[HLA-B27 inflammatory spondylarthropathy, psoriasis and HIV infection].
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Biomedical subjects
Publications and source records attributed to M Dorra.
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Four cases of histologically proven temporal arteritis presenting with atypical clinical, anatomical or evolutive features and raising nosological problems concerning the type of vasculitis involved are presented. In patients with temporal arteritis, some elements are suggestive of periarteritis nodosa. They include peripheral neurological lesions, renal or pleuro-pulmonary lesions and the histological appearance of the temporal artery. Diagnosing periarteritis nodosa in a case of temporal arteritis leads to a special therapeutic strategy.
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A case of multiple intracranial arterial aneurysms occurring in a 43 year-old patient with an 8-year history of Behcet's disease is reported. The diagnosis was based upon the past occurrence of polyarthritis, oral and scrotal aphthous ulcers and cutaneous hypersensitivity. There was a three year remission after a brief treatment with corticosteroids. At the end of this period, a left hemiplegia occurred. CT scan showed an infarction in the right middle cerebral artery territory. Angiography showed fusiform aneurysms of the intracranial right internal carotid, right anterior and middle cerebral arteries. The draining veins and sinuses were normal. Careful angiographic examination of visceral and peripheral arteries showed only bilateral retinal artery aneurysms. Large artery involvement is a rare complication of Behcet's disease, and a thorough review of the literature showed only one reported case of an intracranial aneurysm.
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A 53-year-old man with hepatitis concurrent with cirrhosis was simultaneously positive for hepatitis B surface antigen and heterotypic anti HBs antibody. This could be explained by a state of tolerance, with chronic carrying of B virus (subtype a y w3), but would not preclude B virus (subtype d) reinfection, inducing the synthesis of specific anti-d antibodies. In the same patient, the very high level of 3460 ng/ml was reached for alpha-fetoprotein; this was transitory and returned to normal within 8 months; it was probably due to the acute hepatitis. Thus it appears that, even in cases of cirrhosis, a major rise in this marker does not absolutely imply the presence of hepatocellular carcinoma.
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The diagnosis of temporal arteritis is made after a mean delay of four months. This delay is explained by the absence, in 50% of cases, of any clinical sign in the temporal area, the possible presence of alterations in liver function tests, and the frequency of anemia and loss of weight. These signs often suggest a mistaken diagnosis of hepatobiliary disease or cancer. The authors evaluate the cost of this delay in a personal series of 33 cases confirmed histologically. They recommend biopsy of the temporal artery in each patient aged more than 70 with a marked rise in erythrocyte sedimentation rate unexplained by an infectious disease or dysglobulinemia.
Temporal arteritis is still often overlooked in 1982 as shown by the diagnostic delay reported in a retrospective review of 33 patients and in the series previously published in the medical literature. Clinical features to be especially kept in mind are the occasional nature of temporal signs, the pathognomonic value of jaw claudication and the existence of signs which may constitute a premonitory syndrome of blindness. The frequent rise in alkaline phosphatase levels is often misleading. In patients over 65 with an unexplained inflammatory syndrome, biopsy of both temporal arteries should be promptly performed, prior to the unpleasant and costly investigations designed to detect an occult carcinoma. Corticosteroids are still the best treatment of giant cell arteritis but the optimal initial dosage remains unknown as no data from randomized trials are available. Symptomatic relapses have been reported up to 10 years (5 years for ocular symptoms) after onset of the disease. Because of this protracted course, which makes adequate follow-up difficult to ensure, recovery rates from different series are at variance. The main side-effects of maintenance corticosteroid therapy in these elderly patients are osteoporosis, which warrants systematic countermeasures, and probably an increased rate of atheromatosis.
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Two patients with aortic stenosis had recurrent digestive haemorrhages. Fiberoscopy showed the presence of angiodysplasia of the stomach in one patient and of the colon in the other. This association had been reported in subjects older than 60. It does not seem to be fortuitous. The authors discuss the theoretical and practical implications, including the need for full radiological and endoscopic exploration of patients with recurrent digestive haemorrhages, particularly when associated with aortic stenosis.