Ready to leave? - Adolescents' and parents' perceptions of transition from paediatric to adult rheumatology care.

BACKGROUND: In Sweden, approximately 2000 children live with Juvenile Idiopathic Arthritis (JIA). About half of them continue to have an active disease and need to transfer to adult rheumatology care. This study aimed to investigate Swedish adolescents' and parents´ perceptions of readiness for transition from pediatric to adult rheumatology care. METHODS: The study was a cross-sectional quantitative study. Patients at the pediatric rheumatology clinic at a university hospital in Sweden and members of The Swedish National Organization for Young Rheumatics aged 14-18 and their parents were invited to participate in the study. Data was collected with the Readiness for Transition Questionnaire (RTQ) focusing on adolescents' transition readiness, adolescents' healthcare behaviors and responsibility, and parental involvement. Data were analyzed with descriptive statistics. Comparative analyses were made using non-parametric tests with significance levels of 0.05 as well as factor analyses and logistic regression. RESULTS: There were 106 adolescents (85 girls, 20 boys) and 96 parents answering the RTQ. The analysis revealed that many adolescents and parents experienced that the adolescents were ill-prepared to take over responsibility for several healthcare behaviors, such as booking specialty care appointments, calling to renew prescriptions and communicating with medical staff on phone and to transfer to adult care. Parents and adolescents alike stated that it was especially difficult for the adolescents to take responsibility for healthcare behaviors meaning that the adolescents had to have direct interaction with the healthcare professionals (HCPs) at the paediatric rheumatology clinic, for example to renew prescriptions. It was evident that the adolescents who perceived they were ready to take responsibility for the aspects related to direct interaction with HCPs were more overall ready to be transferred to adult care. CONCLUSION: Adolescents need more support to feel prepared to transfer to adult care. With the results from this study, we can develop, customize, and optimize transitional care programs in Sweden for adolescents.

Measures of knee and gait function and radiographic severity of knee osteoarthritis - A cross-sectional study.

BACKGROUND: Pain reports show at most weak to moderate relationship with structural findings of knee osteoarthritis (OA). Less is known about the relationship between measures of knee and gait function and structural findings of knee OA. RESEARCH QUESTION: To test the hypothesis that patient-reported, performance-based and three-dimensional knee and gait measures can distinguish between individuals with varying degrees of radiographic knee OA severity. METHODS: To increase the spectrum of radiographic severity baseline data of individuals included in a cohort study and in a randomized controlled trial respectively were included in this cross-sectional study. Individuals completed the Knee injury and Osteoarthritis Outcome Score (KOOS), Single Limb Mini Squat (SLMS) test, and three-dimensional gait analysis. Radiographic severity was dichotomized into mild (Kellgren Lawrence (KL) 1-2) or severe (KL 3-4) knee OA. Proxies for medial knee joint loading were peak knee adduction moment (KAM) and KAM impulse, and summary measures of overall gait function were the Gait Deviation Index for kinematics (GDI) and kinetics (GDI-kinetic). Area under the receiver operating characteristic curves (AUC) and logistic regressions were used to evaluate whether KOOS-scores, SLMS test, peak KAM, KAM impulse, and GDI-scores could discriminate radiographic severity of knee OA. RESULTS: The sample (n = 115) consisted of 60% women, mean age 61 years (SD 8). Good discriminating abilities (AUC > 0.7) were demonstrated for all measures of knee function and gait, except for GDI and GDI-kinetic (0.62 and 0.36, respectively). Odds ratios from logistic regressions largely supported the AUC findings. SIGNIFICANCE: With the exception of gait summary measures, discriminating abilities were demonstrated by all measures of knee and gait function. Given the interest in interpreting OA as a multi-factorial disease, this information may assist researchers in selecting the most appropriate outcomes for biomechanical studies.

Telomere length was similar in school-age children with bronchopulmonary dysplasia and allergic asthma.

AIM: Inflammation is a major factor in the pathophysiology of bronchopulmonary dysplasia (BPD), and it contributes to accelerated telomere shortening and cellular ageing. This study aimed to determine its effect on telomere length and lung function in school-aged children born preterm with BPD. METHODS: We examined 29 children with BPD, born preterm in Stockholm county 1998-99, along with 28 children with allergic asthma born at term matched for age and gender. At 10 years of age, we measured relative telomere length (RTL) in blood by quantitative polymerase chain reaction, lung function by spirometry and inflammation by fractional exhaled nitric oxide and blood cytokines. RESULTS: RTL was not different in preterm born with BPD compared to term born children with asthma. The gender effect was strong in both groups; girls had significantly longer median RTL than boys (1.8 versus 1.5, p < 0.01). Short RTL was associated with low forced expiratory flow, also after adjusting for gender, but was not affected by severity of BPD or ongoing inflammation. CONCLUSION: Telomere length was similar in 10-year-old children born preterm with a history of BPD and term born children with allergic asthma. However, impaired lung function and male gender were associated with short telomeres.

European consensus on the concepts and measurement of the pathophysiological neuromuscular responses to passive muscle stretch.

BACKGROUND AND PURPOSE: To support clinical decision-making in central neurological disorders, a physical examination is used to assess responses to passive muscle stretch. However, what exactly is being assessed is expressed and interpreted in different ways. A clear diagnostic framework is lacking. Therefore, the aim was to arrive at unambiguous terminology about the concepts and measurement around pathophysiological neuromuscular response to passive muscle stretch. METHODS: During two consensus meetings, 37 experts from 12 European countries filled online questionnaires based on a Delphi approach, followed by plenary discussion after rounds. Consensus was reached for agreement ≥75%. RESULTS: The term hyper-resistance should be used to describe the phenomenon of impaired neuromuscular response during passive stretch, instead of for example 'spasticity' or 'hypertonia'. From there, it is essential to distinguish non-neural (tissue-related) from neural (central nervous system related) contributions to hyper-resistance. Tissue contributions are elasticity, viscosity and muscle shortening. Neural contributions are velocity dependent stretch hyperreflexia and non-velocity dependent involuntary background activation. The term 'spasticity' should only be used next to stretch hyperreflexia, and 'stiffness' next to passive tissue contributions. When joint angle, moment and electromyography are recorded, components of hyper-resistance within the framework can be quantitatively assessed. CONCLUSIONS: A conceptual framework of pathophysiological responses to passive muscle stretch is defined. This framework can be used in clinical assessment of hyper-resistance and will improve communication between clinicians. Components within the framework are defined by objective parameters from instrumented assessment. These parameters need experimental validation in order to develop treatment algorithms based on the aetiology of the clinical phenomena.

EQ-5D-Y as a health-related quality of life measure in children and adolescents with functional disability in Sweden: testing feasibility and validity.

AIM: The EQ-5D-Y is a newly developed generic instrument measuring health-related quality of life in children and adolescents. The aim of the study was to test the feasibility and validity of the EQ-5D-Y in a Swedish patient sample of children and adolescents with functional motor, orthopaedic and medical disabilities and to compare the results with a general population sample. METHODS: Two samples of children and adolescents answered the EQ-5D-Y and a core set of internationally standardised instruments, variables and socio-demographic questions. Patients with functional disability (n = 71, aged seven to 17 years) and individuals from the general population (n = 407, aged eight to 16 years) were included in this study. RESULTS: There was a low number of missing answers. Significantly more problems were reported in all dimensions in the patient sample, and the mean visual analogue scale (VAS) score was significantly lower. Any problems were reported by 83% of the patients and by 37% of the general population. In the patient sample, 21% reported problems at the most severe level, compared with 1.5% in the general population sample. By subgroup analyses by diagnoses, the frequency of reported problems in the different dimensions varied. CONCLUSION: Even though feasibility and discriminative validity of the EQ-5D-Y were supported in our study, further studies are needed including more patients and patient groups.

Quantifying gait deviations in individuals with rheumatoid arthritis using the Gait Deviation Index.

OBJECTIVES: In this study we evaluated the usability of the Gait Deviation Index (GDI), an index that summarizes the amount of deviation in movement from a standard norm, in adults with rheumatoid arthritis (RA). The aims of the study were to evaluate the ability of the GDI to identify gait deviations, assess inter-trial repeatability, and examine the relationship between the GDI and walking speed, physical disability, and pain. METHOD: Sixty-three adults with RA and 59 adults with typical gait patterns were included in this retrospective case-control study. Following a three-dimensional gait analysis (3DGA), representative gait cycles were selected and GDI scores calculated. To evaluate the effect of walking speed, GDI scores were calculated using both a free-speed and a speed-matched reference set. Physical disability was assessed using the Health Assessment Questionnaire (HAQ) and subjects rated their pain during walking. RESULTS: Adults with RA had significantly increased gait deviations compared to healthy individuals, as shown by lower GDI scores [87.9 (SD = 8.7) vs. 99.4 (SD = 8.3), p < 0.001]. This difference was also seen when adjusting for walking speed [91.7 (SD = 9.0) vs. 99.9 (SD = 8.6), p < 0.001]. It was estimated that a change of ≥ 5 GDI units was required to account for natural variation in gait. There was no evident relationship between GDI and low/high RA-related physical disability and pain. CONCLUSIONS: The GDI seems to useful for identifying and summarizing gait deviations in individuals with RA. Thus, we consider that the GDI provides an overall measure of gait deviation that may reflect lower extremity pathology and may help clinicians to understand the impact of RA on gait dynamics.

Change in Gait Deviation Index after anti-tumour necrosis factor-α treatment in individuals with rheumatoid arthritis: a pilot study.

OBJECTIVES: Anti-tumour necrosis factor-alpha (TNF-α) inhibitors provide fast, effective resolution of rheumatoid arthritis (RA) inflammation. In this study we aimed to quantify the impact of TNF-α treatment on gait dynamics. METHOD: The sample comprised 16 subjects [11 female, median age 56 (range 48-66) years, median disease duration 9.5 (range 4.6-20.6) years] with RA who met the American College of Rheumatology (ACR) criteria, had lower extremity involvement, did not use walking aids, and had started TNF-α treatment within 1 week of baseline gait analysis. Gait analysis focused on three-dimensional (3D) lower extremity joint kinematics, kinetics, time and distance parameters. The Gait Deviation Index (GDI) and GDI-Kinetic were calculated. Data on gait, disease activity, and physical disability were collected at baseline and at 3.5 months. RESULTS: Following treatment with TNF-α, statistically significant improvements were found in disease activity [using the 28-joint Disease Activity Score based on C-reactive protein (DAS28-CRP); median difference (m(d)) = 2.3, p < 0.01], physical disability [Health Assessment Questionnaire (HAQ) m(d) = 0.4, p < 0.01], and pain during walking [visual analogue scale (VAS) m(d) = 11.0, p < 0.05]. Reductions in gait deviations were noted (GDI m(d) = 3.7, p = 0.04; GDI-Kinetic m(d) = 4.1, p = 0.05) along with reductions in dimensionless time and distance parameters. A moderate to good negative correlation existed between baseline GDI and GDI change scores (r(s) = -0.7, p < 0.01). CONCLUSIONS: Treatment with TNF-α improved gait dynamics in adults with RA. Significant gait deviations were, however, still present after treatment. In this study, GDI and GDI-Kinetic scores appeared to be useful outcome measures to quantify changes in gait deviations after this intervention.

Ankle/hindfoot arthrodesis in rheumatoid arthritis improves kinematics and kinetics of the knee and hip: a prospective gait analysis study.

OBJECTIVES: To evaluate the effects of ankle/hindfoot arthrodesis in rheumatoid arthritis (RA) patients on gait pattern of the knee and hip. METHODS: In this prospective follow-up study, 14 RA patients scheduled for ankle/hindfoot arthrodesis (talo-calcaneal, talo-navicular, calcaneo-cuboid and/or talo-crural joints) and 14 age- and sex-matched healthy controls were included. Three-dimensional gait analyses of joint angles, moments and work were performed at the index operation and after 13 months of follow-up. Each patient underwent clinical assessments of pain while walking, overall evaluation of disease activity, Health Related Quality of Life Questionnaire (EQ-5D), activity limitations, maximum walking distance, difficulty with walking surface and gait abnormality. For comparisons of pre- vs post-operative conditions, Wilcoxon's matched pairs test and Friedman ANOVA by rank test were used. RESULTS: At follow-up after ankle/hindfoot fusion surgery, RA patients demonstrated a statistically significant improvement in mean range of joint motions, moments and work in the overlying joints such as the knee and hip. Moreover, there was significantly less pain, disease activity, activity limitation, difficulty with walking surface and gait abnormality. EQ-5D and maximum walking distance were also significantly improved at follow-up. CONCLUSIONS: Our results demonstrate that ankle/hindfoot arthrodesis in RA is an effective intervention to reduce pain and to improve Health Related Quality of Life and functional ability. Moreover, the overlying leg joints experience an improvement in joint motion, muscle-generated joint moments and work during walking. Three-dimensional gait analysis may assist future investigations of the effects of orthopaedic surgery on functional mobility in RA to prevent irreversible disablement.

Eosinophil activation in preterm infants with lung disease.

AIM: We investigated the role of eosinophils in the pathogenesis of bronchopulmonary dysplasia (BPD) in preterm infants. METHODS: Fifteen preterm infants with BPD were compared to 13 preterms with respiratory distress syndrome (RDS) and to 16 healthy preterms. We assessed total eosinophil and neutrophil counts in venous blood samples and the levels of the eosinophilic activity markers eosinophilic cationic protein (ECP) and the cellular surface antigen (CD9). RESULTS: The eosinophil count was greater in BPD compared with RDS and healthy infants (1414 vs. 797 and 471 cells per microlitre, respectively, p = 0.03). ECP levels were elevated (34 vs. 12.8 and 9.8 microg/L, respectively, p = 0.002) and CD9 levels reduced (75 vs. 94 and 86 mean fluorescence intensity units, respectively, p = 0.01) in BPD compared with RDS and healthy infants, suggesting eosinophilic activation in BPD. These findings were not solely explained by differences between gestational age or birth weight of the different groups. ECP levels were positively correlated with the duration of oxygen supplementation in the BPD group. The eosinophil count fell promptly after steroid treatment was commenced in the BPD group. CONCLUSION: The findings suggest that BPD is linked to eosinophil activation, which might contribute to the pathogenesis.

Arthrodesis versus Mayo resection: the management of the first metatarsophalangeal joint in reconstruction of the rheumatoid forefoot.

In a prospective randomised study 31 patients were allocated to either arthrodesis or Mayo resection of the first metatarsophalangeal joint as part of a total reconstruction of the rheumatoid forefoot. Of these, 29 were re-examined after a mean of 72 months (57 to 80), the Foot Function Index was scored and any deformity measured. Load distribution was analysed using a Fscan mat in 14 cases, and time and distance were measured in 12 of these patients using a 3D Motion system. We found excellent patient satisfaction and a significant, lasting reduction of the Foot Function Index, with no statistically significant differences between the groups. There were no significant differences in recurrence of the deformity, the need for special shoes, gait velocity, step length, plantar moment, mean pressure or the position of the centre of force under the forefoot. The cadence was higher and the stance phase shorter in the fusion group. These results suggest that a Mayo resection may be an equally good option for managing the first metatarsophalangeal joint in reconstruction of the rheumatoid forefoot.

Effect of joint injections in children with juvenile idiopathic arthritis: evaluation by 3D-gait analysis.

AIM: To investigate how gait patterns change after intra-articular corticosteroid injections (ICIs) in the lower extremities. METHODS: Eighteen children, aged 5-16 y, with juvenile idiopathic arthritis (JIA) and lower extremity involvement participated in the study. Sixty-four joints in the lower extremities were treated with ICIs. The Visual Analogue Scale (VAS, 0-100 mm) was used to assess pain. A 3D motion analysis system and two force plates were used to measure gait parameters, kinematics and kinetics. The first gait analysis was performed before treatment with ICIs, and a second one was done 8-17 d after treatment. RESULTS: The participants' average rating of pain decreased from 26 mm on the VAS before ICI to 11 mm (p = 0.001) after treatment. The self-chosen walking velocity was significantly faster (p = 0.02) after treatment. The range of knee and ankle joint angles during gait increased significantly (p = 0.03 and 0.04) after treatment. At loading response, the hip extension moment increased (p = 0.01) as did knee flexion moment, and plantar flexion moment at pre-swing increased significantly (p = 0.02 and 0.002) after treatment. The ankle also generated more power (p = 0.005) after treatment. CONCLUSION: The study shows positive effects of treatment with ICIs in the lower extremities--especially regarding pain, walking velocity and joint moments. The data indicate that ICI treatment influences the gait pattern also in joints that have not been injected.

Gait in children with juvenile chronic arthritis. Timing and force parameters.

OBJECTIVES: To examine gait in children with juvenile chronic arthritis (JCA) with reference to velocity, ground reaction forces and temporal parameters. METHODS: Fifteen children with JCA were assigned into two groups (uni- and bilateral involvement and classified as pauci- or polyarticular arthritis). Fourteen healthy children participated in the control group. Light-beams were used to determine walking velocity and the children with JCA rated their pain on a visual analogue scale. Two force plates registered the ground reaction forces and foot-switches were used to obtain temporal parameters. RESULTS: The mean velocity for the children with JCA was significantly less than for the healthy controls. Velocity normalized to height showed a tendency for the children with JCA to walk slower than controls. Differences between JCA children and healthy controls were observed for peak vertical forces during heel contact and push-off. No temporal differences were observed between the groups. CONCLUSIONS: Such kinetic and temporal information may provide the clinician with a sensitive tool for pre- and post assessment of intra-articular steroid injections and/or physical therapy.