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A Ravelli

Publications and source records attributed to A Ravelli.

At least 55 records · Page 3Linked to original sources
PubMed1997

Soluble tumour necrosis factor receptor levels reflect coagulation abnormalities in systemic juvenile chronic arthritis.

The objective was to evaluate tumour necrosis factor (TNF) status in patients with systemic juvenile chronic arthritis (s-JCA). Plasma levels of TNF-alpha, and serum levels of soluble TNF receptor 1 and 2 (sTNFR1 and sTNFR2) were measured using specific immunoassays in 20 patients with s-JCA, 10 with polyarticular JCA and 15 with pauciarticular JCA, and in 20 controls comparable for age. In patients with active s-JCA, circulating levels of TNF-alpha, sTNFR1 and sTNFR2 were significantly (P < 0.001) higher than those of controls. The levels of sTNFR1 and sTNFR2, but not those of TNF-alpha, were associated with the persistence and severity of systemic symptoms and were significantly correlated with prolongation of partial thromboplastin time and decrease in prothrombin activity. In two patients evaluated during a s-JCA-associated macrophage activation syndrome, a marked increase in sTNFR1 and sTNFR2 was found. Our results suggest that in s-JCA, TNF is involved in systemic manifestations, in the subclinical coagulation abnormalities, and in the development of the macrophage activation syndrome.

Adolescent↗
PubMed1997

Cytokines in juvenile rheumatoid arthritis.

Most data concerning the role of cytokines in chronic arthritides have been obtained in adult rheumatoid arthritis. In this paper we review the available evidence regarding the role of the inflammatory cytokines interleukin-1, interleukin-6, and tumor necrosis factor in juvenile rheumatoid arthritis, focusing in particular on the differences among the various onset types. Results from the analysis of cytokine expression may provide the basis for the use of specific anticytokine treatment. Recent clinical trials in adult rheumatoid arthritis have demonstrated the feasibility and clinical usefulness of these treatments.

Arthritis, Juvenile↗
PubMed1997

Correlation between conventional disease activity measures in juvenile chronic arthritis.

OBJECTIVE: To estimate in a cross sectional analysis the degree of colinearity among the disease activity measures more commonly used in juvenile chronic arthritis (JCA). METHODS: This study assessed in a single clinical evaluation three subjective variables, three measures of functional capacity, eight articular indices, and two laboratory indicators of systemic inflammation in 55 consecutive children with JCA. The relation between the clinical measures of JCA activity was determined by Pearson correlation coefficients. An r value of 0.7 or greater was considered evidence of colinearity. RESULTS: Among the subjective variables, parent global assessment of overall well being and parent assessment of pain were correlated with each other; the physician assessment of disease activity did not show evidence of colinearity with any other variable. The functional status measures were correlated with each other, but not with the indices of articular inflammation. There was a high degree of colinearity among the articular variables, with the number of active joints and the overall severity score being correlated with each other as well as with all the single articular indices. The laboratory variables were correlated with each other, but not with any of the articular, functional or subjective variables. CONCLUSION: Our results show a high degree of colinearity among the disease activity measures belonging to the same category, whereas this is uncommon for variables that investigate different domains of disease activity. These data underline the need to include the evaluation of each domain in the assessment of JCA activity.

Adolescent↗
PubMed1997

The clinical significance of antiphospholipid antibodies.

The antiphospholipid syndrome is defined as the association between the presence of antiphospholipid antibodies, detected as anticardiolipin antibodies and/or lupus anticoagulant, and a history of either arterial or venous thrombosis and/or recurrent pregnancy loss. Because thrombosis may occur in virtually any organ system, diagnosing the antiphospholipid syndrome and taking appropriate anticoagulation measures are important considerations in all medical specialties. Antiphospholipid antibody-associated thrombosis tends to recur. Antithrombotic prophylaxis to prevent recurrences is therefore needed. Prophylaxis in individuals with circulating antiphospholipid antibodies who have no history of thrombosis is still controversial. Although direct evidence for a pathogenetic role of antiphospholipid antibodies in the development of thrombosis is still lacking, recent studies suggest that it is causative rather than coincidental. New insights on the possible mechanisms leading to thrombosis were provided by the discovery of the serum cofactor (beta2-GPI), a coagulation inhibitor which is required for binding of anticardiolipin antibodies to cardiolipin. More recently, patients with antiphospholipid antibodies were found to possess autoantibodies directed against other coagulation factors, including prothrombin, protein C and protein S. Future studies should clarify whether these different antigenic specificities are associated with particular clinical events and assess the risk of thrombosis associated with the presence of antiphospholipid antibodies in asymptomatic individuals.

Antibodies, Antiphospholipid↗
PubMed1997

Health education in mental health services.

In the past few years, we have witnessed numerous efforts to develop health education in psychiatry. Many of them, however, were not successfully implemented. In the Netherlands, a health education method was developed in conjunction with seven mental health providers. The method promotes a more successful implementation.

Health Education↗
PubMed1997

Long-term health outcomes and quality of life in American and Italian inception cohorts of patients with juvenile rheumatoid arthritis. I. Outcome status.

OBJECTIVE: To assess the long-term health outcomes and quality of life of patients with juvenile rheumatoid arthritis (JRA) using health and functional assessment questionnaires in 2 populations, one from the USA and one from Italy. METHODS: Patient eligibility criteria: (1) first examined in our units between 1958 and 1990 during the first 6 months after onset of symptoms, (2) diagnosis of JRA by the American College of Rheumatology criteria, (3) disease duration of at least 5 years at the time of assessment of outcome. Instruments used: (1) the Health Assessment Questionnaire (HAQ, short form, or childhood HAQ (CHAQ), and (2) Quality of Life Scales (QOLS, adults only). Eligible patients were identified by computer search and chart review and were then mailed a packet containing a consent/assent form and the assessment instruments. RESULTS: Of 346 patients who met the eligibility criteria were able to locate 301, and 290 verbally agreed to participate and were mailed packets. Signed consent and complete information were received from 227 of the 290 (78%), 178 from the USA and 49 from Italy. Mean duration of disease at the time of outcome assessment was 15 yrs. 127 had pauciarticular, 55 polyarticular, and 45 systemic onset disease. Mean and (median) scores of the outcomes are shown in the table. [table: see text] CONCLUSION: Long-term outcome, as assessed by the instruments used, is very favorable in most patients with JRA 5 years or more after onset of symptoms.

Adolescent↗
PubMed1997

Long-term health outcomes and quality of life in American and Italian inception cohorts of patients with juvenile rheumatoid arthritis. II. Early predictors of outcome.

OBJECTIVE: To determine whether demographic, clinical, and immunogenetic variables measurable during the first 6 months of illness long-term health outcomes and quality of life in patients with juvenile rheumatoid arthritis (JRA). METHODS: Patient eligibility criteria: (1) first examined in our units between 1958 and 1990 within 6 months of onset of symptoms; (2) diagnosis of JRA by American College of Rheumatology criteria; (3) disease duration of at least 5 years at the time of assessment of outcome. Instruments used: (1) the Health Assessment Questionnaire (HAQ, short form), or Childhood HAQ (CHAQ) to measure disability (0-3 scale), (2) pain, and (3) parental assessment of overall well being, each scored on a 15 cm visual analog scale; (4) the Quality of Life Scales (QOLS) (adults only). Independent variables that showed significant results using univariate tests underwent multiple logistic regression analysis. RESULTS: 227 patients were available for analysis. Mean duration of disease at time of assessment of outcome was 15 years (range 5.3-36.1). Univariate tests allowed 11 variables for disability, 9 for pain, 7 for overall well being, and 4 for QOL into the multivariate analysis. The best predictor of higher disability was the articular severity score (odds ratio, OR, 5.69) while antinuclear antibody positivity foretold less disability (OR 0.29). HLA-DR5 positivity conferred the greatest risk for pain (OR 3.34), while HLA-B5, DR3, and C3 were protective (OR 0.25, 0.28, 0.33, respectively). Early hand involvement was the strongest predictor of poorer overall well being (OR 8.75). Only the erythrocyte sedimentation rate was predictive of future QOL, but the model yielded a low C statistic (< 70%) and the OR 95% confidence limits were extreme (OR 9.77; 95% confidence interval, 1.22-77.8). CONCLUSION: Clinical and immunogenetic variables measurable within 6 months of onset of JRA can be used to predict future disability, pain, and well being. QOL appears more difficult to forecast, perhaps due to the multiple domains that make up this outcome. Further study is needed to identify other genetic and laboratory factors that predict outcome in JRA with greater precision.

Adolescent↗
PubMed1996

Defective iron supply for erythropoiesis and adequate endogenous erythropoietin production in the anemia associated with systemic-onset juvenile chronic arthritis.

Systemic-onset juvenile chronic arthritis (SoJCA) is associated with high levels of circulating interleukin-6 (IL-6) and is frequently complicated by severe microcytic anemia whose pathogenesis is unclear. Therefore, we studied 20 consecutive SoJCA patients with hemoglobin (Hb) levels <12 g/dL, evaluating erythroid progenitor proliferation, endogenous erythropoietin production, body iron status, and iron supply for erythropoiesis. Hb concentrations ranged from 6.5 to 11.9 g/dL. Hb level was directly related to mean corpuscular volume (r = .82, P < .001) and inversely related to circulating transferrin receptor (r = -.81, P < .001) suggesting that the severity of anemia was directly proportional to the degree of iron-deficient erythropoiesis. Serum ferritin ranged from 18 to 1,660 microgram/L and was unrelated to Hb level. Bone marrow iron stores wore markedly reduced in the three children investigated, and they also showed increased serum transferrin receptor and normal-to-high serum ferritin. All 20 patients had elevated IL-6 levels and normal in vitro growth of erythroid progenitors. Endogenous erythropoietin (epo) production was appropriate for the degree of anemia as judged by both the observed to predicted log (serum epo) ratio 10.95 +/- 0.12) and a comparison of the serum epo-Hb regression found in these subjects with that of thalassemia patients. Multiple regression analysis showed that serum transferrin receptor was the parameter most closely related to hemoglobin concentration: variation in circulating transferrin receptor explained 61% of the variation in Hb level (P < .001). In 10 severely anemic patients, amelioration of anemia following intravenous iron administration resulted in normalization of serum transferrin receptor. Defective iron supply to the erythron rather than blunted epo production is the major cause of the microcytic anemia associated with SoJCA. A true body-iron deficiency caused by decreased iron absorption likely complicates long-lasting inflammation in the most anemic children, and this can be recognized by high serum transferrin receptor levels. Although oral iron is of no benefit, intravenous iron saccharate is a safe and effective means for improving iron availability for erythropoiesis and correcting this anemia. Thus, while chronically high endogenous IL-6 levels do not appear to blunt epo production, they are probably responsible for the observed abnormalities in iron metabolism. Anemia of chronic disease encompasses a variety of anemic conditions whose peculiar features may specifically correlate with the type of cytokine(s) predominantly released.

Administration, Oral↗
PubMed1996

Neurological disorders, other than stroke, associated with antiphospholipid antibodies in childhood.

We report the results of a systematic study on the association of antiphospholipid antibodies (aPLs) with some neurological disease other than stroke in a childhood population. Patients affected by migraine, benign intracranial hypertension (BIH) or unilateral movement disorders, such as hemichorea and hemidystonia with acute-subacute onset, were screened for aPLs. None of them had clinical or serological evidence of Systemic Lupus Erythematosus (SLE) or other connective tissue disease. Moderate to high levels of anticardiolipin antibodies (aCL) and/or positive Lupus Anticoagulant (LA) were demonstrated in 6 out of 17 patients with migraine, in 3 out of 4 patients with BIH and in all of the 5 patients showing unilateral movement disorders. The association between aPLs and these neurological conditions, usually regarded as cryptogenic, may suggest a possible pathogenetic mechanism.

Adolescent↗
PubMed1996

Pulmonary function in children with systemic lupus erythematosus.

BACKGROUND: Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. METHODS: After a mean of 4.5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). RESULTS: In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage of patients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. CONCLUSIONS: In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease.

Adolescent↗
PubMed1995

Evaluation of response to methotrexate by a functional index in juvenile chronic arthritis.

In juvenile chronic arthritis (JCA) the indices of disease activity, such as joint swelling, pain, tenderness, and limitation of motion, may poorly correlate with the degree of functional impairment. Since the improvement of functional capacity is one of the main objectives of treatment, we evaluated the effect of methotrexate (MTX) in 29 children with JCA by assessing both the articular indices and a functional status measure. After 6 months of therapy, 15 of the 29 patients had a significant (> or = 50%) reduction in the number of joints with active arthritis and/or the articular severity score and were then judged as responders, while 14 did not respond to MTX. In the responder group, the median functional index score decreased from 17 (range: 13-27) at baseline to 13 (range: 13-15) at 6 months (p < 0.001); the median score of the non-responder group was 20 both at baseline and at 6 months (ranges: 14-27, and 13-36, respectively). These results show an improvement of functional impairment in those patients who respond to MTX according to the conventional indices of articular inflammation.

Adolescent↗
PubMed1995

Chronic recurrent multifocal osteomyelitis with unusual features.

We describe two children with chronic recurrent multifocal osteomyelitis who showed unusual extraskeletal manifestations. The first patient developed an asymptomatic pulmonary consolidation; the second had an atypic localization of the associated pustulosis, which involved the trunk, groin, thighs and buttocks, but spared the palms and soles. We conclude that this disease is clinically more heterogeneous than previously thought.

Child↗
PubMed1995

Recurrent chest pain as the presenting manifestation of spinal meningioma.

We report the case of a 9-year-old boy with a spinal cord meningioma whose only manifestations were recurrent episodes of chest pain lasting for 2 years. This case shows that spinal cord meningioma should be considered among the possible causative factors of chronic chest pain in childhood.

Chest Pain↗
PubMed1995

Frequency of relapse after discontinuation of methotrexate therapy for clinical remission in juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the outcome of children with juvenile rheumatoid arthritis (JRA) who discontinued taking methotrexate (MTX) therapy after the achievement of clinical remission. METHODS: We conducted a retrospective review of the clinical course of all consecutive patients with JRA treated with MTX at our department. RESULTS: Seventeen of the 30 patients who responded to treatment entered clinical remission 6 to 30 mo after beginning MTX therapy. All patients were discontinued from MTX within 2 to 5 mo. Five patients relapsed within 9 mo, whereas 12 patients had sustained remission for 12 mo or longer. Four of 5 children with extended pauciarticular JRA relapsed early after MTX discontinuation. These patients were less responsive to a further cycle of MTX, and 3 showed progression of radiographic joint lesions. CONCLUSION: These findings suggest that MTX should be continued in patients with extended pauciarticular JRA even after the achievement of clinical remission.

Adolescent↗