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Biomedical subjects

R G Hull

Publications and source records attributed to R G Hull.

At least 19 recordsLinked to original sources
PubMed1994

Lesch-Nyhan syndrome presenting with renal insufficiency in infancy and transient neonatal hypothyroidism.

A 20-day-old male infant presented with acute renal failure. Three weeks later he developed acutely swollen, hot, red joints and tophi in his hands and feet. The serum uric acid was 2.2 mmol/l (normal 0.13-0.23 mmol/l) and the urinary oxypurine/creatinine ratio was 2.26 mmol (normal < 1.5 mmol). Complete deficiency of hypoxanthine guanine phosphoribosyl transferase (HGPRT) in intact erythrocytes confirmed Lesch-Nyhan syndrome. Neurological development was delayed and self-mutilation was observed at 22 months. Acute renal failure secondary to crystal nephropathy and tophaceous gout are unusual presenting features of this rare condition. This child also had transient neonatal hypothyroidism, which is not a recognized manifestation of the syndrome.

Acute Kidney Injury↗
PubMed1993

D-penicillamine and polymyositis: the significance of the anti-Jo-1 antibody.

A 57-yr-old lady developed polymyositis whilst taking D-penicillamine for RA. D-Penicillamine-induced polymyositis occurs in RA with a greater frequency than idiopathic polymyositis. Anti-acetyl choline receptor antibodies and ANA were positive, consistent with drug-induced disease. Anti-Jo-1 antibodies are considered specific for idiopathic myositis, and their presence was unexpected. Following withdrawal of the drug, the disappearance of the anti-Jo-1 and other antibodies coincident with clinical improvement, suggested that D-penicillamine was responsible for inducing antibody production.

Antibodies↗
PubMed1988

Outcome in juvenile arthritis.

Juvenile chronic arthritis has a number of subtypes with only seropositive juvenile rheumatoid arthritis and systemic juvenile chronic arthritis having equivalents in adult life. In 75% of patients the inflammatory disease has subsided by adulthood, leaving some with degenerative and mechanical problems. Systemic, polyarticular and pauciarticular subgroups, based on mode of presentation, have been related to prognosis. Seropositive polyarticular disease behaves as an aggressive form of adult rheumatoid arthritis. Standard methods of assessment are inappropriate in children. Active joint score is most useful. Radiographs are difficult to interpret because of growth and lack of early erosive disease. Growth and social outcome is important. Death occurs in 7% of cases and is due to infection and cardiac involvement during active systemic disease, and due to secondary amyloidosis later. Slow-acting drugs and surgical procedures may alter outcome. The aetiology of these diseases remains unknown and there is a need for diagnostic tests, particularly to identify those children who will do badly.

Adolescent↗
PubMed1987

Pneumococcal septic arthritis in rheumatoid arthritis.

Septic arthritis is associated with a definite morbidity which may be related to a delay in diagnosis and hence treatment. The cases of three patients with rheumatoid arthritis and chronic chest disease where the joint sepsis was not the predominant feature are presented. The responsible organism was Streptococcus pneumoniae which had spread after recent chest infections. Minimal joint symptoms or general malaise in association with an unexplained rise in erythrocyte sedimentation rate in these circumstances warrant a search for joint sepsis.

Aged↗
PubMed1986

Circulating human leucocyte elastase in rheumatoid arthritis.

Elastase-inhibitor complex (EIC) levels were determined in EDTA-plasma samples of 40 patients with connective tissue disease by a double antibody immuno-assay technique. In active rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), EIC levels were significantly higher than in the normal controls (P less than 10(-8) and fell on remission. The mean EIC level in active RA was significantly greater than in inactive disease (P = 0.0001) but there was no statistically significant difference between the EIC levels in the acute and inactive disease states in SLE (P = 0.49). In active RA, there was a positive correlation between EIC levels and white blood cell count (WBC) but not with erythrocyte sedimentation rate (ESR). In SLE there was no significant correlation between EIC levels and ESR or WBC. EIC measurement may be useful in the objective assessment of activity in RA.

Arthritis, Rheumatoid↗
PubMed1984

Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant.

A woman presented with multiple cerebral thromboses and skin lesions characteristic of Degos' disease. Her serum contained high titres of anticardiolipin antibodies and showed lupus anticoagulant activity, both known to be strong markers of a thrombotic tendency in systemic lupus erythematosus. This finding may have therapeutic implications for this usually fatal disease.

Adult↗