Growth retardation in juvenile rheumatoid arthritis (JRA).
Explore the source record for details and available documents.
Arthritis, Juvenile↗
Biomedical subjects
B H Bernstein
Publications and source records attributed to B H Bernstein.
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.
Adolescent↗
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.
Adolescent↗
Reiter's syndrome in childhood.
Seven boys with Reiter's syndrome are described. Three had diarrhea and 2 had venereal contact as antecedent events. All developed the complete triad of symptoms in a 5- to 24-day period. Joint involvement of the lower extremities was seen in each boy. HLA-B27 typing was positive in 6 of 7 (86%). Serum and synovial fluid levels of CH50 and C3 were elevated in 5 boys and confirm similar findings in adults. Two boys recovered spontaneously without therapy and 3 boys received aspirin with a rapid and complete resolution of symptoms. The two oldest boys had the most severe joint involvement and were receiving phenylbutazone with continuing active arthritis when they were lost to followup. Reiter's syndrome in children may be infrequently reported because its antecedents and the triad symptoms are common occurrences in pediatric practice.
Adolescent↗
Systemic lupus erythematosus in childhood correlations between changes in disease activity and serum complement levels.
Serial complement component (C3 and C4) determinations were performed in 26 children with systemic lupus erythematosus. Twenty-one children with SLE had 52 episodes of C3 depression (mean duration 25 weeks); only 11 of these children had active nephritis when serum concentrations of complement were depressed. Fourteen children had active rash associated with low C3; in seven of these children rash was the only clinical evidence of disease activity. Ten children had active CNS disease; in seven children the CNS involvement correlated with low C3. In general, variations in serum concentrations of C4 did not reflect changes in SLE activity which were not reflected by changes in serum concentrations of C3. Serum C4 occasionally remained depressed longer than C3, perhaps reflecting continuing subclinical disease activity. Increased C3 occurred in 18 of 26 children as doses of corticosteroid were increased, in six of 14 when cyclophosphamide was added, and in two children when hydroxychloroquine was added. Our findings suggest that a wide variety of manifestations of childhood SLE may produce hypocomplementemia. In addition to renal disease, variations in serum concentrations of C3 and C4 can reflect, or occasionally predict, changes in rash and CNS disease.
Adolescent↗
Scleroderma of the foot: orthopaedic treatment.
Four patients with scleroderma involving the feet have been treated at Childrens Hospital of Los Angeles by the authors. All children had severe deformity of the feet secondary to the scleroderma. A review of the literature reveals little past mention of indications or treatments of this deformity in the past with the exception of the use of local steroids. Six feet in four children underwent orthopedic treatment. Treatment was primarily by casting to stretch soft tissues and gain as much correction as possible. Surgical correction of the remaining deformity by releasing the soft tissue contractures was then accomplished. Internal fixation of the foot in the corrected position was carried out in four cases. Three of the four patients showed significant improvement following soft tissue surgery and one patient was unchanged. Surgical treatment of the patient with scleroderma can be carried out safely as long as careful attention is paid to circulatory status and extensive preoperative casting and stretching of "soft" tissues is utilized.
Casts, Surgical↗
High dose methotrexate in the treatment of refractory juvenile rheumatoid arthritis.
OBJECTIVE: To assess the response to and safety of long term, high dose (> or = 1 mg/kg/week or > or = 15 mg/m2/week) methotrexate (MTX) administration, in a cohort of 21 children with longstanding, severe juvenile rheumatoid arthritis (JRA). METHODS: Children received MTX at an average weekly dose of 27 mg for a mean of 15.2 months. Outcome was assessed using a disease activity score based on changes in concomitant therapy, laboratory parameters, physician's global assessment, and radiologic evaluation. RESULTS: Seven patients (33%) improved, including one child who achieved complete remission, while 14/21 children (67%) did not benefit from high dose MTX. Subsequently, 6/14 (43%) of the non-responders discontinued high dose MTX and began cyclosporine. Radiologic progression, regardless of clinical outcome, was documented in 10/15 (67%) of the patients. The drug was well tolerated despite mild gastrointestinal symptoms and transient liver enzyme elevation. CONCLUSION: The results of this open retrospective pilot trial suggest that high dose MTX is well tolerated, but that its role in the treatment of children with refractory JRA may be limited. Radiologic progression, despite improvement in the clinical status or in the laboratory parameters, supports the hypothesis that MTX acts as a potent antiinflammatory agent.
Adolescent↗
T cell receptor rearrangements in juvenile rheumatoid arthritis: a search for oligoclonality.
Southern blot analysis of DNA from paired samples of synovial compartment (membrane and/or fluid) and peripheral blood T cells from nine children with juvenile rheumatoid arthritis (JRA) was carried out. Using a T cell receptor C beta probe, dominant TCR rearrangements were discovered in specimens from three patients: synovial fluid T cells from one, synovial fluid and synovial membrane cells from a second, and synovial membrane and peripheral blood cells from a third. The patient showing dominant bands in peripheral blood as well as in synovium was the only child in the series with systemic disease. Since non-specific T cell recruitment is likely to dilute antigen specific clones to low levels, the finding of dominant rearrangements in three of nine patients may indicate that oligoclonality is indeed a feature of JRA.
Arthritis, Juvenile↗