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Biomedical subjects

V Hanson

Publications and source records attributed to V Hanson.

At least 37 records · Page 2Linked to original sources

Antibodies to nonhistone nuclear antigens and antilymphocyte antibodies among children and adults with systemic lupus erythematosus and their relatives.

Antibodies to nonhistone nuclear antigens are found with increased frequency in the serum of patients with systemic lupus erythematosus (SLE) and their first degree relatives. We measured the prevalence of ANA and antibodies against DNA, RNP, Sm, SSA, and SSB as well as antilymphocyte antibodies (ALA) in the serum of 24 patients with childhood onset SLE and 94 of their first degree relatives and compared the prevalence of these same antibodies in 8 patients with adult onset SLE and 33 of their first degree relatives. Antibodies against DNA, RNP, and Sm occurred more frequently in the serum of adult onset SLE patients. Among the first degree relatives ALA occurred more frequently in the sera of relatives of patients with childhood onset SLE, and parental antibodies to SSA were associated with the occurrence of childhood onset SLE in 3/24 cases. Since antibodies to SSA have been shown to be related to neonatal SLE their possible relationship to childhood SLE requires further study.

Adult↗

Nailfold capillary abnormalities in childhood rheumatic diseases.

The nailfold capillary patterns of 84 patients with a variety of childhood rheumatic diseases and 34 normal control subjects were observed. Distinctive morphologic abnormalities with capillary dilation and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis and with scleroderma (P less than 0.001). Among those with scleroderma, capillary abnormalities were found in all nine patients with systemic disease and in none of 10 patients with cutaneous disease only (Fisher's exact P less than 0.001). Of 25 patients with dermatomyositis for whom muscle biopsies were available for analysis, abnormal nailfold capillary pattern was found with highest prevalence in patients with two or more specific vascular lesions noted on biopsy (Fisher's exact P = 0.041). Nailfold capillary abnormalities are present in distinct populations of childhood rheumatic diseases, reflect the underlying vasculopathy of childhood dermatomyositis, and may be of diagnostic value in distinguishing localized from systemic scleroderma.

Adolescent↗

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↗

The association of antinuclear antibodies, antilymphocyte antibodies, and C4 activation among the relatives of children with systemic lupus erythematosus.

We studied 31 children with systemic lupus erythematosus (SLE) and 108 first-degree relatives of the children to determine if HLA type, familial relationship to patient, or gender influenced the familial aggregation of SLE and the serologic and serum complement abnormalities associated with SLE. There were no significant relationships between the presence of antinuclear antibodies (ANA), antilymphocyte antibodies (ALA), or circulating immune complexes. Furthermore, there was no correlation between the occurrence of ANA, ALA, or circulating immune complexes with HLA type or relationship to patient. Both ANA and ALA were associated with a reduction in the mean serum C4 level, without a corresponding reduction in mean serum C3 or CH50. The reduction in mean serum C4 level with ALA occurred without regard to the subject's relationship to the patient, but the reduction in mean serum C4 level with ANA was significant only among the sisters. Because of these data, it seems that the sisters of patients with SLE are more likely than other relatives to have activation of the complement system in association with serologic abnormalities. Those sisters with ANA and ALA had the greatest amount of complement activation and may be the relatives at greatest risk for the subsequent development of SLE.

Adult↗

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.

Adult↗

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.

Adult↗

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.

Adolescent↗

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. The major complaint was pain; swelling and vasomotor instability were prominent, and exquisite tenderness was characteristic. Chronic trophic changes were not observed. Antecedent illness or trauma could be related to the RND in less than half of the children, but personality factors appeared contributory to the development of RND in most children. Physical therapy was the principal form of treatment; therapy with a corticosteroid or by sympathetic blockade was not employed. Reduction in the evidences of disease, including improvement in function, were present in all children at the termination of therapy; improvement was maintained in all but one child after a mean period of 2.4 years. The excellent response to conservative therapy suggests that RND may be a more benign condition in children than in adults.

Adolescent↗

Neutropenia associated with chrysotherapy for juvenile rheumatoid arthritis.

Severe neutropenia, in the absence of generalized bone marrow depression, is a rare complication in adults receiving chrysotherapy for rheumatoid arthritis and has not been described in children. Isolated, severe neutropenia developed in five children with systemic onset JRA while they were receiving gold injections. This potentially fatal complication occurred within eight weeks of beginning therapy in four patients, and after 24 weeks of well-tolerated therapy in the fifth. Leukopenia preceded neutropenia in two children. Localized infection was successfully treated in one child; septicemia was fatal to a second child. Neutropenia resolved within eight to 14 days of its onset in the four survivors; chelation with dimercaprol in one child did not appear to alter the recovery time. It is suggested that a systemic onset of JRA in children less than 6 years of age identifies a higher risk group developing severe neutropenia during chrysotherapy. Cessation of gold therapy upon recognition of a decreasing neutrophil count may prevent or ameliorate a developing neutropenia; careful observation for, and early treatment of, infection may alter its outcome.

Agranulocytosis↗

Long-term colchicine therapy of familial Mediterranean fever.

Familial Mediterranean fever is a disorder characterized by recurrent fever and polyserositis. Continuous prophylactic colchicine therapy has been effective in suppressing attacks in affected adults. From 30 children with FMF, 14 were selected for colchicine therapy. Eight children continued prophylactic colchicine therapy for 29 months (mean) and experienced a marked decrease in the frequency of attacks. Six other children did not comply with the treatment regimen. Although no deleterious side effects were noted, the safety of long-term colchicine administration in childhood is unknown.

Adolescent↗

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. We report on two children with familial Mediterranean fever with radiographic abnormalities similar to those described in adults. Although sacroiliitis is strongly correlated with the presence of HLA-B27 in most arthropathies, these children were HLA-B27-negative. The diagnosis of familial Mediterranean fever was delayed in both patients because the association of sacroiliitis with familial Mediterranean fever in childhood was not recognized.

Arthritis↗

Aspirin-induced hepatotoxicity and its effect on juvenile rheumatoid arthritis.

Evidence of hepatic disease was sought in 102 children with juvenile rheumatoid arthritis (JRA) who were treated with aspirin. Serum glutamic oxaloacetic transaminase level was elevated (greater than 39 IU/liter) in 59% of the children. The degree and prevalence of SGOT elevations correlated with aspirin dose and serum salicylate level. Nevertheless, increased SGOT values were frequently present in children receiving moderate aspirin doses and having serum salicylate levels less than 25 mg/100 ml. Elevated SGOT values decreased in proportion to the degree of reduction in aspirin dose. The SGOT values above the 100 IU/liter were statistically associated with reduced sedimentation rates. Concomitant improvement in the clinical manifestations of JRA was noted in some children.

Arthritis, Juvenile↗

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. Evidence from animal studies suggests that intensive physical therapy with emphasis on continued weight bearing may have been important in promoting healing in these children. It appears that restoration of severely damaged hip joints in JRA can occur when the disease activity is controlled. Restoration may be favored by a child's greater potential for bone remodeling and by vigorous physical therapy with emphasis upon continued ambulation.

Adolescent↗

Plant thorn synovitis.

Five children with an inflammatory monarthritis due to penetration of the joint by plant thorns are presented. The clinical presentation was that of a transient acute synovitis followed by a relatively asymptomatic period, and later by chronic arthritis often after the thorn injury was forgotten. Histopathology demonstrated a granulomatous synovitis. Polarized light microscopy facilitated identification of the plant tissue within the synovium. Conservative medical therapy was ineffective, but surgical excision of the affected synovium resulted in normal joint function. The differential diagnosis of monarthritis in children in extensive, and awareness of this condition is necessary to elicit a comprehensive history and to avoid delay in treatment.

Arthritis↗