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Biomedical subjects

M Gattinara

Publications and source records attributed to M Gattinara.

16 recordsLinked to original sources

A comparison of response criteria to evaluate therapeutic response in patients with juvenile idiopathic arthritis treated with methotrexate and/or anti-tumor necrosis factor alpha agents.

OBJECTIVE: There are no validated criteria to evaluate clinical response in juvenile idiopathic arthritis (JIA). The purpose of this study was to compare 4 sets of criteria (2 from the American College of Rheumatology [ACR] and 2 from the European League Against Rheumatism [EULAR]) for clinical response evaluation in JIA patients treated with methotrexate and/or anti-tumor necrosis factor alpha drugs. METHODS: Seventy-five patients with JIA were evaluated at baseline and after 6 months of therapy with second-line drugs. Mean age at study onset was 12.8 years (range 2-32.9 years). Diagnoses were systemic JIA (n = 16), rheumatoid factor-positive JIA (n = 5), rheumatoid factor-negative JIA (n = 9), persistent oligoarticular JIA (n = 10), extended oligoarticular JIA (n = 33), and psoriatic arthritis (n = 2). Clinical response was evaluated with the ACR Pediatric 30 criteria and the ACR 20% response criteria (ACR20), and with the EULAR Disease Activity Score (DAS) and 28-joint DAS (DAS28). Patients with EULAR criteria responses of "good" or "moderate" were classified as responders. Responders and nonresponders according to the different criteria were then compared. RESULTS: For patients younger than 16 years, Cohen's kappa varied between 0.51 and 0.72, with a good-to-excellent reproducibility index for all comparisons, except for the DAS28/ACR20 comparison. The best agreement was obtained by comparing the DAS and the ACR Pediatric 30. For patients older than 16 years, the reproducibility index was good or excellent in only 2 cases, i.e., comparing the DAS and the ACR Pediatric 30 and comparing the DAS and the DAS28 (as expected). CONCLUSION: Our study shows a good agreement overall for the different criteria tested. The highest concordance was observed between the DAS and the ACR Pediatric 30, the lowest between the DAS28 and the ACR20. Our data suggest that the ACR Pediatric 30 criteria can be used also in adult patients affected by JIA, and that the original DAS can be an alternative to the ACR Pediatric 30 in both children and young adults with JIA.

Adolescent↗

Efficacy and safety profile of cyclosporin A in the treatment of juvenile chronic (idiopathic) arthritis. Results of a 10-year prospective study.

OBJECTIVE: This open prospective trial was performed in order to assess the efficacy and safety of cyclosporin A in the treatment of patients with juvenile chronic arthritis (JCA). METHODS: Thirty-four of the patients enrolled were affected by systemic-onset disease and seven by chronic anterior uveitis associated with JCA. The cyclosporin dose was usually 3-5 mg/kg per day. The average duration of therapy was 1.4 yr, with a maximum of 7.2 yr. RESULTS: The efficacy of treatment was mainly evident in terms of control of fever and reduction of steroid therapy. The benefits with respect to arthritis, laboratory parameters and uveitis seemed to be less clear-cut. Side-effects were frequent but usually mild or reversible. Sixty-six per cent of the study population withdrew from therapy because of inefficacy or side-effects. Eight systemic patients withdrew from therapy owing to complete remission. CONCLUSION: Cyclosporin can be used in the treatment of JCA, its main benefits being the control of fever and a steroid-sparing effect.

Adolescent↗

Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years.

OBJECTIVE: To investigate the relationships between systemic onset juvenile idiopathic arthritis disease activity, course of the disease, and functional class according to Steinbrocker. METHODS: The records of all children with systemic onset juvenile arthritis (JA) according to the American College of Rheumatology criteria attending our center since 1971 with a minimum followup period of 3 years were reviewed. A cohort of 80 consecutive patients entered the study: 42 males, 38 females, mean age at onset 6.3 years (range 0.7-16), mean followup period 10.7 years (range 3-33). The cumulative duration of the active periods (CDAP) in months was calculated for every patient. RESULTS: Three patterns of disease course were apparent: monocyclic (subtype I), intermittent (subtype II), and persistent (subtype III). At the last control the functional class and disease activity status were evaluated. In all subtype I patients (9 cases) the disease was in remission and no patient was in class II, III, or IV. In subtype II patients (27 cases), 16 were inactive or in remission and 6 in class III. In subtype III (44 cases) 21 were inactive or in remission and 17 were in class III or IV. The equation relating the Steinbrocker class to the CDAP was calculated considering the functional outcome as the dependent variable. The linear regression equation y = 0.0083 x + 1.266 was found with a correlation coefficient r = 0.586 (p < 0.0001). The majority of our patients were treated with disease modifying antirheumatic drugs, which in many cases were effective in reducing the duration of the active phases of disease. CONCLUSION: Systemic onset JA may present with different clinical courses; the functional outcome is always good in subtype I (monocyclic), but can be poor in subtypes II and III. The severity of disability evaluated according to Steinbrocker classes is dependent on the cumulative duration of the active periods of the disease.

Adolescent↗

[Temporomandibular joint involvement in juvenile idiopathic arthritis: treatment with an orthodontic appliance].

OBJECTIVE: About 65% of children suffering from juvenile idiopathic arthritis (JIA) shows a more or less marked involvement of temporo-mandibular joint (TMJ) with altered mandibular growth, resorption of the condyles, occlusary instability, reduced chewing ability and facial dysmorphia. The purpose of our study is to prevent and to treat the progressive evolution of JIA on craniofacial growth and morphology with a functional appliance; surgery should be considered only in so far as the adequacy of TMJ movement is concerned. METHODS: From 1992 until now 72 children with proved JIA and TMJ involvement have been treated (50 females, 22 males, aged 6 to 16 years old). TMJ involvement was bilateral in 61% and unilateral in 39% of patients. A diagnostic workup was carried out involving tomograms of TMJ and cephalometric radiograph and analysis. The authors used a bimaxillary activator in the attempt to modify the unfavourable growth pattern and provide a gradual ante-rotation of the jaw. RESULTS: Almost all JIA patients showed satisfactory long term results, easing of pain, reduced skeletal discrepancy, increased function and good facial profile. CONCLUSIONS: The long term results of this study indicate that orthopaedic therapy might control the vicious circle of the malocclusion in children with JIA, preventing exacerbation of mandibular clockwise rotation. Surgical intervention for the improvement of TMJ function should be considered only if a severe restricted state is imminent.

Adolescent↗

Evaluation by dual-photon absorptiometry of bone mineral loss in rheumatic children on long-term treatment with corticosteroids.

The introduction of reliable and non-invasive methods of measuring bone mass has allowed investigators to study the bone mass loss (ostopenia) related to rheumatic diseases and corticosteroid therapy. Serial measurements of lumbar bone mineral density (BMD) by dual-photon absorptiometry (DPA) is an effective method of checking bone mineralisation. In children, however, the bone mass is not constant over time, so bone densitometric measurements must take into account not only the net loss but also the missing increase in BMD. In order to study the effects of both chronic rheumatic disease and the long-term administration of corticosteroids in children, as a first step the reference curves of bone mineralisation (mean BMD values and annual % BMD growth rates) were calculated from a control population of 79 children (32 males and 47 females) aged 3 to 20 years. All the subjects involved in the study underwent DPA utilising a Norland Bonestar instrument provided with a 153 Gadolinium source and the average BMD (g/cm2) of the lumbar spine was calculated. As a second step, a transverse study was carried out on a series of 157 children and young adults affected by chronic rheumatic diseases with juvenile onset. As a third step, a longitudinal study on the adverse effect of the chronic administration of corticosteroids on bone mineralisation was carried out on a series of 58 patients affected by chronic rheumatic diseases with juvenile onset who were being treated with long-term corticosteroids. Serial measurements of bone mass should help us to improve our strategies in preventing and counteracting osteopenia due to the chronic administration of corticosteroids also in children.

Absorptiometry, Photon↗

[Rheumatic fever].

While rheumatic fever (RF) remains a major problem in underdeveloped countries, in continental United States and Western Europe the incidence of this disease declined markedly in the sixties and reached a nadir during the seventies. However in the last eighties a resurgence of RF has been documented in some areas of the United States with an eightfold increase of incidence over the prior 15 year average. Although a true outbreak of RF has not been documented in Italy, a trend towards an increased number of new cases per year has been observed at the Centre for Rheumatic Children at the Gaetano Pini Institute in Milan. Most of these children presented a mild disease with clinical features rather different from those described in classical textbooks. Most of them satisfied the revised Jones' criteria, in some cases the objective signs of arthritis could not be noticed, but the joint involvement presented as marked arthralgia while other features supported the diagnosis of RF. Rheumatic carditis was observed in more than 1/3 of cases with a high rate of residual valvular heart disease of a mild degree of severity in most cases. In conclusion RF has not completely disappeared in Italy and remains as a possible cause of permanent valvular heart damage in children. It is possible that the increasing morbidity observed in the last eighties may be related to the reduced primary prophylaxis against streptococcal infections due to the wrong conviction that RF is no more a risk in developed countries.

Adolescent↗

Validation of the Italian version of the Stanford Childhood Health Assessment Questionnaire for measuring functional status in children with chronic arthritis.

The Stanford Health Assessment Questionnaire developed by Singh et al. to measure functional status in children with chronic arthritis (CHAQ) was translated into Italian (I-CHAQ), with minor modifications to obtain cross-cultural equivalence. This version was evaluated in a series of 96 patients with juvenile rheumatoid arthritis (JRA), both males and females ranging in age from 3 to 19 years (mean age 9.9 years). All three onset subtypes and all four classes of disability were represented in the sample. The questionnaire was filled in by the parents if the children were less than 8 years of age (23 cases), and by the children themselves in all other cases; a health professional was always present to provide assistance. As expected, JRA patients with a systemic or polyarticular disease onset had higher scores than those with a pauciarticular onset, and there were statistically significant differences in disability index values between patients from different Steinbrocker functional classes. The instrument showed good reproducibility in a test-retest over a two-week period, a high correlation between the child and parent scores, excellent internal reliability, and good convergent and discriminant validity.

Activities of Daily Living↗

A multicenter study on insulin-like growth factor-I serum levels in children with chronic inflammatory diseases.

OBJECTIVE: To study insulin-like factor-I (IGF-I) levels in children and adolescents with connective tissue diseases (CTDs), compare them with values obtained in normal controls, and correlate them with age, sex, steroid treatment, and inflammatory parameters. METHODS: A multicenter, cross-sectional study was performed in 3 Italian pediatric rheumatology centers. The subjects studied comprised 117 patients with juvenile arthritis (53 systemic, 25 pauciarticular and 17 polyarticular) and other CTDs (22), and 78 children without inflammatory conditions. IGF-I levels were measured by radioimmunoassay after acid-ethanol extraction. RESULTS: Mean IGF-I serum levels were 167.6 ng/ml (+/- 132.5) in patients and 214.4 (+/- 142.8) in controls. A significant correlation was found between IGF-I levels and age in the controls (P = 0.001), but not in the patients. Covariance analysis with age as the covariate showed significantly lower IGF-I levels in the patient group (P = 0.001). No significant correlation was found between IGF-I levels and the total quantity of steroid taken. Multiple regression analysis showed that IGF-I levels were inversely correlated with the ESR (P = 0.0001) and positively correlated with age (P = 0.0002) and sex (P = 0.021) in the patient group. CONCLUSION: IGF-I serum levels are decreased in patients with CTDs; inflammation could play a major role.

Adolescent↗