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Biomedical subjects

P Pelkonen

Publications and source records attributed to P Pelkonen.

At least 37 records · Page 2Linked to original sources

Antibodies against neutrophil cytoplasmic components in Kawasaki disease.

The occurrence of antibodies against neutrophil cytoplasmic components in 39 children (23 boys, 16 girls, median age 2.0 years) with Kawasaki syndrome was studied. The conventional indirect immunofluorescence test (ANC-Ab) on alcohol-fixed neutrophils and two commercially available ELISA tests (ANCA-EIA and MPO-EIA) were employed to detect the antibodies. Fourteen (36%) of the 39 patients with Kawasaki disease had antibodies against neutrophil cytoplasmic components in at least one of the three tests used. Eleven patients were identified using the indirect immunofluorescence test. Five patients were positive in the MPO-EIA test and two additional patients in the ANCA-EIA test. The IF staining pattern was cytoplasmic in eight patients and perinuclear in three. The cytoplasmic staining pattern in patients with acute Kawasaki disease is different from that seen in patients with Wegener's granulomatosis. The occurrence of antibodies may assist in the diagnosis of some patients with Kawasaki disease, although neither the positivity itself nor the five different antibody profiles seem to have any other clinical relevance.

Antibodies, Antineutrophil Cytoplasmic↗

Serum trace elements in juvenile chronic arthritis.

We evaluated the serum concentrations of zinc, copper and selenium in 125 patients with juvenile chronic arthritis (JCA). Trace element levels showed distinct abnormalities as compared with those of a large group of healthy children. Serum zinc and selenium concentrations were lower and those of copper higher in children with arthritis than in healthy children and, further, patients with polyarthritis had significantly higher copper and lower zinc levels than those with oligoarthritis. Serum zinc levels showed a direct correlation with hemoglobin and an inverse correlation with values for the erythrocyte sedimentation rate (ESR), whereas copper correlated directly with ESR. Selenium values did not correlate with the activity of the disease, but were low in the patients with arthritis of long duration.

Adolescent↗

Elevated circulating tumor necrosis factor-alpha in patients with Kawasaki disease.

The mechanism of vascular injury in Kawasaki disease (KD) is unclear. Recent studies suggest a role for circulating antibodies that are cytotoxic for endothelial cell antigens inducible by cytokines. Tumor necrosis factor-alpha (TNF, cachectin) is a monocyte- or macrophage-derived cytokine that has an important role as an effector molecule in various inflammatory processes. To study the possible involvement of TNF in KD, we measured the levels of circulating TNF in 39 patients with KD at various stages of the disease by using a newly developed sensitive radioimmunoassay. The TNF levels in sera from the acute and subacute phases of the disease were significantly (p less than 0.001) higher than in sera taken in the convalescence phase or in sera from children without inflammatory disease. In all patients from whom serial samples were available, the TNF level was higher during the acute and subacute phases than during convalescence. Coronary aneurysms developed in four of the patients, and these patients were among those who had the highest levels of circulating TNF during the acute and subacute phase (51.1 +/- 13.6 pg/ml (mean +/- SD) vs 30.4 +/- 15.8 pg/ml in patients without coronary aneurysms; p less than 0.001). No differences in circulating TNF levels were observed between patients who received as treatment aspirin plus intravenous immunoglobulin and those who received aspirin alone. The results show that the levels of circulating TNF are increased in acute KD and support the hypothesis that this cytokine may be involved in the pathogenesis of the vascular injury in KD.

Adolescent↗

Chronic osteomyelitislike disease with negative bacterial cultures.

During a seven-year period we observed 14 children who had chronic osteomyelitislike disease. The bacterial cultures from the bone lesions were negative. In eight patients the findings were compatible with chronic recurrent multifocal osteomyelitis (CRMO), in four the findings were compatible with chronic sclerosing osteomyelitis of Garré, and two had osteomyelitis of the clavicle. In patients with CRMO, lymphocyte subpopulations, the responses to mitogens, and the chemotactic and chemokinetic responses showed no consistent abnormalities. After a mean follow-up of 4.5 years (range, one to ten years), all four patients with osteomyelitis of Garré were symptomatic, and two had complications. Only two of the eight patients with CRMO had active disease. The course had been complicated by growth disturbances in one patient and by thoracic outlet syndrome in another. Wegener's granulomatosis later developed in a patient with CRMO.

Adolescent↗

Synovial fluid lymphocytes in different subtypes of juvenile rheumatoid arthritis.

We studied the subsets of synovial fluid (SF) lymphocytes and their activation states in 4 subtypes of juvenile rheumatoid arthritis. The expression of lymphocyte differentiation antigens and activation markers (Ia and Tac) appeared to be similar in these subgroups. Tac + DNA-synthesizing T blasts represented, at most, 5% of all SF mononuclear cells. This finding was in clear contrast to the high proportion of Ia-positive SF mononuclear cells. There were no differences in Ia and Tac expression or DNA synthesis among the different juvenile rheumatoid arthritis subgroups. This finding suggests that the cell-mediated immune response may represent secondary features of the disease that are involved as a final common pathogenetic pathway.

Arthritis, Juvenile↗

Kawasaki disease: monitoring of circulating immune complexes.

We followed the levels of circulating immune complexes (CIC) in 27 patients with Kawasaki disease (KD) from the acute stage of the disease through convalescence, using the test for platelet-reactive IgG-IC, and C1q-binding and conglutinin-binding enzyme immunoassays. CIC were detected by one or more techniques in all but one patient. Positive results were obtained most often with the test for platelet-reactive IgG-IC. Measurement of complement components C3 and C4 in 14 patients revealed an increase in C3 levels during the first few weeks of the disease and normal levels of C4. The blood platelet count correlated directly with the level of platelet-reactive IgG-IC. The highest levels of CIC were found during weeks 3 through 7 after the onset of disease. Measurement of CIC is, however, not applicable to the clinical follow-up of patients with KD.

Antigen-Antibody Complex↗

HLA antigens in IgA deficient paediatric patients.

HLA antigens (A, B, C and DR loci) were studied in 62 IgA-deficient (IgAd) paediatric patients: 17 with coeliac disease (CD), 13 with juvenile arthritis (JA), 27 with frequent respiratory tract infections (RTI) and five with other diseases. The frequencies of HLA antigens in IgAd patients were compared with those in healthy blood donors, and in CD and JA patients with normal serum IgA levels. The IgA deficiency in the patients showed significant associations with HLA A1, B8, B13, Cw6, DR3 and DR7 (P less than 0.0005, P corr less than 0.02 vs controls) and decreased frequencies of DR2 (P less than 0.0005, P corr less than 0.02 vs controls). The HLA associations typical of coeliac disease, increased frequencies of HLA-B8 and DR3, were evident among the IgAd coeliacs; in contrast to the coeliacs with normal IgA levels, the IgAd coeliacs showed a significant increase of the HLA-Cw6 allele (P less than 0.0005, P corr less than 0.02 vs control coeliacs). Increased frequencies of HLA-A1, B8, B13, Cw6, DR3 and DR7 were noted in the patients with RTI, which can be explained by the frequent occurrence of the haplotypes A1, B8, DR3 and B13, DR7, the latter haplotype often also having the Cw6 allele. Among the IgAd JA patients, the antigen frequencies were similar to those in the JA patients with normal serum immunoglobulins.

Adolescent↗

Venous thrombosis associated with lupus anticoagulant and anticardiolipin antibodies.

We describe deep vein thrombosis associated with lupus anticoagulant and anticardiolipin antibodies in three children aged 10 to 14 years. One of them also had arterial thromboses. None of the patients had systemic lupus erythematosus when the thrombosis first occurred, but one fulfilled the criteria for systemic lupus erythematosus 3 years later. At presentation all had symptoms suggestive of pulmonary embolism and evidence of an autoimmune disease: Addison's disease in one, anti-DNA or antinuclear antibodies in all three, and a positive Coombs' test in two. Two of the three gave a false-positive test for syphilis. In the patient with systemic lupus erythematosus recurrent thrombocytopenia and severe haemolytic anaemia necessitated splenectomy. A child should be tested for lupus anticoagulant or anticardiolipin antibody if venous or arterial occlusion occurs without a known predisposing cause, or if there is pulmonary embolism or symptoms or laboratory findings suggestive of a connective tissue disease.

Adolescent↗

Life changes related to the onset of juvenile rheumatoid arthritis.

Life changes preceding the onset of juvenile rheumatoid arthritis (JRA) were studied in 49 children with prolonged arthritis, and in 58 children with temporary arthritis. In addition, the frequency of changes experienced by patients during their life was compared with the expectancy scores for healthy children. The frequency of changes during the year prior to the onset of the disease did not differentiate children with JRA from children with temporary arthritis. During their lives, the children of both groups had experienced a higher frequency of life changes requiring considerable readjustment than healthy children of the same age.

Adaptation, Psychological↗

Clinical signs and laboratory tests in the differential diagnosis of arthritis in children.

To develop a scheme for primary diagnosis, we analyzed the clinical findings and laboratory test results in 278 children with arthritis by using univariate analysis and multivariate logistic regression analysis. An elevated C-reactive protein (CRP) value, a temperature above 38.5 degrees C, and a high white blood cell count were independent predictors for the diagnosis of septic joint infection in patients with acute monoarthritis. The presence of either of the first two signs had a sensitivity of 100% and a specificity of 87% for septic arthritis. Sixty-seven percent of all patients with arthritis were cured within two weeks from the onset of joint symptoms. In patients whose disease duration exceeded two weeks, a low CRP value, the absence of fever, and an elevated IgG value were independent predictors for the diagnosis of juvenile arthritis. Antinuclear antibodies had a specificity of 100% and a sensitivity of 25% for juvenile arthritis or other connective tissue diseases. We recommend that laboratory tests indicated for all children with joint symptoms include determinations of the erythrocyte sedimentation rate and the CRP value, both total and differential leukocyte counts, urinalysis, and a bacterial culture of a throat smear. When arthritis is prolonged or when enteroarthritis is suspected, tests for antinuclear antibodies and serum immunoglobulins, serologic tests for Yersinia and Salmonella, and stool bacterial cultures should be included.

Adolescent↗

Incidence of arthritis in urban Finnish children. A prospective study.

The incidence of various types of arthritis in children was estimated by a prospective 1-year study in the greater Helsinki area (population under 16 years of age: 148,362). Patients were sought from primary care physicians, and 71% of the patients studied were seen within 1 week of the onset of symptoms. All patients received followup care for at least 3 months; patients whose symptoms were prolonged received followup care for a minimum of 2 years. The incidence per 100,000 children under 16 years of age was as follows: 108.5 for all cases of arthritis, 6.7 for septic arthritis, 5.4 for enteropathic arthritis, 51.9 for transient synovitis of the hip, 18.9 for prolonged arthritis (duration greater than 3 months), and 25.8 for acute transient arthritis. The incidence of juvenile rheumatoid arthritis was 19.6. Oligoarticular disease was prevalent (76%) among the juvenile rheumatoid arthritis patients.

Adolescent↗

Ferritinemia as an indicator of systemic disease activity in children with systemic juvenile rheumatoid arthritis.

Twenty children with systemic juvenile rheumatoid arthritis, aged 0.9-13.7 years, were studied with regard to their serum ferritin concentration at diagnosis and during follow-up, ranging from 2 to 9 years. At diagnosis, during fever, the concentration was extremely high. The median value was 935 micrograms/l. The values were unrelated to other manifestations of the disease or laboratory findings. During glucocorticoid treatment, the serum ferritin concentrations normalized rapidly, usually within a few weeks. The rate of normalization reflected the response of the fever to treatment. Later, subnormal concentrations were found, which were unrelated to the activity of the arthritis. Thus, serum ferritin is a useful guide when tapering glucocorticoid dosage.

Adolescent↗

Outbreak of Kawasaki syndrome in Finland.

During a ten-month period from June 1981 to March 1982 83 patients with Kawasaki syndrome were diagnosed in Finland. The attack rate was 26/100 000 children under five years of age, corresponding to an annual attack rate of 31/100 000 children under five years. The course of the outbreak suggested geographic spreading. 20% of the patients had clinical and ECG evidence of carditis, and ECG abnormalities were found in 59% of the patients. One patient died from a ruptured coronary aneurysm. Neurologic manifestations were seen in 10% of the patients. This is the first reported outbreak of Kawasaki syndrome outside Japan, Korea and the United States of America.

Adolescent↗

Serum-sickness-like disease is a common cause of acute arthritis in children.

Among 283 children in a prospective study of arthritis we found 15 patients with a self-limited serum-sickness-like disease consisting of urticaria or joint erythema and mostly polyarticular arthritis. The mean duration of joint symptoms was 5.9 days. A preceding infection was reported in 12 patients and 12 had received drugs, the therapy starting on average 12.8 days before the onset of joint symptoms. In 9 cases the drug was penicillin. Four patients had recurrent attacks. Circulating immune complexes were detected in the serum of 12 patients, but specific IgE antibodies to penicillin only in 3 patients. The estimated annual incidence of the condition was 4.7/100,000 children under age 16.

Adolescent↗