1 40 1 Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month Backlog URL Address <a href="http://doi.org/10.1212/wnl.57.7.1290" target="_blank" rel="noreferrer">http://doi.org/10.1212/wnl.57.7.1290</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Sleep-disordered breathing and respiratory failure in acid maltase deficiency Publisher An entity responsible for making the resource available Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2001 Subject The topic of the resource Child; Humans; Adult; Middle Aged; Sensitivity and Specificity; Predictive Value of Tests; adolescent; Preschool; Q3 Literature Search; REM; Sleep; Polysomnography; Diaphragm/physiopathology; Glycogen Storage Disease Type II/complications; Respiratory Insufficiency/diagnosis/etiology/physiopathology; Sleep Apnea Syndromes/diagnosis/etiology/physiopathology; Vital Capacity Creator An entity primarily responsible for making the resource Mellies U; Ragette R; Schwake C; et al Description An account of the resource BACKGROUND: Sleep-disordered breathing (SDB) and respiratory failure (RF) are complications of acid maltase deficiency (AMD), a rare hereditary myopathy. OBJECTIVE: To define the relationship between lung and respiratory muscle function, to establish incidence and patterns of SDB, and to determine daytime predictors of SDB. METHODS: Sitting and supine lung and respiratory muscle function tests were obtained in 27 subjects with juvenile and adult AMD (aged 39 +/- 19 years) and compared with outcomes of polysomnography. RESULTS: Ventilatory restriction was present in 17/27 subjects. Inspiratory vital capacity (IVC) correlated (p 50 mm Hg) first in REM sleep, then in non-REM sleep (p < 0.005). SDB was predicted by DW (sensitivity 80%, specificity 86%) and nocturnal hypoventilation by IVC < 40% (sensitivity 80%, specificity 93%). Noninvasive ventilation, instituted for daytime respiratory failure or nocturnal hypoventilation, normalized daytime and nocturnal gas exchange (p < 0.005). CONCLUSION: Vital capacity correlates with respiratory muscle function in AMD. Diaphragm weakness is the major cause of SDB and RF. SDB and nocturnal hypoventilation are predictable from daytime function tests. 2001 Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1212/wnl.57.7.1290" target="_blank" rel="noreferrer">10.1212/wnl.57.7.1290</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). Type The nature or genre of the resource Journal Article 2001 Adolescent Adult Backlog Child Diaphragm/physiopathology et al Glycogen Storage Disease Type II/complications Humans Journal Article Mellies U Middle Aged Neurology Polysomnography Predictive Value of Tests Preschool Q3 Scoping Review Results Ragette R REM Respiratory Insufficiency/diagnosis/etiology/physiopathology Schwake C Sensitivity and Specificity Sleep Sleep Apnea Syndromes/diagnosis/etiology/physiopathology Vital Capacity