Noninvasive ventilation in children with spinal muscular atrophy types 1 and 2

American Journal of Physical Medicine & Rehabilitation
Albino PetroneRenato Cutrera

Abstract

Our aim was to assess the efficacy of noninvasive ventilation (NIV) for the treatment of thoracoabdominal asynchrony during sleep in children with spinal muscular atrophy (SMA) types 1 and 2. Nine subjects underwent assessment for sleep apnea/hypopnea index (AHI), mean oxyhemoglobin saturation (SpO2), oxygen desaturation index, transcutaneous carbon dioxide tension (tcpCO2), and mean phase angle during sleep as a measure of thoracoabdominal coordination. A second sleep study was performed with use of NIV. The nine patients (7 mos of age, range 2-33) had a baseline AHI of 2.1 events per hour (range 0.5-55.8), oxygen desaturation index of 3.7 events per hour (range 1.6-46.1), mean tcpCO2 of 46 mm Hg (range 37-60), and phase angle of 127 degrees (range 72.7-151.7). Comparing baseline and NIV sleep studies, we found significant improvement in oxygen desaturation index (P < 0.010), mean tcpCO2 (P < 0.001), and phase angle (P < 0.001). For five patients, phase-angle improvement became significant when using high-span bilevel positive airway pressure (PAP). NIV improved sleep breathing parameters and thoracoabdominal coordination during sleep in SMA types 1 and 2. Phase-angle improvement correlated with bilevel PAP pressures. Phase an...Continue Reading

References

Nov 1, 1992·The American Review of Respiratory Disease·C L MarcusS L Ward
Sep 1, 1990·The American Review of Respiratory Disease·Yakov SivanC J L Newth
Jan 1, 1989·Journal of Applied Physiology·M A SacknerB Krieger
Mar 1, 1967·Journal of Applied Physiology·K Konno, J Mead
Mar 1, 1995·Archives of Physical Medicine and Rehabilitation·J R Bach, T G Wang
Aug 1, 1996·The Journal of Applied Bacteriology·E A DaviesM R Adams
Apr 5, 2001·Journal of Biomedical Science·M Z HaiderL Reynold
Oct 5, 2001·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·A K KroksmarkM Tulinius
Jul 12, 2002·Pediatric Pulmonology·John R BachBrian Weaver
Sep 23, 2003·Archives of Disease in Childhood·M K M Hardart, R D Truog
Sep 26, 2003·American Journal of Physical Medicine & Rehabilitation·John R Bach, Carlo Bianchi
Oct 30, 2003·The European Respiratory Journal·Uwe MelliesHelmut Teschler
Feb 20, 2004·Journal of Obstetric, Gynecologic, and Neonatal Nursing : JOGNN·Jennifer A MarkowitzKenneth H Fischbeck
Sep 15, 2004·Chest·Christine IoosBrigitte Estournet-Mathiaud
Sep 6, 2005·American Journal of Physical Medicine & Rehabilitation·Maria Beatrice Chiarini TestaRenato Cutrera

Citations

Oct 27, 2012·Neurologic Clinics·Sushanth BhatSudhansu Chokroverty
Feb 2, 2010·Paediatric Respiratory Reviews·Raanan Arens, Hiren Muzumdar
May 5, 2009·Paediatric Respiratory Reviews·J Hammer, C J L Newth
May 25, 2015·Sleep & Breathing = Schlaf & Atmung·Karen T NozoeMonica L Andersen
Dec 19, 2016·Chest·Howard Panitch
Dec 9, 2017·The European Respiratory Journal·Athanasios G KaditisStijn Verhulst
Jun 12, 2020·Pediatric Pulmonology·Athanasios G KaditisChristina Kanaka-Gantenbein
Feb 23, 2007·American Journal of Physical Medicine & Rehabilitation·John R Bach
Feb 28, 2018·Frontiers in Pediatrics·Prabhjot K BediJoanna E MacLean
Jan 18, 2020·Pulmonary Medicine·Athanasios VoulgarisPaschalis Steiropoulos
Oct 27, 2020·Frontiers in Pediatrics·Alessandro OnofriHui-Leng Tan

Related Concepts

Oxyhemoglobin
Positive End-Expiratory Pressure
Sleep, Slow-Wave
Hypersomnia With Periodic Respiration
Muscular Atrophy, Spinal, Type II
Polysomnography

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Lipidomics & Rhinovirus Infection

Lipidomics can be used to examine the lipid species involved with pathogenic conditions, such as viral associated inflammation. Discovered the latest research on Lipidomics & Rhinovirus Infection.

Spatio-Temporal Regulation of DNA Repair

DNA repair is a complex process regulated by several different classes of enzymes, including ligases, endonucleases, and polymerases. This feed focuses on the spatial and temporal regulation that accompanies DNA damage signaling and repair enzymes and processes.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Torsion Dystonia

Torsion dystonia is a movement disorder characterized by loss of control of voluntary movements appearing as sustained muscle contractions and/or abnormal postures. Here is the latest research.

Archaeal RNA Polymerase

Archaeal RNA polymerases are most similar to eukaryotic RNA polymerase II but require the support of only two archaeal general transcription factors, TBP (TATA-box binding protein) and TFB (archaeal homologue of the eukaryotic general transcription factor TFIIB) to initiate basal transcription. Here is the latest research on archaeal RNA polymerases.

Alzheimer's Disease: MS4A

Variants within the membrane-spanning 4-domains subfamily A (MS4A) gene cluster have recently been implicated in Alzheimer's disease in genome-wide association studies. Here is the latest research on Alzheimer's disease and MS4A.

Central Pontine Myelinolysis

Central Pontine Myelinolysis is a neurologic disorder caused most frequently by rapid correction of hyponatremia and is characterized by demyelination that affects the central portion of the base of the pons. Here is the latest research on this disease.