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Biomedical subjects

D B Sullivan

Publications and source records attributed to D B Sullivan.

At least 37 records · Page 2Linked to original sources
PubMed1983

Radiographic findings of scleroderma in childhood.

The radiographs of 12 children with the American Rheumatism Association criteria for scleroderma were analyzed and compared to the adult presentation. Childhood and adult manifestations of the disease differ. Although some similarities exist, tuft resorption and soft-tissue calcifications in the fingers are more common in childhood. In contrast, small bowel involvement, hand contractures, and erosive arthropathy are less frequent. In particular, inflammatory joint destruction was not identified in this small pediatric group.

Adolescent↗
PubMed1983

Immunogenetic studies of juvenile dermatomyositis. HLA antigens in patients and their families.

Typing for HLA-A and -B antigens was performed on 87 children with definite juvenile dermatomyositis (JDMS). A significantly increased frequency of HLA-B8 (estimated relative risk = 2.8, Pc less than 0.01) was observed among White patients, but not among Blacks or Latin Americans with JDMS. No abnormality of HLA haplotype segregation was observed among 38 healthy siblings of the JDMS probands.

Adolescent↗
PubMed1980

Renal papillary necrosis in juvenile rheumatoid arthritis.

Three patients who developed renal papillary necrosis while receiving long-term, high-dose aspirin therapy for juvenile rheumatoid arthritis are presented. It appears that aspirin alone or aspirin in combination with other drugs is the causative agent. The incidence and biologic significance of renal papillary necrosis are insufficient to alter the use of aspirin as the drug of choice in management of JRA. It is recommended that all children with JRA be encouraged to drink ample fluids and be followed with periodic urinalysis and blood pressure measurements. Those children who develop hematuria or hypertension should be evaluated by excretory urography.

Adolescent↗
PubMed1979

The association of autoimmune diseases and anti-IgA antibodies in patients with selective IgA deficiency.

The prevalence of antibodies to immunoglobulin A (IgA) was studied in eighty-three subjects with selective IgA deficiency (SIgAD), thirty-three normal individuals, thirty-two children with juvenile rheumatoid arthritis (JRA) and normal or elevated IgA, seventeen children with systemic lupus erythematosus (SLE) and normal or elevated IgA, and thirteen patients with hypogammaglobulinaemia. Anti-IgA antibodies were detected by haemagglutination of human erythrocytes coated with one of two IgA myelomas by the chromic chloride technique. Antibodies to IgA were not found in significant titre in individuals with normal or elevated IgA or in those with hypogammaglobulinaemia. In IgA deficient patients, elevated titres were found in 25--30% of those who were healthy or who had non-rheumatic diseases, 50% of those with rheumatoid arthritis, 77% of those with JRA and 100% of those with SLE. Patients with rheumatic diseases and SIgAD may therefore be particularly at risk of developing anti-IgA-mediated reactions to blood products.

Agammaglobulinemia↗
PubMed1978

Diagnostic significance of antibody to native deoxyribonucleic acid in children with juvenile rheumatoid arthritis and other connective tissue diseases.

Sera of children with juvenile rheumatoid arthritis and other connective tissue diseases were tested for antibodies to native DNA by a radiolabeled-binding assay. Normal values were obtained in 130 children with JRA, including 28 with uveitis and 14 with selective IgA deficiency. Normal values were also found in sera from children with dermatomyositis, scleroderma, polyarteritis, ankylosing spondylitis, and a variety of other nonconnective tissue diseases. The only sera with elevated DNA-binding assays were from children with systemic lupus erythematosus. On the basis of these data, increased levels of antibodies to native DNA distinguished patients with active SLE from children with JRA.

Adolescent↗
PubMed1977

A syndrome of childhood polyarteritis.

In this report the clinical, laboratory, and histopathologic findings of nine children with polyarteritis are reviewed. All have had evidence of systemic involvement. Eight presented with fever, calf pain, erythematous painful nodules, and elevation of the acute-phase reactants. All were treated with prednisone at a dosage of 2 mg/kg/day. All of the children are alive but have had relapses at least once during the course of tapering the dosage of corticosteroids. Serious complications of disease have included myocardial infarction, hypertension, and impaired renal function.

Adolescent↗
PubMed1977

Dermatomyositis in the pediatric patient.

Forty-one children with dermatomyositis who were treated with corticosteroids and who have been followed over a period of 15 years have been presented. Progressive proximal muscle weakness was seen in all and 60% had muscle pain. The skin rash considered classic for dermatomyositis was seen in 33 children at the time of diagnosis. Elevation of serum muscle enzymes, electromyographic abnormalities, and muscle biopsy evidence of acute myositis were of confirmatory diagnostic value. The course of the disease in this study group has reconfirmed the efficacy of adrenal corticosteroid treatment in conjunction with an individualized physical therapy program and consistent followup. Prognosis for life and minimal functional disability has been good. There have been 3 deaths recorded in this series, only one of which was certain in its relationship to dermatomyositis.

Adolescent↗
PubMed1977

Scleroderma in children.

Fifteen children with scleroderma have been presented. All had characteristic cutaneous abnormalities at onset and during the course of disease. All were girls. All had visceral involvement, primarily of the gastrointestinal tract, heart, and lungs. The presence of visceral disease might have been missed without specific and extensive diagnostic procedures, including gastrointestinal barium studies, esophageal motility, pulmonary function and carbon monoxide diffusing capacity, and plethysmography. Raynaud's phenomenon was frequent and accompanied by evidence of occlusive vascular disease. Contractures around joints were readily evident and arthralgias were common, but evidence of objective arthritis was absent. Sixty percent of the patients in this series had ANA. Overlap syndromes with myositis and SLE were present in 7 patients. Three of 15 children died 6-10 years after onset of disease.

Adolescent↗
PubMed1977

Clinical patterns of chronic iridocyclitis in children with juvenile rheumatoid arthritis.

In the present study we have reconfirmed an earlier observation of the association of iridocyclitis with limited joint involvement in children with JRA. Children who develop iridocyclitis are predominantly girls whose sera are positive for antinuclear antibodies. Iridocyclitis was found to be asymptomatic at onset. Only periodic slit lamp examinations will detect the majority of cases. Iridocyclitis began a median of 2 years after onset of arthritis. Although most episodes occurred within 7 years after onset of arthritis, the risk of this development may never be absent in these children, even after they enter adulthood. In the present study, 16% of the afflicted children experienced either unilateral or bilateral blindness.

Adolescent↗
PubMed1977

Serologic studies in juvenile rheumatoid arthritis: a review.

Careful study of inflammatory and immunologic parameters in JRA and other connective tissue diseases of childhood has contributed to our current understanding of these diseases. Examination of serum for acute phase reactants, and antibodies to immunoglobulin antigens and nuclear antigens, combined with radiologic evaluation of symptomatic joints and, when indicated, examination of joint fluid or synovial membrane, may confirm the diagnosis of JRA. Changes in inflammatory indicators can be used as an index of adequacy of treatment. An ANA positivity in the child with pauciarticular arthritis should always prompt frequent slit-lamp examinations for asymptomatic iridocyclitis. The role played by auto-antibodies in the pathogenesis of JRA is unknown, as is the possible effect of proteins like CRP on the regulation of the inflammatory process in these children.

Alpha-Globulins↗
PubMed1973

Abnormalities in the distribution of serum immunoglobulin concentrations in juvenile rheumatoid arthritis.

Concentrations of serum IgG. IgA, and IgM were determined in 200 patients with juvenile rheumatoid arthritis. The relative frequency distribution of IgG and IgM approached that of a log-normal curve; however, there was marked skewing of the distribution of the serum concentrations of IgA. The prevalence of selective IgA deficiency was 4%. In order to permit further intragroup comparisons, the serum immunoglobulin concentrations were standardized by comparison to a sex-age matched control group. By this process it was found that there was concordance of the serum levels of IgG with IgA, and IgG with IgM. The standardized concentrations of IgA and IgM were less in females than males. The aberration in distribution of serum IgA concentrations found in this study, and the relative inability of females to respond to their disease by increasing specific serum immunoglobulin levels, add further data supporting the concept of immunodeficiency in the pathogenesis of juvenile rheumatoid arthritis.

Adolescent↗