation (NIPPV) in patients with neuromuscular disease.. Before and after 18 +/- 2 months of NIPPV, we measured during the daytime arterial blood gases, lung mechanics, and respiratory muscle strength in 8 patients (51 +/- 5 years; mean +/- SEM). Sleep parameters were also evaluated at 10 +/- 2 months.. All patients tolerated NIPPV and none required hospitalization during follow-up. After NIPPV, daytime arterial PO2 increased (71 +/- 4 to 81 +/- 2 mm Hg; p < 0.05) and arterial PCO2 decreased (46 +/- 3 to 41 +/- 1 mm Hg; p < 0.05). The change of PaO2 after NIPPV was related to its baseline value (r2 = 0.78, p < 0.05). Vital capacity (50 +/- 6% predicted), total lung capacity (63 +/- 4% predicted), alveolar-arterial oxygen gradient (20 +/- 3 mm Hg), and maximal inspiratory (39 +/- 9% predicted) or expiratory (32 +/- 5% predicted) pressures did not change after NIPPV. The apnea-hypopnea index fell from 22 +/- 6 to 1 +/- 1 (p < 0.05), and both sleep architecture and sleep efficiency (from 59 +/- 8% to 83 +/- 5%; p < 0.05) were enhanced. The time spent with an arterial oxygen saturation (SaO2) value below 90% decreased from 160 +/- 53 min to 8 +/- 4 min (p < 0.05). Mean (88 +/- 3 to 95 +/- 1%; p < 0.05) and minimal nocturnal SaO2 (67 +/- 5 to 89 +/- 1%; p < 0.001) improved after NIPPV.. In patients with neuromuscular disease, long-term NIPPV is well tolerated and easy to implement clinically. In these patients, long-term NIPPV improves daytime arterial blood gas values and sleep-disordered breathing. However, it does not modify lung mechanics or respiratory muscle strength.

Topics: Adult; Aged; Carbon Dioxide; Circadian Rhythm; Female; Follow-Up Studies; Humans; Intermittent Positive-Pressure Ventilation; Longitudinal Studies; Lung; Male; Middle Aged; Muscle Contraction; Neuromuscular Diseases; Nose; Oxygen; Partial Pressure; Pressure; Pulmonary Diffusing Capacity; Pulmonary Ventilation; Respiratory Mechanics; Respiratory Muscles; Sleep; Sleep Apnea Syndromes; Total Lung Capacity; Vital Capacity

The measurement of oesophageal pressure during maximal sniffs (Poes sniff) is useful to assess inspiratory muscle strength. The aim of this study was to develop a noninvasive test of inspiratory muscle strength based on the sniff manoeuvre. The sniff nasal inspiratory pressure (SNIP) was measured through a plug occluding one nostril during sniffs performed through the contralateral nostril. In 10 normal subjects and in 12 patients with neuromuscular or skeletal disorders, the SNIP reliably reflected the Poes sniff. Nasal mucosa congestion was induced in four normal subjects by nebulization of increasing doses of histamine. The SNIP accurately reflected Poes sniff when nasal congestion was moderate, but failed to do so when congestion was severe. Reference values of SNIP were established in a group of 160 healthy subjects aged 20-80 years. For both men and women, SNIP was negatively correlated with age, and was similar in the sitting and in the supine positions. SNIP was higher than maximal inspiratory pressure (P1 max) in most subjects, but the wide limits of agreement showed that these two methods are not interchangeable but complementary. The SNIP represents a useful noninvasive test of inspiratory muscle strength.

Topics: Adult; Age Factors; Aged; Aged, 80 and over; Bone Diseases; Esophagus; Female; Histamine; Humans; Inspiratory Capacity; Male; Middle Aged; Nasal Mucosa; Nebulizers and Vaporizers; Neuromuscular Diseases; Nose; Nose Diseases; Posture; Pressure; Reference Values; Reproducibility of Results; Respiratory Muscles; Supine Position

The measurement of esophageal pressure during maximal sniffs (sniff Pes) has been shown useful to assess inspiratory muscle strength. The aim of this study was to validate a noninvasive method for estimating sniff Pes. The sniff nasal inspiratory pressure (SNIP) was measured through a plug occluding one nostril during sniffs performed through the contralateral nostril. Sniff Pes was simultaneously measured with an esophageal balloon. Ten normal subjects performed 338 sniffs of variable intensity. The correlation coefficient of SNIP and sniff Pes was 0.99 +/- 0.01 (p < 0.001). The ratio SNIP/sniff Pes was 0.91 (range, 0.82 to 0.99) and the mean difference between the two measures (SNIP - sniff Pes) was -4.56 cm H2O (-1.2 to -8.6 cm H2O). Twelve patients with neuromuscular or skeletal disorders performed 181 maximal sniffs. The correlation coefficient of SNIP and sniff Pes was 0.96 +/- 0.04 (p < 0.001). The ratio SNIP/sniff Pes was 0.93 (0.77 to 1.07) and the mean difference (SNIP - sniff Pes) was -4.66 cm H2O (+0.47 to -14.26 cm H2O). Nasal mucosal congestion was induced by nebulization of increasing doses of histamine in four normal subjects. The ratio SNIP/sniff Pes was 0.93 (0.72 to 1.02) when nasal peak flow was > 100 L/min, and 0.49 (0.36 to 0.57 L/min) when nasal peak flow fell below 100 L/min. We conclude that SNIP provides a reliable and noninvasive estimation of sniff Pes in normal subjects and in patients with neuromuscular or skeletal disorders. The validity of this method may by impaired by severe nasal congestion.

Topics: Adult; Aged; Esophagus; Female; Humans; Inhalation; Linear Models; Male; Middle Aged; Nasal Provocation Tests; Neuromuscular Diseases; Nose; Pressure; Reference Values; Respiratory Function Tests; Respiratory Muscles

Topics: Humans; Intermittent Positive-Pressure Ventilation; Neuromuscular Diseases; Nose; Respiratory Insufficiency; Tracheostomy

Severe nocturnal hypoxemia may occur in patients with respiratory muscle weakness caused by neuromuscular disorders. Negative pressure ventilators may be partially effective in these patients but can cause upper airway obstructive apneas. We examined the effectiveness of positive pressure ventilation through a nose mask in preventing nocturnal hypoxemia and compared it with negative pressure systems. We reasoned that nasal positive pressure would provide stability for the upper airway. Five patients with neuromuscular disorders underwent a series of all-night sleep studies under control conditions, negative pressure ventilation, and positive pressure ventilation through a comfortable nose mask. Sleep staging and respiratory variables were monitored during all studies. Daytime awake lung function, respiratory muscle strength, and arterial blood gases were also measured. The severe hypoxemia and hypercapnia that occurred under control conditions were prevented by positive pressure ventilation through a nose mask. Negative pressure ventilation improved NREM ventilation in all patients, but did not prevent severe oxyhemoglobin desaturation, which occurred during REM sleep. Negative pressure ventilation appears to contribute to upper airways obstruction during REM sleep as evidenced by cessation of air flow, reduced chest wall movements, falls in arterial oxyhemoglobin saturation, and hypercapnia. With treatment, daytime PaO2 improved from a mean of 70 to 83 mm Hg, and PaCO2 decreased from a mean of 61 to 46 mm Hg. We conclude that nasally applied positive pressure ventilation is a highly effective method of providing nocturnal assisted ventilation because it stabilizes the oropharyngeal airway.

Topics: Evaluation Studies as Topic; Humans; Hypercapnia; Hypoxia; Masks; Neuromuscular Diseases; Nose; Positive-Pressure Respiration; Respiratory Insufficiency; Sleep Apnea Syndromes; Sleep, REM