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Biomedical subjects

A Buoncompagni

Publications and source records attributed to A Buoncompagni.

At least 37 records · Page 2Linked to original sources
PubMed1998

Performance of the preliminary definition of improvement in juvenile chronic arthritis patients treated with methotrexate. Italian Pediatric Rheumatology Study Group.

OBJECTIVE: To investigate the performance of the core set of outcome measures and the preliminary definition of improvement (PDI) in the assessment of response to methotrexate (MTX) treatment in children with juvenile chronic arthritis (JCA). METHODS: Data were obtained from an open label, non-controlled trial designed to investigate the efficacy of MTX in children with JCA. All patients had the core set of variables assessed at baseline and after six months of treatment. Variables in the core set are: (1) physician global assessment of disease activity; (2) parent or patient (if appropriate in age) global assessment of overall well being; (3) functional ability; (4) number of joints with active arthritis; (5) number of joints with limited range of motion; (6) erythrocyte sedimentation rate. The PDI specifies that to be classified as improved, a patient must show at least 30% improvement from baseline in three of any six variables in the core set, with no more than one of the remaining variables worsening by more than 30%. RESULTS: A total of 111 JCA patients were included in the study. According to the PDI, after six months of MTX treatment 73 patients (66%) were classified as improved and 38 (34%) as not improved. Among the core set variables, parent assessment detected the highest percentage of patients improved (72%) and functional assessment the lowest (37%). CONCLUSION: The PDI identifies about two thirds of patients with JCA treated with low dose MTX as improved. This proportion is similar to that expected to improve based upon a previous controlled study of low dose, oral MTX and provides preliminary evidence of the definition's validity.

Adolescent↗
PubMed1998

Serum interleukin 12 concentration in juvenile chronic arthritis.

OBJECTIVES: The aim of this study was to evaluate serum interleukin (IL) 12 concentration in patients with juvenile chronic arthritis (JCA), according to disease subtype, activity, and duration. IL12 has been demonstrated to prime the selective expansion of T helper (Th) cells with a Th1-type pattern of cytokine production. METHODS: Sixty eight serum samples from 50 JCA patients (12 systemic, 12 polyarticular, 26 pauciarticular), 20 serum samples from age matched healthy controls were tested with two different immunoassays specific for total IL12 (p40 and p70 heterodimer) and for IL12 (p70) heterodimer, respectively. The following disease activity parameters were evaluated: (a) presence of arthritis at least in one joint, (b) physician global estimate of disease activity, (c) disability index according to the Childhood Health Assessment Questionnaire (CHAQ), (d) C reactive protein (CRP). RESULTS: Total IL12 (p40 and p70 heterodimer) was significantly higher in JCA active patients than in those on clinical remission and in healthy controls (p < 0.001). Conversely, detectable concentrations of IL12 (p70) heterodimer were found in three active JCA patients only. Moreover, total IL12 (p40 and p70 heterodimer) showed a significant negative correlation both with time from disease diagnosis (r = -0.29, p = 0.04) and, for the pauciarticular subgroup, with disease activity duration (r = -0.71, p < 0.001). CONCLUSIONS: This study shows that the p40 moiety of IL12 is increased in serum samples from active JCA patients, especially in the earliest phases of the disease, whereas biological active IL12 (p70) heterodimer is virtually undetectable.

Adolescent↗
PubMed1998

Prolactin and interleukin 6 in prepubertal girls with juvenile chronic arthritis.

OBJECTIVE: To evaluate prolactin (PRL) serum levels in prepubertal girls affected with 2 different subtypes of pauciarticular onset juvenile chronic arthritis (JCA) and with previous acute postinfectious arthritis in remission (AA), and to correlate the relationship of PRL versus interleukin 6 (IL-6) serum levels. METHODS: Eleven girls with antinuclear antibody (ANA) positive early onset pauciarticular JCA, 8 with ANA negative late onset pauciarticular JCA of various forms (considered to have spondyloarthropathy, SpA), and 7 who had had AA were evaluated for serum concentrations of PRL, IL-6, and thyroid hormones and presence of uveitis. All were prepubertal and without clinical or biological signs of disease activity. RESULTS: Mean serum concentrations of PRL were significantly increased in ANA positive (8.9 +/- 4.0 ng/ml) patients and in patients with SpA (7.8 +/- 2.4 ng/ml) compared to those of AA patients (4.4 +/- 0.3 ng/ml) (p = 0.01 and p = 0.025, respectively) and to controls. Both ANA positive and SpA patients showed increased mean serum concentrations of IL-6 in comparison with AA patients and controls. Significant correlation between PRL and IL-6 concentrations (r = 0.604, p = 0.002) was observed from the whole series. CONCLUSION: We found a direct correlation between serum levels of PRL and IL-6 in both ANA positive JCA patients and in ANA negative SpA patients; thus, hyperprolactinemia correlates better with the chronic course of the disease than with ANA positivity.

Adolescent↗
PubMed1998

Differences in tumor necrosis factor-alpha soluble receptor serum concentrations between patients with Henoch-Schönlein purpura and pediatric systemic lupus erythematosus: pathogenetic implications.

OBJECTIVE: Animal models of immune complex mediated tissue injury have shown different patterns of proinflammatory cytokine production according to the subtype of immunoglobulin involved. The IgA immune complex model differs from the IgG model by the lack of involvement of tumor necrosis factor (TNF) in the pathogenesis of tissue damage. We investigated in age matched patients the possible difference in TNF involvement in a predominantly IgA mediated disease, Henoch-Schönlein purpura (HSP), in comparison with systemic lupus erythematosus (SLE), in which vascular injury is mostly associated with local deposition of IgG immune complexes. METHODS: Serum concentrations of TNF-alpha and its soluble receptors (sTNF-R) p55 and p75 were studied in 20 patients with pediatric SLE at various degrees of disease activity, in 16 patients with highly active HSP, and in 15 healthy controls by enzyme amplified sensitivity immunoassay. SLE disease activity was evaluated using 2 scores, the European Consensus Group Study for SLE Disease Activity Criteria and the SLE Disease Activity Index. RESULTS: Serum concentrations of TNF-alpha fell within the normal range in patients with both SLE and HSP irrespective of disease activity. Conversely, patients with SLE displayed increased serum levels of sTNF-R that correlated positively with the degree of disease activity (r = 0.60, p < 0.001; r = 0.71, p < 0.001, for p55 and p75, respectively). In contrast, no difference in the serum levels of sTNF-R was found between patients with highly active HSP and controls. CONCLUSION: Our study provides the first circumstantial evidence that pediatric SLE and HSP are characterized by differential involvement of TNF in the pathogenesis of tissue damage.

Adolescent↗
PubMed1997

Retinitis pigmentosa, hypopituitarism, nephronophthisis, and mild skeletal dysplasia (RHYNS): a new syndrome?

We report on a 17 6/12-year-old boy with nephronophthisis, retinitis pigmentosa, left upper eyelid ptosis, enopthalmos, transmissive deafness, GH and TSH deficiency, and mild skeletal dysplasia. A similar case was reported by Bianchi et al. [1988: Helv Paediatr Acta 43:449-455] in another Italian patient. Here we confirm the previous observations and argue that both patients might be affected by a new syndrome.

Abnormalities, Multiple↗
PubMed1997

Synovial fluid T cell clones from oligoarticular juvenile arthritis patients display a prevalent Th1/Th0-type pattern of cytokine secretion irrespective of immunophenotype.

The aim of the present study was to investigate the patterns of cytokine production by T cell clones raised from in vivo activated synovial fluid (SF) mononuclear cells (MNC) of five patients with oligoarticular juvenile arthritis (JA). Freshly isolated SF T cells were cultured in vitro with low dose recombinant IL-2 and subsequently cloned by limiting dilution. Sixty-four clones were obtained from the five patients studied. Fifty-nine clones were TCR alpha/beta+, either CD4+ (n = 43) or CD8+ (n = 15). The remaining five clones were TCR gamma/delta+, CD4-, CD8-. Clone immunophenotypes differed in the individual patients. Forty-four T cell clones were stimulated with phytohaemagglutinin (PHA) and phorbol myristate acetate (PMA) and supernatants tested for the presence of IL-2, IL-4, IL-5 and interferon-gamma (IFN-gamma) by ELISA or bioassays. Cytokine mRNA accumulation was tested by reverse transcriptase-polymerase chain reaction (RT-PCR). Most of 44 clones tested released large amounts of IFN-gamma irrespective of the immunophenotype. Of these, 27 were classified as Th1-type and 17 as Th0-type based upon the IFN-gamma/IL-4 ratio in culture supernatants. Finally, when 10 representative T cell clones were tested for pro- and anti-inflammatory cytokines, gene expression by RT-PCR, all of them were found to express the granulocyte-macrophage colony-stimulating factor (GM-CSF), tumour necrosis factor-alpha (TNF-alpha), IL-10 and transforming growth factor-beta 1 (TGF-beta1) genes, and half of them IL-6 and IL-8 mRNA. In conclusion, T cell clones, that represent the progeny of in vivo activated SF T cells from oligoarticular JA patients, display heterogeneous immunophenotypes, but all share the ability to produce large amounts of IFN-gamma, with a predominant Th1/Th0 pattern. The expression of pro- and anti-inflammatory cytokine genes in these clones suggests that in vivo activated SF T cells modulate joint inflammation in a complex fashion.

Adolescent↗
PubMed1996

Serum p55 and p75 tumour necrosis factor receptors as markers of disease activity in juvenile chronic arthritis.

OBJECTIVE: To determine the expression of tumour necrosis factor alpha (TNF alpha) and its soluble receptors (p55 and p75) in the sera and synovial fluid of patients with juvenile chronic arthritis (JCA), and their correlation with disease activity parameters. METHODS: Ninety eight sera from 45 patients with JCA (14 systemic, 12 polyarticular, 19 pauciarticular), 20 sera from age matched healthy controls, and five synovial fluids from five antinuclear antibody (ANA) positive pauciarticular JCA patients were tested for the presence of TNF alpha, soluble TNF receptors p55 and p75 (sTNFRp55, sTNFRp75), and interleukin-6 (IL-6) by an enzyme amplified sensitivity immunoassay. Physician global estimate of disease activity, weekly fever score and joint score, C reactive protein (CRP), erythrocyte sedimentation rate (ESR), and haemoglobin concentration were evaluated as parameters of disease activity. The expression of p55 and p75 on peripheral mononuclear cells (MNCs) from five patients with systemic JCA and synovial MNCs from five ANA positive patients with pauciarticular JCA was evaluated by flow cytometry. RESULTS: TNF alpha serum concentrations did not differ significantly between the patients with active JCA and the control group. No correlation was found between TNF alpha and parameters of disease activity, but both p55 and p75 showed a significant positive correlation with the physician global estimate of disease activity (p < 0.001), ESR (p < 0.001), CRP (p < 0.001), and serum concentrations of IL-6 (p < 0.001). Serum concentrations of haemoglobin correlated inversely with the concentrations of p55 and p75 (p < 0.001). Synovial lymphocytes selectively expressed the p75 surface receptor. CONCLUSIONS: sTNFRp55 and sTNFRp75 each represent a sensitive marker of disease activity in JCA. Their increased expression in biological fluids may support the hypothesis that TNF alpha has a role in the pathogenesis of JCA.

Adolescent↗
PubMed1996

6-methylprednisolone 'mini-pulses': a new modality of glucocorticoid treatment in systemic onset juvenile chronic arthritis.

From 1989 to 1994 we have been following in our Department 22 patients (12 males, 10 females, age range 1-13.5 years) affected by sJCA who underwent glucocorticoid (GC) treatment. These patients were randomised to receive for 6 months two different GC schedules: group A (12) received 6 methylprednisolone (6MP) i.v. for 3 days with 5 mg/kg/d and for an additional 3 days at 2.5 mg/kg/d followed by po prednisone (1 mg/kg/d); group B (10) received po prednisone (1 mg/kg/d). A remarkable decrease of disease activity and fever score and a prompt improvement of joint involvement were observed. A CPR serum concentration decrease associated to a consistent increase of Hb over baseline values were disclosed in group A. GC cumulative daily requirement was significantly lower in group A. Four patients from group B but only one from group A developed a cushingoid appearance: no differences of BMI were found in the two groups. Our study shows that in sJCA patients i.v. GC mini pulses are effective on early clinical and biological parameters of inflammation and requires a lower cumulative daily dosage than conventional oral GC treatment.

Administration, Oral↗
PubMed1995

Antiphospholipid antibodies in paediatric systemic lupus erythematosus, juvenile chronic arthritis and overlap syndromes: SLE patients with both lupus anticoagulant and high-titre anticardiolipin antibodies are at risk for clinical manifestations related to the antiphospholipid syndrome.

Antiphospholipid antibodies (APA) are often associated with severe clinical manifestations, especially in the setting of systemic lupus erythematosus (SLE). Here we have investigated the prevalence of anticardiolipin antibodies (aCL) and lupus anticoagulant (LA) in paediatric patients affected with SLE, JCA and overlap syndromes (OS) and correlated the presence of aCL and LA with clinical features. aCL were assayed by enzyme-limited immunoassay; LA was determined by activated partial thromboplastin time and the kaolin clotting time test. aCL and LA assays were performed in parallel on at least two occasions over a 7-30-month follow-up. Fifteen out of nineteen (79%) SLE patients had aCL and 8/19 (42%) had LA. Six SLE patients displayed manifestations that were APA-related: deep venous thromboses, autoimmune haemolytic anaemia, pulmonary hypertension, neurological alterations. Five out of six symptomatic patients had both LA and high-titre aCL. In contrast, JCA and OS patients had usually low-titre aCL, no detectable LA and no APA-related manifestations. aCL persisted at high titre over time in SLE patients, but was only transiently detected in JCA and OS patients. This study shows that the simultaneous positivity for LA and high-titre aCL allows the identification of paediatric SLE patients who are at risk not only for thrombosis, but also for other APA-related clinical features.

Adolescent↗
PubMed1993

Clinical diversity of Raynaud's phenomenon in childhood: report of six cases.

Six pediatric patients with Raynaud's phenomenon are reported. Three patients had clinical and immunological evidence for a connective tissue disease; one had primary Raynaud's phenomenon associated with antinuclear antibodies and two had Raynaud's phenomenon secondary to exposure to lead and to a vibrating instrument, respectively. The diagnostic and therapeutic implications of these observations will be discussed.

Adolescent↗
PubMed1991

[Infantile dermatomyositis. Clinical aspects and prospective treatments].

Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.

Acute Disease↗