Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis

Vanacor, Roberta; Raimundo, Fabiana Viegas; Marcondes, Natália Aydos; Corte, Bruno Pellini; Ascoli, Aline Maria; Azambuja, Aline Zimmermann de; Scopel, Luciano; Santos, Patrícia Vargas dos; Dalcin, Paulo de Tarso Roth; Faulhaber, Gustavo Adolpho Moreira; Furlanetto, Tânia Weber

doi:10.1590/1806-9282.60.01.012

Cited by 8 publications

(4 citation statements)

References 36 publications

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“…The prevalence of bone mineral density (BMD) z-scores below − 1 SD was 10% in our study, but it has been reported to be as high as 85% in some adult CF studies [9]. On the other hand, there are some cross-sectional surveys showing that pre-pubertal children with CF do not have a bone mineral deficit compared with a weight-and sex-matched control groups [35][36][37][38]. The reasons for the discrepancy in the findings include: differences in measurement and analytic techniques that adjust for impaired growth and maturation in CF patients, disease severity, reference data, and the lack of consensus in defining impaired bone health in children.…”

Section: Discussioncontrasting

confidence: 62%

A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis

Chirita-Emandi

Shepherd

Kyriakou

et al. 2017

Journal of Pediatric Endocrinology and Metabolism

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Background: We aimed to describe the longitudinal changes in bone mineral content and influencing factors, in children with cystic fibrosis (CF). Methods: One hundred children (50 females) had dual X-ray absorptiometry (DXA) performed. Of these, 48 and 24 children had two to three scans, respectively over 10 years of follow-up. DXA data were expressed as lumbar spine bone mineral content standard deviation score (LSBMCSDS) adjusted for age, gender, ethnicity and bone area. Markers of disease, anthropometry and bone biochemistry were collected retrospectively. Results: Baseline LSBMCSDS was > 0.5 SDS in 13% children, between − 0.5; 0.5 SDS, in 50% and ≤ − 0.5 in the remainder. Seventy-eight percent of the children who had baseline LSBMCSDS > − 0.5, and 35% of the children with poor baseline (LSBMCSDS < − 0.5), showed decreasing values in subsequent assessments. However, mean LS BMC SDS did not show a significant decline in subsequent assessments (− 0.51; − 0.64; − 0.56; p = 0.178). Lower forced expiratory volume in 1 s percent (FEV1%) low body mass

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Section: Discussioncontrasting

confidence: 62%

A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis

Chirita-Emandi

Shepherd

Kyriakou

et al. 2017

Journal of Pediatric Endocrinology and Metabolism

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“…Furthermore, stratification of the patients according to faecal elastase‐1 levels resulted in significant variations of vitamin D3 between the subgroups . In cystic fibrosis, a condition regularly associated with pancreatic exocrine insufficiency, a decrease in BMD has been noted in several studies . Furthermore, no improvement in skeletal health was observed in these patients compared to patients from the late 1990s .…”

Section: Systematic Literature Review Of Age‐related Pancreatic Functmentioning

confidence: 99%

The ageing pancreas: a systematic review of the evidence and analysis of the consequences

et al. 2018

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Senior people constitute the fastest growing segment of the population. The elderly are at risk for malnutrition, thought to be caused by reduced food intake or involution of the physiological capacity of the GI tract. Age-related changes are well known in other secretory organs such as liver, kidney and intestine. The pancreas, representing a metabolically active organ with uptake and breakdown of essential nutritional components, changes its morphology and function with age. During childhood, the volume of the pancreas increases, reaching a plateau between 20 and 60 years, and declines thereafter. This decline involves the pancreatic parenchyma and is associated with decreased perfusion, fibrosis and atrophy. As a consequence of these changes, pancreatic exocrine function is impaired in healthy older individuals without any gastrointestinal disease. Five per cent of people older than 70 years and ten per cent older than 80 years have pancreatic exocrine insufficiency (PEI) with a faecal elastase-1 below 200 μg g stool, and 5% have severe PEI with faecal elastase-1 below 100 μg g stool. This may lead to maldigestion and malnutrition. Patients may have few symptoms, for example steatorrhoea, diarrhoea, abdominal pain and weight loss. Malnutrition consists of deficits of fat-soluble vitamins and is affecting both patients with PEI and the elderly. Secondary consequences may include decreased bone mineral density and results from impaired absorption of fat-soluble vitamin D due to impaired pancreatic exocrine function. The unanswered question is whether this age-related decrease in pancreatic function warrants therapy. Therapeutic intervention, which may consist of supplementation of pancreatic enzymes and/or vitamins in aged individuals with proven exocrine pancreas insufficiency, could contribute to healthy ageing.

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“…[16] The association between low BMD and airway clearance impairment and/or decline of respiratory function has not been demonstrated. [11,13,17,18] Some authors consider that osteoporosis may be a barrier to lung transplantation. [19,20] Interestingly, we found no association between spinal pain and low BMD in our study.…”

Section: Discussionmentioning

confidence: 99%

Clinical relevance of low bone density in cystic fibrosis adult patients: A pilot study

et al. 2023

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Survival improvement in cystic fibrosis (CF) is associated with more frequent long-term complications, including CF related bone disease (CFBD). Impact of CFBD on global health outcome remains poorly described. We aimed to assess the relationship between low bone mineral density (BMD) and spinal pain, disability, and quality of life in CF adult patients. This monocentric crosssectional study with prospective data collection was conducted from November 2016 to December 2019 in the Department of Respiratory Diseases at the University Hospital of Reims (NCT02924818). BMD was assessed by X-ray absorptiometry (DXA). Disability was assessed by the Health Assessment Questionnaire (HAQ). Quality of life was assessed by both the St George's Respiratory Questionnaire and the Cystic Fibrosis Questionnaire for teenagers and adults (CFQ 14+). Forty patients were analyzed, 68% of men, with a median age of 25 years, a median body mass index of 21 kg/m² and a median FEV 1 % of 54%. Nine patients (23%) had spinal pain. Ten patients (25%) had a low BMD. Compared with patients with normal BMD, patients with low BMD had a significantly lower BMI (22 vs 19 kg/m²; P = .006) and less vitamin D supplementation (33% vs 0%; P = .035). Low BMD was not associated with spinal pain, disability and quality of life. Low BMD is frequent in CF, affecting 1-quarter of adult patients. No significant association was found between low BMD and spinal pain, disability or quality of life.Abbreviations: BMD = bone mineral density, BMI = body mass index, CF = cystic fibrosis, CFBD = cystic fibrosis related bone disease, CFQ 14+ = cystic fibrosis questionnaire for teenagers and adults, FEV 1 = forced expiratory volume at first second, HAQ = health assessment questionnaire.

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Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis

Cited by 8 publications

References 36 publications

A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis

A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis

The ageing pancreas: a systematic review of the evidence and analysis of the consequences

Clinical relevance of low bone density in cystic fibrosis adult patients: A pilot study

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