Ectopic calcification among families in the Azores: Clinical and radiologic manifestations in families with diffuse idiopathic skeletal hyperostosis and chondrocalcinosis

Objective. Twelve families that were multiply affected with diffuse idiopathic skeletal hyperostosis (DISH) and/or chondrocalcinosis, were identified on the island of Terceira, The Azores, potentially supporting the hypothesis that the 2 disorders share common etiopathogenic factors. The present study was undertaken to investigate this hypothesis. Methods. One hundred three individuals from 12 unrelated families were assessed. Probands were identified from patients attending the Rheumatic Diseases Clinic, Hospital de Santo Espirito, in The Azores. Family members were assessed by rheumatologists and radiologists. Radiographs of all family members were obtained, including radiographs of the dorsolumbar spine, pelvis, knees, elbows, and wrists, and all cases were screened for known features of chondrocalcinosis. Results. Ectopic calcifications were identified in 70 patients. The most frequent symptoms or findings were as follows: axial pain, elbow, knee and metacarpophalangeal (MCP) joint pain, swelling, and/or deformity, and radiographic enthesopathic changes. Elbow and MCP joint periarticular calcifications were observed in 35 and 5 patients, respectively, and chondrocalcinosis was identified in 12 patients. Fifteen patients had sacroiliac disease (ankylosis or sclerosis) on computed tomography scans. Fifty-two patients could be classified as having definite (17%), probable (26%), or possible (31%) DISH. Concomitant DISH and chondrocalcinosis was diagnosed in 12 patients. Pyrophosphate crystals were identified from knee effusions in 13 patients. The pattern of disease transmission was compatible with an autosomal-dominant monogenic disease. The mean age at which symptoms developed was 38 years. Conclusion. These families may represent a familial type of pyrophosphate arthropathy with a phenotype that includes peripheral and axial enthesopathic calcifications. The concurrence of DISH and chondrocalcinosis suggests a shared pathogenic mechanism in the 2 conditions.

Bone properties and skeletal maturity in adolescent males, as assessed by quantitative ultrasound

ABSTRACT Background: Previous studies have implied that weight-bearing, intense and prolonged physical activities optimize bone accretion during the grow^ing years. The majority of past inquiries have used dual-energy X-ray absorptiometry (DXA) to examine bone strength and hand-wrist radiography to determine skeletal maturity in children. Recently, quantitative ultrasound (QUS) technologies have been developed to examine bone properties and skeletal maturity in a safe, noninvasive and cost-effective manner. Objective: The purpose of this study was to compare bone properties and skeletal maturity in competitive male child and adolescent athletes with minimallyactive, age-matched controls, using QUS technology. >. Methods: In total, 224 males were included in the study. The 115 pre-pubertal boys aged 10-12 years consisted of control, minimally-active children (n=34), soccer players (n=26), gymnasts (n=25) and hockey players (n=30). In addition, the 109 late-pubertal boys aged 14-16 years consisted of control, minimally-active adolescents (n=31), soccer players (n=30), gymnasts (n=17) and hockey players (n=31). The athletic groups were elite level players that predominantly trained year-round. Physical activity, nutrition and sports participation were assessed with various questionnaires. Anthropometries, such as height, weight and relative body fat percentage (BF%) were assessed using standard measures. Skeletal strength and age were evaluated using bone QUS. Lastly, salivary testosterone (sT) concentration was measured using Radioimmunoassay (RIA). Results: Within each age group, there were no significant differences between the activity groups in age and pubertal stage. An age effect was apparent in all variables, as expected. A sport effect was noted in all physical characteristics: the child and adolescent gymnasts were shorter and lighter than other sports groups. Adiposity was greater in the controls and in the hockey players. All child subjects were pubertal stage (fanner) I or II, while adolescent subjects were pubertal stage IV or V. There were no differences in daily energy and mineral intakes between sports groups. In both age groups, gymnasts had a higher training volume than other athletic groups. Bone speed of sound (50s) was higher in adolescents compared with the children. Gymnasts had signifieantly higher radial 50S than controls, hockey and soccer players in both age cohorts. Hockey athletes also had higher radial 50S than controls and soccer players in the child and adolescent groups, respectiyely. Child gymnasts and soccer players had greater tibial 50S compared with the hockey players and control groups. Likewise, adolescent gymnasts and soccer players had higher tibial SoS compared with the control group. No interaction was apparent between age and type of activity in any of the bone measures. » Lastly, maturity as assessed by sT and secondary sex characteristics (Tanner stage) was not different between sports group within each age group. Despite the similarity in chronological age, androgen levels and sexual maturity, differences between activity groups were noted in skeletal maturity. In the younger group, hockey players had the highest bone age while the soccer players had the lowest bone age. In the adolescent group, gymnasts and hockey players were characterized by higher skeletal maturity compared with controls. An interaction between the age and sport type effects was apparent in skeletal maturity, reflecting the fact that among the children, the soccer players were significantly less mature than the rest of the groups, while in the adolescents, the controls were the least skeletally mature. Summary and Conclusions: In summary, radial and tibial SOS are enhanced by the unique loading pattern in each sport (i.e, upper and lower extremities in gymnastics, lower extremities in soccer), with no cumulative effect between childhood and adolescence. That is, the effect of sport participation on bone SOS was apparent already among the young athletes. Enhanced bone properties among athletes of specific sports suggest that participation in these sports can improve bone strength and potential bone health.

Comparative analysis between three methods of bone estimating age in individuals with down syndrome by mode of the hand and wrist ray

The wrist and hand region has been the most commonly used for estimating age and osseous development due to the great number of ossification centers. The aim was to determine which method, Tanner & Whitehouse's (TW3), Greulich & Pyle's (GP) or Eklof & Ringertz's, more closely relates to the chronological age in subjects with Down syndrome with chronological ages between 61 and 180 months, using wrist and hand radiographs. The sample consisted of 85 radiographs, 52 of males and 33 of females. Eklof & Ringertz's method was computerized (Radiomemory). Greulich & Pyle's atlas was used and compared with the wrist and hand radiographs. For the TW3 method, 13 ossification centers were evaluated; for each one of them, there are seven or eight development stages to which scores are assigned; these scores are then added and the results are transformed into osseous age values. No statistically significant differences were observed between the male and female genders for methods TW3 and GP, contrasting with the observed differences for the Eklof & Ringertz method. Correlation (r2) between osseous and chronological ages was 0.8262 for TW3 and 0.7965 for GP, while for the method of Eklof & Ringertz, it was 0.7656 for females and 0.8353 for males. The author concluded that the osseous age assessment method that better related to the chronological age was the TW3, followed by Greulich & Pyle's and Eklof & Ringertz's.

Correlation between hand/wrist and panoramic radiographs in severe secondary hyperparathyroidism

Objectives: Hand/wrist and dental radiographs are important for osteoporosis analysis in secondary hyperparathyroidism (SHPT). This study evaluated whether a correlation exists between the effects of the disease on the hands and jaws, and investigated the association between osteoporosis progression in the hands and parathyroid hormone (PTH) levels in chronic kidney disease (CKD) patients. Materials and methods: Four panoramic radiographic parameters (mental index, mandibular cortical index, trabecular bone pattern, and calcification/resorption) and four corresponding hand/wrist radiographic parameters (metacarpal cortical thickness, phalangeal cortical index, trabecular bone pattern, and calcification/resorption) were applied to investigate possible correlation between the effects of SHPT on the jaws and hands/wrists, by Spearman's correlation coefficient. PTH levels and the hand/wrist radiographic parameters were also tested by spearman's correlation coefficient (p < 0.05). The presence of brown tumors, vascular calcifications, and acroosteolysis on the hands was also evaluated. Results: Mandibular cortical index was strongly correlated with the phalangeal cortical index (p = 0.000). Phalangeal cortical index and trabecular bone pattern of hand/wrist correlated with PTH levels (0.002 and 0.000, respectively). Brown tumors occurred in four CKD patients, while both vascular calcifications and acroosteolysis were observed in 19 patients. Conclusion: There is a significant correlation between the morphological changes caused by secondary hyperparathyroidism in hand and jaw bones. The morphological status can be assessed using the mandibular cortical index, besides the phalangeal cortical index. The latter correlates well with parathyroid hormone levels of advanced chronic kidney disease. Clinical relevance: Panoramic images reveal morphological changes in the jaw bone, indicating likewise changes in the hand/wrist in severe secondary hyperparathyroidism. The severity of the bone changes may be a reflection of the parathyroid hormone levels in advanced chronic kidney disease. © 2012 Springer-Verlag.

Use of condition-specific patient-reported outcome measures in clinical trials among patients with wrist osteoarthritis : a systematic review

Background. This paper aimed to identify condition-specific patient-reported outcome measures used in clinical trials among people with wrist osteoarthritis and summarise empirical peer-reviewed evidence supporting their reliability, validity, and responsiveness to change. Methods. A systematic review of randomised controlled trials among people with wrist osteoarthritis was undertaken. Studies reporting reliability, validity, or responsiveness were identified using a systematic reverse citation trail audit procedure. Psychometric properties of the instruments were examined against predefined criteria and summarised. Results. Thirteen clinical trials met inclusion criteria. The most common patient-reported outcome was the disabilities of the arm, shoulder, and hand questionnaire (DASH). The DASH, the Michigan Hand Outcomes Questionnaire (MHQ), the Patient Evaluation Measure (PEM), and the Patient-Reported Wrist Evaluation (PRWE) had evidence supporting their reliability, validity, and responsiveness. A post-hoc review of excluded studies revealed the AUSCAN Osteoarthritis Hand Index as another suitable instrument that had favourable reliability, validity, and responsiveness. Conclusions. The DASH, MHQ, and AUSCAN Osteoarthritis Hand Index instruments were supported by the most favourable empirical evidence for validity, reliability, and responsiveness. The PEM and PRWE also had favourable empirical evidence reported for these elements. Further psychometric testing of these instruments among people with wrist osteoarthritis is warranted.

Comparison between electrically-evoked and voluntary wrist movements on sensorimotor and prefrontal cortical activation : A multi-channel time domain fNIRS study

Neuromuscular electrical stimulation (NMES) has been consistently demonstrated to improve skeletal muscle function in neurological populations with movement disorders, such as poststroke and incomplete spinal cord injury (Vanderthommen and Duchateau, 2007). Recent research has documented that rapid, supraspinal central nervous system reorganisation/neuroplastic mechanisms are also implicated during NMES (Chipchase et al., 2011). Functional neuroimaging studies have shown NMES to activate a network of sub-cortical and cortical brain regions, including the sensorimotor (SMC) and prefrontal (PFC) cortex (Blickenstorfer et al., 2009; Han et al., 2003; Muthalib et al., 2012). A relationship between increase in SMC activation with increasing NMES current intensity up to motor threshold has been previously reported using functional MRI (Smith et al., 2003). However, since clinical neurorehabilitation programmes commonly utilise NMES current intensities above the motor threshold and up to the maximum tolerated current intensity (MTI), limited research has determined the cortical correlates of increasing NMES current intensity at or above MTI (Muthalib et al., 2012). In our previous study (Muthalib et al., 2012), we assessed contralateral PFC activation using 1-channel functional near infrared spectroscopy (fNIRS) during NMES of the elbow flexors by increasing current intensity from motor threshold to greater than MTI and showed a linear relationship between NMES current intensity and the level of PFC activation. However, the relationship between NMES current intensity and activation of the motor cortical network, including SMC and PFC, has not been clarified. Moreover, it is of scientific and clinical relevance to know how NMES affects the central nervous system, especially in comparison to voluntary (VOL) muscle activation. Therefore, the aim of this study was to utilise multi-channel time domain fNIRS to compare SMC and PFC activation between VOL and NMESevoked wrist extension movements.

Positive magnetic resonance imaging findings in the asymptomatic wrist

BACKGROUND: Magnetic resonance imaging (MRI) is being increasingly utilized to define pathology and guide treatment in patients presenting with wrist pain. The clinical relevance of MRI identified or confirmed pathology has not been established, and the prevalence of asymptomatic MRI pathology is not known. METHODS: Twenty volunteers with no previous wrist injury or symptoms underwent bilateral MRI wrist studies in this exploratory diagnostic study. The scans were reported by an experienced musculoskeletal radiologist and an experienced wrist surgeon, with a consensus reached on each report. RESULTS: There were 3.15 positive MRI findings per wrist. There were 126 positive findings (range 1-6 per wrist). Sixty-eight ganglia were identified. Eleven ligament tears or perforations were also identified. Increased joint fluid was seen at many sites, most frequently adjacent to the piso-triquetral joint. CONCLUSION: The accuracy of MRI in identifying triangular fibrocartilage complex tears, intercarpal ligament tears and carpal bone osteonecrosis is rapidly being refined. Positive MRI findings are common and may be coincidental in patients with wrist pain. MRI findings need to be correlated closely with clinical examination and history.

Machine learning for activity recognition : hip versus wrist data

Problem addressed Wrist-worn accelerometers are associated with greater compliance. However, validated algorithms for predicting activity type from wrist-worn accelerometer data are lacking. This study compared the activity recognition rates of an activity classifier trained on acceleration signal collected on the wrist and hip. Methodology 52 children and adolescents (mean age 13.7 +/- 3.1 year) completed 12 activity trials that were categorized into 7 activity classes: lying down, sitting, standing, walking, running, basketball, and dancing. During each trial, participants wore an ActiGraph GT3X+ tri-axial accelerometer on the right hip and the non-dominant wrist. Features were extracted from 10-s windows and inputted into a regularized logistic regression model using R (Glmnet + L1). Results Classification accuracy for the hip and wrist was 91.0% +/- 3.1% and 88.4% +/- 3.0%, respectively. The hip model exhibited excellent classification accuracy for sitting (91.3%), standing (95.8%), walking (95.8%), and running (96.8%); acceptable classification accuracy for lying down (88.3%) and basketball (81.9%); and modest accuracy for dance (64.1%). The wrist model exhibited excellent classification accuracy for sitting (93.0%), standing (91.7%), and walking (95.8%); acceptable classification accuracy for basketball (86.0%); and modest accuracy for running (78.8%), lying down (74.6%) and dance (69.4%). Potential Impact Both the hip and wrist algorithms achieved acceptable classification accuracy, allowing researchers to use either placement for activity recognition.

'A slap on the wrist'? The conservative agenda in Queensland, Australia

Recent amendments to youth justice legislation in Queensland include opening Children’s Court proceedings, removing the Principle of Detention as a Last Resort, facilitating transfers of 17 year-old offenders to adult prisons, instigating new bail offences, and introducing mandatory boot camp orders. This article examines the context of these changes including the inadequacies of the public policy process, and the impassioned political rhetoric imbued with simplistic slogans. This is a case study of regressive youth justice policy and the article reflects on the many causes underlying the reactive winding back of reform.

Feasibility and acceptability of wrist actigraph in assessing sleep quality and sleep quantity: A home-based pilot study in healthy volunteers

Purpose This study aimed to determine the feasibility and acceptability of actigraphy to monitor sleep quality and quantity in healthy self-rated good sleeper adults at home-based settings. Method Sixteen healthy volunteers (age > 18) were invited to participate. Each participant was provided with a wrist actigraph device to be worn for 24-hour/day for seven consecutive days to monitor their sleep-wake patterns. Actigraphy data were downloaded using-proprietary software to generate an individual-sleep report. Participants also completed a set of self-reported Health Related Quality of Life (HRQOL) using WHO (five) Well Being Index (WBI) questionnaires. Results Actigraphy was well accepted by all participants. Only 43.8% of the participants achieved normal total sleep time (TST) and 62.5% had a mean sleep efficiency value below the normal range. Despite a reduced quality of sleep among the participants, the self-reported HRQOL scores produced by the WHO-5 WBI showed a “fair” to “good” among the participants. Conclusions To maintain healthy well-being, it is vital to have efficient and quality sleep. Insufficient and poor sleep may contribute to various health problems and hazardous outcomes. People often believe they have normal and efficient sleep, not realising they may be developing poor sleep habits. This study found that actigraphy can be easily utilized to monitor sleep-wake patterns at home-based settings. We proposed that actigraphy could be adapted for use in the primary care settings (e.g. community pharmacy) to improve the sleep health management in the community.

Transnationalism and the Karen wrist-tying ceremony: An ethnographic account of Karen settlement practice in Brisbane

When settling, people often use cultural schema from their original homeland to build familiarity in unfamiliar surrounds. This paper draws on ethnographic fieldwork conducted by the first author in Brisbane, with the Karen community from Burma, during which participant observation and interview methods were used. We present an ethnographic account of the Brisbane Karen wrist-tying ceremony. The ceremony acts as an insight into the challenges for Karen whilst settling into Australia. It reflects multiple accounts of history and tradition, but simultaneously speaks to emerging, contemporary Karen contexts. This research contributes to richer understandings of settlement: it frames transnational cultural practice as a flexible mode of integration, rather than an exclusionary mode of othering. We propose that the integrative discourse of the ceremony creates familiarity and social connection in local and diasporic spaces. This acts as a counter to the challenges of Karen settlement including the negotiations of local/global identity politics.

Bone health in children with inflammatory bowel disease : adjusting for bone age

OBJECTIVES: Clinical results of bone mineral density for children with inflammatory bowel disease are commonly reported using reference data for chronological age. It is known that these children, particularly those with Crohn disease, experience delayed growth and maturation. Therefore, it is more appropriate to compare clinical results with bone age rather than chronological age. MATERIALS AND METHODS: Areal bone mineral density (aBMD) was measured using dual energy x-ray absorptiometry, and bone age was assessed using the Tanner-Whitehouse 3 method from a standard hand/wrist radiograph. Results were available for 44 children ages 7.99 to 16.89 years. Areal bone mineral density measurements were converted to z scores using both chronological and bone ages for each subject. RESULTS: Areal bone mineral density z scores calculated using bone age, as opposed to chronological age, were significantly improved for both the total body and lumbar spine regions of interest. When subjects were grouped according to diagnosis, bone age generated z scores remained significantly improved for those with Crohn disease but not for those diagnosed with ulcerative colitis. Grouping of children with Crohn disease into younger and older ages produced significantly higher z scores using bone age compared with chronological for the older age group, but not the younger age group. CONCLUSIONS: Our findings, in accordance with those presented in the literature, suggest that aBMD results in children with Crohn disease should include the consideration of bone age, rather than merely chronological age. Bone size, although not as easily available, would also be an important consideration for interpreting results in paediatric populations. © 2009 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Novel ANKH amino terminus mutation (Pro5Ser) associated with early-onset calcium pyrophosphate disease with associated phosphaturia

This report describes a 32-year-old woman presenting since childhood with progressive calcium pyrophosphate disease (CPPD), characterized by severe arthropathy and chondrocalcinosis involving multiple peripheral joints and intervertebral disks. Because ANKH mutations have been previously described in familial CPPD, the proband's DNA was assessed at this locus by direct sequencing of promoter and coding regions and revealed 3 sequence variants in ANKH. Sequences of exon 1 revealed a novel isolated nonsynonymous mutation (c.13 C>T), altering amino acid in codon 5 from proline to serine (CCG>TCG). Sequencing of parental DNA revealed an identical mutation in the proband's father but not the mother. Subsequent clinical evaluation demonstrated extensive chondrocalcinosis and degenerative arthropathy in the proband's father. In summary, we report a novel mutation, not previously described, in ANKH exon 1, wherein serine replaces proline, in a case of early-onset severe CPPD associated with metabolic abnormalities, with similar findings in the proband's father.

The validity of the GENEActiv wrist-worn accelerometer for measuring adult sedentary time in free living

OBJECTIVES Based on self-reported measures, sedentary time has been associated with chronic disease and mortality. This study examined the validity of the wrist-worn GENEactiv accelerometer for measuring sedentary time (i.e. sitting and lying) by posture classification, during waking hours in free living adults. DESIGN Fifty-seven participants (age=18-55 years 52% male) were recruited using convenience sampling from a large metropolitan Australian university. METHODS Participants wore a GENEActiv accelerometer on their non-dominant wrist and an activPAL device attached to their right thigh for 24-h (00:00 to 23:59:59). Pearson's Correlation Coefficient was used to examine the convergent validity of the GENEActiv and the activPAL for estimating total sedentary time during waking hours. Agreement was illustrated using Bland and Altman plots, and intra-individual agreement for posture was assessed with the Kappa statistic. RESULTS Estimates of average total sedentary time over 24-h were 623 (SD 103) min/day from the GENEActiv, and 626 (SD 123) min/day from the activPAL, with an Intraclass Correlation Coefficient of 0.80 (95% confidence intervals 0.68-0.88). Bland and Altman plots showed slight underestimation of mean total sedentary time for GENEActiv relative to activPAL (mean difference: -3.44min/day), with moderate limits of agreement (-144 to 137min/day). Mean Kappa for posture was 0.53 (SD 0.12), indicating moderate agreement for this sample at the individual level. CONCLUSIONS The estimation of sedentary time by posture classification of the wrist-worn GENEActiv accelerometer was comparable to the activPAL. The GENEActiv may provide an alternative, easy to wear device based measure for descriptive estimates of sedentary time in population samples