Antiphospholipid antibodies in children with idiopathic cerebral ischaemia.
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Adolescent↗
Biomedical subjects
A Ravelli
Publications and source records attributed to A Ravelli.
Intravenous iron therapy for severe anaemia in systemic-onset juvenile chronic arthritis.
The role of iron supplementation in treating the anaemia of systemic-onset juvenile chronic arthritis is not clear. Eight affected children with severe persistent anaemia unresponsive to oral iron therapy were treated with intravenous iron saccharate. From a median post-oral-iron value of 8.0 g/dL (range 6.5-9.5), haemoglobin rose to 11.0 g/dL (10.1-12.1) (p = 0.01). The concentration of serum transferrin receptor, an indicator of iron deficiency, before intravenous therapy correlated with the increase in haemoglobin (r = 0.88, p < 0.01). Intravenous iron saccharate could be an effective treatment for chronic anaemia in this condition, especially with iron deficiency not responsive to oral iron.
Adolescent↗
Anticardiolipin antibodies in pediatric systemic lupus erythematosus.
OBJECTIVE: To investigate the prevalence and the clinical significance of anticardiolipin antibodies (ACLs) in a group of children with systemic lupus erythematosus (SLE). DESIGN: Cross-sectional and longitudinal study. SETTING: Pediatric Clinic, University of Pavia, Italy. PARTICIPANTS: Thirty children (aged 4.9 to 16.5 years) with SLE. MEASUREMENTS AND MAIN RESULTS: Twenty-six (87%) of the 30 patients were initially positive for either IgG or IgM ACLs; 24 (80%) of 30 had IgG ACLs, and 15 (50%) of 30 had IgM ACLs. The cross-sectional analysis showed a trend for IgG ACLs to be positively associated with autoimmune cytopenia and negatively associated with renal disease. The levels of ACLs, particularly of the IgG isotype, tended to correlate with SLE activity as expressed by the complement fraction C3, the erythrocyte sedimentation rate, or the SLE Activity Measure, but not by the SLE Disease Activity Index or the anti-DNA antibodies. Serial determinations of ACL levels in 20 patients revealed frequent fluctuations. High levels of IgG ACLs (> 50 arbitrary units) were observed in nine patients; all nine had active disease and eight had one or more clinical features that have been previously associated with antiphospholipid antibodies: neuropsychiatric manifestations in six patients, autoimmune cytopenia in two patients, and avascular necrosis of bone in one patient. Only one patient experienced an overt episode of vascular thrombosis; IgG ACLs were positive at a medium level 6 months before the thrombotic event, but their level was unchanged when the thrombosis was discovered; the lupus anticoagulant test was positive at time of the thrombosis. CONCLUSIONS: Our results show that in pediatric SLE, ACLs are frequently found, high levels of IgG ACLs are often associated with central nervous system involvement, and ACLs have a low predictive value in the development of vascular thrombosis.
Adolescent↗
Antiphospholipid antibodies in paediatrics.
Antiphospholipid antibodies, i.e. circulating auto-antibodies to negatively charged phospholipids, are mainly observed in patients with systemic lupus erythematosus, but may also occur in individuals who lack evidence of a well-defined systemic disease. Several studies have suggested that they may play a direct role in the pathogenesis of recurrent vascular thrombosis, repeated abortions, and thrombocytopenia (so-called antiphospholipid antibody syndrome), although the mechanism by which this occurs is still poorly understood. The management of patients with antiphospholipid antibody-related thrombosis includes anti-aggregants, anticoagulants, corticosteroids, and cytotoxic drugs, but the optimum treatment is still controversial. Here we review the test systems used to detect antiphospholipid antibodies and their significance in paediatrics.
Adolescent↗
Hypocomplementemic urticarial vasculitis syndrome with severe systemic manifestations.
We describe two children with clinical and laboratory features of hypocomplementemic urticarial vasculitis syndrome. Both patients had severe, life-threatening manifestations: rapidly progressive glomerulonephritis (patient 1) and pulmonary hemorrhage (patient 2). We conclude that this syndrome may be a potentially severe multisystem disease.
Adolescent↗
Antiphospholipid antibody syndrome as a cause of venous thrombosis in childhood.
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Antiphospholipid Syndrome↗
Immune responses to the Escherichia coli dnaJ heat shock protein in juvenile rheumatoid arthritis and their correlation with disease activity.
Patients with juvenile rheumatoid arthritis frequently have abnormal immune responses to the hsp65 class of bacterial heat shock proteins. However, lymphocytes from children with other inflammatory diseases may also recognize hsp65, and the role of these antigens in juvenile rheumatoid arthritis remains controversial. We have studied humoral and cellular immune responses to a distinct, recently described bacterial heat shock protein, designated dnaJ. The Escherichia coli dnaJ gene was cloned and expressed, and the purified recombinant protein was used as an antigen. Neither normal children nor children with various chronic inflammatory diseases had lymphocyte proliferative responses to recombinant dnaJ. However, lymphocytes from patients with polyarticular, pauciarticular, and systemic manifestations of juvenile rheumatoid arthritis responded strongly to the antigen. Cellular immune responses to dnaJ were higher in synovial fluid than in blood and higher in children with active disease than in children in remission. These data show that increased immune reactivity to dnaJ is characteristic of juvenile rheumatoid arthritis and that the magnitude of the immune response is linked to disease activity. The results suggest that an abnormal immune response to antigens on commensal gut bacteria may contribute to the generation of chronic inflammation in juvenile rheumatoid arthritis.
Adolescent↗
Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis.
We retrospectively investigated, in 19 children with systemic-onset juvenile chronic arthritis (SoJCA), the possible influence on the outcome of methotrexate (MTX) therapy of several independent variables, including age at onset of juvenile chronic arthritis, disease duration and severity of the disease at baseline. The dosage of MTX ranged from 7.5 to 11.0 mg/m2/week (median 9.3 mg/m2/week) and was given as a single, oral weekly dose. After 6 months of treatment, 12 (63%) patients were judged as responders on grounds of a > or = 50% reduction in the number of joints with active arthritis and/or an articular severity score; 7 (37%) did not respond to therapy. When the baseline values of the selected variables were compared, we found that the responder group had, with respect to the non-responder group, a lower percentage of radiographic lesions (p < 0.005), a shorter duration of the disease (p < 0.05) and a lower number of joints with limitation of motion (p < 0.01), functional limitation score (p < 0.05) and articular severity score (p < 0.05). A threshold value of disease duration of two years and the presence/absence of radiographic lesions gave a correct classification with respect to the treatment outcome of 73.7% and 83.3%, respectively. The predictive value of these two variables was confirmed by a multivariate analysis. We conclude that earlier treatment with MTX, possibly before the appearance of radiographic changes, may favourably influence the outcome of MTX treatment in those patients with SoJCA who require a second-line drug.
Adolescent↗
High prevalence of antiphospholipid antibodies in children with idiopathic cerebral ischemia.
BACKGROUND: The presence of circulating antiphospholipid antibodies (aPLs) is frequently associated with thromboembolic phenomena. OBJECTIVE: To investigate the prevalence of aPLs, detected as lupus anticoagulant (LA) or anticardiolipin antibody (aCL), in a group of unselected children with idiopathic cerebral ischemia. DESIGN: Prospective, case series. SETTING: A pediatric neurology department. PATIENTS: Thirteen children with cerebral ischemia (eight with stroke, three with transient ischemic attacks, and two with ocular ischemia). Age-matched apparently healthy children served as controls. MEASUREMENTS: LA and aCL determination was performed within 3 days after the occurrence of the ischemic event and was repeated after 3 to 6 months. To be defined as aPL-positive, patients had to have either a positive LA test or positive IgG and/or IgM aCL at moderate/high level in both determinations. MAIN RESULTS: Ten (76%) of the 13 patients were positive for either LA or aCL. No differences were found between aPL-positive and aPL-negative patients with respect to clinical manifestations or radiological features. Six (46%) of the 13 patients had a history of multiple ischemic events. CONCLUSIONS: Our results show a very high prevalence of aPLs in children with idiopathic cerebral ischemia. Because the presence of these antibodies has relevant therapeutic implications, their determination in children with cerebral ischemia is recommended.
Adolescent↗
Chronic intoxication by ethane-1-hydroxy-1,1-diphosphonate (EHDP) in a child with myositis ossificans progressiva.
A child with myositis ossificans progressiva was treated for 8 years with ethane-1-hydroxy-1,1-diphosphonate (EHDP) 30-40 mg/kg per day. Latterly he complained of severe, progressive bone and joint pain which made standing and walking almost impossible. A radiographic skeletal survey showed diffuse ricket-like lesions. Withdrawal of EHDP therapy produced substantial improvement in his general condition as well as in the radiographic appearance of the bones. Multiple exostoses were observed in this case and, particularly those around the knees, presented a peculiar morphology. This supports the theory that exostoses originate from a defect of metaphyseal modelling.
Adolescent↗
Anti-DNA antibodies in the primary antiphospholipid syndrome.
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Adolescent↗
Plasma levels after oral methotrexate in children with juvenile rheumatoid arthritis.
Plasma levels of methotrexate (MTX) after oral administration of 6.4 to 11.2 mg/m2/week (mean 8.5 mg/m2/week) were studied in 33 children with severe juvenile rheumatoid arthritis (JRA). MTX concentrations were measured by a fluorescence polarization immunoassay (TDx) at 1, 2, 3, and 24 h after administration. The maximum level was observed in most patients after 1 h. No significant correlation was found between MTX dosage and the 1, 2, and 3-h plasma levels. No patient showed values in the range of probable toxicity 24 h after administration. A stepwise multiple regression analysis on 1, 2, and 3-h MTX levels, selected clinical features, dosage and duration of MTX therapy, and concomitant drug treatment showed that MTX concentrations at the different time points tend to be closely related; among the other variables, only concurrent treatment with salicylates was found to affect significantly the 3-h level. Serial determinations performed in 20 patients at the same oral dosage showed a wide interindividual and intraindividual variability of the plasma levels from the first dose to the next. Variable and unpredictable levels were observed also in most of the 8 patients studied after one or more increases of MTX dosage. No difference in MTX concentrations was observed between patients who responded to treatment and those who failed to respond, and between patients who had serum transaminase elevation and those who did not. Our results suggest that, until the pharmacokinetics of low dose MTX is clarified, routine therapeutic monitoring of MTX has a limited value in the clinical management of children with JRA.
Administration, Oral↗
[The Schönlein-Henoch syndrome with severe multisystemic involvement].
A case of Henoch-Schönlein purpura (HSP) characterized by several unusual complications and exceedingly prolonged course is reported. A 6-year-old boy, hospitalized with a typical clinical picture of HSP, developed after a few days a severe gastrointestinal vasculitis leading to digestive hemorrhages and food intolerance. The treatment included continuous gastrointestinal infusion, parenteral nutrition, and corticosteroids during the exacerbations of symptoms. Intestinal ultrasonography showed diffuse submucous edema and widespread mural hematomas; hydrops of the gallbladder and edematous swelling of the pancreas were also detected. A gastro-duodenoscopy revealed diffuse and severe mucosal vasculitis with ulcers and petechial lesions. One week after admission the occurrence of weight gain, hyponatremia, and hypoprotidemia in the absence of proteinuria suggested a protein-losing enteropathy. The elevation of serum and urine amylases was consistent with pancreatic involvement. The course was characterized by recurrent exacerbations of gastrointestinal manifestations; symptoms subsided slowly to such a degree that oral feeding could be gradually restarted only seven weeks after admission. The use of high-frequency intestinal ultrasonography proved useful and sensitive in monitoring the evolution of intestinal involvement. During hospitalization the child complained of acute painful scrotal swelling. Ultrasonography showed scrotal hemorrhage and testicular swelling but no signs of testicular torsion, thus helping to avoid surgical exploration. In the recovery phase the child complained of colicky abdominal pain with vomiting. A plain abdominal roentgenogram showed bilateral staghorn pelvic urolithiasis. This finding was confirmed by an intravenous pyelography which also revealed a bilateral pyeloureteritis with edema of the ureteral wall and partial stenosis of the lumen.(ABSTRACT TRUNCATED AT 250 WORDS)
Acute Disease↗
Recurrent juvenile dermatomyositis and cutaneous necrotizing arteritis with molecular mimicry between streptococcal type 5 M protein and human skeletal myosin.
An adult patient had a syndrome associating the features of juvenile dermatomyositis and cutaneous polyarteritis nodosa that followed a cyclic course from childhood; recurrences were always associated with a rise of serum antistreptococcal antibodies. Regions of homology between streptococcal type 5 M protein and skeletal myosin were found. These findings suggest that streptococcal infection, possibly through a molecular mimicry mechanism, played a role in the pathogenesis of the disease in our patient.
Acute Disease↗
Antiphospholipid antibody associated thrombosis in juvenile chronic arthritis.
A child with systemic onset juvenile chronic arthritis (JCA) who developed a bilateral femuropopliteal vein thrombosis after plaster immobilisation following a tibial fracture is described. When the thrombosis was diagnosed, antiphospholipid antibodies detected either as lupus anticoagulant and anticardiolipin antibodies were found. This suggests that short term prophylatic antithrombotic treatment should be considered in antiphospholipid antibody positive JCA patients who require immobilisation after fractures of demineralised bones.
Antibodies, Antiphospholipid↗
Superior vena cava thrombosis in a child with antiphospholipid syndrome.
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Adolescent↗
Pulmonary function in childhood connective tissue diseases.
The term connective tissue diseases (CTD) defines a group of illnesses characterized by the presence of immune abnormalities and by widespread inflammation involving various organs and tissues including the lung. These diseases are not frequent in the paediatric age group. Very few data on pulmonary function are available in paediatric CTD. We investigated possible early lung function abnormalities and any likely relationship with clinical activity of the disease in a group of 81 paediatric CTD patients, without clinical or radiological evidence of pulmonary involvement. Measurement of lung volumes and diffusion lung capacity were performed. A sample of 65 subjects, defined as normal on the basis of history and clinical examination, and matched by age and height with the group of patients, was chosen as control group. CTD patients did not show significant deviations from the control distribution with respect to functional residual capacity (FRC) and maximal expiratory flow at 75% of the forced vital capacity (MEF75) values. On the contrary, both vital capacity (VC) and diffusing capacity of the lungs for carbon monoxide (DLCO) were quite impaired in most CTD during the active phase of the disease. Our results show a functional lung impairment in most children with clinically active CTD, even in absence of abnormalities on chest X-ray pictures.
Adolescent↗
Correlation of serum interleukin-6 levels with joint involvement and thrombocytosis in systemic juvenile rheumatoid arthritis.
We measured interleukin-6 (IL-6) levels in 70 serum samples obtained from 25 patients with systemic-onset juvenile rheumatoid arthritis (JRA), using the hybridoma cell line B9. Patients with systemic-onset JRA had significantly elevated serum IL-6 levels during active disease (mean +/- SD 92.1 +/- 75.1 hybridoma growth factor units/ml; P less than 0.00001 versus healthy age-matched controls), but not during remission. Serum IL-6 levels correlated with the extent and severity of joint involvement (P less than 0.001) and with platelet counts (P less than 0.05). Our data suggest that IL-6 plays a significant role in the pathogenesis of systemic-onset JRA.
Adolescent↗