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Biomedical subjects

B M Ansell

Publications and source records attributed to B M Ansell.

At least 55 records · Page 3Linked to original sources
PubMed1987

A genetic marker for systemic amyloidosis in juvenile arthritis.

A genetic marker of susceptibility to systemic amyloidosis has been identified. A DNA polymorphic site, 5' to the serum amyloid P component gene, has been found to be significantly associated with amyloidosis in juvenile arthritic patients. When genomic DNA was cut with the restriction enzyme MspI and probed with the cDNA for serum amyloid P component, homozygosity for the 5.6 kb restriction fragment length polymorphic (RFLP) band was absent in all 28 amyloid patients. Furthermore, the proportion who were homozygous for the 8.8 kb RFLP band was greater (p = 0.008) than that among 89 normal subjects. The distribution of this polymorphic site among 19 juvenile arthritic patients without amyloidosis was the same as that in the normal group. Thus the 8.8 kb RFLP band represents a genetic predisposition to reactive amyloidosis in juvenile arthritis and may apply to amyloidosis associated with more common inflammatory conditions.

Adult↗
PubMed1987

Vertebral collapse in juvenile chronic arthritis: its relationship with glucocorticoid therapy.

Forty-six patients with juvenile chronic arthritis maintained on glucocorticoid therapy have been reviewed retrospectively for evidence of vertebral collapse. The 23 patients who had sustained vertebral fractures had received a daily dose 2.3 times higher than those without. No patients sustained a vertebral collapse until they had received a prednisolone cumulative dose of at least 5 g. It would appear that the ideal dose, if collapse of vertebrae is to be avoided, should be not more than 5 mgs daily, probably best given as 10 mg on alternate days; unfortunately, some children with serious systemic disease cannot be controlled on these low doses. In such cases it seems justified to investigate the possible use of the oxazoline derivative of prednisolone (deflazacort) in view of its reported bone-sparing properties in adults.

Arthritis, Juvenile↗
PubMed1987

Anaemia in juvenile chronic arthritis: serum inhibition of normal erythropoiesis in vitro.

Serum from patients with juvenile chronic arthritis (JCA) was shown to inhibit colony formation by normal erythropoietic progenitor cells cultured in vitro. The inhibition was proportional to the degree of anaemia and to certain indices of activity of the arthritis and systemic disease. It occurred in a dose dependent manner with increasing serum concentration and was independent of previous blood transfusion or administered drugs. Erythropoietic progenitor cells from the bone marrows of anaemic patients with JCA showed normal requirements for accessory cells (T lymphocytes and macrophages) in culture, and autologous accessory cells were not deficient in providing normal growth requirements.

Adolescent↗
PubMed1987

Scoliosis in juvenile chronic arthritis.

Structural scoliosis occurs more commonly in patients with juvenile chronic arthritis than in the normal population. We have reviewed 32 patients with both juvenile arthritis and a scoliosis and suggest that structural curves may arise from postural curves associated with asymmetrical involvement of lower limb joints.

Adolescent↗
PubMed1987

Juvenile chronic arthritis.

Juvenile Chronic Arthritis is a relatively uncommon childhood disease. There are no absolute diagnostic tests and many classification criteria have evolved (4, 15, 7) based variably on joint number, disease course, associated clinical features and rheumatoid factor seropositivity. These are of no help diagnostically, but do allow investigative and follow-up studies to compare like with like. It is generally accepted that there are three main modes of onset, the commonest being pauci-articular with less than five joints in the first 3 to 6 months. Arising from the population immediately around the old MRC rheumatism unit at the Canadian Red Cross Memorial Hospital, Taplow, this accounted for 68%, with a poly-articular onset in 20% and systemic onset in 12%. Despite this low incidence of systemic illness, it remains a great challenge.

Adolescent↗
PubMed1987

A chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome. A specific entity analysed in 30 patients.

We have studied the clinical presentation and course of a chronic inflammatory disease occurring in childhood and observed in 30 patients. The first symptoms were generally present at birth, except in a few patients where they were first noticed in early infancy. All the patients had the association of three main symptoms: neurological, cutaneous and articular. The skin rash was the first symptom observed in all the patients and looked like a chronic non pruritic urticaria varying during the day. The articular manifestations involved knees, ankles and feet, elbows, wrists and hands unaffecting the other joints. They could be mild giving arthritis during flare-ups or severe with major radiological modifications affecting the epiphysis, metaphysis and growth cartilage. The neurological manifestations were characterized by a chronic meningitis and symptoms indicating meningeal irritation: headaches, seizures, spasticity of legs. Most patients had a cerebral atrophy and a low IQ. Sensory organ involvement occurred progressively during the follow-up: ocular inflammation with optic atrophy, deafness and hoarseness. Common morphological features characterized these patients with short stature, head enlargement, saddle back nose and short and thick extremities with clubbing of fingers. The course was that of a chronic inflammatory disease with numerous flare-ups associating fever, splenomegaly and adenomegaly. Except for a high level of eosinophils in blood, CSF and tissues, the biology was non specific and only exhibited features of inflammation. Except for two families, the disease was sporadic. A high frequency of prematurity with features resembling a foetal infection was observed but no proof of a possible causal virus has so far been found so that etiology remains unknown.

Adolescent↗
PubMed1987

Immunogenetics of juvenile chronic arthritis.

The current knowledge of the relationship between the HLA system and the different forms of Juvenile Chronic Arthritis is reviewed: Of the different forms the Early Onset Pauciarticular JCA is associated with DRw8, DR5 and A2, the Polyarticular Onset Rheumatoid Factor positive JCA shows the same pattern of association as does the Adult Rheumatoid Arthritis namely with DR4 and DR1. Juvenile Spondylitis is strongly related to the presence of B27. For the other clinical forms of Juvenile Chronic arthritis the data are not sufficiently well established to give a definite association. In Early Onset Pauciarticular JCA the association with the DR antigens DRw8 and DR5 is independent of that with HLA-A2. Thus there must be at least two HLA linked regions contributing to susceptibility to this form of JCA, one in the region of HLA-A and the other one in the HLA-DR region. If Early Onset Pauciarticular JCA is analysed according to the type of onset (Monoarticular, with large joints, with large and small joints, with small joints only and with Iridocyclitis) there are no significant differences in the frequencies for the associated DR alleles DR5 and DRw8. The antigen DR4 is not found at all in patients with Monoarticular Onset and in increasing frequencies with the increased number of involved joints. Analysis of the same patients according to the clinical course of the disease (Persistent Pauciarticular, Extended Pauciarticular, Polyarticular) shows that again there are no significant deviations for the associated antigens A2, DR5, DRw8. DR4 is not found in the group with Persistent Pauciarticular Disease.

Adolescent↗
PubMed1987

Sacroiliitis in juvenile chronic arthritis. A 10-year follow-up.

66 children with early juvenile chronic arthritis underwent HLA A and B typing in 1974; 16 carried HLA B27. Ten of these had a pauciarticular onset, 3 systemic and 3 poly-articular. At follow-up 10 years later bilateral sacroiliitis had developed in 9 of the 16 B27 positive children, all of whom had a pauciarticular onset. Males predominated and these children had an older age of onset and a predominantly lower limb arthropathy. Enthesopathy and acute iritis further distinguished this group clinically. Other sacroiliac changes, usually simple fusion associated with severe hip involvement, were seen in 7 of the patients, 6 were girls and 6 were HLA B27 negative. Back pain and limitation had developed in 5 children, 4 of whom were B27 positive, after an average of 8 years' disease and at a mean age of 17.3 years. It is concluded that the presence of HLA B27 in boys aged 9 or over, with an onset of pauciarticular disease, signifies that they belong to the spondylitic group.

Arthritis, Juvenile↗
PubMed1987

Microcytic anaemia in juvenile chronic arthritis.

The degrees of anaemia and microcytosis in 100 patients with juvenile chronic arthritis (JCA) were analysed according to the pattern of disease and its activity. Microcytosis was common (40% of patients had a mean corpuscular volume (MCV) of less than 75 fl), sometimes severe (MCV less than 65 fl in 14 patients), and closely correlated with the severity of the anaemia. The degrees of microcytosis and anaemia were both directly related to disease activity as measured by the ESR, and were most severe in cases with the systemic form of JCA. In 50 patients iron status was assessed. The serum iron concentration was directly related to the MCV, whereas the serum ferritin was inversely related to MCV and haemoglobin concentration and directly related to the ESR. Bone marrow iron stores were normal or increased in 6 patients with severe microcytic anaemia. These data suggest that the anaemia of JCA is typically microcytic, and that this microcytosis is associated with the disturbance of iron metabolism seen in the 'anaemia of chronic disorders' rather than overall depletion of body iron.

Adolescent↗
PubMed1987

Perspectives in pediatric systemic lupus erythematosus.

As in the adult, systemic lupus erythematosus is a disorder with many manifestations and a number of subgroups, but in any personal series the total is usually small so that these are not always obvious. There is thus an urgent need for multicenter collection of data. This is important not only for appraisal of different aspects of the disease, particularly central nervous system involvement but also to look at prognosis. Assessment of therapy on the different aspects of this disease also requires multicenter cooperation.

Autoantibodies↗
PubMed1986

Multicentre study of piroxicam versus naproxen in juvenile chronic arthritis, with special reference to problem areas in clinical trials of nonsteroidal anti-inflammatory drugs in childhood.

Clinical trials of nonsteroidal anti-inflammatory drugs (NSAIDs) are necessary in juvenile chronic arthritis (JCA) but pose certain problems highlighted and discussed in this study, including recruitment, the assessment of efficacy, and the heterogeneity of the disease. In a multicentre 8-week double-blind cross-over study using the double-dummy technique, piroxicam was compared with naproxen in 47 children with seronegative JCA aged 5-16 years. No significant difference between the two treatments was found in either the clinical variables measured or the parent/patient and physician preference at the end of the study. Side-effect profiles of the two drugs were similar, mainly gastrointestinal disturbances. Piroxicam may be a useful alternative NSAID in JCA, particularly in view of its once-daily dosage.

Adolescent↗
PubMed1986

Intensive immunosuppression in intractable rheumatoid arthritis.

Twelve patients with intractable rheumatoid arthritis were treated with antilymphocyte globulin (ALG), prednisolone and a cytotoxic agent, usually azathioprine, and were followed for 1 year. There was a significant (p less than 0.05) improvement in the mean score for early-morning stiffness, grip strength and the severity of nodules and vasculitis at 6 weeks and 3 months when compared to the initial visit. However, in most patients, this benefit was not sustained despite continued cytotoxic and steroid therapy. A rise in the haemoglobin and fall in ESR was maintained throughout the study period.

Antilymphocyte Serum↗
PubMed1986

Antinuclear antibody studies in juvenile chronic arthritis.

This study of children with chronic arthritis confirms that patients at greatest risk for chronic iridocyclitis have a young age of onset and positive antinuclear antibodies (ANA). Children who are ANA negative have a low risk of iridocyclitis. When it occurs it is at a significantly older age. No child developing juvenile chronic arthritis over 9, even with positive ANA, had chronic iritis. There was remarkable variation in ANA titres; these correlated with erythrocyte sedimentation rate, active arthritis, and iridocyclitis. Maximum ANA titres were often seen very early in the disease but at this time were usually not associated with a poor prognosis. Histocompatibility antigens A2 and DRw8 were significantly increased, suggesting primary associations in pauciarticular arthritis with ANA. HLA-DR5 was associated with mild forms of arthritis and with absence of, or only mild, iritis. HLA-B15/w62 was associated with severe forms of eye involvement.

Adolescent↗
PubMed1986

Overlap connective tissue syndromes.

Twenty six children with overlapping features of more than one connective tissue disorder are reported. The median age of onset was 9.5 years and median duration of follow up 7.5 years. Common presenting symptoms included arthritis, tenosynovitis. Raynaud's phenomenon, myositis, and rashes. At follow up 14 patients had developed sclerodermatous skin changes, but significant systemic involvement was uncommon. Only 16 of the 26 cases had antibodies to nuclear ribonucleoprotein; therefore, 10 did not satisfy criteria for mixed connective tissue disease. It was not possible to differentiate clinical patterns by the presence or absence of any particular antibody profile.

Adolescent↗