Case 27. Storage Wars

Thomas Gustafson; Snigdha Pusalavidyasagar

doi:10.1007/978-3-031-18374-4_27

Case 27. Storage Wars

Thomas Gustafson, Snigdha Pusalavidyasagar

Research output: Chapter in Book/Report/Conference proceeding › Chapter

Abstract

A 61-year-old female presented with a long history of difficulty maintaining sleep, muscle weakness, morning headaches, orthopnea and daytime sleepiness. She was hospitalized for acute hypoxic/hypercapnic respiratory failure, required initial invasive followed by non-invasive ventilation (NIV) with supplemental oxygen. Muscle biopsy was positive for glycogen storage disease type II, and patient was diagnosed with late-onset Pompe disease. Sleep related breathing disorder was treated with Bi-level PAP device and oxygen for few years. Gradual worsening of pulmonary function, necessitated switch in treatment with NIV set in volume assured pressure support mode due to high likelihood of eventual need for on-demand ventilation.

Original language	English (US)
Title of host publication	Sleepless and Sleepy
Subtitle of host publication	50 Challenging Sleep Medicine Cases
Editors	Alcibiades Rodriguez
Publisher	Springer International Publishing
Pages	119-122
Number of pages	4
ISBN (Electronic)	9783031183744
ISBN (Print)	9783031183737
DOIs	https://doi.org/10.1007/978-3-031-18374-4_27
State	Published - Jan 1 2023

Bibliographical note

Publisher Copyright:
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2023.

Keywords

Hypercapnia
Hypoxemia
Late onset Pompe disease
Non-invasive ventilation
Sleep related breathing disorder

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10.1007/978-3-031-18374-4_27

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abstract = "A 61-year-old female presented with a long history of difficulty maintaining sleep, muscle weakness, morning headaches, orthopnea and daytime sleepiness. She was hospitalized for acute hypoxic/hypercapnic respiratory failure, required initial invasive followed by non-invasive ventilation (NIV) with supplemental oxygen. Muscle biopsy was positive for glycogen storage disease type II, and patient was diagnosed with late-onset Pompe disease. Sleep related breathing disorder was treated with Bi-level PAP device and oxygen for few years. Gradual worsening of pulmonary function, necessitated switch in treatment with NIV set in volume assured pressure support mode due to high likelihood of eventual need for on-demand ventilation.",

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AU - Pusalavidyasagar, Snigdha

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KW - Hypoxemia

KW - Late onset Pompe disease

KW - Non-invasive ventilation

KW - Sleep related breathing disorder

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Case 27. Storage Wars

Abstract

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