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Biomedical subjects

B M Ansell

Publications and source records attributed to B M Ansell.

At least 19 recordsLinked to original sources

Juvenile rheumatoid arthritis, juvenile chronic arthritis, and juvenile spondyloarthropathies.

Immunogenetics are supporting the marked heterogeneity of chronic arthritis in children. Thus DRw13-DRw18 and DQw6-DQw18 were associated with persistent pauciarticular disease in children with an early onset of disease. Several studies have shown DPw2 as an additional susceptibility factor in this subgroup. Standardization of diagnostic criteria for juvenile onset spondyloarthropathy and psoriatic arthritis is necessary; various studies are in progress, and although HLA-B27 provides the common marker, this may only apply to a small group of juvenile psoriatics who have spondyloarthropathy. In the management of juvenile rheumatoid arthritis, methotrexate in moderate doses has been shown to be superior to lower doses of methotrexate and placebo in controlling polyarthritis. Methotrexate may be of particular value in treating the polyarthritis that follows a pauciarticular onset. The possible value of sulfasalazine in a B27 group with persistent polyarthritis has been suggested. Highlights of corticosteroid therapy were intra-articular injections, particularly in pauciarticular disease, the suggestion that deflazacort has a calcium sparing effect, and the possible role of intravenous methylprednisone in the management of severe disease.

Adrenal Cortex Hormones

Juvenile dermatomyositis.

Juvenile dermatomyositis is a relatively rare, multisystem disease characterized by a nonsuppurative myositis which causes symmetrical weakness, rash and vasculitis; this last can affect the gastrointestinal tract and the myocardium. Late development of calcinosis is seen in approximately two thirds of patients. Its etiology is unknown, although there are clues that it may be an unusual response to a viral infection. Some 50% of children will have a very acute, rapidly progressive disease, while the remainder may present subacutely with rash and a gradually progressive weakness of muscles, joint contractures and very occasionally calcinosis. When there is acute muscle damage, the creatine phosphokinase will be raised, but it is not uncommon to have a normal erythrocyte sedimentation rate, and antinuclear antibodies are usually present. Early in acute cases immune complexes will often be detected. In the presence of vasculitis, monitoring the disease by levels of von Willebrand's factor 8 antigen may be helpful. Although the prognosis for survival has steadily improved, it remains a serious illness and death can occur in the acute phase due to myocarditis, progressive unresponsive myositis, perforation of the bowel as a sequel to vasculitis ulceration or occasionally lung involvement. Intercurrent infections during the course of the disease also give rise to problems. In its management, there is still a question as to whether intravenous pulses of methylprednisone might be more valuable than oral corticosteroids; in either case it must be given in adequate amounts early in the course of the disease to control muscle inflammation. Once this is controlled rehabilitation commences.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones

Juvenile chronic arthritis: classification, differential diagnosis and prognosis.

Although the nomenclature and classification of juvenile chronic polyarthritis is not uniform, the division into systemic, polyarticular and pauciarticular forms is generally accepted. The characteristic symptoms, the division into subgroups according to age, clinical course and serology as well as the differential diagnostic considerations pertaining to each form are described. Most of the children with the pauciarticular form have a good prognosis; however, in 15% of these cases the disease changes into polyarticular JCA. The systemic form has the least favorable prognosis, while the prognosis of polyarthritic JCA depends upon the presence of the IgM-Rheuma factor. In seronegative patients the prognosis also depends on the age at onset of the disease and on the quality of the treatment of the joints during the acute phases.

Adolescent

The wrist in juvenile arthritis.

Patients with juvenile arthritis affecting the wrist have been reviewed six months or more after treatment, which has included steroid injection, synovectomy, arthroplasty, arthrodesis and distraction lengthening of the ulna. The pattern of wrist involvement is described, the general management reviewed, and the indications, techniques and results of these various procedures are presented.

Arthritis, Juvenile

Juvenile chronic arthritis and juvenile spondyloarthropathy.

Clinically recognized subgroups of juvenile chronic arthritis and the juvenile spondyloarthropathies are gradually being shown to be immunogenetically distinct; greater subdivision may ultimately be required. Mechanisms by which the association of certain genes work await further elucidation. Meanwhile, therapy such as intravenous gamma globulin may be most effective in systemic disease. Sulfasalazine is recommended for patients with later-onset pauciarticular disease, particularly HLA-B27 patients. Methotrexate should probably be reserved for severe unresponsive disease, particularly polyarthritis, until side effect profiles are better evaluated.

Arthritis

Panniculitis: a report of four cases and literature review.

Panniculitis is a disease with many causes and associations. The classification of clinical subtypes is unsatisfactory and hampered by the use of eponyms. Four children with recurring panniculitis are described and their histology presented. Three had subcutaneous fat atrophy with lobular panniculitis on biopsy; all responded well to corticosteroids. The fourth child had a septal panniculitis with no atrophy of subcutaneous tissues and only a partial response to treatment with corticosteroids. A widely accepted precise histological classification of panniculitis is needed to enable accurate predictions of the outcome of this serious disorder.

Adipose Tissue

Total hip replacement for juvenile chronic arthritis.

We review the results of 96 primary total hip replacements in 54 patients with juvenile chronic arthritis at five years or longer after surgery. The mean age at operation was 16.7 years (range 11.25 to 26.6); the follow-up period averaged 11.5 years. The clinical results in terms of pain, range of movement, mobility and function are presented. A revision procedure was required in 24 hips (25%) in 18 patients at an average of 9.5 years after the primary operation. A further 17 hips had radiographic signs of loosening. The factors thought to contribute to this relatively high failure rate in patients with juvenile chronic arthritis are discussed.

Adolescent

Juvenile dermatomyositis.

Juvenile dermatomyositis is a multisystem disease characterized by muscular inflammation affecting primarily the skin and muscle; it presents as a nonsuppurative myositis causing symmetrical weakness and typical skin rashes that affect the face and hands in particular, but can occur at any site. In the last few years, a number of reviews have been published, this article only highlights some points of interest.

Adolescent

Still's disease.

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Arthritis, Juvenile

Randomized, double-blind trial of deflazacort versus prednisone in juvenile chronic (or rheumatoid) arthritis: a relatively bone-sparing effect of deflazacort.

Thirty-four children with juvenile chronic (rheumatoid) arthritis were recruited to a randomized, double-blind study of deflazacort (an oxazolone derivative of prednisone) vs prednisone. All had been receiving glucocorticoid therapy for at least 1 year and required at least 5 mg/d of prednisolone (usually as 10 mg every 2 days). Thirty-one children completed the study. Bone density trends were measured in the spine by dual photon absorptiometry and in the forearm by single photon quantitative computed tomography at 3-monthly intervals. Trends (velocities) in bone and soft tissue growth were calculated. In the spine, bone growth correlated well with indices of soft tissue growth, but covariance analysis showed a significant advantage (P less than .007) of deflazacort when spinal bone mineral growth was compared to body surface area and weight. In part this was due to a temporary interruption in weight by children receiving deflazacort, whose gain in height was comparable with that of the prednisone group. Some children in both groups improved clinically and showed catch-up growth; in these children relative spinal bone mineral growth velocities were about twice those observed for height and weight. It is concluded that during the first year of deflazacort, their spinal bone mineral content at a level that was appropriate for their height and weight. Further observations are required to establish whether this advantage can be maintained subsequently. The anti-inflammatory effects of the two glucocorticoids appeared similar.

Adolescent

Rapid periarticular bone loss in rheumatoid arthritis. Possible promotion by normal circulating concentrations of parathyroid hormone or calcitriol (1,25-dihydroxyvitamin D3).

For approximately 2 years, bone loss was measured in women with early stages of rheumatoid arthritis (RA) and in control subjects, using serial computed tomography and dual photon absorptiometry. Rapid trabecular bone loss from the distal radius was observed in the RA patients but not the controls. The bone loss correlated with initial plasma levels of parathyroid hormone and 1,25-dihydroxyvitamin D3 (calcitriol) concentrations. It has been suggested that these humoral factors may interact with cytokines or other mediators produced in the adjacent wrist joint. Losses of the cortical bone of the radial midshaft and the lumbar spine were modest and were comparable in the 2 groups. Indices relating to both bone formation and bone resorption predicted bone loss at these 2 sites, but changes in the parathyroid hormone and calcitriol concentrations did not.

Absorptiometry, Photon

Tarsometatarsal involvement in juvenile spondyloarthropathy.

Patients with juvenile spondyloarthropathy who carry HLA B27 antigen or have a family history of ankylosing spondylitis may have marked tarsometatarsal arthritis. This may be associated with hindfoot involvement and result in a supination deformity. Spontaneous fusion with complete obliteration of the tarsometatarsal joints may occur.

Adolescent

Recurrent acute proptosis in atypical systemic lupus erythematosus.

A patient is described who developed acute proptosis, a rare complication of systemic lupus erythematosus, 16 years after the onset of the disease. Treatment with intravenous methylprednisolone produced rapid improvement, but swelling recurred a few days later. Intravenous corticosteroid was again effective and was continued orally without further episodes of proptosis. This patient is unusual in the long period of mild disease before the development of systemic complications, and in having antibodies to nuclear ribonucleoprotein but persistently normal titres of DNA-binding antibodies in her serum.

Acute Disease