Decreased bone density, elevated serum osteoprotegerin, and beta-cross-laps in Wilson disease.

First Department of Medicine, Semmelweis University, Budapest, Hungary.
Journal of Bone and Mineral Research (Impact Factor: 6.13). 12/2002; 17(11):1961-7. DOI: 10.1359/jbmr.2002.17.11.1961
Source: PubMed

ABSTRACT Osteopathia has been reported in Wilson disease (WD), but bone density has not been measured; therefore, we performed bone mineral density (BMD), bone mineral content (BMC), and quantitative bone ultrasound (QUS) assessments, as well as measured the serum levels of osteocalcin (OCN), beta-cross-laps (beta-CTx's), and the recently discovered osteoprotegerin (OPG) and its ligand RANKL to investigate the underlying mechanism of osseous disorders. Serum OCN, beta-CTx, OPG, and RANKL levels were measured by ELISA in 21 WD patients and in 20 age- and gender-matched healthy subjects. BMD, BMC, and QUS parameters were also determined. Osteoporosis was present in 9/21 (43%) WD patients. Abnormal QUS parameters were found in 7 (33%) of the patients. Although serum OCN levels were similar in patients and controls (29.93 +/- 24.65 mg/ml vs. 29.84 +/- 6.89 mg/ml), beta-CTx and OPG levels were significantly increased in WD compared with the healthy controls (625.4 +/- 312.3 pg/ml vs. 423.6 +/- 144.3 pg/ml and p = 0.022 and 7.2 +/- 3.4 pM vs. 3.5 +/- 1.0 pM and p < 0.001, respectively). No difference was observed in the RANKL level. There was a positive correlation between OCN and beta-CTx (r = 0.55; p = 0.01). We proved high occurrence of osteoporosis in WD. Negative bone remodeling balance is a consequence of increased bone resorption, which is indicated by elevated beta-CTx. The novel finding of elevated serum OPG may reflect a compensatory reaction to enhanced osteoclast activity, despite the normal OCN level.

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    ABSTRACT: PURPOSE: Strenuous, endurance exercise increases biochemical markers of bone resorption but not formation, although the effect of recovery duration between consecutive bouts of exercise is unknown. We examined the effect of recovery duration on the bone metabolic response to two bouts of running. METHODS: Ten physically-active men completed two, 9-d trials. On days 4 and 5, participants completed two, 60 min bouts of running at 65% VO2max separated by either a 23 h (LONG) or 3 h (SHORT) recovery period. Osteoprotegerin (OPG), parathyroid hormone (PTH), albumin-adjusted calcium (ACa), and phosphate (PO4) were measured from blood samples obtained before and for 3 h after exercise, and on four follow-up days (days 6-9). Markers of bone resorption [C-terminal telopeptide region of collagen type 1 (β-CTX)] and bone formation [N-terminal propeptides of procollagen type 1 (P1NP) and bone alkaline phosphatase (bone ALP)] were measured in early morning, fasted samples on days 4 to 9. RESULTS: There were no significant changes in β-CTX, P1NP or bone ALP with either protocol. OPG, PTH, ACa and PO4 concentrations increased with all exercise bouts but the response to the second bout was not altered by recovery duration. CONCLUSION: Two, 60 min bouts of running at 65% VO2max separated by either 23 h or 3 h had no effect on markers of bone resorption or formation from 1 to 4 days postexercise. Reducing recovery duration from 23 h to 3 h between two bouts of running did not alter the increase in OPG, PTH, ACa and PO4 to the second bout.
    Medicine and science in sports and exercise 10/2012; · 4.48 Impact Factor
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    ABSTRACT: OBJECTIVES: Patients with Wilson's disease (WD) develop osseous changes such as osteoporosis, spontaneous fractures, areas of sclerosis and demineralization of maxillary and mandibular bones, and neurologic symptoms including swallowing dysfunctions, which may affect dento-facial growth. However, dento-maxillo-facial structures of these patients have never been investigated. The present study aimed to discover if subjects with WD have different dentofacial structures. METHODS: Lateral cephalometric films of 13 children (5 males and 8 females) with WD and of 15 normal subjects (6 males and 9 females) were evaluated. Mean ages of the patients and controls were 12.62±3.09 years and 12.01±1.38 years, respectively. Lateral cephalometric cranial films of all subjects were taken in the same cephalostat in a habitual and unstrained body posture. Thirteen linear and 11 angular parameters were measured to describe the craniofacial characteristics of the subjects. RESULTS: Statistical analysis showed that there is no statistically significant difference between parameters of normal children and children with WD, with the exception of palatal plane inclination. The inclination of palatal plane was higher in children with WD than in normal subjects. CONCLUSIONS: Children with WD and healthy children have approximately the same dento-maxillo-facial structures. However, increased palatal plane inclination may be a finding of WD.
    International journal of pediatric otorhinolaryngology 06/2013; · 0.85 Impact Factor
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    ABSTRACT: Individuals often perform exercise in the fasted state, but the effects on bone metabolism are not currently known. We compared the effect of an overnight fast with feeding a mixed meal on the bone metabolic response to treadmill running. Ten, physically-active males aged 28±4y (mean±SD) completed two, counterbalanced, 8d trials. After 3d on a standardised diet, participants performed 60min of treadmill running at 65% VO(2max) on Day 4 following an overnight fast (FAST) or a standardised breakfast (FED). Blood samples were collected at baseline, before and during exercise, for 3h after exercise, and on four consecutive follow-up days (FU1-FU4). Plasma/serum were analysed for the c-terminal telopeptide region of collagen type 1 (β-CTX), n-terminal propeptides of procollagen type 1 (P1NP), osteocalcin (OC), bone alkaline phosphatase (bone ALP), parathyroid hormone (PTH), albumin-adjusted calcium, phosphate, osteoprotegerin (OPG), cortisol, leptin and ghrelin. Only the β-CTX response was significantly affected by feeding. Pre-exercise concentrations decreased more in FED compared with FAST (47% vs 26%, P<0.001) but increased during exercise in both groups and were not significantly different from baseline at 1h post-exercise. At 3h post-exercise, concentrations were decreased (33%, P<0.001) from baseline in FAST and significantly lower (P<0.001) than in FED. P1NP and PTH increased, and OC decreased during exercise. Bone markers were not significantly different from baseline on FU1-FU4. Fasting had only a minor effect on the bone metabolic response to subsequent acute, endurance exercise, reducing the duration of the increase in β-CTX during early recovery, but having no effect on changes in bone formation markers. The reduced duration of the β-CTX response with fasting was not fully explained by changes in PTH, OPG, leptin or ghrelin.
    Bone 08/2012; 51(6):990-9. · 3.82 Impact Factor

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