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Behcet syndrome: with immunologic evaluation.

A case of Behcet syndrome with immunologic evaluation, including screening of a vulvar ulcer for IgG, IgM, IgA, and fibrinogen by direct fluorescent microscopy is presented. Attempts were made to demonstrate cellular and humoral immune responses to mucosal antigens by lymphoblast transformation in the presence of cadaver esophageal mucosal extracts and indirect immunofluorescence using autologous serum and mucosal tissue. Serial measurements of percentages of total T, active T, and B lymphocyte populations, and lymphocyte response to phytohemagglutinin (PHA) stimulation during the course of Behcet syndrome are also presented. Clinical evaluation, histology of a Behcet vulvar ulcer, and a 2-year followup with good response to chlorambucil are reviewed.

Adult

Familial Behcet's syndrome.

Behcet's syndrome is reported in two families (one English and the other Egyptian). A mother and two of her sons of the English family and two brothers of the Egyptian family are described. The contradictory opinions concerning the incidence of the familial occurrence of this syndrome are partly due to uncertainty about the nosologic relationship of recurrent aphthal and Behcet's syndrome. The question of whether this syndrome should be included in a large group of aphthoses or be regarded as a true clinical entity is discussed.

Adolescent

A case of vasculo-Behcet's syndrome.

A case of Behcet's syndrome with vascular complications is presented. This case had a familial occurrence of Behcet's syndrome. His vascular complications were deep vein thrombophlebitis of both legs, aneurysms of the left common iliac artery and the left femoral artery and the occlusion of the left subclavian artery. He was successfully operated on for the aneurysms. This paper discusses the problems accompanying the aneurysm of Behcet's syndrome. The authors pointed out that the subclavian steal syndrome may be diagnosed as neuro-Behet's syndrome and stressed the importance of vascular survay in Behcet's syndrome when the patient complaints of thrombophlebitis.

Aneurysm

Platelet function, blood coagulation and fibrinolysis in Behcet's syndrome.

In 10 patients with Behcet's syndrome, various parameters of platelet function, blood coagulation and fibrinolysis were studied. With varying frequency the following abnormalities were found: increased retention of platelets in glass bead column, reduced platelet aggregation to low concentrations of adenosine diphosphate, elevated plasma levels of fibrinogen concentration and factor VIII activity, increased plasma antiheparin activity and impairment of fibrinolytic activity. The above abnormalities were found long after the last thrombotic episode and were more frequent in patients with a history of thrombophlebitis. It is suggested that certain hemostatic abnormalities accompany or form part of Behcet's syndrome and that they are related to the thrombotic complications characteristic of this syndrome.

Adenosine Diphosphate

A family study of Behcet's syndrome.

The first-degree family members of patients with Behcet's syndrome were investigated to determine (1) whether there was a familial aggregation of Behcet's syndrome or of its component features, (2) whether there was an increased incidence of sacro-iliitis in these families, and (3) whether a link exists between Behcet's syndrome and spondarthritides in first-degree relatives. Of the 226 relatives and patients seen, 21 patients with definite Behcet's syndrome and their 71 relatives were analysed in some detail. Orogenital ulceration was found in 2 mothers of probands; 1 patient had psoriasis and Behcet's but neither disease featured in the pedigree. Another family showed numerous manifestations of psoriasis and psoriatic arthritis, 1 member having severe sacro-iliitis but the proband had Behcet's syndrome only. Family trees including HLA pedigrees, where known, are presented. Spouses suffered few of the complaints found in patients, and to a less extent in relatives, but showed a high incidence of backache, perhaps of psychogenic origen.

Adult

Ulcerative esophagitis and colitis in a pediatric pantient with Behcet's syndrome. Response to steroid therapy.

The gastrointestinal manifestations of Behcet's syndrome including colitis and esophageal ulceration have been recognized in recent years. In the present case, a girl with Behcet's syndrome was followed since age 11 for oral, vaginal, perianal ulcers and neurological involvement. At age 16 she developed esophageal ulceration and colitis, was treated with steroids and improved. Endoscopic and radiographic findings are presented, previously reported cases are reviewed and treatment is discussed.

Adolescent

Obstructive lung disease in Behcet's syndrome.

Obstructive lung disease in two patients with Behcet's syndrome is presented. In both cases increased airways resistance, impaired PEF and FEV1.0 were found. In addition, diffusion capacity was slightly decreased in spite of enlarged total lung capacity and increased residual volumes. Both patients had mild hypoxaemia despite slight hyperventilation; a normal chest X-ray, and a decreased serum complement C3 level. Corticosteroids had a beneficial effect on VC in one of the two cases but failed to have any significant effect on the impaired ventilatory function in the other.

Adrenal Cortex Hormones

Synovial pathology in Behcet's syndrome.

Eight specimens of synovial membrane from 6 patients with 'definite' Behcet's syndrome were available for histological examination. Only the superficial zones of the synovia were affected, all except one being replaced by dense inflamed granulation tissue composed of lymphocytes mingled with macrophages, vascular elements, fibroblasts, and neutrophils. There was a marked plasma cell infiltrate and lymphoid follicle formation in one synovium only, and there was no evidence of infection. Pannus and erosive change were present in the three specimens which included the articular surface, the erosive change being visualised radiologically in two of these. It is suggested that these appearances are characteristic of Behcet's syndrome and should be added to the list of diagnostic criteria.

Adult

Acute phase proteins and C9 in patients with Behcet's syndrome and aphthous ulcers.

Estimation of the concentration of C9, C-reactive protein (CRP) and alpha1-antitrypsin in forty sera from patients with Behcet's syndrome and recurrent oral ulcers showed significantly increased amounts of C9 and CRP in Behcet's syndrome. The concentration of C9 was also significantly raised in recurrent oral ulceration, though to a lesser extent than in Behcet's syndrome. The assay C9 and CRP might be useful in the differential diagnosis of Behcet's syndrome, especially from recurrent oral ulcers. It is suggested that during epithelial inflammation in recurrent oral ulcers some of the acute phase proteins are increased and in some patients these may modulate the immunological mechanism in such a way as to induce a transition from focal oral ulceration to the multifocal Behcet's syndrome.

Behcet Syndrome

Damaged membrane fragments and immune complexes in the blood of patients with Behcet's syndrome.

Electron microscopic examination of centrifuged pellets of serum from patients with Behcet's syndrome and recurrent oral ulcers revealed the presence of a large number of membrane fragments. Some of these membranes showed numerous 10 nm holes that were identical to lesions produced by the action of complement. An attempt was made to correlate complement levels, antibodies and cellular immunity with the presence of the membrane fragments, without success. However, a significant correlation was found between the membranes and the IgG class of immune complexes. The finding of membrane fragments with complement-induced damage predominantly in the blood of patients with Behcet's syndrome, and the association with soluble immune complexes suggest that the latter may generate C5b-9 complexes which may bind to the surface of cells in result in cell lysis.

Adult