Correction to: Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Netherlands.

Correction to:Neth Heart J 2016 https://doi.org/10.1007/s12471-016-0849-z Unfortunately the original version of this article contained Electronic Supplementary Material which should not have been published with the article due to copyright reasons.The original version has been updated and the ESM ….

Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Netherlands.

BACKGROUND: The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the first disease-specific instrument for pulmonary arterial hypertension (PAH) to assess patient-perceived symptoms, activity limitations and quality of life. To be able to use this questionnaire in the Netherlands, the aim of the study was to translate and validate this instrument for the Dutch-speaking population. METHODS: First the CAMPHOR was translated into Dutch (by means of a bilingual and a lay panel) and field-tested by means of cognitive debriefing interviews with ten PAH patients. For psychometric evaluation, 80 patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH) were asked to complete the CAMPHOR twice over a two-week period. To test for construct validity, participants also completed the Nottingham Health Profile (NHP). RESULTS: The Dutch version of the CAMPHOR showed high internal consistency for all scales (Cronbach's alpha 0.89-0.91) and excellent reproducibility over two weeks (reliability coefficients 0.87-0.91). Concurrent validity showed that the CAMPHOR scales correlated as expected with the NHP scales. The CAMPHOR was able to distinguish between patient groups based on self-reported general health status, disease severity and NYHA classification demonstrating evidence of known group validity. The CAMPHOR activity limitations scale correlated moderately with the distance walked during the 6­minute walk test (r = -0.47, p < 0.01) and the symptoms scale with the Borg dyspnoea score (r = 0.51, p < 0.01). CONCLUSION: The Dutch version of the CAMPHOR is a reliable and valid measure of quality of life and health status in patients with PAH and CTEPH is recommended for use in routine care and in clinical research.

Assessing the feasibility of a web-based registry for multiple orphan lung diseases: the Australasian Registry Network for Orphan Lung Disease (ARNOLD) experience.

BACKGROUND: We investigated the feasibility of using an online registry to provide prevalence data for multiple orphan lung diseases in Australia and New Zealand. METHODS: A web-based registry, The Australasian Registry Network of Orphan Lung Diseases (ARNOLD) was developed based on the existing British Paediatric Orphan Lung Disease Registry. All adult and paediatric respiratory physicians who were members of the Thoracic Society of Australia and New Zealand in Australia and New Zealand were sent regular emails between July 2009 and June 2014 requesting information on patients they had seen with any of 30 rare lung diseases. Prevalence rates were calculated using population statistics. RESULTS: Emails were sent to 649 Australian respiratory physicians and 65 in New Zealand. 231 (32.4%) physicians responded to emails a total of 1554 times (average 7.6 responses per physician). Prevalence rates of 30 rare lung diseases are reported. CONCLUSIONS: A multi-disease rare lung disease registry was implemented in the Australian and New Zealand health care settings that provided prevalence data on orphan lung diseases in this region but was limited by under reporting.

Applying Rasch analysis to evaluate measurement equivalence of different administration formats of the Activity Limitation scale of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR).

BACKGROUND: Electronic formats of patient-reported outcome (PRO) measures are now routinely used in clinical research studies. When changing from a validated paper and pen to electronic administration it is necessary to establish their equivalence. This study reports on the value of Rasch analysis in this process. METHODS: Three groups of US pulmonary hypertension (PH) patients participated. The first completed an electronic version of the CAMPHOR Activity Limitation scale (e-sample) and this was compared with two pen and paper administrated samples (pp1 and pp2). The three databases were combined and analysed for fit to the Rasch model. Equivalence was evaluated by differential item functioning (DIF) analyses. RESULTS: The three datasets were matched randomly in terms of sample size (n = 147). Mean age (years) and percentage of male respondents were as follows: e-sample (51.7, 16.0 %); pp1 (50.0, 14.0 %); pp2 (55.5, 40.4 %). The combined dataset achieved fit to the Rasch model. Two items showed evidence of borderline DIF. Further analyses showed the inclusion of these items had little impact on Rasch estimates indicating the DIF identified was unimportant. CONCLUSIONS: Differences between the performance of the electronic and pen and paper administrations of the CAMPHOR Activity Limitation scale were minor. The results were successful in showing how the Rasch model can be used to determine the equivalence of alternative formats of PRO measures.

Co-calibrating quality-of-life scores from three pulmonary disorders: implications for comparative-effectiveness research.

Background Efficient use of health resources requires accurate outcome assessment. Disease-specific patient-reported outcome (PRO) measures are designed to be highly relevant to patients with a specific disease. They have advantages over generic PROs that lack relevance to patient groups and miss crucial impacts of illness. It is thought that disease-specific measurement cannot be used in comparative effectiveness research (CER). The present study provides further evidence of the value of disease-specific measures in making valid comparisons across diseases. Methods The Asthma Life Impact Scale (ALIS, 22 items), Living with Chronic Obstructive Pulmonary Disease (LCOPD, 22 items) scale, and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR, 25 items) were completed by 140, 162, and 91 patients, respectively. The three samples were analyzed for fit to the Rasch model, then combined into a scale consisting of 58 unique items and re-analyzed. Raw scores on the three measures were co-calibrated and a transformation table produced. Results The scales fit the Rasch model individually (ALIS Chi(2) probability value (p-Chi(2)) = 0.05; LCOPD p-Chi(2 )=( )0.38; CAMPHOR p-Chi(2 )=( )0.92). The combined data also fit the Rasch model (p-Chi(2 )=( )0.22). There was no differential item functioning related to age, gender, or disease. The co-calibrated scales successfully distinguished between perceived severity groups (p < 0.001). Limitations The samples were drawn from different sources. For scales to be co-calibrated using a common item design, they must be based on the same theoretical construct, be unidimensional, and have overlapping items. Conclusions The results showed that it is possible to co-calibrate scores from disease-specific PRO measures. This will permit more accurate and sensitive outcome measurement to be incorporated into CER. The co-calibration of needs-based disease-specific measures allows the calculation of γ scores that can be used to compare directly the impact of any type of interventions on any diseases included in the co-calibration.

Comparing the impact of psoriasis and atopic dermatitis on quality of life: co-calibration of the PSORIQoL and QoLIAD.

BACKGROUND: Disease-specific patient-reported outcome (PRO) measures are designed to be highly relevant to one disease. It is widely believed that comparisons of outcomes between patients with different diseases are only possible using generic measures. The present study employs a novel method of using Rasch analysis to co-calibrate scores from different disease-specific PRO measures, allowing scores to be compared across diseases. METHODS: Psoriasis patients (n = 146, mean age = 44.4, males = 50 %) completed the Psoriasis Quality of Life scale (PSORIQoL) and atopic dermatitis patients (n = 146, mean age = 45.5, males = 50 %) the Quality of Life in Atopic Dermatitis scale (QoLIAD). Both measures employ the needs-based model of QoL, and they share five common items-providing a link between assessments. The groups were analysed separately, and then combined to test fit to the Rasch model. RESULTS: Both scales showed good fit to the Rasch model after minor adjustments (PSORIQoL: χ (2) p = 0.25; QoLIAD: χ (2) p = 0.51). For the combined dataset, one common item showing differential item functioning by disease was removed and fit to the Rasch model was achieved (χ (2) p = 0.08). The co-calibrated scale successfully distinguished between perceived severity groups (p < 0.001). CONCLUSIONS: It is possible to co-calibrate scores on the PSORIQoL and QoLIAD. This is one of the first studies in health research to demonstrate how Rasch analysis can be used to make comparisons across diseases using different disease-specific measures. Such an approach maintains the greater relevance and, consequently, accuracy associated with disease-specific measurement.

Greek adaptation and validation of the Ankylosing Spondylitis Quality of Life (ASQoL) measure.

BACKGROUND: Ankylosing Spondylitis (AS) is a chronic rheumatic disease that has a significant impact on patient's quality of life (QoL). The Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire is a disease-specific patient-reported outcome measure for assessing QoL in AS. While the ASQoL has been adapted for use in 46 countries worldwide, a Greek language version of the measure has not been available and was required for an international clinical trial. AIM: The aim was to develop and assess the psychometric properties of a Greek language version of the ASQoL. METHODS: The adaptation of the ASQoL into Greek involved three procedures: translation, assessment of face and content validity, and formal validation. The measure was translated into Greek using two translation panels. Cognitive debriefing interviews were employed to determine face and content validity. Finally, the translation's psychometric properties were examined by administering it on two occasions, with a 14-day interval. The Nottingham Health Profile (NHP) was used as a comparator measure. RESULTS: The ASQoL proved straightforward to translate into Greek and interviewees found it relevant, comprehensible and easy to complete.  The measure had good internal consistency (α =0.92) and test-retest reliability (r =0.98). Predicted correlations with the NHP provided evidence of the convergent validity of the two measures. Construct validity was confirmed by the measure's ability to distinguish groups of AS patients varying by perceived disease severity and general health. CONCLUSIONS: The Greek ASQoL has been shown to be well-accepted, reliable and valid and can be recommended for use in clinical studies and routine clinical practice in AS. Hippokratia 2015; 19 (2):119-124.

Adapting the Asthma Life Impact Scale (ALIS) for use in Southern European (Italian) and Eastern European (Russian) cultures.

BACKGROUND: The Asthma Life Impact Scale (ALIS) is a disease-specific measure used to assess the quality-of-life of people with asthma. It was developed in the UK and US and has proven to be acceptable to patients, to have good psychometric properties, and to be unidimensional. OBJECTIVE: This paper reports on the adaptation and validation of the ALIS for use in representative Southern European (Italian) and Eastern European (Russian) languages. METHODS: The ALIS was translated for both cultures using the dual-panel process. The newly translated versions were then tested with asthma patients to ensure face and content validity. Psychometric properties of the new language versions were assessed via a test?re-test postal survey conducted in both countries. LIMITATIONS: It is possible that some cultural or language differences still exist between the different language versions. Further research should be undertaken to determine responsiveness. Further studies designed to determine the clinical validity of the Italian ALIS would be valuable. RESULTS: Linguistic nuances were easily resolved during the translation process for both language adaptations. Cognitive debriefing interviews (Russia n=9, male=11.1%, age mean (SD)=55.4 (13.2); Italy n=15, male=66.7%, age mean (SD)=63.5 (11.2)) indicated that the ALIS was easy to read and acceptable to patients. Psychometric testing was conducted on the data (Russia n=61, age mean (SD)=40.7 (15.4); Italy n=71, male=42.6%, age mean (SD)=49.5 (14.1)). The results showed that the new versions of the ALIS were consistent (Russian and Italian Cronbach's alpha=0.92) and reproducible (Russian test-re-test=0.86; Italian test-re-test=0.94). The Italian adaptation showed the expected correlations with the NHP and the Russian adaptation showed strong correlations with the CASIS and CAFS and weak-to-moderate correlations with %FEV1 and %PEF. In both adaptations the ALIS was able to distinguish between participants based on self-reported general health, self-reported severity, and whether or not they were hospitalized in the previous week.

The effect of weight training on bone mineral density and bone turnover in postmenopausal breast cancer survivors with bone loss: a 24-month randomized controlled trial.

SUMMARY: This study examined whether 24 months of weight training exercises enhanced the effectiveness of risedronate, calcium, and vitamin D in maintaining or improving bone mineral density (BMD) in 223 postmenopausal breast cancer survivors. Subjects who were > or =50% adherent to exercise had no improvement in BMD but were less likely to lose BMD. INTRODUCTION: This study examined whether (1) postmenopausal breast cancer survivors (BCS) with bone loss taking 24 months of risedronate, calcium, and vitamin D had increased bone mineral density (BMD) at the total hip, femoral neck, L1-L4 spine, total radius and 33% radius, and decreased bone turnover; (2) subjects who also participated in strength/weight training (ST) exercises had greater increases in BMD and greater decreases in bone turnover; and (3) subjects who also exercised were more likely to preserve (at least maintain) BMD. METHODS: Postmenopausal BCS (223) were randomly assigned to exercise plus medication or medication only groups. Both groups received 24 months of 1,200 mg of calcium and 400 IU of vitamin D daily and 35 mg of risedronate weekly, and the exercise group additionally had ST exercises twice weekly. RESULTS: After 24 months, women who took medications without exercising had significant improvements in BMD at the total hip (+1.81%) and spine (+2.85%) and significant decreases in Alkphase B (-8.7%) and serum NTx (-16.7%). Women who also exercised had additional increases in BMD at the femoral neck (+0.29%), total hip (+0.34%), spine (+0.23%), total radius (+0.30%), and additional decreases in Alkphase B (-2.4%) and Serum NTx (-6.5%). Additional changes in BMD and bone turnover with exercise were not significant. Subjects who were > or =50% adherent to exercise were less likely to lose BMD at the total hip (chi-square [1] = 4.66, p = 0.03) and femoral neck (chi-square [1] = 4.63, p = 0.03). CONCLUSION: Strength/weight training exercises may prevent loss of BMD in postmenopausal BCS at risk for bone loss.

The development and validation of the Unidimensional Fatigue Impact Scale (U-FIS).

BACKGROUND: The multidimensional assessment of fatigue is complicated by the interrelation of its multiple causes and effects. OBJECTIVE: The purpose of the research was to develop a unidimensional assessment of fatigue (U-FIS). METHODS: Data collected with the Fatigue Impact Scale (FIS) were subjected to Rasch analysis to identify potential problems with the scale. Additional items for the U-FIS were generated from interviews with UK MS patients. The U-FIS was tested for face and content validity in patient interviews and included in a validation survey to determine dimensionality (Rasch model), reliability and validity. RESULTS: The original FIS was not unidimensional when subscale items were combined. The modification of the FIS and addition of a number of items allowed the development of a 22-item unidimensional scale (U-FIS) that was reliable (Cronbach Alpha = 0.96; test-retest = 0.86,) and valid given correlations with the Nottingham Health Profile and ability to distinguish between MS severity groups. There was no significant difference in U-FIS scores according to MS type. CONCLUSION: It is valid to conceptualize the functional impact of fatigue as unidimensional. The U-FIS is a reliable and valid questionnaire that will allow the measurement of this construct in clinical studies.

The development of patient-reported outcome indices for multiple sclerosis (PRIMUS).

BACKGROUND: Complex diseases such as multiple sclerosis (MS) present dilemmas over the choice of patient-reported outcome measures as no single scale can inform on all types of MS impact from the patient's perspective. OBJECTIVE: To develop an outcome tool, the Patient-Reported Indices for Multiple Sclerosis (PRIMUS), to assess MS symptoms, activities, and quality of life. METHODS: PRIMUS content was derived from qualitative interviews with UK MS patients and checked by clinical experts. Semi-structured cognitive debriefing interviews assessed scale face and content validity. PRIMUS scaling properties, reliability, and construct validity were assessed by a test-retest postal survey. RESULTS: Cognitive debriefing interviews (n = 15) demonstrated scale clarity, relevance, and comprehensiveness. The postal survey was completed by 135 patients with MS. After removal of misfitting items and those exhibiting differential item functioning, all scales fitted the Rasch model, confirming unidimensionality. For all scales, test-retest reliability exceeded 0.80. Scale scores were related to perceived MS severity, general health, and symptoms of depression. Moderate correlations were observed between PRIMUS and Nottingham Health Profile scores. CONCLUSIONS: Clinicians and researchers can have confidence in scores obtained by respondents on the PRIMUS. The PRIMUS will aid the assessment of the impact of MS from the patient's perspective.

Longitudinal pulmonary function of childhood bronchiectasis and comparison with cystic fibrosis.

BACKGROUND: Little has been published on the progression of non-cystic fibrosis bronchiectasis (BX), especially in childhood. Data are needed for prognosis and evaluation of the effectiveness of treatments. A study was undertaken to evaluate the change in lung function over time in children with BX, and to consider covariates and compare them with the local cystic fibrosis (CF) population. METHODS: Children with BX or CF and > or =3 calendar years of lung function data were identified from hospital clinics. Diagnosis was made by high resolution CT scans, sweat tests, and genetic studies. Lung function performed on a single plethysmograph between 6 and 15 years of age and > or =6 weeks after diagnosis was analysed longitudinally (linear mixed model). The impact of reference equation and "best annual" versus "all data" approaches were evaluated. RESULTS: There were 44 children in each of the BX and CF groups with an overall mean 5.7 calendar years follow up data. The estimated forced expiratory volume in 1 second (FEV(1)) in the BX group had an intercept of 68% predicted (Polgar) at 10 years of age which fell at a rate of 1.9% per annum using "best annual" data compared with 63% and 0.9% using "all data". Those with post-infectious BX or chronic Haemophilus influenzae infection had more severe disease. In CF the FEV(1) ("best annual") intercept was 85% predicted with a slope of -2.9% per annum. The choice of reference equation affected the magnitude of the result but not the conclusions. CONCLUSION: Children with BX have significant airway obstruction which deteriorates over time, regardless of analysis strategy or reference. Effective interventions are needed to prevent significant morbidity and adult mortality.

New Zealand national incidence of bronchiectasis "too high" for a developed country.

AIMS: To prospectively estimate the incidence of bronchiectasis among New Zealand (NZ) children, to consider aetiology and severity, and to evaluate regional and ethnic variation. METHODOLOGY: NZ paediatricians were surveyed monthly for new cases of bronchiectasis during 2001 and 2002 via the NZ paediatric surveillance unit (with coverage of >94% of NZ paediatricians). Notified cases had their computed tomography scans reviewed and scored for severity. Confirmed cases were followed up by postal questionnaire one year after diagnosis. Demographic, aetiological, and severity data were collected. RESULTS: Ninety nine notifications were received. Sixty five cases were confirmed. An overall incidence of 3.7 per 100,000 under 15 year old children per year was estimated. Incidence was highest in Pacific children at 17.8 compared with 4.8 in Maori, 1.5 in NZ European, and 2.4 other per 100,000 per year. Incidence varied significantly by region. The median age at diagnosis was 5.2 years; the majority had symptoms for more than two years. Eighty three per cent had bilateral disease, with a median of three lobes affected, mean FEV1 of 77% predicted, and modified Bhalla HRCT score of 18. CONCLUSIONS: The incidence of bronchiectasis is high in NZ children, nearly twice the rate for cystic fibrosis and seven times that of Finland, the only other country reporting a childhood national rate. Incidence varied substantially between ethnicities. Most cases developed disease in early childhood and had delayed diagnosis.

A functional role for intra-axonal protein synthesis during axonal regeneration from adult sensory neurons.

Although intradendritic protein synthesis has been documented in adult neurons, the question of whether axons actively synthesize proteins remains controversial. Adult sensory neurons that are conditioned by axonal crush can rapidly extend processes in vitro by regulating the translation of existing mRNAs (Twiss et al., 2000). These regenerating processes contain axonal but not dendritic proteins. Here we show that these axonal processes of adult sensory neurons cultured after conditioning injury contain ribosomal proteins, translational initiation factors, and rRNA. Pure preparations of regenerating axons separated from the DRG cell bodies can actively synthesize proteins in vitro and contain ribosome-bound beta-actin and neurofilament mRNAs. Blocking protein synthesis in these regenerating sensory axons causes a rapid retraction of their growth cones when communication with the cell body is blocked by axotomy or colchicine treatment. These findings indicate that axons of adult mammalian neurons can synthesize proteins and suggest that, under some circumstances, intra-axonal translation contributes to structural integrity of the growth cone in regenerating axons. By immunofluorescence, translation factors, ribosomal proteins, and rRNA were also detected in motor axons of ventral spinal roots analyzed after 7 d in vivo after a peripheral axonal crush injury. Thus, adult motor neurons are also likely capable of intra-axonal protein synthesis in vivo after axonal injury.

Bone mineral density in postmenopausal breast cancer survivors.

PURPOSE: The overall purpose of this longitudinal 18-month study was to test the feasibility and effectiveness of a multicomponent intervention for prevention and treatment of osteoporosis. The purpose of this article is to describe the baseline bone mineral density (BMD) findings for 30 postmenopausal women and to compare these BMD findings to time since menopause, body mass index, and tamoxifen use. DATA SOURCES: Baseline data of BMD findings for 30 postmenopausal women, who have had a variety of treatments including surgery, adjuvant chemotherapy and or tamoxifen, and are enrolled in the 18-month longitudinal study. A demographic questionnaire and a three day dietary record were used to collect baseline data. CONCLUSIONS: Eighty percent of the women with breast cancer history had abnormal BMDs at baseline (t-scores below -1.00 SD). Thinner women showed a greater risk for accelerated trabecular bone loss at the spine and hip. IMPLICATIONS FOR PRACTICE: These findings suggest the need for early BMD assessments and for aggressive health promotion intervention strategies that include a multifaceted protocol of drug therapy for bone remodeling, 1500 mg of daily calcium, 400 IU vitamin D and a strength weight training program that is implemented immediately following chemotherapy treatment and menopause in this high risk population of women.

Assessing community change at multiple levels: the genesis of an evaluation framework for the California Healthy Cities Project.

More than 40 cities have participated in the California Healthy Cities Project since its inception in 1988. Because Healthy Cities efforts are community driven, these cities address diverse health and social issues using a wide variety of strategies. This complexity, in addition to the usual difficulties associated with evaluating community interventions, creates many challenges for evaluation. Given the community building and process orientation of Healthy Cities, it may be most appropriate to measure intermediate community changes that have been linked to health outcomes in previous research or, at a minimum, theoretically. The California Healthy Cities evaluation framework conceptualizes change at five levels: individual, civic participation, organizational, interorganizational, and community. The framework, developed collaboratively with Healthy Cities participants, attempts to synthesize current thinking and practice on evaluation of community projects by applying concepts from community capacity/competence, social ecology, and urban planning.

Twelve years and counting: California's experience with a statewide Healthy Cities and Community program.

California Healthy Cities and Communities is the longest running statewide program of its kind in the nation. After providing a brief history the authors give an overview of the supporting activities and resources the Program provides to Healthy Cities and Communities initiatives throughout California.

Translational control of ribosomal protein L4 mRNA is required for rapid neurite regeneration.

Under some circumstances neurons can be primed to rapidly regenerate injured neuritic processes independent of new gene expression. Such transcription-independent neurite extension occurs in adult rat sensory neurons cultured after sciatic nerve crush and in NGF-differentiated PC12 cells whose neurites have been mechanically sheared. In the PC12 cells, neurite regeneration occurs by means of translational control of mRNAs which were transcribed prior to neurite injury. The survival of such translationally regulated mRNAs is relatively short in the differentiated PC12 cells (< or =10 h). By subtractive hybridization, we have isolated a short-lived mRNA from differentiated PC12 cells. This mRNA, which encodes the ribosomal protein L4, is translationally regulated during neurite regeneration in PC12 cells. Antisense oligonucleotides to L4 mRNA inhibit neurite regeneration from the differentiated PC12 cells as well as axonal elongation from conditioned sensory neurons, indicating that ongoing translation of L4 mRNA is needed for these forms of rapid transcription-independent neurite growth. Taken together, these data point to the importance of translational regulation of existing neuronal mRNAs in the regenerative responses to neuronal injury. Although there are other examples of neuronal translational control, there are no other known neuronal proteins whose levels are regulated predominantly by translational rather than transcriptional control.

Evaluating the association of two breastfeeding assessment tools with breastfeeding problems and breastfeeding satisfaction.

This pilot study evaluated how the scores from each of two breastfeeding assessment tools correlated with breastfeeding satisfaction and problems. A convenience sample of 30 first-time breastfeeding mothers participated. Mothers were randomly assigned to use either the LATCH or Infant Breastfeeding Assessment Tool (IBFAT). The Maternal Breastfeeding Evaluation Scale (MBFES) and Potential Early Breastfeeding Problem Tool (PEBPT) were used to evaluate the association of the scores of each assessment tool with breastfeeding satisfaction and breastfeeding problems. As scores on the both the LATCH and IBFAT increased, maternal satisfaction scores tended to increase, but not significantly, and breastfeeding problem scores tended to decrease (r = -0.5, p = 0.06 and r = -0.49, p = 0.06; respectively.