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Leukaemia can present with joint swelling in the absence of abnormal haematological findings. Arthritis as a presenting sign of lymphoma, however, is extremely rare. Three children with non-Hodgkin's lymphoma who had joint swelling at the onset of their disease are reported. Two cases showed histological features of anaplastic large cell lymphoma (Ki-1/CD30 positive), and one of angioimmunoblastic T cell lymphoma. In all patients the unusual presentation delayed correct diagnosis. [ABSTRACT FROM AUTHOR]

Very few double-blind trials of oral immunotherapy have been reported. The majority of these have been performed with pollen extracts and the results have often been equivocal. The major weaknesses of these studies have been the short periods of the trials, the low doses of allergen employed and inadequate evaluation of efficacy. The present study has involved a placebo-controlled double-blind trial of oral immunotherapy for three years with Dermatophagoides pteronyssinus at relatively high doses in 18 paediatric patients. Throughout the trial clinical parameters (symptom and medication scores) and immunological parameters (specific IgE, IgG1 and IgG4 levels) were monitored in order to assess the safety and efficacy of the treatment. The treatment was well tolerated by all patients and no side-effects were experienced. Clinical improvement was evident after the second year of therapy and this was confirmed by a significant reduction in conjunctival reactivity assessed by a specific conjunctival provocation test. In addition, there were significant changes in the immunological parameters with a reduction in specific IgE and increased levels of lgG4 and IgG1, results in keeping with previous studies of oral and subcutaneous immunotherapy. Although the results do not provide an explanation of the basis of successful oral immunotherapy, they clearly demonstrate the efficacy and safety of the treatment and suggest that it may be a useful and more acceptable alternative for patients than the traditional subcutaneous immunotherapy. [ABSTRACT FROM AUTHOR]

After a two week baseline, 209 asthmatic children (mean age 10 years, range 6-17) were randomly allocated to receive 4 mg nedocromil sodium (n = 110) or placebo (n = 99) four times daily for 12 weeks in addition to their current treatment. The children completed daily diary cards and visited the clinic at four week intervals. Statistically significant differences in favour of nedocromil sodium were seen for clinician assessment of asthma severity and diary card symptom scores, pulmonary function and inhaled beta 2 bronchodilator use. Total symptom score decreased by 50% from baseline in the nedocromil sodium group and by 9% in the placebo group during the final four weeks. Nedocromil sodium was considered very or moderately effective by 78% of children/parents (placebo 59%) and 73% of clinicians (placebo 50%). Nausea, headache and sleepiness, and dyspnoea led to withdrawal of one child from nedocromil sodium and placebo treatments, respectively. Reports of sore throat and headache were marginally greater with the nedocromil sodium treatment. It is concluded that nedocromil sodium was both effective and safe in the treatment of asthma in children. [ABSTRACT FROM AUTHOR]

Twenty six children with candidiasis, aged between 5 months and 14 years, were treated with different formulations and regimens of ketoconazole. Fifteen children had alimentary tract candidiasis, two had oesophagitis, one had urinary tract candidiasis, two vaginitis, two septicaemia, one endo-ophthalmitis, and three had chronic pulmonary illness with persistence of Candida albicans in sputum. Daily drug doses ranged from 3 to 13 mg/kg and duration of treatment from seven days to 18 months. Pharmacokinetic studies in 22 of the children are reported. A total of 3 mg/kg/day given in three divided doses did not yield sufficiently high concentrations, which were achieved with a daily dose of 8 to 10 mg/kg. The effectiveness of treatment was proved by negative mycological tests (cultures or specific antibodies, or both) in 88%, by cure in 73%, and improvement in 11%. In three patients evaluation was not possible due to an insufficiently proved diagnosis. Nausea and pyrosis in four patients were the only side effects noted and no laboratory abnormalities were found. To achieve therapeutic concentrations of ketoconazole in children we suggest a daily dosage of 7 to 10 mg/kg in two or three divided doses. [ABSTRACT FROM AUTHOR]

Twenty five children with chronic idiopathic thrombocytopenic purpura followed from 6-96 months in 7 Italian paediatric departments were treated with high dose immunoglobulin according to a multicentre protocol. Positive responses were observed in 20 of 25 patients (80%) and negative responses in 5 of 25 (20%). On previous steroid treatment 7 of 10 positive responders were steroid resistant and 13 of 15 were steroid dependent. Within four weeks of beginning treatment 16 of 20 patients (80%) relapsed, while 4 of 20 (20%) maintained normal platelet values after 4-12 months' follow up. Statistical analysis of the platelet count on day five of treatment enabled us to divide positive responders into three groups: good, intermediate, and poor. The possible mode of action and clinical application of high dose immunoglobulin are discussed. [ABSTRACT FROM AUTHOR]

Plasma salicylate concentration was monitored in 42 children on long-term salicylate therapy for rheumatoid arthritis. A given dose of salicylate per kg resulted in large variations in plasma levels, both between individuals and for a single individual at different times. The factors responsible for such variations were studied; in 6 cases urinary metabolites of salicylate were analysed. The relation between salicylate dosage and plasma half-life accounts for the fact that small changes in dosage can result in large changes in plasma concentration. The addition of corticosteroid or ACTH therapy results in lower plasma levels of salicylate, and necessitates higher dosage of salicylate. After the introduction of routine monitoring of plasma salicylate, the incidence of toxic symptoms fell sharply. [ABSTRACT FROM AUTHOR]

Objective: To analyze clinical characteristics, cardiac outcome and response to therapy of an Italian pediatric population affected with acute rheumatic fever (ARF) in the last 15 years., Methods: 135 patients with ARF (aged 2-14.9 years, mean 8.4+/-2.5 years) diagnosed from 1992 to 2006 at the Pediatric Rheumatology Centre of the University of Milan (Italy) were retrospectively reviewed. All patients underwent physical examination, laboratory evaluation, electrocardiogram and echocardiography. Patients were divided into 2 groups: group 1 - patients with complete adherence to updated Jones criteria (107 patients), and group 2 - exceptions (28 patients). Echocardiographic criteria were used to confirm the presence of carditis and to evaluate severity of mitral (MR) and aortic regurgitation (AR) at diagnosis and after treatment with steroids or acetylsalicylic acid (ASA)/non-steroidal anti-inflammatory drugs (NSAIDs)., Results: We observed a persistence of ARF in the last 15 years (mean 9 new cases/year with a peak of 19 cases in 2000). Carditis and arthritis were the main major criteria observed (102/135 and 71/135 patients respectively), then chorea (29/135), erythema marginatum (8/135) and subcutaneous nodules (1/135). Arthritis and chorea resolved completely with various therapies. At the last follow-up (> or =5 years) in group 1, loss of MR was observed in 46% steroid-treated (26/56 cases) and in 39% ASA/NSAID-treated (7/18 cases) patients and loss of AR in 59% steroid-treated (22/38) and 2/7 ASA/NSAID-treated patients (p>0.05)., Conclusion: Incidence of ARF is clinically important currently in the area of northern Italy. Non-suppurative complications of streptococcal pharyngitis should be considered when deciding therapy in a pediatric patient that presents with sore throat.

Objective: Alendronate treatment for 12 months in pediatric patients with rheumatic diseases and secondary low bone mass was reported to result in a substantial increase in bone mineral density (BMD). In this study, we evaluated the changes in bone metabolism and disease activity markers in 45 patients ages 5 to 18 years (31 female, 14 male) with rheumatic diseases treated with alendronate for 12 months., Methods: Variables analyzed included demographic and anthropometric data, biochemical markers of bone metabolism, disease activity indexes, and BMD values. For all variables, the differences between levels at baseline and at 12 months were calculated; correlations between the variables and between the BMD variation over 12 months and baseline levels of the different variables were also evaluated., Results: There was a statistically significant decrease of both bone resorption and bone formation markers over the 12 month treatment period. By contrast, no disease activity index changed significantly over one year. BMD Z score change over one year did not correlate with erythrocyte sedimentation rate, matrix metalloproteinase-3, interleukin 6, or C-reactive protein variations over the same period., Conclusion: These results support the conclusion that alendronate treatment is accompanied by a reduction of bone turnover in pediatric patients and that the observed BMD increase is not secondary to a reduction of inflammatory activity.

Objective: To compare the efficacy and safety of methotrexate (MTX) after oral and intramuscular administration in children with juvenile chronic arthritis (JCA)., Methods: Pediatric rheumatology centers in Italy participated in this short-term, prospective, open trial. Each investigator was allowed to choose the oral or intramuscular route of administration according to his personal preference in everyday clinical practice. Patients enrolled by each center were given MTX through the same method of administration. All patients received 10 mg/m2 of MTX each week for six months., Results: A total of 257 patients with JCA (127 treated orally and 130 intramuscularly) were enrolled in the trial by 11 Italian centers. The response rate after 6 months of MTX therapy was 58% in the oral and 61% in the intramuscular cohort. The frequency of adverse side effects did not differ significantly between the two treatment groups., Conclusion: The results of this study suggest that MTX at the conventional dose regimen is equally effective and has a similar safety profile in children with JCA when administered orally or by intramuscular injections.

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.

Lymphedema, a well known extraarticular manifestation of rheumatoid arthritis, has been rarely described in children with idiopathic chronic arthritis. We describe 12 cases of lymphedema and idiopathic arthritis of childhood seen at 4 different pediatric rheumatology centers. Eight patients were girls, 4 boys; the age at appearance of lymphedema ranged from 2.3 to 17 years. In all patients except one, lymphedema was localized to the lower limbs. The outcome of lymphedema was variable, but not always related to the arthritis course, and was mostly independent of any specific therapy. Lymphography was performed in only one patient, and revealed lack of lymphatic drainage in the affected leg. We conclude that the association of lymphedema and idiopathic arthritis of childhood is not rare; this association is unlikely to be coincidental, even though the pathogenetic mechanisms are currently not well understood.

Forty pediatric patients, ranging from 5-13 years of age and suffering from grass pollen rhinoconjunctivitis, were submitted to local nasal preseasonal (12 weeks) immunotherapy, either with a grass pollen extract or with placebo. After 1 year, 15 of these patients (5 previously treated with active product and 10 with placebo) were treated with the grass pollen extract preseasonally for 2 consecutive years. Before and after treatment, serum total IgA and IgE, and specific IgG and IgE were assayed as well as carrying out nasal provocation tests (NPT) with extracts at different concentrations, endpoint evaluations by rhinomanometry and prick tests with different concentrations of extract. After only 1 year, the actively treated patients showed a significant decrease of daily nasal and conjunctival signs and symptoms-as judged by a 1 to 3 score-in comparison with the control group. The placebo group showed the same results after the 3rd year. The improvement was confirmed by a significant increase of the dose threshold in NPT. No immunological alterations were evident.

Objective: To measure the levels of two adhesion molecules (AM), soluble intercellular adhesion molecule 1 (sICAM-1) and soluble E-selectin (sE-selectin), in serum and synovial fluid (SF) of patients with juvenile chronic arthritis (JCA)., Methods: Both soluble AM levels were tested, in serum and synovial fluid (SF) samples, with an enzyme-linked immunosorbent assay (ELISA) method., Results: Serum levels of sICAM-1 and sE-selectin in JCA patients were not significantly different from those of a control group. Synovial fluid levels of sICAM-1, but not of sE-selectin, assayed significantly higher (p < 0.05) in JCA patients than in controls. Moreover SF levels of both molecules correlated negatively with disease duration, being higher in the earliest phases. No significant correlations were found between JCA sICAM-1 and sE-selectin levels and leukocyte count or ESR., Conclusions: These observations may signify a more important role of ICAM-1 than E-selectin in the migration of inflammatory cells into JCA SF. The negative correlation of both AMSF levels in JCA patients with disease duration could reflect a higher expression of ICAM-1 and E-selectin during the earliest phases of the disease.

Of 5,500 newborn infants whose family histories were screened, 900 were found to have anamnestic risk. Cord-blood IgE was evaluable in 4,677 of these newborns, of which 394 had levels > or = 1 IU/mL; 84 infants had both anamnestic risk and elevated cord-blood IgE levels. Parents of infants with anamnestic risk were informed of their child's risk of atopy. Additionally, for 391 infants at two of the three participating hospitals, a preventive diet was prescribed that recommended breastfeeding for the first 6 months of life, with maternal diet restricted to no more than 200 dL of cow milk per day, no more than one egg per week, and no tomato, fish, shellfish, nuts, or foods allergenic to the mother. Only soy formula was recommended, and introduction of solid foods was also carefully prescribed. Furthermore, doctors recommended against exposure to tobacco smoke, animal allergens, and early entrance into daycare. Evaluable infants whose parents complied with the prescribed diet were found to have a lower incidence of atopy during the first year of life (13.3%, n = 158) than infants whose parents had ignored the prescribed diet (54.7%, n = 86) or infants whose parents were offered no dietary recommendations (28.9%, n = 218). Differences between the compliant group and the two groups with unrestricted diets were significant, indicating that this prescribed diet may protect against or delay onset of food allergies during the first year of life.

Calciotropic hormone assays and bone mineral density (BMD) evaluations were carried out on a group of 36 children affected with juvenile chronic arthritis (JCA) (13 with pauciarticular, 8 with polyarticular and 15 with systemic onset subtype) and in a matched control group. The plasma levels of calcium, phosphate and alkaline phosphatase were normal in all the groups. Basal 25-OH D plasma levels were significantly decreased both in the polyarticular and in the systemic groups; the latter showed a further decrease after 1 year. There was a significant decrease in both the basal and the 1-year plasma levels of 1-25-OH D only in the systemic group. The BMD was decreased in the systemic group alone and the percentage increase after 1 year was significantly inferior to that in the other groups and in the controls. There was a clear relationship between the decrease in hormones and the cumulative dose of steroids administered, as well as between the BMD decrease and the physical activity of the patients (according to Steinbrocker's classification).

An immunological imbalance is probably one of the major pathogenetic causes of rheumatoid arthritis in adults as well as in children. This aspect is the rationale for the use of immuno-modulating drugs. In our study we evaluated the effects of intravenously-administered thymopentin on systemic and local features in 10 children affected with systemic onset juvenile chronic arthritis (JCA). We also considered the effects of intra-articular thymopentin in 3 children affected with pauciarticular onset JCA.

Intradermal skin test antigen (Varidase, Mumps, Candidine) and contact sensitization with 2,4-dinitrochlorobenzene (DNCB) were employed in 35 previously untreated children with neuroblastoma and malignant lymphoma, to evaluate delayed cutaneous hypersensitivity. Cutaneous responses were correlated to stage of disease and survival. The limited number of patients prevents any definitive conclusion. It is apparent, however, that in childhood non-Hodgkin's lymphoma anergy to DNCB is correlated to advanced stages with rapid progressive disease. In neuroblastoma and Hodgkin's disease patients skin test sensitization seems to be of questionable clinical value.

We describe an unusual case of a child who had had incontinentia pigmenti from birth and developed the clinical picture of Behçet's syndrome at five years of age. Among the various investigations performed, chemotactic activity of the polymorphonuclear leukocyte was found to be low. We discuss the possibility that there are common immunological abnormalities in the two syndromes.

A nine-year-old girl had several ex novo episodes of acute glomerulonephritis with clinical evidence of rapid progression in two of them. Guillain-Barré syndrome was diagnosed 10 days after the second episode of acute glomerulonephritis. Two renal biopsies (performed at about a four-year interval) gave morphological evidence of new episodes of acute glomerulonephritis. To our knowledge this is the first report of an association between ex novo episodes of acute glomerulonephritis and Guillain-Barré syndrome.

67 children affected with acute lympocytic leukemia were immunologically evaluated for lymphocytic markers, serum immunoglobulins and delayed hypersensitivity skin tests at the onset, in remission and after cessation of therapy. E, EA rosettes and surface Ig assayed significantly lower in leukemic children than in matched controls, except for three cases of T-cell leukemia in which E rosettes were very high. After cessation of therapy almost normal results were obtained. As for serum Ig, the only abnormal finding was that of low IgM during therapy. The skin tests with Varidase, Candidine, Mumps antigen and DNCB were not significantly different at onset and in remission. As for DNCB test, the negative responses at onset often became positive in remission, but only when the test was performed before any treatment (anamnestic-like response?). One of the three patients with T-cell leukemia relapsed after 8 months: strangely enough, no surface marker could be detected on that occasion. We could not find any relationship between various immunological tests, or between these tests and prognosis; chemotherapy proved active in suppressing cellular immunity, especially the primary cellular response.