Course of treated juvenile dermatomyositis.

Sixty-six patients with possible juvenile dermatomyositis (JDMS) were observed at the Children's Hospital of Los Angeles from 1960 to 1982. In patients initially given high doses of corticosteroids followed by low-dose therapy, three different clinical courses had previously been observed: monocyclic, polycyclic, and chronic continuous. We reviewed the records of 32 patients who met study criteria. The course of JDMS was monocyclic in eight children, chronic polycyclic in 10, and chronic continuous in 14. Of these children, 25 are well and not receiving medication; one has mild JDMS, without corticosteroid therapy; four have active JDMS despite corticosteroid therapy (one is severely handicapped); and two have died. Our results support the improved prognosis of JDMS after corticosteroid therapy, but also the great clinical variability of the disease. Understanding of this variability, as reflected in the three disease courses, facilitates physician choice of the optimal treatment with the least drug toxicity for the individual patient, continuing efforts to clarify the disease pathogenesis, and research efforts to improve current treatment programs for the patient with severe JDMS.

Affective learning in medical education.

The purpose of this study was to test a theory of affective learning and to develop a data-gathering instrument for evaluation of affective learning. Two primary areas of affective learning were conceptualized for study: coping with feelings about professional knowledge and coping with feelings about the necessity for development of professional attitudes toward illness and disability and toward death and dying. This two-year study project (1977-1979) involved a total of 70 first-year medical students. Data were obtained by means of observing students in small group discussions. The observation instrument consisted of a checklist of behaviors. Observer reliability was tested. Study results suggested that students progress through phases of affective learning as described in the theory. Implications of the findings are discussed.

The role of antibodies directed against double-stranded DNA in the manifestations of systemic lupus erythematosus in childhood.

The specificity of antibodies directed against dsDNA for SLE in a childhood population was tested by analyzing sera from 62 children with lupus and 283 children with other known or suspected autoimmune diseases. The role of these antibodies in the manifestations of SLE was then examined by correlating dsDNA Ab titer with clinical manifestations in 311 sera from 20 children followed for a mean of 51 months. Antibodies to dsDNA were found to be highly specific for SLE. The presence of antibodies in titers of 1:80 or greater correlated with the presence of active disease, arthritis, and rash, but not with azotemia, proteinuria, or increasing proteinuria; this indicated that their role in the induction of lupus nephritis was different from that in the induction of rash and arthritis. This may be due to a requirement for small immune complex formation during times of antigen excess in the initiation of lupus nephritis.

Serum complement abnormalities in the antinuclear antibody-positive relatives of children with systemic lupus erythematosus.

Serum C3, C4 and total hemolytic complement (CH50) levels were measured for 21 children with systemic lupus erythematosus (SLE) and 81 first degree relatives. The mean serum C4 and CH50 levels of the 12 relatives with antinuclear antibodies (ANA( were depressed to levels equal to those of the index cases. A similar depression was not found for C3, nor was there a depression of C3, C4, or CH50 in the relatives without ANA. If preexistent depression of C4 levels can be documented in the ANA-positive relatives of index cases, it may provide an explanation for the inherited predisposition to SLE in some families.

Total hip replacement in children with arthritis.

Fourteen children with juvenile rheumatoid arthritis and two with ankylosing spondylitis received 29 total hip replacements (THR). The ages at THR were 12 to 18 years, the median duration of prior hip disease was 7.1 years, and the minimum followup was one year (range 1--4 years, median 2.2 years). The primary indication for THR was pain in 5 hips and severe malposition or flexion contractures in 24 hips. (Sixteen of these also had some degree of pain.) All 29 hips demonstrated improved postoperative range of motion, and all were free of pain. In children, active rheumatic disease in other joints, prosthesis longevity, and possible lack of adequate motivation all merit special consideration prior to THR, but the results are encouraging.

Mixed connective tissue disease in childhood. A clinical and serologic survey.

Mixed connective tissue disease is a syndrome with overlapping clinical features of SLE, scleroderma, and polymyositis. Only one other child with MCTD has been described in detail. In this study 14 children with MCTD are described. Each had overlapping clinical findings that evolved over an extended period of observation, and all 14 had high serum titers of speckled ANA and antibodies to RNP. A serologic survey of 127 children with various rheumatic diseases confirmed the specificity of high titer of speckled ANA and antibodies to RNP for MCTD in children. Significant cardiac and renal involvement, and thrombocytopenia, may be more common in affected children than in adults with MCTD, may lead to longer therapy with higher doses of a corticosteroid, and may contribute to a more serious prognosis than in adults.

The clinical spectrum of systemic lupus erythematosus in childhood.

The onset and course of 108 children with systemic lupus erythematosus have been analysed. There were more black patients than one would expect from hospital population statistics. There was a greater preponderance of boys with onset of the disease at less than 12 years of age and there is a large number of familial cases. Major signs and symptoms differed from those observed in adults only in the greater degree of reticuloendothelial involvement and in a possibly greater propensity for children to change renal biopsy category. Diffuse proliferative renal lesions remain a major contributor to death both in children and in adults, but the importance of the extrarenal mortality factors plus the greater proportion of male deaths is emphasized.

Mixed connective tissue disease in children.

Seven girls and 3 boys with MCTD have been described. As a group their clinical characteristics and serological findings are similar to those reported in adults, with several important differences. Children with MCTD may have marked thrombocytopenia and more frequently they have RF. Significant cardiac and renal involvement are more common in children, may lead to longer and higher dose corticosteroid therapy, and may contribute to a less optimistic prognosis than that described in adults.

Consistency in ratings of clinical performance of the same students throughout medical school and internship.

Ratings of student clinical performance were analyzed to determine consistency of supervisor and coordinator judgements of the same students as they progressed through medical school and on into the internship. The results suggested that students were consistently and progressively rated higher.