Evaluation of Body Composition in Paediatric Osteogenesis Imperfecta

Gilani, M Sadighi; Shepherd, Sheila; Nichols, Ben; Gerasimidis, Konstantinos; Wong, Sze Choong; Mason, Avril

doi:10.1016/j.jocd.2021.01.012

Cited by 8 publications

(6 citation statements)

References 38 publications

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“…Impaired muscle function was found in our OI patients, which was consistent with weaker muscle force of lower limbs in OI type I children of other studies ( 23 , 24 ). Patients with OI type I and IV were found to have lower LMI and higher FMI than healthy controls ( 25 ). These results all indicated that there was a significant abnormal body composition and muscle strength in OI patients.…”

Section: Discussionmentioning

confidence: 99%

The role of osteocalcin in regulation of glycolipid metabolism and muscle function in children with osteogenesis imperfecta

Zheng

Zhao

et al. 2022

Front. Endocrinol.

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ObjectiveOsteoblasts are discovered to secrete hormones with endocrine effects on metabolism, and osteocalcin (OC) is the most abundant non-collagenous protein in bone. We investigate the relationship between serum OC levels and glycolipid metabolism and muscle function in children with osteogenesis imperfecta (OI).MethodsA total of 225 children with OI and 80 healthy controls matched in age and gender were included in this single center study. Serum levels of fasting blood glucose (FBG), triglyceride (TG), total cholesterol (TC), low- and high-density lipoprotein cholesterol (LDL-C, HDL-C) were measured by automated analyzers. Serum levels of fasting insulin (FINS) were measured using an automated electrochemiluminescence system. Serum levels of OC and undercarboxylated osteocalcin (ucOC) were measured by enzyme-linked immunosorbent assay. Grip strength and timed-up-and-go (TUG) test were measured. Bone mineral density (BMD) and body composition were measured using dual-energy X-ray absorptiometry.ResultsOI patients had significantly higher body mass index (BMI), FBG, and HOMA-IR, but lower HDL-C levels, lower grip strength and longer TUG than control group (all P<0.05). Serum OC, ucOC levels, and ucOC/OC in OI type III patients were significantly lower than those in OI patients with type I and IV. Serum levels of OC, ucOC, and ucOC/OC were negatively correlated to BMI, FBG, insulin levels, and HOMA-IR (all P<0.05). The ratio of ucOC/OC was positively correlated to grip strength (r=0.512, P=0.036), lean mass percentage (%LM) of the total body and limbs, and negatively correlated to fat mass percentage (%FM) of the total body, %FM and fat mass index (FMI) of the trunk (all P<0.05).ConclusionsObesity, glucolipid metabolic abnormalities, and reduced grip strength were common in children with OI. Circulating osteocalcin and ucOC may play an important role in the regulation of glucose metabolism, as well as the muscle function of children with OI.

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Section: Discussionmentioning

confidence: 99%

The role of osteocalcin in regulation of glycolipid metabolism and muscle function in children with osteogenesis imperfecta

Zheng

Zhao

et al. 2022

Front. Endocrinol.

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“…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%

“…Obesity induces restrictive respiratory pattern while promoting obstructive apneas during sleep [ 14 – 16 ]. Individuals with OI may experience overweight or even obesity secondary to physical inactivity, small body size and, possibly, inappropriate caloric intake and dietary quality [ 17 , 18 ]. However, information on the nutritional status, body composition, and dietary habits of these patients is scarce, particularly in adult population.…”

Section: Introductionmentioning

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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“…Changes in the DXA can be used to guide treatment with respect to dosing and interval (67). Importantly, the presence of metal implants skews the measurements obtained on DXA (68). Because intramedullary rodding is used to promote bone strength in patients with OI (69), serial DXA evaluation can be challenging; changes in measurements over time must take this variable into consideration.…”

Section: Osteogenesis Imperfectamentioning

confidence: 99%

Transition of young adults with metabolic bone diseases to adult care

Ross

Bowden

et al. 2023

Front. Endocrinol.

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As more accurate diagnostic tools and targeted therapies become increasingly available for pediatric metabolic bone diseases, affected children have a better prognosis and significantly longer lifespan. With this potential for fulfilling lives as adults comes the need for dedicated transition and intentional care of these patients as adults. Much work has gone into improving the transitions of medically fragile children into adulthood, encompassing endocrinologic conditions like type 1 diabetes mellitus and congenital adrenal hyperplasia. However, there are gaps in the literature regarding similar guidance concerning metabolic bone conditions. This article intends to provide a brief review of research and guidelines for transitions of care more generally, followed by a more detailed treatment of bone disorders specifically. Considerations for such transitions include final adult height, fertility, fetal risk, heritability, and access to appropriately identified specialists. A nutrient-dense diet, optimal mobility, and adequate vitamin D stores are protective factors for these conditions. Primary bone disorders include hypophosphatasia, X-linked hypophosphatemic rickets, and osteogenesis imperfecta. Metabolic bone disease can also develop secondarily as a sequela of such diverse exposures as hypogonadism, a history of eating disorder, and cancer treatment. This article synthesizes research by experts of these specific disorders to describe what is known in this field of transition medicine for metabolic bone diseases as well as unanswered questions. The long-term objective is to develop and implement strategies for successful transitions for all patients affected by these various conditions.

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Evaluation of Body Composition in Paediatric Osteogenesis Imperfecta

Cited by 8 publications

References 38 publications

The role of osteocalcin in regulation of glycolipid metabolism and muscle function in children with osteogenesis imperfecta

The role of osteocalcin in regulation of glycolipid metabolism and muscle function in children with osteogenesis imperfecta

Eat, breathe, sleep with Osteogenesis Imperfecta

Transition of young adults with metabolic bone diseases to adult care

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