Pirkko Pelkonen

Recurrent pericarditis in children and adolescents: Report of 15 cases

OBJECTIVES The aim of this study was to analyze the clinical findings, course, and treatment of recurrent pericarditis (RP) in patients with onset in childhood and adolescence. BACKGROUND Recurrent pericarditis is a chronic condition that has presented problems in management. Knowledge about this disease is based on observations in adults, and no series of children has previously been published. METHODS Fifteen children (nine males, six females) in whom pericarditis had recurred at least twice were encountered in the period 1985 to 1998. Their age at onset was 6.5 to 16.8 years (mean 11.6 years), and the follow-up was 4.0 to 16.2 years (mean 8.0 years). RESULTS Recurrent pericarditis was preceded by open-heart surgery by 1 month to 5 years earlier in 7 of 15 patients. The six children with an atrial septal defect (ASD) had an operation at an older age (mean 9.9 years) than usual (mean 4.8 years). The risk of RP in children operated on for ASD at the age of six years or later was 5%. An initial attack of pericarditis was associated with pleuritis and/or pneumonia in 10 of 15 patients and with colitis in 2 of 15 patients During follow-up, the patients had 2 to 30 recurrences (mean 9.9). Later attacks tended to be milder. At the end of follow-up, 7 patients had been without attacks for >or=4 years, whereas after 4 to 16 years, the remaining patients still had active disease. No instance of constriction was found. Altogether, 11 of 15 patients were treated with corticosteroids. However, corticosteroids, whether alone or with methotrexate (n = 5), azathioprine (n = 1), cyclosporine (n = 1), or colchicine (n = 4) did not prevent recurrences. CONCLUSIONS The most frequent background for RP in children was the closure of ASD after the age of six years. Its course was unpredictable and often chronic, irrespective of the underlying cause or the therapy given. Colchicine did not prevent relapses.

Ferritinemia as an Indicator of Systemic Disease Activity in Children with Systemic Juvenile Rheumatoid Arthritis

ABSTRACT. 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 μg/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.

Intra-articular glucocorticoids in early juvenile chronic arthritis

The efficacy of intra‐articular glucocorticoid injections in the early phase of knee joint synovitis was studied in 79 children with juvenile chronic arthritis (42 girls and 37 boys). Half of the injections were given within the first six months from the onset of the disease. The probability of a patient staying in remission was much higher in triamcinolone‐treated patients than in patients receiving methylprcdni‐solone (p<0.0005, Breslow statistics). Using multivariate analysis there was a significant association between the length of remission and the synovial fluid polymorphonuclcar leucocyte proportion (SF‐PMN%. Patients with a high SF‐PMN% tended to have shorter remissions than those with a low SF‐PMN% (improvement of the fit in stcpwise model: chi‐square = 8.81, p<0.005). The difference between triamcinolone and methylprednisolone groups was still clearly evident two years after injection.

Incidence of juvenile idiopathic arthritis in children in Estonia: a prospective population‐based study

Objective: To study the incidence rate of juvenile idiopathic arthritis (JIA) and its clinical subtypes in Estonia, to follow the course of the disease in newly diagnosed patients for 2 years, and to find the frequency of human leucocyte antigens (HLA) B27, DR1 and DR4 in JIA patients. Method: A population‐based study involving prospective registration of new cases of JIA in 1998–2000 and their clinical follow‐up for 2 years. Results: In 1998–2000, 162 new cases of JIA were diagnosed. The mean annual incidence rate of JIA was 21.7 per 100 000 children aged 0–15 years (22.9 in girls and 19.3 in boys). During the investigation period, the incidence rate rose 3.5‐fold. Oligoarthritis was the most frequent subtype (mean annual incidence rate of 11.7 per 100 000), followed by seronegative polyarthritis (4.4 per 100 000). HLA‐DR1, B27 and DR4 were found respectively in 44.4, 28.6 and 11.1% of cases in which the analysis was performed. In HLA‐B27‐positive patients, inflammation markers of blood remained at a high level for a longer period compared with HLA‐B27‐negative patients. Conclusions: This is the first population‐based study on the epidemiology of juvenile arthritis in Estonia in which the new classification criteria defined by the International League of Associations for Rheumatology (ILAR) have been used. In addition to environmental factors, an increase in awareness among family doctors is a probable reason for the rise in incidence during the study period. HLA‐B27 might have predictive value as a marker of chronicity of inflammation.

Persistent and Transient Iga Deficiency in Juvenile Rheumatoid Arthritis

Twenty-five children with serum IgA levels of less than 0.1 g/l (below the 2.5% confidence limit at 2 years of age) were found among approximately 350 cases of juvenile rheumatoid arthritis (JRA). During follow-up, 10 of them proved to have persistent IgA deficiency, 13 were classified as having transient IgA deficiency, and 2 had consistently low serum IgA. Transient IgA deficiency occurred during treatment, in 9 cases with gold and in 2 with antimalarials. The gold-induced IgA deficiency usually developed abruptly soon after institution of gold therapy, and its duration varied from a few months (4 cases) to several years (3 cases). In 6 cases a low IgA level has returned to normal despite continuing gold therapy. In half the patients with persistent IgA deficiency the course has been mild and oligoarticular, and after a mean duration of 8.8 years only one has active disease. In contrast, in the patients with transient IgA deficiency the disease was characterized by early onset (mean age 3.0 years), a polyarticular course (10/13) and prolonged activity (7/13, mean duration 9.6 years). Coeliac disease was diagnosed in 2 patients, both with persistent IgA deficiency.

CLINICAL FINDINGS AND INTESTINAL IMMUNOGLOBULINS IN CHILDREN WITH PARTIAL IgA DEFICIENCY

Abstract. We studied the intestinal morphology, and the jejunal and rectal immunoglobulins of 16 children with partial IgA deficiency, defined as serum IgA concentration more than two standard deviations below the mean for age, but higher than the lower limit of sensitivity of single radial immunodiffusion (0.02 g/l). Five of the patients had been treated with phenytoin, 2 had juvenile rheumatoid arthritis, 2 had ulcerative colitis and 5 had recurrent upper respiratory tract infections. The jejunal morphology was normal in every case. In 6 cases normalization of serum IgA occurred during the follow‐up, while in one patient with ulcerative colitis the concentration fell below 0.02 g/l. In patients with recurrent infections, there was a decreased frequency of infections when the level of serum igA increased. In 4 patients, IgM‐containing cells predominated in both the jejunal and rectal mucosa, and IgM was increased in the intestinal juice. In 6 patients a significant increase in IgM‐containing cells or a decrease in IgA‐containing cells or both were seen in either the rectal or jejunal mucosa. There was no correlation between the number of IgA‐containing cells in the intestinal mucosa and the serum level of IgA.

Outbreak of Kawasaki Syndrome in Finland

ABSTRACT. During a ten‐month period from June 1981 to March 1982 83 patients with Kawasaki syndrome were diagnosed in Finland. The attack rate was 26/100000 children under five years of age, corresponding to an annual attack rate of 31/100000 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.

INTESTINAL MALABSORPTION: A CLINICAL STUDY OF 22 CHILDREN OVER 2 YEARS OF AGE

A classic clinical picture and steatorrhoea with gluten intolerance are the criteria usually applied in the diagnosis of coeliac disease. Hence, it is difficult to detect the existence of atypical symptomatology in the disease. Nevertheless, more or less atypical forms have been described by many authors, expecially in older children (2, 3, 8). When the finding of villous atrophy of the intestinal mucosa is taken as the diagnostic criterion of coeliac disease, it is possible to evaluate the clinical manifestations in coeliac disease and even detect other conditions associated with intestinal mucosal villous atrophy. This report presents the clinical symptomatology and findings in respect of 22 children over 2 years of age, in whom significant villous atrophy was found. The authors believe that these patients are all suffering from coeliac disease, although this name has not been applied because the diagnosis has not been properly verified in each case. We have previously presented a series of infants with similar findings (11).

Fatal unresponsive villous atrophy of the jejunum, connective tissue disease and diabetes in a girl with intestinal epithelial cell antibody.

ABSTRACT. The patient presented with a diabetes at the age of 3 years. At the age of 5 years she got persistent diarrhoea, lost weight and showed symptoms or arthritis and pericarditis. She was found to have total villous atrophy of the jejunum, which did not respond to dietary treatment, total parental nutrition, prednisone and cyclophosphamide medication. She had high titres of antinuclear antibodies and elevated serum IgG, but antibodies to DNA and to ribonuclearprotein were negative. A low titre of antibodies to human intestinal epithelial cells was found. The patient died of overwhelming fungal sepsis. We propose that the intestinal damage is part of the autoimmune disease. Careful study of jejunal biopsy specimens is helpful in distinguishing this type of patient from patients with coeliac disease.

Serum‐Sickness‐Like Disease is a Common Cause of Acute Arthritis in Children

ABSTRACT. 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 4.7/100000 children under age 16.