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Biomedical subjects

D G James

Publications and source records attributed to D G James.

At least 145 records · Page 8Linked to original sources

Bone sarcoidosis.

Sarcoidosis of the bone has been observed in 24 patients for up to 43 yr. It was always present in hands and/or feet and also elsewhere in 4 other patients. Soft-tissue swelling preceded the radiological abnormality for up to 4 yr in 9 patients accompanied it once, followed it once, and was absent in 13 (55%). Bone involvement was usually an incidental finding when sarcoidosis presented elsewhere, including lupus pernio (50%) and other chronic skin lesions (41%), lungs (75%), eyes (51%), lymph nodes (21%), liver (17%), spleen (13%), parotids (13%), facial palsy in 2 patients and erythema nodosum in 1. Pulmonary infiltration, with or without lymphadenopathy, was evident in three-fifths of the patients and hilar adenopathy alone in one-fifth. Sarcoid tissue was observed in the bone, but not in the surrounding soft-tissue swelling of 2 amputated fingers. There were 3 types of bone lesions: a) Lytic in 20 patients; minute 1-mm cortical defects or larger, usually rounded defects that on healing become corticated leading to punched-out "cysts". b) Permeative in 8 patients; these showed progressive cortical tunnelling with remodelling of trabecular and cortical architecture. c) Destructive in 3 patients, with secondary joint-surface involvement and in one a periosteal reaction.

Adult↗

Scleritis and temporal arteritis.

Thirty consecutive patients with severe scleritis or episcleritis were admitted as in-patients to the Medical Ophthalmology Unit and assessed for systemic disease. There were seventeen women and thirteen men. The mean age was 53 with a median of 57 (range 23-83). Eighteen of the patients had scleritis: eleven of these had evidence of connective tissue disease and three of them had temporal arteritis. Twelve patients had episcleritis: six of them had a collagen disease and one of them developed temporal arteritis. This high incidence of temporal arteritis in association with scleritis has not been previously reported. It is important to diagnose and treat overt temporal arteritis early with parenteral steroids so that ischaemic papillopathy can be avoided. A higher incidence of collagen diseases than previously described is reported in episcleritis. It is thought that this is secondary to selection since patients with the usual self-limiting episcleritis are not normally referred for further in-patient investigation. In no patient was more than one significant diagnosis made. There was no significant medical illness in only 11% of patients with scleritis and 33% of patients with episcleritis. The majority of the non-collagen diseases (e.g. hypertension) were not previously recognized. In none of the patients with temporal arteritis was the diagnosis made before admission. It is concluded that full examination and investigation for underlying disease is indicated in both scleritis and severe episcleritis.

Aged↗

Sarcoidosis of the upper respiratory tract and its association with lupus pernio.

In a series of 34 patients with sarcoidosis affecting the upper respiratory tract and nose, 26 had lupus pernio (LP) and 17 had sarcoidosis of the upper respiratory tract (SURT). In nine patients these features coexisted. A patient presenting with SURT carried a 50% risk of developing LP although one feature could be present without the other. Both were disorders of women of the child-bearing years of life. SURT, like LP, was an indicator of chronic fibrotic sarcoidosis, developing insidiously and progressing indolently over the years. It was complicated by ulceration, septal perforation, and LP. Three patients had nasal septal perforations, in two instances following submucous resection. This operation is contraindicated in patients with active sarcoidosis, particularly when granulomas are found on nasal biopsy. The Kveim-Siltzbach skin test was positive in all patients with SURT, making it invaluable in the differential diagnosis of granuloma of the nasal cavity.

Adolescent↗

Ocular sarcoidosis.

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Adrenal Cortex Hormones↗

Uveitis. A series of 368 patients.

During the first 10 years (1963-74) of a new Medical Ophthalmology Unit, 368 of 3,930 admissions had endogenous uveitis, anterior in 215 (58 per cent.), posterior in 65 (18 per cent.), and generalized in 88 (24 per cent.). Acute uveitis in 153 patients tended to have a short course counted in months, whereas in 215 with chronic uveitis the condition was measured over years, punctuated by relapses. Sex distribution was equal. At the time of initial presentation, 60 per cent. of patients were aged 20 to 40 years, 8 per cent. were under 20, and 10 per cent. were over 60 years of age.

Adolescent↗

Virus infections.

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Adrenal Cortex Hormones↗

Immunology of sarcoidosis.

The cardinal immunologic changes in sarcoidosis consist of depression of delayed-type hypersensitivity, hyperreactive circulating antibody responses and the Kveim-Siltzbach skin test phenomenon. Depression of delayed-type hypersensitivity is demonstrated by skin tests using tuberculin, mumps, pertussis, trichophytin, oidiomycin, dinitrochlorobenzene and Californian keyhole limpet hemocyanin. The cultured lymphocytes from patients with depression of delayed-type hypersensitivity react poorly to phytohemagglutinin, and there is a close correlation between anergy of lymphocytes in culture and by cutaneous anergy. In vivo cutaneous anergy mirrors in vitro cellular hyporeactivity. Other technics used to expose immunologic defects in peripheral lymphocytes of patients with sarcoidosis include tests of T and B cell function, rosetie formation and migration inhibition. Whereas there is cutaneous anergy and impaired cellular immunity in patients with sarcoidosis, the reverse holds for circulating factors. There are increased circulating immunoglobulin levels, increased circulating antibody levels to Epstein-Barr, herpes simplex, rubella, measles and parainfluenza viruses, increase antibody response to mismatched blood and occasional false-positive Wassermann reactions, but there is no increase in circulating autoan tibodies. There is no evidence that patients with sarcoidosis belong predominantly to any particular histocompatibility locus. Worldwide figures for the Kveim-Siltzbach skin test are presented. They provide evidence of its specificity in various international series. The causes of nonspecific reactions are discussed.

Antibodies, Viral↗

Neuro-ophthalmic sarcoidosis.

Sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. Fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.

Adult↗