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  1. Article ; Online: Equipment options for cough augmentation, ventilation, and noninvasive interfaces in neuromuscular respiratory management.

    Boitano, Louis J

    Pediatrics

    2009  Volume 123 Suppl 4, Page(s) S226–30

    Abstract: This is a summary of the presentation on equipment options for cough augmentation, ventilation, and noninvasive interfaces in neuromuscular respiratory management presented as part of the program on pulmonary management of pediatric patients with ... ...

    Abstract This is a summary of the presentation on equipment options for cough augmentation, ventilation, and noninvasive interfaces in neuromuscular respiratory management presented as part of the program on pulmonary management of pediatric patients with neuromuscular disorders at the 30th annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008.
    MeSH term(s) Child ; Cough ; Equipment Design ; Humans ; Masks ; Mucociliary Clearance ; Neuromuscular Diseases/therapy ; Positive-Pressure Respiration/instrumentation ; Respiratory Insufficiency/therapy ; Respiratory Therapy/instrumentation ; Respiratory Therapy/methods ; Suction
    Language English
    Publishing date 2009-05
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 207677-9
    ISSN 1098-4275 ; 0031-4005
    ISSN (online) 1098-4275
    ISSN 0031-4005
    DOI 10.1542/peds.2008-2952F
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Managing the patient with neuromuscular disease and respiratory insufficiency.

    Boitano, Louis J

    Respiratory care

    2008  Volume 53, Issue 11, Page(s) 1434–1435

    MeSH term(s) Humans ; Neuromuscular Diseases/complications ; Neuromuscular Diseases/therapy ; Respiration, Artificial/methods ; Respiratory Insufficiency/etiology ; Respiratory Insufficiency/therapy ; Treatment Outcome
    Language English
    Publishing date 2008-11
    Publishing country United States
    Document type Comment ; Editorial
    ZDB-ID 603252-7
    ISSN 0020-1324 ; 0098-9142
    ISSN 0020-1324 ; 0098-9142
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Pulmonary issues in patients with chronic neuromuscular disease.

    Benditt, Joshua O / Boitano, Louis J

    American journal of respiratory and critical care medicine

    2013  Volume 187, Issue 10, Page(s) 1046–1055

    Abstract: Patients with chronic neuromuscular diseases such as spinal cord injury, amyotrophic lateral sclerosis, and muscular dystrophies experience respiratory complications that are cared for by the respiratory practitioner. An organized anatomical approach for ...

    Abstract Patients with chronic neuromuscular diseases such as spinal cord injury, amyotrophic lateral sclerosis, and muscular dystrophies experience respiratory complications that are cared for by the respiratory practitioner. An organized anatomical approach for evaluation and treatment is helpful to provide appropriate clinical care. Effective noninvasive strategies for management of hypoventilation, sleep-disordered breathing, and cough insufficiency are available for these patients.
    MeSH term(s) Chronic Disease ; Humans ; Neuromuscular Diseases/complications ; Respiration Disorders/complications ; Respiration Disorders/therapy ; Respiration, Artificial/methods
    Language English
    Publishing date 2013-05-15
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1180953-x
    ISSN 1535-4970 ; 0003-0805 ; 1073-449X
    ISSN (online) 1535-4970
    ISSN 0003-0805 ; 1073-449X
    DOI 10.1164/rccm.201210-1804CI
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Management of airway clearance in neuromuscular disease.

    Boitano, Louis J

    Respiratory care

    2006  Volume 51, Issue 8, Page(s) 913–22; discussion 922–4

    Abstract: The clearance of airway secretions from the lungs is normally supported by the mucociliary escalator and by cough. These protective mechanisms provide an effective means of pulmonary-hygiene maintenance in healthy individuals. Patients with neuromuscular ...

    Abstract The clearance of airway secretions from the lungs is normally supported by the mucociliary escalator and by cough. These protective mechanisms provide an effective means of pulmonary-hygiene maintenance in healthy individuals. Patients with neuromuscular disease that affects the respiratory pump (the muscles of breathing) can experience mild to profound limitation in both ventilation and cough. Neuromuscular respiratory insufficiency, when left untreated, can substantially impact quality of life and life expectancy. In most cases of neuromuscular disease, respiratory failure and pneumonia are the primary causes of death. Invasive mechanical ventilation and tracheal suctioning have been successfully used when needed to support respiratory insufficiency in this population. These modalities, though supportive, have been associated with substantial morbidity when used in patients with neuromuscular disease. The advent of noninvasive ventilation as a means of supporting chronic neuromuscular respiratory insufficiency has spurred the development of noninvasive cough-augmentation therapy to support airway clearance. Unfortunately, the need to support cough clearance is not always addressed, and few guidelines for the management of cough insufficiency have existed until relatively recently. An understanding of neuromuscular respiratory pathophysiology and the modes of effective noninvasive cough support are key in the evaluation and management of neuromuscular diseases. This review is meant to provide a basic understanding of cough mechanics, and the pathophysiology and management of neuromuscular cough insufficiency.
    MeSH term(s) Combined Modality Therapy ; Cough/physiopathology ; Drainage/methods ; Humans ; Mucus ; Neuromuscular Diseases/physiopathology ; Neuromuscular Diseases/therapy
    Language English
    Publishing date 2006-08
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 603252-7
    ISSN 0020-1324 ; 0098-9142
    ISSN 0020-1324 ; 0098-9142
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  5. Article: Not to invade: a better strategy?

    Boitano, Louis J / Benditt, Joshua O

    Respiratory care

    2011  Volume 56, Issue 6, Page(s) 878

    MeSH term(s) Cause of Death ; Cough ; Humans ; Intubation, Intratracheal ; Muscular Dystrophy, Duchenne/mortality ; Muscular Dystrophy, Duchenne/physiopathology ; Muscular Dystrophy, Duchenne/therapy ; Respiration, Artificial/methods ; Tracheotomy
    Language English
    Publishing date 2011-06
    Publishing country United States
    Document type Comment ; Editorial
    ZDB-ID 603252-7
    ISSN 0020-1324 ; 0098-9142
    ISSN 0020-1324 ; 0098-9142
    DOI 10.4187/respcare.01337
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: An evaluation of home volume ventilators that support open-circuit mouthpiece ventilation.

    Boitano, Louis J / Benditt, Joshua O

    Respiratory care

    2005  Volume 50, Issue 11, Page(s) 1457–1461

    Abstract: Background: Open-circuit mouthpiece ventilation (MPV) is a form of noninvasive ventilation that can be used to provide portable daytime ventilatory support for neuromuscular patients with chronic respiratory failure. MPV has been reported to reduce the ... ...

    Abstract Background: Open-circuit mouthpiece ventilation (MPV) is a form of noninvasive ventilation that can be used to provide portable daytime ventilatory support for neuromuscular patients with chronic respiratory failure. MPV has been reported to reduce the risk of respiratory infection due to tracheostomy, and to improve cough and voice function and patient quality of life. Despite these potential benefits, mouthpiece ventilation is not widely used. This may be due in part to the fact that little information is available as to which ventilators can support this application.
    Objective: To determine which volume-cycled portable home ventilators currently available in the United States will support MPV, and what peak inspiratory flow rates create adequate circuit pressure to prevent low-pressure alarming.
    Methods: We used a commercially available MPV breathing circuit with a set tidal volume range of 500\N1,000 mL with each of 8 ventilators currently available in the United States.
    Results: Six of the 8 ventilators supported MPV: Respironics Lifecare PLV-100 and PLV Continuum, Mallinckrodt Achieva PSO2, Pulmonetics LTV800, Newport HT50, and Uni-Vent Eagle 754.
    MeSH term(s) Equipment Design ; Home Care Services ; Humans ; Neuromuscular Diseases/physiopathology ; Quality of Life ; Tidal Volume ; Ventilators, Mechanical
    Language English
    Publishing date 2005-11
    Publishing country United States
    Document type Evaluation Studies ; Journal Article
    ZDB-ID 603252-7
    ISSN 0020-1324 ; 0098-9142
    ISSN 0020-1324 ; 0098-9142
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  7. Article: Limits of effective cough-augmentation techniques in patients with neuromuscular disease.

    Toussaint, Michel / Boitano, Louis J / Gathot, Vincent / Steens, Marc / Soudon, Philippe

    Respiratory care

    2009  Volume 54, Issue 3, Page(s) 359–366

    Abstract: Background: Manual and mechanical cough-augmentation techniques can improve peak cough flow (PCF) in patients with respiratory insufficiency caused by neuromuscular disease.: Methods: We studied cough-augmentation techniques in 179 clinically stable ... ...

    Abstract Background: Manual and mechanical cough-augmentation techniques can improve peak cough flow (PCF) in patients with respiratory insufficiency caused by neuromuscular disease.
    Methods: We studied cough-augmentation techniques in 179 clinically stable patients with various neuromuscular diseases. We measured vital capacity (VC), maximum expiratory pressure (MEP), and PCF, with and without 3 cough-augmentation techniques: manually assisted cough (MAC); breath-stacking (in a subgroup of 60 patients receiving noninvasive mechanical ventilation); and breath-stacking in combination with MAC (also in the 60-patient subgroup). We analyzed the data with the receiver operating characteristic (ROC), to predict the lower limits (assisted PCF > or = 180 L/min) and upper limits (assisted PCF < unassisted PCF) of effectiveness of the 3 cough-augmentation techniques.
    Results: The lower limit of effective assisted cough with MAC, breath-stacking, and breath-stacking plus MAC was best predicted by VC > 1,030 mL (ROC 0.86, P < .001), VC > 558 mL (ROC 0.92, P < .001), and VC > 340 mL (ROC 0.90, P < .001). The upper limit of effective MAC was best predicted by MEP > 34 cm H(2)O (ROC 0.89, P < .001), whereas the ROC prediction of the upper limit of effective cough with breath-stacking and with breath-stacking plus MAC was not better than random. With each of the cough-augmentation techniques the benefits decreased linearly with increasing MEP and VC (P < .001). Compared to MAC and breath-stacking alone, breath-stacking plus MAC best improved unassisted PCF (P < .001).
    Conclusions: In clinically stable patients with neuromuscular diseases, the effectiveness of cough-augmentation techniques can be predicted with measurements of maximum respiratory capacity. Patients with VC > 340 mL and MEP < 34 cm H(2)O would optimally benefit from the combination of breath-stacking plus manually assisted cough to improve PCF to > 180 L/min.
    MeSH term(s) Adult ; Cough ; Cross-Sectional Studies ; Female ; Humans ; Male ; Neuromuscular Diseases/physiopathology ; Peak Expiratory Flow Rate/physiology ; Pressure ; Prospective Studies ; ROC Curve ; Respiratory Muscles/physiopathology ; Respiratory Therapy/methods ; Treatment Outcome ; Vital Capacity/physiology
    Language English
    Publishing date 2009-03
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 603252-7
    ISSN 0020-1324 ; 0098-9142
    ISSN 0020-1324 ; 0098-9142
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  8. Article: American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation.

    Birnkrant, David J / Panitch, Howard B / Benditt, Joshua O / Boitano, Louis J / Carter, Edward R / Cwik, Valerie A / Finder, Jonathan D / Iannaccone, Susan T / Jacobson, Lawrence E / Kohn, Gary L / Motoyama, Etsuro K / Moxley, Richard T / Schroth, Mary K / Sharma, Girish D / Sussman, Michael D

    Chest

    2007  Volume 132, Issue 6, Page(s) 1977–1986

    Abstract: This statement on the management of patients with Duchenne muscular dystrophy (DMD) undergoing procedural sedation or general anesthesia represents the consensus opinion of a multidisciplinary panel convened under the auspices of the American College of ... ...

    Abstract This statement on the management of patients with Duchenne muscular dystrophy (DMD) undergoing procedural sedation or general anesthesia represents the consensus opinion of a multidisciplinary panel convened under the auspices of the American College of Chest Physicians. Expert recommendations on this subject are needed for several reasons. First, patients with DMD have an increased risk of complications when they undergo sedation or general anesthesia. In addition, due to improved cardiopulmonary therapies, patients with DMD are experiencing an unprecedented duration of survival. As a result, it is more common for them to require procedures involving sedation or general anesthesia. The risks related to anesthesia and sedation for DMD patients include potentially fatal reactions to inhaled anesthetics and certain muscle relaxants, upper airway obstruction, hypoventilation, atelectasis, congestive heart failure, cardiac dysrhythmias, respiratory failure, and difficulty weaning from mechanical ventilation. This statement includes advice regarding the highly interrelated areas of respiratory, cardiac, GI, and anesthetic management of patients with DMD undergoing general anesthesia or procedural sedation. The statement is intended to aid clinicians involved in the care of patients with DMD and to be a resource for other stakeholders in this field, including patients and their families. It is an up-to-date summary of medical literature regarding this topic and identifies areas in need of future research.
    MeSH term(s) Anesthesia, General/adverse effects ; Anesthesia, General/standards ; Conscious Sedation/adverse effects ; Conscious Sedation/standards ; Humans ; Muscular Dystrophy, Duchenne/complications ; Muscular Dystrophy, Duchenne/physiopathology ; Respiration, Artificial/standards ; Risk Factors
    Language English
    Publishing date 2007-12
    Publishing country United States
    Document type Journal Article ; Practice Guideline ; Research Support, Non-U.S. Gov't
    ZDB-ID 1032552-9
    ISSN 1931-3543 ; 0012-3692
    ISSN (online) 1931-3543
    ISSN 0012-3692
    DOI 10.1378/chest.07-0458
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  9. Article: Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials.

    Standaert, Thomas A / Boitano, Louis / Emerson, Julia / Milgram, Laura J H / Konstan, Michael W / Hunter, Janice / Berclaz, Pierre-Yves / Brass, Lois / Zeitlin, Pamela L / Hammond, Keith / Davies, Zoe / Foy, Carla / Noone, Peadar G / Knowles, Michael R

    Pediatric pulmonology

    2004  Volume 37, Issue 5, Page(s) 385–392

    Abstract: Patients with cystic fibrosis (CF) can be discriminated from healthy subjects by measurement of the nasal potential difference, which has become a useful outcome measure for therapies directed toward correcting defective electrolyte transport in CF. A ... ...

    Abstract Patients with cystic fibrosis (CF) can be discriminated from healthy subjects by measurement of the nasal potential difference, which has become a useful outcome measure for therapies directed toward correcting defective electrolyte transport in CF. A standard operating procedure was developed by a CF Foundation clinical trials network, to be followed by all sites performing collaborative studies. Key variables in the measurement included type of voltmeter, exploring probe, reference electrodes, and solutions used to assess both sodium transport and chloride conductance. Eight sites submitted data on 3-8 normal and 4-5 CF subjects. Baseline voltage, an index of sodium transport, was -18.2 +/- 8.3 mV (mean +/- SD) for normals, and -45.3 +/- 11.4 mV for CF patients. There was no CFTR-mediated chloride secretion in CF subjects, as evidenced by the lack of response to perfusion with zero chloride + beta agonist solutions (+3.2 +/- 3.5 mV) vs. that in normals (-23.7 +/- 10.2 mV). The standardized nasal potential difference measurement minimizes variability between operators and study sites. Valid and consistent results can be attained with trained operators and attention to technical details. These data demonstrate the procedure to be sufficient for multicenter studies in the CF Foundation network.
    MeSH term(s) Adult ; Clinical Protocols ; Clinical Trials as Topic ; Cystic Fibrosis/diagnosis ; Feasibility Studies ; Female ; Humans ; Ion Transport ; Male ; Membrane Potentials ; Nasal Mucosa/physiopathology ; Outcome Assessment (Health Care) ; Reagent Kits, Diagnostic ; Sodium Chloride/metabolism
    Chemical Substances Reagent Kits, Diagnostic ; Sodium Chloride (451W47IQ8X)
    Language English
    Publishing date 2004-05
    Publishing country United States
    Document type Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.10448
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