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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
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
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URL Address
<a href="http://doi.org/10.1097/00005792-198807000-00002" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/00005792-198807000-00002</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
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Respiratory complications of mucopolysaccharide storage disorders
Publisher
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Medicine
Date
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1988
Subject
The topic of the resource
Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Retrospective Studies; Sleep Apnea Syndromes/et [Etiology]; Mucopolysaccharidoses/co [Complications]; Respiratory Tract Diseases/et [Etiology]; Airway Obstruction/et [Etiology]; Anesthesia General/ae [Adverse Effects]; Cardiovascular Diseases/et [Etiology]; Intubation Intratracheal/ae [Adverse Effects]; Lung Diseases/et [Etiology]; Lung Diseases/pp [Physiopathology]; Mucopolysaccharidoses/mo [Mortality]; Mucopolysaccharidoses/pp [Physiopathology]; Respiratory Tract Diseases/pp [Physiopathology]; Respiratory Tract Diseases/ra [Radiography]; Spinal Diseases/et [Etiology]; Spinal Diseases/ra [Radiography]; breathing difficulties; MLII; MLIII; MPSI; MPSII; MPSVI; surgical interventions; pharmacologic interventions; tonsillectomy; adenoidectomy; tracheostomy; diuretics; theophylline; digoxin; beta adrenergic blockers; calcium antagonists; respiratory problems
Creator
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Semenza G L; Pyeritz R E
Description
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Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i) upper airway narrowing by hypertrophied tongue, tonsils, adenoids, and mucous membranes; (ii) lower airway narrowing by glycosaminoglycan deposition within the tracheobronchial mucosa; (iii) decreased thoracic dimensions due to scoliosis and thoracic hyperkyphosis; and (iv) decreased abdominal dimensions due to lumbar hyperlordosis, gibbus formation and hepatosplenomegaly. Cardiac and neurologic involvement, while present, did not play primary roles in the development of respiratory disease. The functional consequences of these findings included increased risk of developing: (i) respiratory tract infections; (ii) airway compromise during or after anesthesia or sedation; (iii) dyspnea on exertion; (iv) obstructive lung disease; (v) obstructive sleep apnea; and (vi) cor pulmonale. A management approach is presented which can reduce the morbidity and mortality experienced by these patients.
Identifier
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<a href="http://doi.org/10.1097/00005792-198807000-00002" target="_blank" rel="noreferrer noopener">10.1097/00005792-198807000-00002</a>
Rights
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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).
1988
adenoidectomy
Adolescent
Adult
Airway Obstruction/et [Etiology]
Anesthesia General/ae [Adverse Effects]
beta adrenergic blockers
breathing difficulties
calcium antagonists
Cardiovascular Diseases/et [Etiology]
Child
Child Preschool
digoxin
diuretics
Female
Humans
Infant
Intubation Intratracheal/ae [Adverse Effects]
Lung Diseases/et [Etiology]
Lung Diseases/pp [Physiopathology]
Male
Medicine
MLII
MLIII
MPSI
MPSII
MPSVI
Mucopolysaccharidoses/co [Complications]
Mucopolysaccharidoses/mo [Mortality]
Mucopolysaccharidoses/pp [Physiopathology]
pharmacologic interventions
Pyeritz R E
respiratory problems
Respiratory Tract Diseases/et [Etiology]
Respiratory Tract Diseases/pp [Physiopathology]
Respiratory Tract Diseases/ra [Radiography]
Retrospective Studies
Semenza G L
Sleep Apnea Syndromes/et [Etiology]
Spinal Diseases/et [Etiology]
Spinal Diseases/ra [Radiography]
surgical interventions
theophylline
Tonsillectomy
Tracheostomy