Macrophage migration inhibitory factor (MIF) and oligoarticular juvenile idiopathic arthritis (o-JIA): association of MIF promoter polymorphisms with response to intra-articular glucocorticoids.

Objectives: To address the clinical relevance of macrophage migration inhibitory factor (MIF) promoter polymorphisms in oligoarticular juvenile idiopathic arthritis (o-JIA) by evaluating their associations with serum and SF MIF levels, with response to intra-articular glucocorticoid injections and with outcome of the disease.

Methods: Seventy-five Caucasian patients with o-JIA were studied. Alleles of the -794 CATT variable number of tandem repeats (VNTR) and of the -173 G/C single nucleotide polymorphism (SNP) were identified by capillary electrophoresis following fluorescently labelled PCR and by allelic discrimination assay, respectively.

Agreement between physicians and parents in rating functional ability of children with juvenile idiopathic arthritis.

Objective: To investigate concordance between physicians and parents in rating the degree of functional ability of children with juvenile idiopathic arthritis (JIA).

Methods: The attending physician and a parent were asked to rate independently the level of physical functioning of 155 patients with disease duration >/= 5 years on a 6-point scale ranging from 1 = no disability (i.e.

Juvenile idiopathic arthritis: will etanercept be an improvement over current therapies?

Overexpression of cytokines in inflamed joints plays an important role in joint inflammation and in damage to articular tissue. Biological agents aimed at specifically antagonising tumour necrosis factor (TNF) are effective in the treatment of adult rheumatoid arthritis. A recent trial of etanercept, a genetically engineered fusion protein consisting of the Fc domain of human IgG1 and the TNF receptor p75, has demonstrated that this agent is also well tolerated and effective in patients with juvenile idiopathic arthritis (JIA).

A longitudinal analysis of physical functional disability over the course of juvenile idiopathic arthritis.

Objective: To describe the longitudinal course of physical functioning in children with juvenile idiopathic arthritis (JIA) and identify predictors of long-term functional impairment.

Methods: Between January 1987 and December 2002, 227 patients had two or more functional ability questionnaires completed by a parent. The total number of questionnaires was 1356 and the follow-up between first and last questionnaire administration was 949.

Antiphospholipid syndrome in pediatrics.

APS is recognized increasingly as a leading cause of vascular thrombosis in the pediatric population. With the obvious exception of pregnancy morbidity, most of the clinical features that may occur in adults with APS have been described also in children. Because the coincident prothrombotic factors that are common in adults have little or no impact in children, pediatric patients with APS constitute a suitable sample to investigate the relationship of aPL with the associated clinical manifestations, such as thrombocytopenia, hemolytic anemia, chorea, and livedo reticularis, and the specificities of aPL that are more linked to thrombosis.

Methotrexate improves the health-related quality of life of children with juvenile idiopathic arthritis.

Objectives: To examine the change in health-related quality of life (HRQOL) and its determinants in children with juvenile idiopathic arthritis (JIA) treated with methotrexate (MTX).

Methods: Patients were extracted from the PRINTO clinical trial which aimed to evaluate the efficacy and safety profile of MTX administered in standard, intermediate or higher doses (10, 15 and 30 mg/m(2)/week respectively). Children with polyarticular-course JIA, who were less than 18 years and had a complete HRQOL assessment were included.

A randomized, placebo-controlled trial of infliximab plus methotrexate for the treatment of polyarticular-course juvenile rheumatoid arthritis.

Objective: To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA).

Methods: This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo for 14 weeks, after which all children received infliximab through week 44.

Adapted versions of the Sharp/van der Heijde score are reliable and valid for assessment of radiographic progression in juvenile idiopathic arthritis.

Objective: To develop adapted versions of the Sharp/van der Heijde radiographic scoring system for use in juvenile idiopathic arthritis (JIA), and to investigate their validity in JIA patients with polyarticular disease.

Methods: The study group comprised 177 patients with polyarticular JIA. Radiographs of the wrist/hand of each patient were obtained at baseline (first observation) and then at 1, 3, 5, 7/8, and 10 years and were assessed independently by 2 pediatric rheumatologists according to different adaptations of the Sharp/van der Heijde method.

The Paediatric Rheumatology International Trials Organization (PRINTO).

In this review we describe an international project, conducted by the Paediatric Rheumatology International Trials Organization (PRINTO) that was aimed to identify, validate and promulgate core sets of measures and a definition of improvement for the evaluation of response to therapy in clinical trials and in daily clinical practice in patients with juvenile systemic lupus erythematosus (JSLE). The following clinical measures were included in the PRINTO core set of outcome measure for the evaluation of response to therapy: 1) physician's global assessment of disease activity; 2) global disease activity measure; 3) 24-hour proteinuria; 4) parent's global assessment of the overall patient's well-being; 5) health-related quality of life assessment. The measures included in the core set were found to be feasible and not redundant, to have good construct validity, discriminative ability, internal consistency, with fair responsiveness to clinically important change in disease activity, and to be associated strongly with treatment outcome.

Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication.

Probabilistic record linkage is a valid and transparent tool to combine databases without a patient identification number.

Objective: To describe the technical approach and subsequent validation of the probabilistic linkage of the three anonymous, population-based Dutch Perinatal Registries (LVR1 of midwives, LVR2 of obstetricians, and LNR of pediatricians/neonatologists). These registries do not share a unique identification number.

Study Design And Setting: A combination of probabilistic and deterministic record linkage techniques were applied using information about the mother, delivery, and child(ren) to link three known registries.

Development and validation of a new short and simple measure of physical function for juvenile idiopathic arthritis.

Objective: To develop and validate a new short and simple measure of physical function in children with juvenile idiopathic arthritis (JIA).

Methods: The Juvenile Arthritis Functionality Scale (JAFS) is a 15-item questionnaire that explores physical function in 3 body areas (lower limbs, hand/wrist, and upper segment). Validation of the Italian version of the instrument was accomplished by evaluating 211 consecutive JIA patients ages 2.

The magnitude of early response to methotrexate therapy predicts long-term outcome of patients with juvenile idiopathic arthritis.

Objective: To investigate the relationship between the magnitude of clinical response in the first 6 months of methotrexate (MTX) therapy and long-term outcome in children with juvenile idiopathic arthritis (JIA).

Methods: The clinical charts of 125 JIA patients who were started with MTX and then followed for at least 5 years were reviewed. Based on the level of American College of Rheumatology (ACR) Pediatric response at 6 months, patients were divided in four mutually exclusive groups: (1) non-responders, (2) responders at 30%, (3) responders at 50%, and (4) responders at 70%.

Physicians' and parents' ratings of inactive disease are frequently discordant in juvenile idiopathic arthritis.

Objective: To investigate discrepancies between physicians' and parents' ratings of inactive disease in children with juvenile idiopathic arthritis (JIA) and the determinants of the discrepancy.

Methods: Study data were obtained from the clinical database generated at the study unit. Each patient visit included a standardized assessment of JIA outcome measures.

Colonic lymphoid nodular hyperplasia in children: relationship to food hypersensitivity.

Background & Aims: The clinical significance of lymphoid nodular hyperplasia (LNH) of the lower gastrointestinal tract is unclear. The aim of this study was to define the frequency and clinical significance of LNH in pediatric patients undergoing colonoscopy.

Methods: Two hundred forty-five children (101 male, 144 female; median age, 8.

Juvenile idiopathic arthritis.

Juvenile idiopathic arthritis is a broad term that describes a clinically heterogeneous group of arthritides of unknown cause, which begin before 16 years of age. This term encompasses several disease categories, each of which has distinct methods of presentation, clinical signs, and symptoms, and, in some cases, genetic background. The cause of disease is still poorly understood but seems to be related to both genetic and environmental factors, which result in the heterogeneity of the illness.

The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis.

Objective: To develop criteria for the classification of systemic sclerosis (SSc) in children (juvenile SSc).

Methods: The study consisted of 3 phases: 1) collection of data on the signs and symptoms of actual patients with juvenile SSc that are useful for defining involvement of a particular organ; 2) selection of the parameters essential for the classification of juvenile SSc and preparation of a set of provisional classification criteria (PCC) using 2 Delphi surveys; 3) consensus conference consisting of 2 steps: discussion and rating of clinical profiles of 160 patients with definite juvenile SSc, possible juvenile SSc, or other fibrosing diseases as "having or not having juvenile SSc," using nominal group technique, and defining those PCC with the best statistical performance and highest face validity by using the clinical profiles of patients with definite juvenile SSc as the gold standard.

Results: In phase 1, 55 centers submitted clinical data on 153 patients with juvenile SSc.

Results from the pediatric European register for treatment of Helicobacter pylori (PERTH).

Background And Aim: Data on the eradication treatment for childhood Helicobacter pylori are scanty. A register was established on the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) website to collect data on treatment performed by European pediatricians to ascertain what is practiced in the field.

Subjects: From January 2001 to December 2002, information on 597 children were entered by 23 European Centers, but only data of 518 treated children were completed and analyzed (86.

Proxy-reported health-related quality of life of patients with juvenile idiopathic arthritis: the Pediatric Rheumatology International Trials Organization multinational quality of life cohort study.

Objective: To investigate the proxy-reported health-related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA).

Methods: In this multinational, multicenter, cross-sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. Potential determinants of HRQOL included demographic data, physician's and parent's global assessments, measures of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate.

Record linkage: making the most out of errors in linking variables.

This paper presents a refinement of the probabilistic medical record linking algorithm. We introduced "close agreement" to account for typical errors in administrative variables used for record linkage. Linking data on early pregnancy determinants with data on late child outcomes was used as a case study.

Cyclosporine A in juvenile idiopathic arthritis. Results of the PRCSG/PRINTO phase IV post marketing surveillance study.

Objective: To investigate the clinical use patterns, clinical effect and safety of cyclosporine A (CSA) in juvenile idiopathic arthritis (JIA) in the setting of routine clinical care.

Methods: An open-ended, phase IV post marketing surveillance study was conducted among members of the Pediatric Rheumatology Collaborative Study Group (PRCSG) and of the Paediatric Rheumatology International Trials Organisation (PRINTO) to identify patients with polyarticular course JIA who had received CSA during the course of their disease.

Results: A total of 329 patients, half of whom had systemic JIA, were collected in 21 countries.

Correlation between juvenile idiopathic arthritis activity and damage measures in early, advanced, and longstanding disease.

Objective: To compare the correlation between juvenile idiopathic arthritis (JIA) measures of disease activity and damage in patients with early and late disease.

Methods: Three cohorts of patients with JIA disease duration < or =1 year (early disease, n = 70), 5-9.9 years (advanced disease, n = 114), and > or =10 years (longstanding disease, n = 39) were studied.

Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database.

Objective: To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group of children and describe the clinical evolution of the disease and compare it with the adult form.

Methods: Data on 153 patients with juvenile SSc collected from 55 pediatric rheumatology centers in Europe, Asia, and South and North America were analyzed. Demographic, clinical, and immunologic characteristics of children with juvenile SSc at the onset, at diagnosis, and during the disease course were evaluated.

Pulmonary aspiration shown by scintigraphy in gastroesophageal reflux-related respiratory disease.

Objectives: Gastroesophageal reflux (GER) may underlie respiratory manifestations via vagally mediated airway hyperresponsiveness or microaspiration, and intraesophageal pH monitoring is generally used to identify GER in patients with such manifestations. We aimed to establish the frequency of retrograde pulmonary aspiration in patients with unexplained respiratory manifestations.

Methods: Fifty-one patients with refractory respiratory symptoms (cough, n = 18; pneumonia, n = 14; apnea, n = 8; asthma, n = 7; and laryngitis, n = 4) were prospectively evaluated.

Remission in juvenile idiopathic arthritis.

Until recently, no uniform and widely accepted criteria for defining remission in juvenile idiopathic arthritis (JIA) were available. In recent years, a set of preliminary criteria for clinical remission in JIA was developed through an international collaborative effort. These criteria enable the classification of patients in the states of inactive disease, clinical remission with medication, and clinical remission without medication.