Weight loss surgery improves quality of life in pediatric patients with osteogenesis imperfecta

Zani, Augusto; Ford‐Adams, Martha; Ratcliff, P.-H. D. Megan; Bevan, Denise; Inge, Thomas H.; Desai, Ashish

doi:10.1016/j.soard.2015.11.029

Cited by 7 publications

(2 citation statements)

References 21 publications

(20 reference statements)

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“…Surgical advancement of lower jaw or both jaws to increase pharyngeal airway space would likewise be unfeasible in OI [ 26 ]. Weight reduction in patients with low ambulatory ability may also be very difficult [ 27 ]. PAP treatment therefore is the best and nearly exquisite therapy option for OI patients with sleep apnea.…”

Section: Discussionmentioning

confidence: 99%

Positive airway pressure therapy for obstructive sleep apnea in patients with Osteogenesis imperfecta: a prospective pilot study

Arponen

Bachour

Bäck

et al. 2021

BMC Musculoskelet Disord

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Background Obstructive sleep apnea (OSA) is prevalent in individuals with Osteogenesis imperfecta (OI). To date, no study has investigated treatment of OSA in adult individuals with OI using positive airway pressure (PAP). This observational pilot study examined the adherence of adults with OI to treatment of OSA with PAP therapy, and the evolution of self-experienced sleepiness and depression symptoms before and after treatment. Methods We included 20 patients, with a mean age of 51 years, who represented varying severity of OI and displayed an apnea and hypopnea index ≥ 5 /sleeping hour as recorded by an overnight polysomnography. PAP therapy was proposed to all patients. Epworth Sleepiness Scale (ESS) questionnaire to evaluate daytime sleepiness, and a validated self-rating depression questionnaire to identify possible depression, were completed prior to PAP therapy and repeated after a minimum of one year. The datasets supporting the conclusions of this article are included within the article. Results From the 20 patients, 15 initiated PAP therapy, and two patients later interrupted it. The mean PAP follow-up period was 1230 days. At baseline, an abnormally high ESS score was reported by 29% of the respondents, and an abnormally high number of symptoms suggesting depression by 29%. Follow-up questionnaires were completed by 60% of the patients, of whom 83% were adherent to PAP treatment. ESS score and depression symptoms did not decrease significantly with PAP therapy. Conclusions Patients with OI accepted well PAP therapy and remained compliant. Sleepiness and depression persisted unaltered despite good PAP adherence. These unexpectedly poor improvements in symptoms by PAP therapy may be due to subjective depression symptoms and the complexity of factors underlying persisting sleepiness in OI. Further research is needed to confirm this novel finding.

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Section: Discussionmentioning

confidence: 99%

Positive airway pressure therapy for obstructive sleep apnea in patients with Osteogenesis imperfecta: a prospective pilot study

Arponen

Bachour

Bäck

et al. 2021

BMC Musculoskelet Disord

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“…Surgery to control weight had been reported. Weight loss surgery effectively treated severe obesity in 2 OI patients and substantially improved mobility and HRQoL, theoretically reducing fracture risk [19] .…”

Section: Low Educational Level and Their Familymentioning

confidence: 99%

Investigation on the Current Situation and Health-Related Quality of Life of Children and adolescents with OI in southwest China

mo²

2021

Preprint

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Background: Osteogenesis imperfecta（OI）is a chronic, non-infectious disease that accompanies the patient for life. OI will result in reduced bone mass, fractures, thereby leading to bone deformities and reduced patient the health-related quality of life (HRQoL). With the endocrine therapy and surgical orthopedic had been recommended, the disease was effectively controlled. The disease status is discrepancy in different regions. This study aims to assess the diagnosis and treatment status, evaluate the HRQoL of children and adolescents with OI in southwest China, and analyze the influencing factors, so as to provide reference for health care consideration for OI population in southwest China.Methods: This cross-sectional study was performed using the Pediatric Quality of Life InventoryTM (PedsQL TM) to assess the HRQoL of children and adolescents with OI. The demographic and clinical data were collected. This study was conducted in Children’s Hospital of Chongqing Medical University, the National Children's Regional Medical Centre (SOUTH-WEST) and the National Clinical Research for child health. Healthy children and adolescents aged 2 to 18 years lived in southwest China as a reference.Results: A total of 27 children and adolescents with OI were included in this study. The current status of diagnosis and treatment such as the treatment, education, family, BMI, pain, steep walking and friends and so on had been investigated. The PedsQLTM of OI population was lower in all dimensions, and they were significantly different in physiological and social functioning and the total score, compared with 54 healthy children and adolescents. Factors associated with HRQoL of OI patients were the steep walking and fixing a playmate at less.Conclusions: The HRQoL of children and adolescents with OI had been improved with the endocrine therapy and surgical orthopedic. But there was an imbalance around the world for the less effective management. Health care education should be more professional, effectively and continuously. Training patients to gain steep walking ability and peer companionship could be a way to improve the HRQoL of children and adolescents with OI in southwest China. The medical staff also should pay attention to their life and growth after discharge.

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Optimising Health-Related Quality of Life in Children With Osteogenesis Imperfecta

Hill,

Ford,

Baker

2024

Calcif Tissue Int

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Weight loss surgery improves quality of life in pediatric patients with osteogenesis imperfecta

Cited by 7 publications

References 21 publications

Positive airway pressure therapy for obstructive sleep apnea in patients with Osteogenesis imperfecta: a prospective pilot study

Positive airway pressure therapy for obstructive sleep apnea in patients with Osteogenesis imperfecta: a prospective pilot study

Investigation on the Current Situation and Health-Related Quality of Life of Children and adolescents with OI in southwest China

Optimising Health-Related Quality of Life in Children With Osteogenesis Imperfecta

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