Congenital muscular dystrophy with rigid spine syndrome (CMD-RSS): reversal of severe pulmonary hypertension and survival for 13 years using nocturnal bilevel positive airway pressure and oxygen

R. J. Farney, G. Elliott, J. M. Walker (Salt Lake City, United States Of America)

Source: Annual Congress 2005 - Noninvasive ventilation in chronic respiratory failure
Session: Noninvasive ventilation in chronic respiratory failure
Session type: Thematic Poster Session
Number: 630
Disease area: Pulmonary vascular diseases, Respiratory critical care, Sleep and breathing disorders

Congress or journal article abstract

Rating: 0
You must login to grade this presentation.

Share or cite this content

Citations should be made in the following way:
R. J. Farney, G. Elliott, J. M. Walker (Salt Lake City, United States Of America). Congenital muscular dystrophy with rigid spine syndrome (CMD-RSS): reversal of severe pulmonary hypertension and survival for 13 years using nocturnal bilevel positive airway pressure and oxygen. Eur Respir J 2005; 26: Suppl. 49, 630

You must login to share this Presentation/Article on Twitter, Facebook, LinkedIn or by email.

Member's Comments

No comment yet.
You must Login to comment this presentation.


Related content which might interest you:
Noninvasive ventilation for chest wall and neuromuscular disorders
Source: Eur Respir J 2002; 20: 480-487
Year: 2002



Physiological differences in patients with kyphoscoliosis, post tuberculosis sequelae and obesity hypoventilation syndrome when starting long-term noninvasive positive pressure ventilation (NPPV)
Source: Annual Congress 2008 - Noninvasive ventilation in the chronic setting
Year: 2008

Noninvasive mechanical ventilation in acute exacerbation of restrictive thoracic disease
Source: ISSN=1025-448x, ISBN=1-904097-17-0, page=33
Year: 2001

Long-term echocardiographic changes with positive airway pressure therapy in obesity hypoventilation syndrome
Source: Virtual Congress 2020 – Burden and predictive factors for chronic lung diseases and comorbidity
Year: 2020


Effects of continuous positive airway pressure (nCPAP) on pulmonary hemodynamics in COPD patients with overlap syndrome
Source: Eur Respir J 2004; 24: Suppl. 48, 568s
Year: 2004

Comparison of exercise capacity, pulmonary functions, respiratory and peripheral muscle strength between patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome
Source: Annual Congress 2012 - Assessment of physical activity, exercise, muscle function and clinical characteristics as outcomes in physiotherapy and rehabilitation
Year: 2012

Assessment of inspiratory muscular pressure in patients with chronic obstructive pulmonary disease
Source: Eur Respir J 2002; 20: Suppl. 38, 351s
Year: 2002

Efficacy of continous positive airway pressure (CPAP) in the treatment of the obesity hypoventilation syndrome
Source: Eur Respir J 2005; 26: Suppl. 49, 726s
Year: 2005

NIV and chronic respiratory failure secondary to restrictive thoracic disorders (obesity excluded)
Source: Eur Respir Mon; 2008: 41: 240–250
Year: 2008

Influence of continuous positive airway pressure therapy on endothelial function and blood pressure in patients with arterial hypertension and obstructive sleep apnoea syndrome
Source: Eur Respir J 2006; 28: Suppl. 50, 814s
Year: 2006

Pulmonary arterial hypertension masquerading as severe refractory asthma
Source: Eur Respir J 2008; 32: 513-516
Year: 2008



Efficacy of bi-level positive airways pressure ventilation for treatment of obstructive sleep apnea/hypoventilation syndrome in Prader-Willi syndrome (PWS)
Source: Eur Respir J 2005; 26: Suppl. 49, 85s
Year: 2005

Echocardiographic changes with positive airway pressure in obesity hypoventilation syndrome. Pickwick study
Source: International Congress 2017 – CPAP therapy
Year: 2017

Does bi-level positive airway pressure affect heart rate and pulse transit time in children with spinal muscular atrophy?
Source: Annual Congress 2009 - Noninvasive ventilation in chronic respiratory failure
Year: 2009

Sniff nasal inspiratory pressure in children with muscular, chest wall or lung disease
Source: Eur Respir J 2009; 33: 113-117
Year: 2009



Continuos positive airway pressure by helmet in exacerbation of idiopathic pulmonary fibrosis
Source: Annual Congress 2013 –Mechanical ventilation and weaning
Year: 2013


Continuous positive airway pressure or NIV in patients with acute and chronic obesity hypoventilation syndrome?
Source: Sleep and Breathing Conference 2021
Year: 2021


Comparison of arterial and venous blood gases in cases with obesity hypoventilation syndrome and neuromuscular disease
Source: International Congress 2018 – Management of chronic cough and respiratory muscle dysfunction
Year: 2018


High-span bi-level positive pressure ventilation for treatment of thoraco-abdominal asynchrony in children with spinal muscular atrophy type 1 and 2
Source: Eur Respir J 2007; 30: Suppl. 51, 320s
Year: 2007

Falls in blood pressure in patients with obstructive sleep apnea syndrome after long-term nasal continuous positive airway pressure treatment
Source: Eur Respir J 2005; 26: Suppl. 49, 38s
Year: 2005