Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS)

Muhlebach, Marianne S.; Shaffer, Christopher B.; Georges, Linda S.; Abode, Kathy; Muenzer, Joseph

doi:10.1002/ppul.22629

Cited by 31 publications

(30 citation statements)

References 22 publications

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“…At our center, patients with MPS often undergo flexible bronchoscopy at the time of surgical interventions which allows assessment of the upper and lower airway for GAG deposits. Review of these bronchoscopies has revealed a high prevalence of widespread deposits, frequently involving the lower airway [24]. Future studies might employ direct visualization or imaging of the lower airway along with polysomnography to better characterize these processes.…”

Section: Discussionmentioning

confidence: 99%

Relationship of Sleep to Pulmonary Function in Mucopolysaccharidosis II

Wooten

Muenzer

Vaughn

et al. 2013

The Journal of Pediatrics

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Objective To study the sleep characteristics, pulmonary function, and their relationships in an enzyme naive population of patients with mucopolysaccharidosis (MPS) II (Hunter syndrome). Study design The analyzed subjects (30 patients with MPS II with a median age of 9 years) had been enrolled in an MPS II natural history study and a phase I/II enzyme replacement clinical study in which they underwent standard polysomnography including spirometry and plethysmography, if cooperative. Descriptive statistics and nonparametric correlation were performed for demographic, sleep, and pulmonary function variables. Results Median apnea-hypopnea index (AHI) was 6.4, with obstructive sleep apnea (OSA) observed in 27/30 subjects. Sleep architecture was characterized by diminished rapid-eye movement (REM) sleep duration (median 13%), and decline in sleep efficiency and slow-wave sleep (SWS) duration in older individuals. Oxygen desaturation below 90% occurred in 26/30 subjects, and hypoventilation above 50 torr occurred in 11/23 subjects with accurate end-tidal carbon dioxide (ETCO2) recordings. Of fifteen subjects with reliable spirometry, median forced expiratory volume in 1 second (FEV1) was below 80% predicted in 12/15 subjects. FEV1 in percent-predicted (FEV1 %) was inversely related to AHI and increase from baseline ETCO2 (p=0.023, rs= −0.58), (p<0.001, rs= −0.82). Conclusion Sleep in MPS II is characterized by OSA, altered sleep architecture, and impaired gas exchange. Sleep disruption is related to daytime pulmonary function, thus both systems should be evaluated when sleep abnormalities are suspected.

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Section: Discussionmentioning

confidence: 99%

Relationship of Sleep to Pulmonary Function in Mucopolysaccharidosis II

Wooten

Muenzer

Vaughn

et al. 2013

The Journal of Pediatrics

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“…From a research perspective, airway center team members have collaborated with researchers on a project to develop predictive models for treatment of upper airway obstruction. [36][37][38][39] Studies focusing on inhalation injury in pediatric burn patients, 40 airway management of complex patients, 41,42 and the experience of the school-aged child with a tracheostomy 43 were also supported by airway center data and resources. Multidisciplinary children's airway centers address the needs of a complex patient population and comprise unique patients relying on multiple providers and extensive resources.…”

Section: Discussionmentioning

confidence: 99%

A Multidisciplinary Children’s Airway Center: Impact on the Care of Patients With Tracheostomy

et al. 2016

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Ms Abode conceptualized and drafted the initial manuscript, developed clinical protocols and quality metrics, and carried out data collection and initial analysis; Drs Drake, Zdanski, and Retsch-Bogart developed clinical protocols and quality metrics, and reviewed and revised the manuscript; Ms Gee carried out data collection and reviewed and revised the manuscript; Dr Noah developed clinical protocols and quality metrics, carried out data analysis, and critically reviewed and revised the manuscript; and all authors approved the fi nal manuscript as submitted.

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“…Flexible bronchoscopic intubation represents the most frequent primary indication for interventional bronchoscopy in paediatric intensive care patients [145,146], particularly for critically ill [147] and syndromic [148] patients. In children nasal intubation tends to be easier than oral intubation, because the angle around the base of the tongue is less sharp than in adults.…”

Section: Endoscopic Intubationmentioning

confidence: 99%

ERS statement: interventional bronchoscopy in children

et al. 2017

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Paediatric airway endoscopy is accepted as a diagnostic and therapeutic procedure, with an expanding number of indications and applications in children. The aim of this European Respiratory Society task force was to produce a statement on interventional bronchoscopy in children, describing the evidence available at present and current clinical practice, and identifying areas deserving further investigation. The multidisciplinary task force panel performed a systematic review of the literature, focusing on whole lung lavage, transbronchial and endobronchial biopsy, transbronchial needle aspiration with endobronchial ultrasound, foreign body extraction, balloon dilation and occlusion, laser-assisted procedures, usage of airway stents, microdebriders, cryotherapy, endoscopic intubation, application of drugs and other liquids, and caregiver perspectives. There is a scarcity of published evidence in this field, and in many cases the task force had to resort to the collective clinical experience of the committee to develop this statement. The highlighted gaps in knowledge underline the need for further research and serve as a call to paediatric bronchoscopists to work together in multicentre collaborations, for the benefit of children with airway disorders.

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Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS)

Cited by 31 publications

References 22 publications

Relationship of Sleep to Pulmonary Function in Mucopolysaccharidosis II

Relationship of Sleep to Pulmonary Function in Mucopolysaccharidosis II

A Multidisciplinary Children’s Airway Center: Impact on the Care of Patients With Tracheostomy

ERS statement: interventional bronchoscopy in children

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