Predictors of longitudinal outcomes for children using long‐term noninvasive ventilation

Bedi, Prabhjot K.; DeHaan, Kristie; MacLean, Joanna E.; Castro‐Codesal, Maria L.

doi:10.1002/ppul.25188

Cited by 8 publications

(5 citation statements)

References 34 publications

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“…During this period, the number of children with CNS disorders treated by NIV/CPAP has been multiplied by 16, from 11 to 182 children 18 . Similar trends have been observed in Canada, Italy, and Spain 23,25–27 …”

Section: Discussionsupporting

confidence: 80%

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Pediatric long‐term noninvasive respiratory support in children with central nervous system disorders

Carrara,

Aubertin,

Khirani

et al. 2023

Pediatric Pulmonology

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RationaleThe use of long‐term noninvasive respiratory support is increasing in children along with an extension of indications, in particular in children with central nervous system (CNS) disorders.ObjectiveThe aim of this study was to describe the characteristics of children with CNS disorders treated with long‐term noninvasive respiratory support in France.MethodsData were collected from 27 French pediatric university centers through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ≥3 months on 1st June 2019.Main ResultsThe data of 182 patients (55% boys, median age: 10.2 [5.4;14.8] years old [range: 0.3–25]) were collected: 35 (19%) patients had nontumoral spinal cord injury, 22 (12%) CNS tumors, 63 (35%) multiple disabilities, 26 (14%) central alveolar hypoventilation and 36 (20%) other CNS disorders. Seventy five percent of the patients were treated with noninvasive ventilation (NIV) and 25% with continuous positive airway pressure (CPAP). The main investigations performed before CPAP/NIV initiation were nocturnal gas exchange recordings, alone or coupled with poly(somno)graphy (in 29% and 34% of the patients, respectively). CPAP/NIV was started in an acute setting in 10% of the patients. Median adherence was 8 [6;10] hours/night, with 12% of patients using treatment <4 h/day. Nasal mask was the most common interface (70%). Airway clearance techniques were used by 31% of patients.ConclusionCPAP/NIV may be a therapeutic option in children with CNS disorders. Future studies should assess treatment efficacy and patient reported outcome measures.

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Section: Discussionsupporting

confidence: 80%

“…18 Similar trends have been observed in Canada, Italy, and Spain. 23,[25][26][27] Nocturnal gas exchange recordings were the main investigation performed before NIV/CPAP initiation. In 29% of children, it was the sole investigation.…”

Section: Discussionmentioning

confidence: 99%

Pediatric long‐term noninvasive respiratory support in children with central nervous system disorders

Carrara,

Aubertin,

Khirani

et al. 2023

Pediatric Pulmonology

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“…11 We found that obesity was a risk factor for decreased PAP usage over time, similar to a previous 10-year multicentred retrospective review that evaluated longitudinal PAP adherence among 622 children aged 0-18 years old, which showed that children with obesity or metabolic syndrome were more likely to decline PAP therapy (OR =2.077 [95% CI 1.110-3.888]). 39 The reasons for these findings are unclear; however, there is one study suggesting lack of perceived benefits of PAP in this population may contribute to decreased use. 16 Of concern, obesity is an independent risk factor for susceptibility to and severity of COVID-19 which is related to worsening outcomes including increased risk of hospitalization, critical care admission and mortality.…”

Section: Discussionmentioning

confidence: 97%

To Wear or Not Wear the Mask: Decline in Positive Airway Pressure Usage in Children with Sleep Disordered Breathing During the COVID-19 Pandemic

Sunkonkit¹,

Selvadurai²,

Voutsas³

et al. 2022

NSS

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Positive airway pressure (PAP) therapy is an effective treatment prescribed to children with sleep disordered breathing (SDB); however, PAP adherence remains challenging. Given that COVID-19 pandemic continues to impact sleep and daily life, the aim of this study was to evaluate longitudinal trajectory of PAP usage in children during the COVID-19 pandemic. Patients and Methods: This was a retrospective study. Children aged 1-18 years with SDB prescribed PAP at The Hospital for Sick Children (Toronto, Canada) were evaluated for PAP adherence. Demographics, medical history and PAP adherence data during four consecutive 3-month time periods from December 2019 to December 2020 were collected. These four time periods included i) prior to COVID-19 lockdown, ii) during the first three months of lockdown, iii) summer and iv) return to school period. Percentage of days where PAP was used for ≥4 hours and average nightly usage of PAP were primary outcomes. Results: A total of 149 children (61.7% male, mean (±SD) age=12.8 ± 4.1 years, BMI (±SD) z-score=1.45±1.43) were enrolled. Compared to prior to lockdown, the median (IQR) of percentage of PAP usage ≥4 hours and average nightly usage of PAP declined significantly during the summer and return to school periods (p<0.001 for all). By the end of the return to school period, only 69/149 (46%) showed sustained PAP usage and 80/149 (54%) had decreased PAP usage. Obesity was a risk factor for a decline in PAP usage after returning to school (β=−15.36, p=0.03). Conclusion: Compared to COVID-19 pre-pandemic PAP usage, there was a significant decline in PAP usage across COVID-19 pandemic. There is critical under usage of PAP in children diagnosed with SDB, resulting in an urgent need to address barriers to mitigate poor adherence to PAP long-term. Targeted strategies are required to optimize PAP adherence in children with SDB.

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“…Hip and elbow joints cannot be fully extended, while finger, knee and ankle joints are hyperextended. Because of the reduced size of the chest, breathing is often impaired [18,38,39]. The narrowness of the nasopharynx often causes infections, especially of the upper respiratory tract [18,38,39].…”

Section: Introductionmentioning

confidence: 99%

“…Because of the reduced size of the chest, breathing is often impaired [18,38,39]. The narrowness of the nasopharynx often causes infections, especially of the upper respiratory tract [18,38,39]. Middle ear infections may also occur.…”

Section: Introductionmentioning

confidence: 99%

How do we Treat Aberrant Excessive Signaling in FGF3 Receptor Pathway to Cure Achondroplasia in Childhood?

Bittmann

2023

AJBSR

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In 1878, a French physician named Joseph Marie Jules Parrot coined the term achondrodysplasia. This term is purely historical and does not explain the cause or symptoms of the disorder. Other names that were once used but are now obsolete include chondrodystrophy, chondrodystrophia fetalis, Parrot syndrome, and Kaufmann syndrome, named after the German pathologist Eduard Kaufmann. Achondroplasia is the most common form of genetic short stature, occurring in 1 out of every 20,000 births. It is considered a rare disease. Achondroplasia is caused by a specific mutation in the fibroblast growth factor receptor gene FGFR-3. This gene is located on chromosome 4p 16.3. The mutation in achondroplasia leads to an overexpression of the FGFR3 receptor, inhibiting the proliferation of chondrocytes, which are cells responsible for cartilage formation. In 96% of cases, there is a specific G (1138) A point mutation in the FGFR-3 gene. Researchers are currently focusing on developing targets that can reduce the overexpression of the FGF3 receptor and inhibit chondrocyte formation. This mutation disrupts cartilage formation, causing the growth plates in bones to ossify prematurely. This results in restricted growth, particularly in the arms and legs, leading to a disproportionate short stature where the trunk and head are relatively longer than the extremities. This article provides an overview of achondroplasia, with a special focus on current therapies and future treatment options for pediatric patients. Various targets in the FGFR3 signaling pathway are being studied in clinical research, and their potential for treating achondroplasia will be analyzed in detail. The ultimate goal is to find a cure by targeting the aberrant excessive FGF3 receptor signaling pathways. The article will discuss the FGFR3 receptor pathways in detail and mention possible clues for curing the disease.

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Predictors of longitudinal outcomes for children using long‐term noninvasive ventilation

Cited by 8 publications

References 34 publications

Pediatric long‐term noninvasive respiratory support in children with central nervous system disorders

Pediatric long‐term noninvasive respiratory support in children with central nervous system disorders

To Wear or Not Wear the Mask: Decline in Positive Airway Pressure Usage in Children with Sleep Disordered Breathing During the COVID-19 Pandemic

How do we Treat Aberrant Excessive Signaling in FGF3 Receptor Pathway to Cure Achondroplasia in Childhood?

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