Nocturnal hypoventilation: predictors and outcomes in childhood progressive neuromuscular disease

Katz, Sherri L.; Gaboury, Isabelle; Keilty, Krista; Banwell, Brenda; Vajsar, Jiri; Anderson, Peter; Ni, Andy; MacLusky, Ian

doi:10.1136/adc.2010.182709

Cited by 67 publications

(68 citation statements)

References 34 publications

(31 reference statements)

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“…Cependant, les TRS et l'insuffisance respiratoire nocturne peuvent se manifester de manière insidieuse, c'est-à-dire en l'absence de tout symptôme clinique [7][8][9]. Plusieurs études ont recherché un lien entre la pré-sence et/ou la sévérité des symptômes cliniques diurnes et les TRS, une hypoxémie et/ou une hypercapnie nocturnes.…”

Section: Relation Entre Trs Et Symptômes Cliniquesquestionnairesunclassified

“…Parmi plus d'une vingtaine d'études concernant des populations d'enfants/adolescents avec une DMD, une SMA ou diverses MNM, on retiendra l'étude de Toussaint et al [17] qui a montré chez 114 adolescents et jeunes adultes avec DMD que la capacité vitale s'avère utile pour détecter une hypercapnie nocturne, bien qu'avec une faible spécificité. La capacité vitale (en position assise ou couchée) est d'ailleurs l'un des paramètres les mieux corrélés aux gaz du sang nocturnes [7,11,17,18] ou aux TRS [18]. Seule l'étude de Ragette et al [18] s'est intéressée à la variation de la capacité vitale de la position assise à la position couchée (CV) -qui indique une faiblesse diaphragmatique lorsque le CV est supérieur à 25 % -et a montré que la faiblesse diaphragmatique représentait un facteur de risque important pour les TRS et l'insuffisance respiratoire nocturne et diurne.…”

Section: Relation Entre Trs Et Fonction Respiratoire-muscles Respiratunclassified

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Les explorations des muscles respiratoires sont-elles utiles pour poser l’indication d’une étude du sommeil chez l’enfant neuromusculaire ?

Khirani

Ramirez²,

Olmo-Arroyo

et al. 2015

Med Sci (Paris)

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Section: Relation Entre Trs Et Symptômes Cliniquesquestionnairesunclassified

Section: Relation Entre Trs Et Fonction Respiratoire-muscles Respiratunclassified

Les explorations des muscles respiratoires sont-elles utiles pour poser l’indication d’une étude du sommeil chez l’enfant neuromusculaire ?

Khirani

Ramirez²,

Olmo-Arroyo

et al. 2015

Med Sci (Paris)

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“…Compensatory tachypnea with small tidal volumes may cause lung atelectasis with a consequent reduction of the compliance of the respiratory system and increase the mechanical load on already weakened respiratory muscles (6). The final result is alveolar hypoventilation with increased partial arterial pressure of carbon dioxide (PaCO2) (7). These mechanisms are the main responsible for hypoxemic and hypercapnic respiratory failure during NMD (8).…”

Section: Muscular Weaknessmentioning

confidence: 99%

Management of patients with neuromuscular disorders and acute respiratory failure

Antonaglia¹,

Garuti²,

Torregiani³

2014

SOB

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showed in term of lower mortality, treatment failure, duration of ICU in several studies. Home mechanical noninvasive ventilation and MAC may result dramatic reduction in the need for hospitalization and a prolongation of life expectancy of these patients. To avoid the progression of respiratory failure an early identification of respiratory deterioration is needed, as such as prevention of acute episodes. So, especially in the progressive diseases, is very important to monitor respiratory status with periodic lung function tests. Any respiratory tract infection in neuromuscular disease could trigger acute respiratory failure so it is mandatory take any aggressive measures to prevent and treat this complication. Some of principal preventive measures include vaccination against Pneumococcus Pn. and influenza virus, early antibiotic treatment when bacterial infection is suspected, chest physiotherapy for removal airway secretions or mechanical assisted maneuvers to increase cough efficiency. In conclusion, a proactive, preventive approach is the key for optimal management acute respiratory problems in neuromuscular disorders.KEY WORDS: acute respiratory failure; neuromuscular disorders; noninvasive ventilation; mechanically assisted coughing; intensive care unit. IntroductionAcute respiratory failure (ARF) may be a life-threatening event during the course of several neuromuscular disorders (NMD). It can be the first manifestation of respiratory involvement or most frequently a worsening episode during chronic respiratory impairment. Deterioration of lung function during NMD varies in term of severity and time course according to different diseases. However, some general issues for the management of ARF in NMD should be kept in mind by the physicians to reduce time to recovery and to minimize the risks for the patient. Neuromuscular disorders affecting respiratory functionNeuromuscular disorders are numerous and represent a group of disease involving the muscle, the nerve or the neuromuscular junction ( SummaryAcute respiratory failure (ARF) is a common cause of morbidity and mortality in most advanced neuromuscular disorders (NMD). Deterioration of lung function during NMD may have different severity and time course. During slowly progressive neuromuscular disease an acute event can precipitate the respiratory failure, while rapidly progressive NMD may present as ARF at the onset of the disease. During ARF a patient with NMD may be affected by inspiratory muscles weakness responsible for decreased ventilation, expiratory muscles weakness responsible for altered coughing effectiveness, and bulbar involvement responsible for altered swallowing and airway protection. Neuromuscular disorders may also affect central control of breathing, sleep disorder, bones and joints deformities with consequent ventilatory dysfunction. Usually ARF in NMD is prompted by precipitating factors such as upper airways infections, pneumonia, atelectasis etc. It is important to distinguish respiratory failure due to an acute event or to a ...

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“…Though recent guidelines support the physical benefits of NIV for NMD (Hull et al, 2012), studies suggest that NIV may have both positive (Rousseau, Pietra, Blaya, & Catala, 2011) and negative impacts on psychosocial wellbeing and quality of life (QoL) (Katz et al, 2010;Mah, Thannhauser, McNeil, & Dewey, 2008) Similar to youth with other chronic conditions (Björquist, Nordmark, & Hallström, 2015;Sonneveld et al, 2013) , participants expected to become more autonomous in their health management. In relation to being able plan for the future, participants appreciated the efforts of specialist NMD doctors' to identify their individual diagnosis and the focus this gave.…”

Section: Theme I: Finely Balanced Healthmentioning

confidence: 99%

A fine balance and a shared learning journey: Exploring healthcare engagement through the experiences of youth with Neuromuscular Disorders

Travlos

Bulsara

Patman

et al. 2016

NRE

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Nocturnal hypoventilation: predictors and outcomes in childhood progressive neuromuscular disease

Abstract: 15% of subjects had clinically unsuspected NH, predicted by moderate pulmonary function test impairment and scoliosis. Over 1 year those with NH had increased gas trapping, decline of muscle strength and worse perception of health status, despite NPPV.

Cited by 67 publications

References 34 publications

Les explorations des muscles respiratoires sont-elles utiles pour poser l’indication d’une étude du sommeil chez l’enfant neuromusculaire ?

Les explorations des muscles respiratoires sont-elles utiles pour poser l’indication d’une étude du sommeil chez l’enfant neuromusculaire ?

Management of patients with neuromuscular disorders and acute respiratory failure

A fine balance and a shared learning journey: Exploring healthcare engagement through the experiences of youth with Neuromuscular Disorders

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