Bone Histomorphometry in Adult Patients With Cystic Fibrosis

Haworth, Charles; Webb, A K; Egan, J. J.; Selby, Peter L.; Hasleton, Philip; Bishop, Paul; Freemont, Tony

doi:10.1378/chest.118.2.434

Cited by 82 publications

(40 citation statements)

References 19 publications

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“…Haworth et al [18] found reduced bone volume (trabecular and cortical) and evidence for accelerated bone breakdown (increased osteoclast numbers) and inadequate bone formation (decreased osteoblast number and activity) in CF patients with advanced disease. Elkin et al [7] also found decreased bone formation by tetracycline labeling and an increased mineralization lag time in adults with CF, but osteomalacia was rare.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Bone Turnover in Cystic Fibrosis Adults

Aris

Ontjes²,

Buell³

et al. 2002

Osteoporosis International

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Cystic fibrosis (CF) patients often have low bone mineral density (BMD) and may suffer from fractures and kyphosis. The pathogenesis of low BMD in CF is multifactorial. To study bone metabolism, we collected fasting serum and urine from 50 clinically stable CF adults (mean age 28 years) and 53 matched controls to measure markers of bone formation and bone resorption. The CF subjects had moderate lung disease (FEV1: 46.1 +/- 18.6% predicted) and malnutrition (BMI: 20.0 +/- 3.3 kg/m2). Only 3 subjects had normal BMD. CF subjects had higher urinary N-telopeptides of type I collagen (81.0 +/- 60.0 vs 49.0 +/- 24.2 nm BCE/mmol creatinine, p = 0.0006) and free deoxypyridinoline (7.3 +/- 5.0 vs 5.3 +/- 1.9 nM/mM, p = 0.004) levels than controls. Serum osteocalcin levels were similar in the two groups, a result confirmed by two immunoassays that recognize different epitopes on osteocalcin. Serum bone-specific alkaline phosphatase levels were elevated in CF patients (32.0 +/- 11.3 vs 21.8 +/- 7.0 U/l, p < 0.0001), but were much more closely associated with serum total alkaline phosphatase levels (r = 0.51, p = 0.001) than with age or gender. Parathyroid hormone levels were elevated (p = 0.007) and 25-hydroxyvitamin D levels were depressed (p = 0.0002) in the CF patients in comparison with controls. These results indicate that adults with CF have increased bone resorption with little change in bone formation. Medications that decrease bone resorption or improve calcium homeostasis may be effective therapies for CF bone disease.

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Section: Discussionmentioning

confidence: 99%

Abnormal Bone Turnover in Cystic Fibrosis Adults

Aris

Ontjes²,

Buell³

et al. 2002

Osteoporosis International

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“…In transiliac bone biopsies in adults, decreased bone formation [161], with or without increased bone resorption [162] have been observed, with reductions in trabecular and cortical bone mass. Interestingly, despite the high prevalence of vitamin D deficiency in CF, there were no histological signs of osteomalacia in these samples.…”

Section: Pathogenesismentioning

confidence: 98%

Chronic pediatric inflammatory diseases: Effects on bone

Viswanathan

Sylvester

2007

Rev Endocr Metab Disord

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In children, chronic inflammatory diseases present a significant challenge to long-term skeletal health. These conditions are often associated with poor appetite and suboptimal overall nutrition, altered nutrient utilization, delayed puberty, inactivity, and reduced muscle mass, all of which can alter bone metabolism. In addition, bone cell activity is susceptible to the effects of the immune response that characterizes these diseases. Moreover, drugs used to treat these maladies, notably glucocorticoids, may have negative effects on bone formation and on linear growth in developing children. As a result, predicted peak bone mass may not be achieved, and fracture risk may be increased in the short term or in the future. Studies using primarily dual energy X-ray absorptiometry have documented that deficits in bone mass are common in these diseases. However, there are wide variations in the prevalence of low bone mass, largely due to differences in the characteristics of each study population. Recent studies provide insight into the pathogenesis of decreased bone mass in these conditions. In this paper we will provide an overview of the effects of chronic inflammatory conditions on bone mass in children. We will also present relevant data from adult patients, when pediatric data are scant or not available.

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“…To date, bone biopsies have only been collected in adults with moderate to severe lung disease [ 65 ] or post-mortem [ 66,67 ] . The histological features of 19 adults with CF [FEV 1 = 38.6% (SD = 14)] indicated that the bone disease in adults with CF was characterized by low bone turnover and reduced bone formation at a cellular level [ 65 ] .…”

Section: Bone Health Outcomesmentioning

confidence: 99%

Cystic Fibrosis

Buntain¹,

Chang²

2012

Vitamin D and the Lung

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The features of cystic fi brosis (CF) include chronic infection and in fl ammation of the lungs and malabsorption due to pancreatic insuf fi ciency. In people with CF, the many factors contributing to vitamin D de fi ciency include reduced sun exposure, pancreatic insuf fi ciency, reduced levels of vitamin D binding protein, reduced fat stores, liver disease, accelerated enterohepatic excretion and in fl uence of CF transmembrane conductance regulator (CFTR). The clinical impact of vitamin D de fi ciency on bone metabolism is known; however, its effects beyond the skeletal system is less recognized. The evidence for routine vitamin D supplementation and management in the presence of vitamin D de fi ciency are discussed.

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Bone Histomorphometry in Adult Patients With Cystic Fibrosis

Cited by 82 publications

References 19 publications

Abnormal Bone Turnover in Cystic Fibrosis Adults

Abnormal Bone Turnover in Cystic Fibrosis Adults

Chronic pediatric inflammatory diseases: Effects on bone

Cystic Fibrosis

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