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Biomedical subjects

V Hamuryudan

Publications and source records attributed to V Hamuryudan.

At least 37 records · Page 2Linked to original sources
PubMed1998

Genetic anticipation in Behçet's syndrome.

OBJECTIVE: To examine the presence of genetic anticipation in families with Behçet's syndrome (BS). METHODS: A total of 18 families with 40 affected members in two successive generations were evaluated by interviewing them for their ages at the onset of the first symptom of BS and for their ages at the time they fulfilled the diagnostic criteria. RESULTS: It was noted that the age of onset of the first symptom was lower in the second generation in 14 families (p = 0.01) with a mean (SD) age of 20.57 (7.47) years in the children compared with 33.29 (9.92) years in the parents (t = 7.79, p < 0.0001), whereas the diagnostic criteria were fulfilled at an earlier age in the children in 15 families (p = 0.01) with a mean age of 21.2 (6.74) years in the children compared with 36.4 (9.55) years in the parents (t = 7.41, p < 0.0001). CONCLUSION: Genetic anticipation was present in 15 of 18 (84%) of the families with BS in the form of earlier disease onset in the children compared with their parents.

Adolescent↗
PubMed1998

Fibromyalgia in Behçet's syndrome.

OBJECTIVE: To ascertain the frequency of fibromyalgia (FM) in Behçet's syndrome (BS) and to evaluate the relationship of FM to Behçet's disease activity. METHOD: Self-questionnaires were completed by 108 patients with BS. Each patient was evaluated by an observer blinded to diagnosis; evaluation included assessment of tender points by palpation. Another observer determined the disease activity of patients at that time. RESULTS: Ten of 108 patients (9.2%) met the American College of Rheumatology criteria for FM. Nine of the patients who met the criteria for FM were women. In contrast to patients without FM, patients with FM had mild to moderate disease activity in which musculoskeletal complaints were common. CONCLUSION: There is a trend for an increased frequency of FM in female BS patients.

Adult↗
PubMed1997

Azathioprine in Behcet's syndrome: effects on long-term prognosis.

OBJECTIVE: To assess the effect of azathioprine (AZA) treatment on long-term prognosis in Behçet's syndrome. METHODS: Patients (all male) who took part in a double-blind, placebo-controlled trial of AZA a mean +/- SD of 94 +/- 10 months previously were reevaluated. RESULTS: The emergence of blindness (log rank chi2 = 5.6, P = 0.02) and a 2-line drop in the visual acuity of the right eye (log rank chi2 = 5.9, P = 0.015) occurred significantly more frequently among the patients originally allocated to the placebo group compared with patients who originally received AZA, despite posttrial treatment for patients in both groups when needed. There was also a trend toward more frequent occurrence of extraocular complications in the placebo group. The beneficial effect of AZA was especially pronounced among patients who had eye involvement of short duration prior to their entry into the trial. CONCLUSION: Early treatment with AZA tends to favorably affect the long-term prognosis of Behçet's syndrome.

Adult↗
PubMed1997

Management of aneurysms in Behçet's syndrome: an analysis of 24 patients.

BACKGROUND: The surgical therapy of Behçet aneurysms is often unsuccessful, resulting in graft occlusions, anastomoses, and/or new aneurysms. METHODS: Twenty-nine aneurysms were documented in 24 Behçet's patients during a period of 19 years. All patients were male, ranging in age from 20 to 53 years (mean, 35 +/- 7.3 years). The mean duration of disease was 9 +/- 5 years. There were nine abdominal aorta, four iliac, three common femoral, five superficial femoral, four popliteal, one subclavian, one carotid, and one posterior tibial artery aneurysm. In addition, in one patient an aneurysm developed from the arterialized venous conduit that had been inserted for a common femoral artery aneurysm elsewhere. Five patients were already under immunosuppressive therapy for ocular problems at the time of diagnosis. Fifteen patients received immunosuppressive therapy after operation. We performed one abdominal aneurysmorrhaphy, two iliac artery PTFE graft interpositions, two aortobiliac bypasses (PTFE), six aortic tube graft (three PTFE, three Dacron) interpositions, one avrtofemoral bypass (PTFE), two iliofemoral bypasses (PTFE), two superficial femoral artery graft (PTFE) interpositions, and three popliteal graft interpositions (one PTFE, two vein graft). Also as an initial procedure one carotid, one subclavian, four superficial femoral, one popliteal, and one posterior tibial artery were ligated. RESULTS: Nineteen patients were followed up for a mean duration of 47.3 +/- 27 months (range, 1 to 108 months). The patient with a subclavian aneurysm died of massive bleeding on postoperative day 15. Four patients were lost to follow-up. In the abdominal aortic aneurysm group one patient died of gastrointestinal bleeding 4 years after the operation. Another patient from the same group died 5 years after operation without any vascular disease. In the common femoral artery group the patient with an occluded iliofemoral graft died of an exsanguinating pulmonary artery aneurysm in the first year after operation. Overall, there were five anastomotic aneurysms. In addition, after the initial operation two iliofemoral, one aortofemoral, and one popliteal interposition graft were occluded without disabling ischemia. CONCLUSIONS: Aneurysms limited to the extremities could be ligated without disabling ischemia. Abdominal aortic aneurysms could be treated with tube graft insertion, giving satisfactory results. Patients could tolerate graft occlusion without major ischemia.

Adult↗
PubMed1997

Other forms of vasculitis and pseudovasculitis.

Behçet's syndrome can involve all sizes and kinds of blood vessels. There is an association between arterial involvement and venous thrombosis. Pulmonary arterial aneurysms and neurological involvement have a definite influence on mortality. Male sex and young age are indicators of a more severe disease course. Immunosuppressive treatment early in the disease may affect the long term prognosis favourably. Patients with familial Mediterranean fever may develop manifestations of vasculitis. The most common associations are with Schönlein-Henoch purpura and polyarteritis nodosa. In some patients the diagnosis of vasculitis precedes that of familial Mediterranean fever. Kawasaki disease, although rare, can be seen in adults. The coronary sequela of childhood disease can affect the prognosis later in life. Many conditions, like myxoma, cholesterol embolism, calciphylaxis may mimic vasculitic syndromes. These conditions should always be kept in mind because their pathophysiology and treatment are different from true vasculitides.

Animals↗
PubMed1996

The frequency and clinical characteristics of methotrexate (MTX) oral toxicity in rheumatoid arthritis (RA): a masked and controlled study.

To assess the frequency and clinical characteristics of methotrexate (MTX) oral toxicity in rheumatoid arthritis (RA) patients, 51 RA patients receiving MTX and 46 RA patients not receiving MTX were studied. A questionnaire, the credibility of which was tested on four separate patient groups including a group with Behcet's Syndrome, was used as a tool to determine the prevalence of stomatitis by a blind observer. In this first controlled study of the oral toxicity of MTX, prevalence of stomatitis was found in 37.2% in the group taking MTX and 19.5% in the group not taking MTX (P = 0.054). No statistical differences were detected with respect to number, duration, frequency, and site of stomatitis. Two of the 51 MTX taking patients temporarily reduced their MTX dosage and only one patient temporarily terminated MTX treatment. MTX and toxicity is usually of no major clinical concern in the treatment of RA.

Administration, Oral↗
PubMed1996

Calciphylaxis: a condition mimicking necrotizing vasculitis.

A patient with end stage renal disease developed ischaemic skin necrosis and digital gangrene. He had diffuse arterial calcification associated with hyperparathyroidism secondary to renal failure. The patient received inappropriate cyclophosphamide therapy as he had been misdiagnosed as having an inflammatory vasculitis. This clinical picture, previously named "calciphylaxis" should come into the differential diagnosis of systemic vasculitis in a uraemic patient with hyperparathyroidism.

Arterioles↗
PubMed1996

Surgical therapy of pulmonary arterial aneurysms in Behçet's syndrome.

Two patients with Behçet's syndrome were operated for exsanguinating pulmonary artery aneurysm into a bronchus. Lower lobectomy was performed in the first patient. He died of hypoxia and sepsis on the 8th postoperative day. Pneumonectomy was undertaken in the second patient, who is well 30 months after the operation.

Adult↗
PubMed1996

The ten-year mortality in Behçet's syndrome.

We surveyed the 10-yr mortality among 152 Behçet's syndrome (BS) patients who had registered at a BS out-patient clinic and compared it to the expected mortality in the general population. Information on mortality was available in 79% of the study group, among whom six patients (all males) had died. The observed mortality of two patients in the 15-24 yr age bracket was significantly above that expected in the general population. BS is a cause of increased mortality in the young male patients

Adolescent↗
PubMed1996

Monocyte activity in Behçet's disease.

Monocytes obtained from patients with Behçet's disease (BD) were examined for differentiation markers (expression of CD14 and antigens reacting with monoclonal antibodies 25F9 and G16/1) and for expression of selected adhesion molecules. There was significantly raised expression of the CD14 molecule, and increased staining with 25F9 and G16/1 antibodies in monocytes obtained from patients with BD. A monocyte activation marker, soluble CD14, was also found to be raised in patients' serum compared with normal serum (8.1 +/- 9.2 vs 1.4 +/- 0.7 micrograms/ml). Furthermore, BD patients' monocyte culture supernatants caused significantly increased adhesion of normal neutrophils to endothelial cell monolayers in vitro. All these findings show that BD patients' monocytes are active in vivo and produce a number of pro-inflammatory cytokines which may play a role in the chronic inflammation found in these patients.

Behcet Syndrome↗
PubMed1996

Gastrointestinal involvement in Behçet's syndrome: a controlled study.

OBJECTIVE: To make a retrospective and prospective analysis of the frequency of symptomatic inflammatory bowel disease in patients with Behçet's syndrome (BS). METHODS: The medical records of the first 1000 patients with BS were reviewed retrospectively for past or present history of diarrhoea. The past and present history of diarrhoea was also elicited prospectively among 147 consecutive patients with BS and 78 diseased controls (42 with rheumatoid arthritis, 17 with systemic lupus erythematosus, seven with seronegative spondylarthropathy, and 12 with miscellaneous rheumatic diseases). Inflammatory mucosal changes were sought in rectal biopsy specimens from 75 patients with BS, 47 diseased controls (29 with nephrotic syndrome, eight with rheumatoid arthritis, six with familial Mediterranean fever, and four with ankylosing spondylitis), and 14 patients with ulcerative colitis. RESULTS: In chart review there were only seven Behçet's patients with diarrhoea; none of them had inflammatory bowel disease. In the prospective survey there were no significant differences between the BS and control groups in the past and present history of diarrhoea. There were no significant differences in the rectal mucosal histology between patients with BS and controls, while patients with ulcerative colitis showed pronounced differences. CONCLUSION: Symptomatic inflammatory bowel disease is not common in BS patients from Turkey.

Adult↗
PubMed1995

Anti-endothelial cell antibodies, endothelial proliferation and von Willebrand factor antigen in Behçet's disease.

Endothelial cell dysfunction has previously been demonstrated in Behçet's disease which has vasculitic features. In this study we investigated anti-endothelial cell antibodies (AECA) and von Willebrand factor antigen (vWF) levels in patients with Behçet's disease. In vitro effects of patient sera on endothelial cell proliferation were also evaluated. AECAs were present in 29% of 70 Behçet's disease patients (Binding Index: 25 +/- 29% vs 9 +/- 7% in normal controls, p < 0.005). 95% of AECA positive patients were clinically active compared to 74% of AECA negative patients (p = 0.04). Among specific organ manifestations only active arthritis correlated with AECA positivity (6 of 7 patients vs 14 of 63, p = 0.002). AECA positive patients had a significantly higher mean ESR (37 +/- 24 mm/h vs 21 +/- 17 mm/h, p:0.006). Mean vWF levels were also significantly higher in patients compared to controls (166 +/- 75% vs 84 +/- 34%, p < 0.0001). No correlations were observed between AECA titres and vWF levels. No significant differences were observed between patients and controls when endothelial cell proliferation was studied (Proliferation Index: 1.25 +/- 0.28 vs 1.12 +/- 0.25, p = 0.5). Our results suggest that AECA may be related to disease activity in Behçet's disease. The presence of vWF, even in patients in complete remission, might be related to factors other than endothelial damage for vWF release from endothelial cells.

Adolescent↗
PubMed1994

Pulmonary arterial aneurysms in Behçet's syndrome: a report of 24 cases.

Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 +/- 11 S.D. yr (range 17-59 yr). Their mean disease duration was 5 +/- 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 +/- 11 S.D. months (range 1-36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 +/- 24 S.D. months (range 1-78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment.

Adolescent↗