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.

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.

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.

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.

Reflex neurovascular dystrophy in childhood.

Reflex neurovascular dystrophy has rarely been recognized in children. During the past eight years we have observed 24 instances of RND in 23 children. Lower extremity involvement was manifested in 20 of them and upper extremity in four.

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.

HLA-B27-negative sacroiliitis: a manifestation of familial Mediterranean fever in childhood.

Familial Mediterranean fever is a polysystemic disease seen most frequently in persons of Mediterranean ancestry. Arthritis is one of the common manifestations. Both symptomatic and asymptomatic sacroiliitis have been reported in adults.

Hip joint restoration in juvenile rheumatoid arthritis.

Six children with long-standing juvenile rheumatoid arthritis and radiographic evidence of severe hip joint damage were found 6 months to 5 years afterward to have radiographic evidence of significant hip joint space widening and remodeling of articular surfaces. The disease activity had decreased and hip joint function had improved in all children. Each child had received various antiinflammatory drugs, but no specific medication could be implicated in the improvement.

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.

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.

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.

Prognosis of juvenile rheumatoid arthritis.

The true prognosis of JRA is unknown. The best interpretation of reports to this date may be that at any given time of examination between 5 and 15 years after onset, 30-50% of children will have grossly active disease and that 70-90% of patients will be in class I-II functional status. Published studies, however, are not comparable because of differing criteria and selection of support data to be reported.

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.