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B H Athreya

Publications and source records attributed to B H Athreya.

At least 19 recordsLinked to original sources

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study.

OBJECTIVE: Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. METHODS: A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. RESULTS: Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. CONCLUSION: This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.

Adolescent↗

Calcium apatite crystals in synovial fluid rice bodies.

BACKGROUND: Rice bodies can occur in the joints in many rheumatic conditions, but they are most common in rheumatoid arthritis. They are generally believed to occur rarely in patients with osteoarthritis, but one study reported rice bodies with apatite crystals. OBJECTIVE: To report on a series of joint fluids with rice bodies containing apatite clumps and examine their clinical pictures. METHODS: All synovial fluid analysis reports for 10 years were reviewed for rice bodies and eight patients were reported on. A series of patients with a variety of diseases with synovial fluid rice bodies found to contain calcific material is described. All were examined by compensated polarised light and alizarin red stain, and four were examined by electron microscopy. RESULTS: The eight patients all had alizarin red S chunks embedded throughout the rice body. Transmission electron microscopy disclosed the presence of a matrix of collagen, fibrin, and amorphous materials containing typical apatite crystals. Clinical diagnoses, radiographic findings, and leucocyte counts varied, but six of the eight patients had had previous repeated corticosteroid injections into the joints. CONCLUSION: Aggregates of apatites may be more common than previously recognised in rice bodies as they are not routinely sought. Whether they are a result of joint damage or depot steroid injections and whether that might contribute to further joint injury now needs to be investigated.

Anthraquinones↗

Point prevalence of cardiac abnormalities in children with systemic lupus erythematosus.

OBJECTIVE: To determine the point prevalence and pattern of silent cardiac abnormalities and associations with suspected risk factors in a sample of children with systemic lupus erythematosus (SLE). METHODS: Cross-sectional analysis of 19 children with SLE from a referral-based rheumatology clinic at an urban children's hospital. Patients were eligible if they were 20 years of age or younger and classified with SLE using the revised criteria of the American College of Rheumatology. Each patient completed a survey, physical examination, standard 12-lead electrocardiogram (ECG), echocardiogram, and had laboratory determinations of complement, triglyceride, and cholesterol levels. RESULTS: Six patients (32%) had cardiac abnormalities on ECG or echocardiogram. In 3, the abnormalities were mild and considered within the normal range. In 5, the abnormalities were considered silent. These abnormalities included ischemic changes (3 patients), valvular insufficiency (3 patients), ventricular repolarization defects (2 patients), cardiac enlargement (1 patient), and ventricular dysfunction (1 patient). Only a recent history of palpitations was significantly associated with the presence of cardiac abnormalities (p = 0.04). CONCLUSIONS: Silent cardiac abnormalities can occur in children with SLE. A recent history of palpitations may be associated with cardiac abnormalities. Routine evaluation of children with SLE using ECG and echocardiogram may help screen for these abnormalities. However, future studies comprising larger sample sizes and longitudinal followup will be required to determine the natural history of cardiac abnormalities in children with SLE and to identify risk factors.

Adolescent↗

Use of ELISA to measure antinuclear antibodies in children with juvenile rheumatoid arthritis.

OBJECTIVE: To compare a series of commercial ELISA tests with an indirect immunofluorescent antibody (IFA) test for the detection of antinuclear antibodies (ANA) in children with juvenile rheumatoid arthritis (JRA). METHODS: Sera from 178 patients with JRA (88 pauciarticular, 68 polyarticular, 22 systemic) were compared with 26 healthy pediatric subjects. Twenty-one samples from patients with systemic lupus erythematosus (SLE) were also tested. All samples were analyzed by IFA and by 3 commercial ELISA methods. Concordance of ELISA results with IFA results (selected standard) were used as a measure of performance. Sensitivity and specificity were calculated for each test and likelihood ratios (LR) were established for IFA and ELISA in pauciarticular and polyarticular JRA sera. The increment in pretest probability was then obtained for each test as an additional measure of test performance. RESULTS: IFA rendered positive results on 18-77% of the JRA sera depending upon the subset, 100% of SLE sera, and 15% of normal patient sera. Using IFA as the standard, correspondence with positive results among patients with JRA ranged from 0 to 74% for the 3 ELISA tests, while it ranged from 5 to 73% in IFA negative sera. IFA tests showed intermediate range likelihood ratios (0.3, 0.5, 3.5, and 5) and increments in pretest probability ranging from 25 to 45%. While one of the ELISA tests attained 50% of increment in pretest probability for the positive test, it showed 0% increment as a negative test. The other 2 ELISA tests incremented the pretest probability from 0 to 25%. CONCLUSION: Our findings indicate that in JRA, the lack of correspondence with the historic standard IFA precludes the use of ELISA tests for detection of ANA. In addition, IFA out-performs ELISA by a substantial degree when "clinical utility" analysis of test performance is utilized. Detection of ANA in children with JRA should either continue to rely on IFA or be based on a different set of antigens if an ELISA format is chosen.

Antibodies, Antinuclear↗

Hypophyseal-pituitary-adrenal axis in autoimmune and rheumatic diseases.

This article discusses the effects of sex steroids and anterior pituitary hormones on the immune system. Data from clinical and experimental studies on the effects of CRH, FSH, LH, and prolactin are reviewed. This is followed by a summary of results from our studies on the effects of FSH, LH, and prolactin on PBMC, CD4+ cells, and CD8+ cells in vitro.

Adolescent↗

Computer-assisted diagnosis of pediatric rheumatic diseases.

OBJECTIVE: AI/RHEUM is a multimedia expert system developed originally to assist in the diagnosis of rheumatic diseases in adults. In the present study we evaluated the usefulness of a modified version of this diagnostic decision support system in diagnosing childhood rheumatic diseases. METHODOLOGY: AI/RHEUM was modified by the addition of 5 new diseases to the knowledge base of the system. Criteria tables for each of the diseases included in the knowledge base were modified to suit the needs of children. The modified system was tested on 94 consecutive children seen in a pediatric rheumatology clinic. RESULTS: AI/RHEUM made the correct diagnosis in 92% of the cases when the diagnosis was available in the knowledge base of the system. It was also shown to be effective in the education of pediatric trainees through its multimedia features. CONCLUSIONS: AI/RHEUM is an expert system that may be helpful to the nonspecialist as a diagnostic decision support system and as an educational tool.

Child↗

Juvenile rheumatoid arthritis-like polyarthritis in chromosome 22q11.2 deletion syndrome (DiGeorge anomalad/velocardiofacial syndrome/conotruncal anomaly face syndrome).

OBJECTIVE: To investigate the association of polyarthritis and chromosome 22q11.2 deletions. METHODS: Eighty patients with chromosome 22q11.2 deletion syndrome followed up at The Children's Hospital of Philadelphia were examined for evidence of arthropathy or arthritis. Patients with chromosome 22q11.2 deletion syndrome and polyarthritis underwent laboratory evaluations of immunologic function to determine the relationship of their immunodeficiency to the polyarthritis. RESULTS: The prevalence of polyarthritis in patients with chromosome 22q11.2 deletion syndrome was markedly increased over the prevalence of polyarticular juvenile rheumatoid arthritis (JRA) in the general population. All 3 patients with polyarthritis had evidence of impaired T cell function. Two of the patients with polyarthritis also had IgA deficiency. CONCLUSION: The chromosome 22q11.2 deletion syndrome represents a primary T cell disorder which can be associated with a JRA-like polyarthritis. All 3 patients with polyarthritis had evidence of more extensive immunoregulatory derangements than those typically seen in patients with chromosome 22q11.2 deletion, and these derangements may have predisposed to the development of polyarthritis.

Arthritis↗

New-onset juvenile dermatomyositis: comparisons with a healthy cohort and children with juvenile rheumatoid arthritis.

OBJECTIVE: To determine, in a case-control study, if patients with new-onset juvenile dermatomyositis (juvenile DM) have increased symptoms prior to onset, exposure to certain environmental conditions, frequency of familial autoimmune diseases, or antibody titers, compared with 2 control groups. METHODS: A structured interview with the families of 80 children with juvenile DM, 40 children with juvenile rheumatoid arthritis (JRA), or 23 healthy children, from the same geographic area as the children with juvenile DM, was conducted. All children's sera were tested for antibody to Toxoplasma gondii, herpes simplex virus (HSV), or coxsackievirus B (CVB). RESULTS: A high proportion of children with juvenile DM had constitutional symptoms 3 months before the disease-onset date (P = 0.013 versus control children). Children with JRA had more relatives with rheumatoid arthritis (P = 0.0001) and pernicious anemia (P = 0.003) than did children with juvenile DM or healthy children. Among children < or =7 years of age, elevated enteroviral titers were more frequent in those with juvenile DM (81%) and in healthy controls (90%) than in those with JRA (64%), suggesting a common environmental exposure. Titers to T gondii, HSV, or CVB 1-6 were normal. CONCLUSION: Frequencies of familial autoimmune disease, exposure to environmental factors, or elevated antibody titers to T gondii, HSV, or CVB are not increased in juvenile DM. Children with juvenile DM do have symptoms of illness 3 months before the disease-onset date, and young patients have elevated enteroviral titers, as do young geographic controls.

Animals↗

Syndromes and arthritis.

There are many questions and no clear answers raised by these children. These syndromes, however, seem to be biologic experiments of nature and present unique opportunities to study the various elements involved in the pathogenesis of arthritis. Pediatric rheumatologists are in a unique position to study these syndromes.

Adolescent↗

Clinical characteristics of antiphospholipid antibody syndrome in children.

OBJECTIVE: To evaluate the clinical features and outcome of antiphospholipid syndrome (APS) in children. STUDY DESIGN: Retrospective chart review of patients seen at the Children's Hospital of Philadelphia and Children's Seashore House Pediatric Rheumatology Center between 1988 and 1993. RESULTS: Nine patients with ages ranging from 8 months to 17 years are presented. Clinical features of five patients with primary APS, described in detail, were digital ischemia, stroke, chorea, Addison disease, and pulmonary vaso-occlusive disease. The four children with secondary APS had systemic lupus erythematosus. Clinical features of these patients include livedo reticularis, deep venous thrombosis, and pulmonary hypertension. Antiphospholipid titers, results of coagulation studies, and serologic findings did not predict outcome. CONCLUSION: APS in children has diverse clinical features similar to those in adults and should be considered in cases of unexplained vaso-occlusive disease.

Adolescent↗

Lyme disease.

Explore the source record for details and available documents.

Arthritis, Infectious↗

Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A.

Cytophagic histiocytic panniculitis (CHP) can be a severe variant of Weber-Christian disease characterized by the histopathologic appearance of lobular panniculitis infiltrated by histiocytes containing blood cell fragments and by a clinical course with marked systemic features including multiorgan failure, hypertriglyceridemia, and coagulopathy, which may lead to death. Therapy of CHP includes standard treatment for panniculitis, such as antimalarials, plus immunosuppressives for more severe cases. The response to treatment, however, is unpredictable. In several recent reports, cyclosporine A has been successfully used to treat panniculitis. We report a patient and review the literature on CHP and the use of cyclosporine A as therapy. Published reports reveal that in instances of severe CHP when cyclosporine A was not given, 19 of 27 patients died (70% mortality). When severe CHP was treated with cyclosporine A, rapid remission was achieved in our patient and all five previously published cases (0% mortality). We believe cyclosporine A is the drug of choice in severe CHP and should be considered in all such patients.

Adolescent↗

Vasculitis in children.

Our understanding of the pathogenesis of vasculitis has improved significantly in recent years. The study of cytokines, endothelium, and adhesion molecules in the initiation of inflammatory responses has opened new avenues of investigation and treatment of these disorders. The recognition of newer antibodies such as antineutrophil cytoplasmic antibody has given us newer classification of some of the vasculitides. In this paper, articles published in 1995 on childhood vasculitis with both clinical and research importance are reviewed.

Child↗

Vasculitis in children.

Common vasculitic disorders in children include those associated with infections (e.g., Rickettsiae, subacute bacterial endocarditis), Schonlein-Henoch purpura, and Kawasaki disease. Recent advances have occurred in understanding the pathogenesis of vasculitides. In this review, the reader will be exposed to some of the developments in adhesion molecules, antineutrophil cytoplasmic antibodies, antiendothelial antibodies, and antiphospholipid antibodies. Classification criteria and diagnostic strategies are also summarized.

Child↗

Clinically significant gastropathy associated with nonsteroidal antiinflammatory drug use in children with juvenile rheumatoid arthritis.

OBJECTIVE: To estimate the frequency of documented clinically significant gastrointestinal (GI) side effects secondary to nonsteroidal antiinflammatory drugs (NSAID) therapy and to describe the adverse events. METHODS: Computerized medical records of 702 patients with juvenile rheumatoid arthritis (JRA) administered NSAID were searched for the occurrence of clinically significant gastropathy (esophagitis, gastritis, peptic ulcer disease). RESULTS: Five children were identified who had a total of 10 events of gastropathy documented by either barium swallow or endoscopy, and thought to be attributable to NSAID therapy. Each child had at least 2 separate events of clinically significant gastropathy. CONCLUSION: Although mild GI disturbances are frequent side effects associated with NSAID therapy, the number of children with JRA who experience clinically significant gastropathy appears to be low.

Adolescent↗

Measurement of health status in children with juvenile rheumatoid arthritis.

OBJECTIVE: To develop and validate a self- or parent-administered instrument for measuring functional status in children with juvenile rheumatoid arthritis (JRA). METHODS: We adapted the Stanford Health Assessment Questionnaire (HAQ) for use in children ages 1-19 years, by adding several new questions, such that for each functional area, there was at least 1 question relevant to children of all ages. The face validity of the instrument was evaluated by a group of 20 health professionals and parents of 22 healthy children. The questionnaire was then administered to parents of 72 JRA patients (mean age 9.1 years, onset type systemic in 16, polyarticular in 21, pauciarticular in 35). RESULTS: The instrument showed excellent internal reliability (Cronbach's alpha = 0.94), with a mean inter-item correlation of 0.6. The convergent validity was demonstrated by strong correlations of the Disability Index (average of scores on all functional areas) with Steinbrocker functional class (Kendall's tau b = 0.77, P < 0.0001), number of involved joints (Kendall's tau b = 0.67, P < 0.0001), and morning stiffness (Kendall's tau b = 0.54, P < 0.0001). Spearman's correlation coefficient between Disability Index scores from questionnaires administered to parents and those from questionnaires administered to older children (> 8 years) was 0.84 (n = 29; P < 0.001), showing that parents can accurately report for their children. The test-retest reliability, studied at a 2-week interval, revealed virtually identical Disability Index scores measured on the 2 occasions (0.96 versus 0.96; P > 0.9 by paired t-test; Spearman's correlation coefficient = 0.8, P < 0.002). CONCLUSION: The Childhood HAQ, which takes less than 10 minutes to complete, is a valid, reliable, and sensitive instrument for measuring functional status in children with JRA.

Arthritis, Juvenile↗