A novel magnetic resonance imaging scoring system for active and chronic changes in children and adolescents with juvenile idiopathic arthritis of the hip.

BACKGROUND: Hip involvement predicts severe disease in juvenile idiopathic arthritis (JIA) and is accurately assessed by MRI. However, a child-specific hip MRI scoring system has not been validated. OBJECTIVE: To test the intra- and interobserver agreement of several MRI markers for active and chronic hip changes in children and young adults with JIA and to examine the precision of measurements commonly used for the assessment of growth abnormalities. MATERIALS AND METHODS: Hip MRIs from 60 consecutive children, adolescents and young adults with JIA were scored independently by two sets of radiologists. One set scored the same MRIs twice. Features of active and chronic changes, growth abnormalities and secondary post-inflammatory changes were scored. We used kappa statistics to analyze inter- and intraobserver agreement for categorical variables and a Bland-Altman approach to test the precision of continuous variables. RESULTS: Among active changes, there was good intra- and interobserver agreement for grading overall inflammation (kappa 0.6-0.7). Synovial enhancement showed a good intraobserver agreement (kappa 0.7-0.8), while the interobserver agreement was moderate (kappa 0.4-0.5). Regarding acetabular erosions on a 0-3 scale, the intraobserver agreement was 0.6 for the right hip and 0.7 for the left hip, while the interobserver agreement was 0.6 for both hips. Measurements of joint space width, caput-collum-diaphyseal angle, femoral neck-head length, femoral width and trochanteric distance were imprecise. CONCLUSION: We identified a set of MRI markers for active and chronic changes in JIA and suggest that the more robust markers be included in future studies addressing clinical validity and long-term patient outcomes

Imaging assessment of children presenting with suspected or known juvenile idiopathic arthritis : ESSR-ESPR points to consider

Juvenile idiopathic arthritis (JIA) is the most common paediatric rheumatic disease. It represents a group of heterogenous inflammatory disorders with unknown origin and is a diagnosis of exclusion in which imaging plays an important role. JIA is defined as arthritis of one or more joints that begins before the age of 16 years, persists for more than 6 weeks and is of unknown aetiology and pathophysiology. The clinical goal is early suppression of inflammation to prevent irreversible joint damage which has shifted the emphasis from detecting established joint damage to proactively detecting inflammatory change. This drives the need for imaging techniques that are more sensitive than conventional radiography in the evaluation of inflammatory processes as well as early osteochondral change. Physical examination has limited reliability, even if performed by an experienced clinician, emphasising the importance of imaging to aid in clinical decision-making. On behalf of the European Society of Musculoskeletal Radiology (ESSR) arthritis subcommittee and the European Society of Paediatric Radiology (ESPR) musculoskeletal imaging taskforce, based on literature review and/or expert opinion, we discuss paediatric-specific imaging characteristics of the most commonly involved, in literature best documented and clinically important joints in JIA, namely the temporomandibular joints (TMJs), spine, sacroiliac (SI) joints, wrists, hips and knees, followed by a clinically applicable point to consider for each joint. We will also touch upon controversies in the current literature that remain to be resolved with ongoing research

A Patient-Specific Foot Model for the Estimate of Ankle Joint Forces in Patients with Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is the leading cause of childhood disability from a musculoskeletal disorder. It generally affects large joints such as the knee and the ankle, often causing structural damage. Different factors contribute to the damage onset, including altered joint loading and other mechanical factors, associated with pain and inflammation. The prediction of patients' joint loading can hence be a valuable tool in understanding the disease mechanisms involved in structural damage progression. A number of lower-limb musculoskeletal models have been proposed to analyse the hip and knee joints, but juvenile models of the foot are still lacking. This paper presents a modelling pipeline that allows the creation of juvenile patient-specific models starting from lower limb kinematics and foot and ankle MRI data. This pipeline has been applied to data from three children with JIA and the importance of patient-specific parameters and modelling assumptions has been tested in a sensitivity analysis focused on the variation of the joint reaction forces. This analysis highlighted the criticality of patient-specific definition of the ankle joint axes and location of the Achilles tendon insertions. Patient-specific detection of the Tibialis Anterior, Tibialis Posterior, and Peroneus Longus origins and insertions were also shown to be important

Imaging of the hip in juvenile idiopathic arthritis

Hip involvement is common and estimated to occur in approximately 35\u201363% of children with juvenile idiopathic arthritis (JIA). It is more prevalent in the aggressive systemic subtypes, with irreversible changes occurring as early as within 5 years of diagnosis. Whilst clinical parameters and joint examination can be useful for assessing disease severity, subclinical disease is known to exist and delayed treatment may herald a lifetime of disability and pain. Early recognition of JIA changes is therefore crucial in determining treatment options. Validated scoring systems in the radiologic assessment of the hip for clinical drug trials may inform treatment outcomes, although robust tools for analysis are still lacking. This review article details the modalities utilised for imaging the hip in children with JIA with particular efforts focused upon reliability and validity in their assessment of joint disease. We conclude with a short literature review on the potential future techniques being developed for hip joint imaging in JIA

Echocardiographic alterations in systemic sclerosis: a longitudinal study.

OBJECTIVE To investigate the evolution of cardiac alterations in systemic sclerosis (SSc). METHODS Echocardiographic and echo-Doppler findings from 77 unselected SSc patients were analyzed at the first clinical observation and after a follow-up period of 65 * 36 months. Data were compared with those obtained from 45 normal subjects matched for age and sex. RESULTS Baseline left ventricular (LV) systolic function was normal in all patients and controls while LV diastolic dysfunction (expressed by an inverted E/A ratio which represents early and late filling of the LV during atrial contraction) was present in 23 patients and in 1 control (P < 0.001). At the end of the follow-up period, while LV systolic function declined in 1 case alone, 6 further patients developed an inverted E/A ratio. Moreover, in the group of SSc patients mean A-wave values, E/A ratio, left atrial dimension, and LV wall thickness significantly changed, all indicating the progression of heart involvement. The alteration of LV diastolic function was independent of other known causes potentially affecting LV relaxation. Moreover, impairment of LV filling parameters was detected in the first phase of follow-up, while the anatomical changes occurred in the last phase. CONCLUSIONS Our data confirm the significant prevalence of LV diastolic dysfunction in SSc patients and the role of primary myocardial involvement. The long-term follow-up demonstrates that LV filling dysfunction is progressive and precedes the occurrence of LV remodelin

Diagnostic value of faecal calprotectin in paediatric gastroenterology clinical practice

Background. Faecal calprotectin (FC) is a new marker of intestinal inflammation. Data on FC in paediatric gastroenterology clinical practice are still scarce. Aims. To assess FC values in different paediatric gastrointestinal diseases comparing them with those obtained in healthy children. Patients. Two hundred and eighty-one children (age range 13-216 months) consecutively referred for gastrointestinal symptoms. Seventy-six healthy controls (age range 13-209 months). The exclusion criteria in healthy children were the following: any known underlying chronic disease or a history of abdominal pain, diarrhoea, acute respiratory tract infection, intake of non-steroidal anti-inflammatory drugs, gastric acidity inhibitors, antibiotics, drugs influencing gut motility, and menstrual or nasal bleeding in the last 3 weeks. Methods. Stool samples stored, prepared and analyzed by an ELISA assay. Results. In healthy children the median FC value was 28.0 mug/g (15-57 interquartile range) with a 95th percentile value of 95.3 mug/g. All increase in FC concentration was observed in all diseases characterized by gastrointestinal mucosa inflammation, and the active inflammatory bowel disease patients showed the higher FC values. All children affected by functional bowel disorders or by non-inflammatory diseases showed normal values. We calculated all optimized FC cut off value of 102.9266 mug/g (revealed by the receiver operating characteristic curve) to distinguish patients with active organic/inflammatory disorders from healthy Subjects and from patients with functional bowel disorders. Conclusions. Calprotectin is a sensitive, but not disease specific, marker to easily detect inflammation throughout the whole gastrointestinal tract. It may help in identifying an organic disease characterized by intestinal mucosa inflammation and in the differential diagnosis of functional bowel disorders. (C) 2004 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved

XL Premio Scanno - Provincia de L'Aquila "Riccardo Tanturri"

Il Premio Scanno nasce per iniziativa di Riccardo Tanturri De Horatio, professore universitario di Lingua e Letteratura Italiana, scrittore, poeta e giornalista. Nel 1972 diventa presidente della locale Azienda di soggiorno e turismo di Scanno e da allora si dedica a promuovere l'immagine del piccolo centro abruzzese nel mondo, fino alla sua morte. Successivamente, il coronamento del suo desiderio viene affidato a un prestigioso Comitato d'onore e a un efficiente Comitato promotore, che perpetuano il Premio e le accrescono la fama

Left ventricular mass and function in children with growth hormone (GH) deficiency before and during 12 months GH replacement therapy

OBJECTIVE: This open, prospective study was designed to evaluate the effect of GH deficiency (GHD) on left ventricular (LV) mass (LVM) and performance, by echocardiography, and on lipid profile during childhood. SUBJECTS: Twelve prepubertal children with GHD (eight boys and four girls) aged 8.1 +/- 1.7 years were studied before and after 6 and 12 months of GH replacement therapy at a dose of GH of 30 micro g/kg/day. Twelve healthy children sex-, height-, weight- and body surface area-matched with the patients, served as controls. METHODS: Echocardiography was performed at study entry and after 12 months both in GHD children and in controls. Only in GHD children, echocardiography was repeated also after 6 months of GH replacement. In all subjects, we measured LV posterior wall thickness (LVPWT), LV end-diastolic diameter (LVEDD), LVM index (LVMi), LV systolic and diastolic function. RESULTS: At study entry, LVPWT (5.3 +/- 0.8 vs. 6.2 +/- 1.1 mm, P < 0.05), LVEDD (34.0 +/- 2.4 vs. 36.7 +/- 2.1 mm, P < 0.007) and LVMi (47.0 +/- 6.9 vs. 59.6 +/- 9.5 g/m2, P < 0.005) were significantly lower in GHD children than in controls. Lipid profile, heart rate, blood pressure, LV systolic function and indices of ventricular filling were similar in patients and controls. After 12 months of GH replacement therapy, LVPWT (6.1 +/- 0.7 mm, P < 0.0005), LVEDD (38.8 +/- 4.3 mm, P < 0.002) and LVMi (71.5 +/- 12.7 g/m2, P < 0.0005) significantly increased in GHD children compared to pretreatment values. In particular, after 12 months of therapy GHD children achieved a normal LVMi when compared to controls (60.7 +/- 8.6, P = ns). LVMi increase was significantly correlated with the increase in IGF-I level (r = 0.49; P < 0.004). LV systolic performance, diastolic filling and blood pressure did not change significantly during GH therapy. After 12 months of treatment, the atherogenic index, measured as total/high-density lipoprotein-cholesterol ratio (2.7 +/- 0.8) was significantly lower than both pretreatment (3.4 +/- 0.3, P < 0.03) and control values (3.8 +/- 1.1, P < 0.04). CONCLUSIONS: GH deficiency in children affects heart morphology, by inducing a significant decrease in cardiac size, but does not modify cardiac function and lipid profile. Twelve months of GH replacement treatment normalizes cardiac mass, and reduces the atherogenic index