ABSTRACT
The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Greek language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographics, clinical data, and the JAMAR from 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). The Greek JAMAR was fully cross-culturally adapted with two forward and three backward translations. A total of 272 JIA patients (5.9% systemic, 57.7% oligoarticular, 21.3% RF negative poly-arthritis, 15.1% other categories), and 100 healthy children were enrolled in all centres. The JAMAR components discriminated well-healthy subjects from JIA patients; notably, there was no significant difference between healthy subjects and their affected peers in psychosocial quality of life and school-related items. All JAMAR components revealed good psychometric performances. In conclusion, the Greek version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and in clinical research.
Subject(s)
Arthritis, Juvenile/diagnosis , Disability Evaluation , Patient Reported Outcome Measures , Rheumatology/methods , Adolescent , Age of Onset , Arthritis, Juvenile/physiopathology , Arthritis, Juvenile/psychology , Arthritis, Juvenile/therapy , Case-Control Studies , Child , Child, Preschool , Cultural Characteristics , Female , Greece , Health Status , Humans , Male , Parents/psychology , Patients/psychology , Predictive Value of Tests , Prognosis , Psychometrics , Quality of Life , Reproducibility of Results , TranslatingABSTRACT
Iron overload in hemoglobinopathies is secondary to blood transfusions, chronic hemolysis, and increased iron absorption and leads to tissue injury requiring the early use of chelating agents. The available agents are parenteral deferoxamine and oral deferiprone and deferasirox. There are limited data on the safety and efficacy of deferiprone at a very young age. The aim of our study was the presentation of data regarding the use of oral solution of deferiprone in 9 children (mean age 6.5, range 2-10) with transfusion dependent hemoglobinopathies (6 beta thalassemia major, 1 thalassemia intermedia, and 2 sickle cell beta thalassemia). The mean duration of treatment was 21.5 months (range 15-31). All children received the oral solution without any problems of compliance. Adverse reactions were temporary abdominal discomfort and diarrhea (1 child), mild neutropenia (1 child) that resolved with no need of discontinuation of treatment, and transient arthralgia (1 child) that resolved spontaneously. The mean ferritin levels were significantly reduced at the end of 12 months (initial 2440 versus final 1420 µ g/L, P < 0.001). This small study shows that oral solution of deferiprone was well tolerated by young children and its use was not associated with major safety concerns. Furthermore, it was effective in decreasing serum ferritin.
ABSTRACT
OBJECTIVE: Chronic inflammatory diseases in adults have been associated with increased cardiovascular risk and impaired vascular function. We aimed to assess the presence of early vascular dysfunction in patients with juvenile idiopathic arthritis (JIA) and investigate the role of inherent inflammatory process of JIA in vascular health. METHODS: Thirty patients with JIA (age range 7-18 years) were compared to 33 age- and sex-matched controls. Endothelial function (brachial artery flow-mediated dilation [FMD]), carotid intima-media thickness (IMT), and arterial stiffness were examined. Endothelial inflammation was assessed by intercellular adhesion molecule 1 (ICAM-1) and P-selectin measurements. RESULTS: Patients with JIA showed decreased FMD compared to controls (P = 0.001), independent of age (P = 0.9 among age subgroups). Baseline differences in erythrocyte sedimentation rate, ICAM-1, and glucose between the 2 groups accounted for the difference in FMD. The presence of systemic JIA was associated with greater IMT compared to patients with oligoarticular disease, polyarticular disease, or controls (P = 0.014, P = 0.069, and P = 0.046, respectively). The difference in IMT between systemic versus oligoarticular/polyarticular JIA was attributed to the following risk factors: age, body mass index, blood pressure, disease activity, and corticosteroids use. There were no differences in arterial stiffness indices between JIA patients and controls or between patients with systemic versus nonsystemic disease. CONCLUSION: Endothelial function is impaired in patients with JIA at a very young age, while IMT is increased only in the presence of systemic JIA. Vascular dysfunction may be partly attributed to the effects of disease-related characteristics (inflammation, disease activity, and medications).
Subject(s)
Arthritis, Juvenile/complications , Brachial Artery/physiopathology , Carotid Artery, Common/pathology , Endothelium, Vascular/physiopathology , Tunica Intima/pathology , Tunica Media/pathology , Vascular Diseases/etiology , Vasodilation , Adolescent , Age Factors , Analysis of Variance , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/immunology , Arthritis, Juvenile/physiopathology , Biomarkers/blood , Brachial Artery/diagnostic imaging , Brachial Artery/immunology , C-Reactive Protein/analysis , Carotid Artery, Common/diagnostic imaging , Carotid Artery, Common/immunology , Case-Control Studies , Child , Cross-Sectional Studies , Endothelium, Vascular/diagnostic imaging , Endothelium, Vascular/immunology , Female , Greece , Humans , Inflammation Mediators/blood , Intercellular Adhesion Molecule-1/blood , Linear Models , Male , Manometry , P-Selectin/blood , Risk Assessment , Risk Factors , Tunica Intima/diagnostic imaging , Tunica Intima/immunology , Tunica Media/diagnostic imaging , Tunica Media/immunology , Ultrasonography, Doppler , Vascular Diseases/diagnosis , Vascular Diseases/immunology , Vascular Diseases/physiopathologyABSTRACT
OBJECTIVE: To evaluate the characteristics and visual prognosis of juvenile idiopathic arthritis-associated uveitis (JIA). METHODS: A retrospective review was performed on 56 patients who met the criteria for JIA to identify those with uveitis and related complications. Patients were referred to and were examined in the Pediatric Department of the University Hospital of Ioannina, between 1995 and 2007. RESULTS: The prevalence of JIA-associated uveitis was high. Despite this and the related complications, the final visual outcome was satisfactory in the majority of the cases. Authors did not observe any correlation between prognosis and sex, age at the onset of uveitis or arthritis, pattern of arthritis, or positivity for antinuclear antibodies (ANA). CONCLUSION: We found a remarkably high prevalence of uveitis and related ocular complications in 7 (28%) of the patients, and the rate of poor visual outcome was 12%.
