Biomedical subjects
Publications and source records attributed to R G Gray.
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A young Black woman with mixed connective tissue disease (MCTD) developed an aseptic meningitis after receiving ibuprofen. The meningeal reaction, reported infrequently in systemic lupus erythematosus (SLE) and only once previously in MCTD, was characterized by a predominantly polymorphonuclear cerebrospinal fluid (CSF) pleocytosis and depression of CSF glucose. Reversible renal insufficiency also occurred. Features suggestive of a hypersensitivity reaction included pruritus, conjunctivitis, facial oedema, desquamation of the palms and soles, and subsequent near total alopecia. Meningeal signs responded rapidly to systemic corticosteroid therapy. Patients with MCTD as well as those with SLE may be at peculiar risk of developing this uncommon reaction to ibuprofen.
This study evaluated the influence of a family history of coronary heart disease (CHD) and CHD risk factors on the total serum cholesterol/high density lipoprotein (TChol/HDL) and the low density lipoprotein/HDL (LDL/HDL) ratios of 84 black children (ages 7-11 years) and adolescents (ages 12-15 years). Fasting lipid determinations were measured on all subjects. A questionnaire was administered to each subject's parent to determine the frequency of myocardial infarction, heart disease, hypertension, stroke, atherosclerosis, diabetes, and obesity in first- and second-degree blood relatives. Based on three-way analysis of variance tests, significant stroke and age, diabetes and age, and obesity and age interactions in TChol/HDL and LDL/HDL were found. There was also an obesity and age and sex interaction effect on T.Chol/HDL. The results indicated that a family history of stroke, diabetes, or obesity can have an unfavorable effect on the TChol/HDL and LDL/HDL ratios in black adolescents.
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Affected twins with acute hereditary tyrosinaemia type I are described. Attempts at therapy with a phenylalanine-tyrosine-methionine restricted diet supplemented with cysteine, vitamin E and ascorbic acid failed to influence the course of the disorder. The bleeding diathesis was due to a morbid reduction of a number of clotting factors, particularly factor VII, and this was associated with impaired platelet aggregation and release. The liver of one showed a marked reduction in fumarylacetoacetate lyase activity and her urine contained a potent inhibitor of red cell delta-aminolaevulinic acid dehydratase. Biochemical investigations of cultured fibroblasts suggest that these do not express the disorder and are unlikely to prove useful diagnostically.
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A new case of alpha-aminoadipic aciduria had an apparent immunodeficiency and died at the age of 4 months. The urine contained large amounts of alpha-aminoadipate and smaller quantities of alpha-keto- and alpha-hydroxyadipate. Post mortem, the highest concentrations of alpha-aminoadipate were found in liver and kidney. Enzymatic studies on liver and cultured fibroblasts failed to demonstrate the expected deficiency of alpha-amino-adipate aminotransferase, a result perhaps explicable by the presence of cytoplasmic aminotransferase activity.
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We identified bilateral carpal tunnel syndrome in 19 of 43 living persons of a nonconsanguineous family. No single common etiologic feature was seen. Sixty-three percent of the afflicted kindred had symptomatic digital flexor tenosynovitis. Noninflammatory thickening of the flexor retinaculum or tendon sheaths, or both, was the commonest surgical finding. The 44% prevalence, early age of onset, and equal sex ratio differ from idiopathic carpal tunnel syndrome. Family pedigree suggests an inheritable disorder transmitted by an autosomal dominant gene with a high degree of penetrance.
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Telescoped digits of the hands and feet developed in a 69-year-old male with severe chronic tophaceous gout during allopurinol treatment. Extensive osseous tophi, resorbed rapidly during therapy with this xanthine oxidase inhibitor and not replaced by new bone matrix, were responsible for the deformity. Several hypotheses are advanced to explain the failure of bone erosions to heal.
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Tenosynovitis of one or more flexor tendons of the hand (mean 3.1 tendons per patient) was noted in 55% of 100 patients with rheumatoid arthritis (RA) examined periodically during a mean period of 5 years. The third flexor tendon was involved most frequently (71% of patients), followed by the second (62%), fourth (53%), fifth (27%), and first (13%). Patients with flexor tendonitis (FT) had a significantly higher prevalence of rheumatoid nodules (56% vs 33%), carpal tunnel syndrome (47% vs 13%), wrist extensor tenosynovitis (47% vs 9%), and elbow epicondylitis (22% vs 7%) than patients without FT. Dupuytren's contracture, DeQuervain's tenovaginitis, flexor carpi radialis and ulnaris tendonitis, and Achilles tendonitis were found exclusively in patients with FT. A control group of 50 non-RA patients with FT had statistically fewer diseased tendons per patient (mean 1.5) and a different digital distribution, the thumb being affected more frequently (P less than 0.05) than in RA patients.
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The activities of the urea cycle enzymes in the liver of a female patient with hyperammonemia were determined (Table 1). Ornithine transcarbamylase (OTC, EC. 2.1.3.3) was reduced to 5-10% of normal and the residual enzyme showed an apparent Kmorn of 0.69 (normal 0.37 +/- 0.10) mmol liter. The pH dependence was normal. The patient's mother also showed hyperammonemia but was not clinically affected. Consideration of the genetics of the disease suggested that many female patients should have a mixture of normal and mutant enzymes. Electrophoresis of the patient's liver extract showed an additional band of OTC activity probably due to this mutant enzyme. The ratio of plasma glutamate-pyruvate transaminase to OTC was abnormal in four clinically affected patients with OTC deficiency (Fig. 4B) but not in two of their mothers without clinical signs.
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