Calcium intake improvement after nutritional intervention in paediatric patients with osteogenesis imperfecta

Zambrano, Marina Bauer; Félix, Têmis Maria; Mello, Elza de

doi:10.1111/jhn.12657

Cited by 5 publications

(3 citation statements)

References 22 publications

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“…Olers are more prone to experience overweight or even obesity secondary to physical inactivity [45], small body size and inappropriate dietary habits [18,46,47]. This happens not only in paediatric patients [19], but also in adulthood as shown in this study.…”

Section: Discussionmentioning

confidence: 65%

Eat, Breathe, Sleep With Osteogenesis Imperfecta

LoMauro

Landoni

Fraschini

et al. 2021

Preprint

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Background: Although Osteogenesis Imperfecta (OI) affects the connective tissue causing extremely brittle bones with consequent skeletal deformities, it is important to go beyond bones. Indeed, the quality of life in OI does not only depends on bones status, as OI might affect also other important functions. We have therefore implemented a multidisciplinary study to assess lung function, breathing pattern, sleep quality and nutritional status in 27 adult OI type III and IV patients (median age: 34.6 years; 19 women; 14 type III).Results: According to nocturnal oxygen desaturation, two groups were identified: 13 patients with (OI_OSA, incidence: 48.2%) and 14 without (no_OSA) obstructive sleep apnea. The former was characterized by higher spinal and ribcage deformity, by more restrictive lung function, by paradoxical thoracic breathing in supine position, by rapid and shallow breathing, by higher body mass index, by longer neck and waist circumferences; by higher abdominal volume and by greater percentage of body fat mass, particularly localized in the trunk. The best predictor of OI_OSA was the negative value of the supine ribcage contribution to tidal volume, followed by the ratio between the neck and the waist circumferences with body height and the supine thoraco-abdominal volumes phase shift angle. Conclusions: The pathophysiology of OI ensued a dangerous vicious circle, in which breathing, sleep and nutritional status are tightly linked, and they might all end up in negatively affecting the quality of life. The vicious circle is fed by some intrinsic characteristics of the disease (thoracic, cranial and mandibular deformities) and some bad daily habits of the patients (i.e. physical inactivity and low dietary quality). The former impacts on restricting the respiratory function, the latter makes Olers more prone to experience overweight or obesity. The main consequence is a high incidence of obstructive sleep apnea, which remains an underdiagnosed disorder in individuals with severe OI who are obese, with a neck to height ratio over than 31.6%, and characterized by paradoxical breathing in supine position. A multidisciplinary approach, including evaluations of breathing, sleep and nutrition, is required to better manage the disease and fulfil the maximizing well-being of OI patients.

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Section: Discussionmentioning

confidence: 65%

Eat, Breathe, Sleep With Osteogenesis Imperfecta

LoMauro

Landoni

Fraschini

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Olers are more prone to experience overweight or even obesity secondary to physical inactivity [ 45 ], small body size and inappropriate dietary habits [ 18 , 46 , 47 ]. This happens not only in paediatric patients [ 19 ], but also in adulthood as shown in this study.…”

Section: Discussionmentioning

confidence: 99%

“…It is also the only one to simultaneously consider three vital functions (nutrition, breathing and sleeping) that have an important impact on the quality of life. Indeed, other authors have separately investigated sleep [ 12 , 13 , 41 , 44 ] or respiratory impairment [ 6 – 11 ] or nutrition [ 17 – 19 , 46 , 47 ]. The latter was shown to negatively impact the psychosocial wellbeing in the OI population and to limit daily physical activity [ 53 ].…”

Section: Discussionmentioning

confidence: 99%

Eat, breathe, sleep with Osteogenesis Imperfecta

LoMauro

Landoni

Fraschini

et al. 2021

Orphanet J Rare Dis

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Background Although Osteogenesis Imperfecta (OI) affects the connective tissue causing extremely brittle bones with consequent skeletal deformities, it is important to go beyond bones. Indeed, the quality of life in OI does not only depend on bones status, as OI might affect also other important functions. We have therefore implemented a multidisciplinary study to assess lung function, breathing pattern, sleep quality and nutritional status in 27 adult OI type III and IV patients (median age: 34.6 years; 19 women; 14 type III). Results According to nocturnal oxygen desaturation, two groups were identified: 13 patients with (OI_OSA, incidence: 48.2%) and 14 without (no_OSA) obstructive sleep apnea. The former was characterized by higher spinal and ribcage deformity, by more restrictive lung function, by paradoxical thoracic breathing in supine position, by rapid and shallow breathing, by higher body mass index, by longer neck and waist circumferences; by higher abdominal volume and by greater percentage of body fat mass, particularly localized in the trunk. The best predictor of OI_OSA was the negative value of the supine ribcage contribution to tidal volume, followed by the ratio between the neck and the waist circumferences with body height and the supine thoraco-abdominal volumes phase shift angle. Conclusions The pathophysiology of OI ensued a dangerous vicious circle, in which breathing, sleep and nutritional status are tightly linked, and they might all end up in negatively affecting the quality of life. The vicious circle is fed by some intrinsic characteristics of the disease (thoracic, cranial and mandibular deformities) and some bad daily habits of the patients (i.e. physical inactivity and low dietary quality). The former impacts on restricting the respiratory function, the latter makes Olers more prone to experience overweight or obesity. The main consequence is a high incidence of obstructive sleep apnea, which remains an underdiagnosed disorder in individuals with severe OI who are obese, with a neck to height ratio over than 31.6%, and characterized by paradoxical breathing in supine position. A multidisciplinary approach, including evaluations of breathing, sleep and nutrition, is required to better manage the disease and fulfil the maximizing well-being of OI patients.

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