Are erythema nodosum‐like lesions and superficial thrombophlebitis prodromal in terms of visceral involvement in Behcet's disease?

Erythema nodosum (EN)-like lesions and superficial thrombophlebitis (ST) are skin symptoms that are frequently observed in Behcet's disease. In most of the patients, skin symptoms precede serious visceral involvement. The study included 78 patients whose mean age was 33.48 +/- 6.16 (21-48). Of the cases, 37 were females and 41 were males. Forty-two patients (53.8%) had EN-like lesion and 12 (15.3%) had ST. Six of 54 patients, who had EN-like lesion and ST, were found to have no visceral involvement. In six of the remaining 48 patients, it was established that visceral involvement developed before lesions. It was found in 42 patients that these two lesions preceded visceral involvement. In addition, 18 of 24 patients who did not have these two lesions, do not still have visceral involvement. Therefore, we think that EN-like lesion and ST can be guiding in predicting serious visceral involvement and complications that can develop in advanced stages of Behcet's disease.     

单核细胞/高密度脂蛋白胆固醇比值与白塞病疾病活动性的相关性

目的 研究单核细胞/高密度脂蛋白胆固醇比值(MHR)与白塞病疾病活动性的相关性,进一步探讨其作为评估白塞病活动性的潜在标志物的可能性。方法 选取2019年1月至2020年12月青海大学附属医院风湿免疫科白塞病初治住院患者43例为病例组,体检中心43例健康者为对照组,根据电子病历疾病活动性指数(EMRAI)将病例组分为高活动性组和低活动性组,比较病例组和对照组的C反应蛋白(CRP)、红细胞沉降率(ESR)和MHR水平,分析MHR与CRP、ESR的相关性,采用ROC曲线分析MHR对白塞病疾病活动性度的判断效能。结果 病例组的CRP、ESR及MHR水平均显著高于对照组,差异有统计学意义(P<0.01)。高疾病活动性组的MHR水平高于低疾病活动性组[(0.64±0.28)×10⁹/mmol vs (0.36±0.20)×10⁹/mmol,P<0.01]。Spearman相关分析表明,MHR与EMRAI、ESR、CRP分别呈正相关关系(r=0.504、0.311、0.555,P<0.01)。ROC曲线分析显示,MHR区分低疾病活动性与...     

Assessment of homocysteine, neopterin and nitric oxide levels in Beh?et's disease.

BACKGROUND: Beh?et's disease is a multisystemic immunoinflammatory disease with a wide variety of clinical manifestations, whereas recurrent aphthous stomatitis is a local oral disease. The aim of this study was to examine the distribution of homocysteine levels in patients with active Beh?et's disease, possible association of homocysteine with nitric oxide and neopterin levels, and to characterize the differences between patients with Beh?et's disease and those with recurrent aphthous stomatitis in terms of these parameters compared with healthy controls. METHODS: A total of 23 patients with active Beh?et's disease, 25 patients with recurrent aphthous stomatitis as positive controls, and 21 healthy subjects were included in this study. Serum homocysteine and neopterin levels were measured flourimetrically by HPLC. Serum nitric oxide production was assayed by measuring total nitrite levels with Griess reagent. RESULTS: Significantly higher homocysteine (12.9+/-3.3 micromol/L) and lower nitric oxide (41.5+/-10.9 micromol/L) and neopterin (6.4+/-1.0 nmol/L) levels were observed in patients with Beh?et's disease compared with healthy controls (10.7+/-2.0 micromol/L, 49.7+/-16.2 micromol/L, 8.7+/-2.2 nmol/L, respectively) (p<0.03 for neopterin, p<0.04 for homocysteine and nitric oxide). However, homocysteine, nitric oxide, biopterin and neopterin levels and the neopterin/biopterin ratio for recurrent aphthous stomatitis patients were not significantly different compared to healthy controls. A significant positive correlation was observed between serum homocysteine and serum neopterin/biopterin ratio in patients with Beh?et's disease (r=0.975, p<0.005). CONCLUSIONS: In contrast to recurrent aphthous stomatitis, there is a higher prevalence of hyperhomocysteinemia in Behcet's disease. Homocysteine may have deleterious effects on the pathology of Behcet's disease by decreasing nitric oxide levels and interfering with the immune system.     

Cognitive event-related potential and neuropsychological findings in Behcet's disease without neurological manifestations

Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers and uveitis. BD patients without neurological involvement frequently have mild neurological symptoms. The aim of this study was to evaluate whether BD patients without neurological involvement have any changes in cognitive functions. Twenty BD patients without neurological involvement and 13 control subjects were included in the study and were analyzed by neurophysiological and neuropsychological examinations. The cognitive event-related potentials (P300) were recorded from the frontal, central and parietal areas of the right and left hemispheres of the patients and control subjects. Likewise, all individuals were evaluated with neuropsychological tests. In contrast to a study with similar design, we did not find any difference between the cognitive event-related potentials values of BD patients without neurological involvement and the control subjects. All BD patients without neurological involvement exhibit normal results of the neuropsychological test. In conclusion, the results of neuropsychological tests and cognitive event-related potentials values in BD patients without neurological involvement are indistinguishable from those in control subjects and no alteration of cognitive functions is present.     

Decreased total antioxidant response and increased oxidative stress in Behcet's disease

Behcet's disease (BD) is a chronic systemic inflammatory disease. Inflammatory reactions trigger the oxidative stress and oxidants decrease the level of antioxidants. In the present study, we aimed to determine serum oxidative/antioxidative status in patients with BD. Serum antioxidative status was evaluated by measuring total antioxidant capacity (TAC), paraoxonase 1, arylesterase, sulfhydryl groups and ceruloplasmin in patients with BD and in healthy controls. Serum oxidative status was evaluated by measuring total peroxide (TP), lipid hydroperoxides and oxidative stress index (OSI). OSI was calculated by percent ratio of TP to TAC. Serum levels of TAC, sulfhydryl groups and activities of paraoxonase 1, arylesterase, and ceruloplasmin were significantly lower in patients than in controls (p < 0.001 for all). In contrast, TP, lipid hydroperoxides and OSI values were significantly higher in patients than in controls (p < 0.001 for all). Further, the level of TAC was negatively correlated with the levels of TP, lipid hydroperoxides and OSI both in the BD (r = -0.578, p < 0.01; r = -0.559, p < 0.01 and r = -0.552, p < 0.01, respectively) and the control groups (r = -0.469, p < 0.05; r = -0.351, p < 0.05 and r = -0.391, p < 0.05, respectively). These results indicate that the oxidant parameters increased and antioxidant parameters decreased in patients with BD; therefore, these patients might have been exposed to oxidative stress. We suggest that impaired oxidant/antioxidant balance should be taken into consideration in the follow-up of patients with BD.     

Hyperhomocysteinaemia is associated with uveitis but not with deep venous thrombosis in Behcet's disease.

Plasma homocysteine was assessed in Behcet's disease (BD) patients in order to determine the prevalence of hyperhomocysteinaemia in BD and to test its association with clinical manifestations of the disease. The study included 59 patients with BD and 118 age- and sex-matched healthy subjects. Plasma homocysteine, vitamin B(12) and folate were assessed by automated immunoassay methods. Hyperhomo-cysteinaemia was defined as plasma homocysteine >15 micromol/l. Plasma homocysteine concentrations and the prevalence of hyperhomocysteinaemia were significantly higher in BD patients than in controls [median (5th-95th percentile), 11.3 (6.6-28.1) vs. 10.6 (6.6-17.1) micromol/l, and 25.4% vs. 9.3%, respectively]. In BD patients, hyperhomocysteinaemia was related to male gender, disease severity and uveitis [odds ratio (OR), 5.32; 95% confidence interval (CI), 1.43-21.61; p = 0.008], but not to age, smoking, disease activity, deep venous thrombosis, arthritis or neurological involvement. The association between uveitis and hyperhomocysteinaemia persisted (multi-adjusted OR, 7.46; 95% CI, 1.03-54.3; p = 0.05) after adjusting for gender, age, disease activity and duration, smoking, deep venous thrombosis, and serum concentrations of creatinine, vitamin B(12) and folate. Plasma homocysteine should be measured in patients with BD, and the effect of B-vitamin supplementation should be tested in those with hyperhomo-cysteinaemia.     

Behcet's Disease: Presentation With Sagittal Sinus Thrombosis Diagnosed Noninvasively

The standard evaluation of patients with intracranial hypertension frequently does not reveal a discrete pathophysiologic process, leading in these cases to classification of the syndrome as "benign." We present a 35-year-old woman with a recent diagnosis of pseudotumor cerebri who presented with headache, emesis, and blurring of vision. Her symptoms were progressive despite two lumbar punctures that revealed normal cerebrospinal fluid under high pressure. Contrast and noncontrast CT scans were normal; both the cerebrospinal fluid and CT neuroimaging were thus consistent with benign intracranial hypertension. An MRI, however, supported the presence of sagittal sinus thrombosis, a finding which was confirmed by MR venography. Further workup for an underlying cause of sinus thrombosis disclosed symptoms and signs fulfilling the diagnostic criteria for Behcet's disease. Cerebral venous (or sinus) thrombosis should be considered in the differential diagnosis of intracranial hypertension. Behcet's disease, while extremely rare, should be considered as a potential cause of cerebral venous thrombosis. Magnetic resonance venography can serve as a useful diagnostic study in situations where confirmation or exclusion of sinus thrombosis is required.     

Electrocardiographic findings in Emergency Department patients with pulmonary embolism

To assess the pre-study, null hypothesis that there is no difference in the electrocardiogram (EKG) findings for Emergency Department (ED) patients who rule in vs. rule out for suspected pulmonary embolism, a retrospective review of a cohort of patients with pulmonary embolism and their controls was conducted in an academic, suburban ED. Patients who were evaluated in the ED during a one-year study period for symptoms suggestive of pulmonary embolism were eligible for inclusion. All patients with pulmonary embolism and sex- and age-matched controls comprised the final study groups. Two board-certified cardiologists reviewed each patient's EKG. There were 350 eligible patients identified; 49 patients with pulmonary embolism and 49 controls were entered into the study. The most common rhythm observed in both groups was normal sinus rhythm (67.3% cases vs. 68.6 % controls; p = 1.0). Abnormalities believed to be associated with pulmonary embolism occurred with similar frequency in both case and control groups (sinus tachycardia [18.8 % vs. 11.8%, respectively; p = 0.40]), incomplete right bundle branch block (4.2% vs. 0.0%, respectively; p = 0.24), complete right bundle branch block (4.2% vs. 6.0, respectively; p = 1.0), S1Q3T3 pattern (2.1 vs. 0.0, respectively; p = 0.49), S1Q3 pattern (0.0 vs. 0.0), and extreme right axis (0.0 vs. 0.0). New EKG changes were identified more frequently for patients with pulmonary embolism (33.3% vs. 12.5% controls; p = 0.03), but specific findings were rarely different between cases and controls. In our cohort of ED patients, we did not identify EKG features that are likely to help distinguish patients with pulmonary embolism from those who rule out for the disease.     

Involvement of immunoglobulin FcgammaRIIA and FcgammaRIIIB gene polymorphisms in susceptibility to rheumatic fever

OBJECTIVES: To assess the impact of the human FcgammaRIIA and FcgammaRIIIB gene polymorphisms on the risk of rheumatic fever (RF). DESIGNS AND METHODS: FcgammaRIIA-R/H-131 and FcgammaRIIIB-NA1/NA2 genotypes were determined using polymerase chain reaction in 66 RF cases and 117 healthy controls in this case control study. RESULTS: Compared with healthy controls, the RR genotype was enriched in the entire group of RF cases (odds ratio [OR] 4.98, 95% confidence interval [95% CI] 1.81-13.70). RF patients were more frequently HR heterozygotes rather than HH homozygotes (OR 3.09 vs. 0.11). The results of this study show that patients who have RF are more likely to have the RR and HR genotypes than control children. These probabilities show that RR is associated with the greatest risk for rheumatic fever and HR is associated with an intermediate risk. For the distribution of FcgammaRIIIB NA2 genotypes, a nonsignificant increase was found in RF patients (39.31% vs. 51.51%; OR 1.64, P = 0.1226). CONCLUSION: The FcgammaRIIA-R/H-131 polymorphism may be an important marker in determining predisposition to RF.     

The activities of serum adenosine deaminase and xanthine oxidase enzymes in Behcet's disease

BACKGROUND: Adenosine deaminase (AD) and xanthine oxidase (XO) are enzymes of purine catabolism that catalyze the conversion of adenosine to inosine, deoxyadenosine to deoxyinosine, hypoxanthine to xanthine and xanthine to uric acid, respectively. AD is known to be an important enzyme in the maturation and function of T lymphocytes. The aim of this prospective study was to evaluate whether there are changes in serum AD activity as an index of T lymphocyte function in Behcet's disease (BD) which is known as having T cell-mediated immune response. METHODS: A total of 32 patients and 26 sex- and age-matched healthy control subjects were analysed for AD and XO activities. The patients with BD were divided into two subgroups: BD with and without eye lesions. Twelve patients with complete BD and four patients with incomplete BD had eye complications. AD and XO activities in serum were measured with spectrophotometric methods. RESULTS: There was a remarkable increase in AD activity and moderate increase in XO in patients with BD compared to controls indicating T cell activation and increased maturation. Serum AD activity of complete BD was higher than that of incomplete BD. There was no difference in XO activity between the subgroups of BD. Significant positive correlation was found between AD and XO in BD, although there was no correlation in control group. CONCLUSIONS: The results indicate that increased AD and XO activities may provide an additional benefit for the diagnosis of BD and subtyping of the disease as having eye complication or not and complete and incomplete BD. Further studies are needed to bring to light the exact mechanism of AD and XO activity elevation.     

载脂蛋白B基因XbaI、EcoRI位点多态性与胆石病关系的研究

目的：研究载脂蛋白B基因XbaI、EcoRI位点多态性和胆石病之间的关系。方法：通过病例对照研究设计，采用PCR－RFLP技术对106例胆石病患者和105例对照者进行基因分析。结果：胆石病组和对照组ApoB基因XbaI位点X＋X－、X－X－基因型构成显著不等；胆石病组X＋等位基因频率显著高于对照组（0.104vs 0.052)；胆石病组和对照组ApoB基因EcoRI位点E＋E－和E＋E＋基因型频率构成差异显著，病例组E－等位基因频率显著高于对照组。结论：胆石病发生与ApoB基因Xba I、EcoRI位点多态性存在关联，XbaI X 等位基因和EcoRI E-等位基因可能为胆石病的易感基因。     

Altered urinary excretion of pteridines in neoplastic disease. Determination of biopterin,neopterin, xanthopterin,and pterin

Urinary pteridine concentrations in healthy control subjects and patients with cancer and non-malignant diseases were determined by HPLC and TLC after partial purification by ion exchange and Sephadex chromatography. Elevated concentrations of neopterin were found in 70% of the 50 cancer patients investigated. In patients with non-Hodgkin's lymphoma (seven cases) or with liver metastases (12 cases) neopterin concentrations were significantly higher than in control subjects (p < 0.01). Biopterin was less frequently increased (22%). Xanthopterin was generally raised when neopterin and/or biopterin excretion was high. Neopterin/biopterin ratios were higher in some patients with cancer or with severe renal insufficiency than in controls. These findings suggest that alterations in pteridine metabolism are common in malignant disease. The pathogenic, diagnostic and therapeutic significance of these changes remains to be established.     

Migraine with aura and reproductive life events: a case control study

The course of migraine without aura (MO) is greatly influenced by the events of female reproductive life. Much less is known about migraine with aura (MA). The aim of this study was to evaluate the relationship between MA and the milestones of reproductive life. A retrospective case control study was carried out on 100 women affected by migraine with typical aura (cases) and 200 age-matched women with MO (controls). Premenstrual syndrome was found to be much more common among the patients with MA (odds ratio (OR) 6.0; confidence interval (CI) 3.1-11.6). Menstrually triggered migraine was more frequently encountered among MO than among MA patients (MA 15.0%; MO 53.5%; OR 0.1; CI 0.1-0.3). In both forms of migraine, pregnancy had a favourable effect; however, a lower percentage of MA (43.6%) than MO patients (76.8%; OR 0.2; CI 0.1-0.5) showed improvement or remission. The use of oral contraceptives worsened migraine in MA more frequently than in MO patients (MA 56.4%; MO 25.3%; OR 3.8; CI 1.6-9.3). The course of MA seems to be influenced by female reproductive life events, but in a different way with respect to MO.     

Intra-Hisian Block Associated with Unusual Etiologies

One hundred and sixty-nine patients underwent electrophysiologic study for atrioventricular block. Forty-five (27%) had intra-Hisian block. Four of these 45 patients had unusual etiologies: rheumatoid arthritis, cysticercosis cellulosae, Behcet's disease, and Takayasu aorto-arteritis. Their clinical and electrophysiologic features are described in detail.     

心血管白塞病的外科治疗

目的:总结心血管白塞病的外科治疗经验及教训,探讨改进心血管白塞痛的外科治疗的方法.方法:2004年10月至2010年11月,我院心血管外科共收治了主动脉辩关闭不全心血管白塞病的患者10例,有4例进行了1次手术,6例进行了2次手术.结果:10例患者第1次行主动脉瓣置换术后,1例患者因为多器官功能衰竭死亡,9例出院继续药物治疗.第1次手术治疗出院后的9例患者中,有6例出现了术后辩周漏,其中1例经过第2次行心脏原位移植,术后因为多器官功能衰竭死亡;另5例经过第2次主动脉瓣置换手术,其中4例术后,再出现瓣周漏,最终因为严重低心排出量综合征而死亡;本组共4例长期存活.结论:外科治疗心血管白塞病,易并发瓣周漏,病死率高,故手术治疗应慎重,正确选择手术时机和手术方式,有助于减少术后并发症的发生.     

Patients with Behcet's disease carry a higher risk for microvascular involvement in active disease period

BACKGROUND: Behcet's disease (BD) is characterized with remissions and exacerbations. However, to date, there is no study to investigate a possible association of disease activity (active versus inactive disease period) with cardiovascular complications. METHODS: Forty patients with BD were evaluated in both active and in inactive disease period. For the control group 45 healthy volunteers, age and sex matched, were registered. Subjects with at least a 15-day lesion-free period were regarded in inactive disease period, and subjects with any oral, skin, and/or genital lesion was regarded as in active disease period. In each subject coronary diastolic peak flow velocities (DPFV) were measured at baseline and after dipyridamole infusion (0.84 mg/kg over 6 minutes) using an Acuson Sequoia C256 echocardiography system. Coronary flow reserve (CFR) was defined as the ratio of hyperemic to baseline DPFV. RESULTS: CFR values were significantly lower in BD patients compared to the controls (2.57+/-0.50 versus 2.87+/-0.53, P = 0.006). In active disease period, basal DPFV (24.6+/-7.5 versus 27.3+/-6.6, P = 0.019) was significantly higher than in the inactive disease period. In the active disease period hyperemic DPFV (61.7+/-14.9 versus 56.8+/-16.7, P = 0.015) values decreased significantly. Therefore, in the active disease period CFR significantly decreased from 2.57+/-0.50 to 2.09+/-0.46, P<0.001. The only independent predictor of CFR within the active disease period was the disease duration (beta = -0.384, P = 0.012). CONCLUSION: Within the active disease period, coronary microvascular function is more prominently impaired in BD patients. Therefore, BD patients are possibly more vulnerable to cardiovascular manifestations when they are in an active disease period.     

Increased incidence of duodenitis in chronic renal failure

Forty-six consecutive patients with chronic renal failure (CRF) were assessed radiographically, endoscopically and histologically for duodenal inflammation. Their gastric secretion was also examined. Thirty-eight patients with non-ulcer dyspepsia served as controls. Histologically established duodenitis was more common in CRF patients than in controls. Among the non-dialyzed patients its prevalence reached 28% (9/32), while in the controls it was 5% (2/38) (p less than 0.05). The CRF patients with duodenitis had upper GI symptoms, hypersecretion of acid (p less than 0.05) and duodenal ulcer disease (p less than 0.05) more frequently compared with the CRF subjects with normal duodenal mucosa. The correlation of radiographically and endoscopically detected duodenitis with that found microscopically was poor.     

Determination of malondialdehyde, reduced glutathione levels and APOE4 allele frequency in late-onset Alzheimer's disease in Denizli, Turkey

OBJECTIVES: The aim of the present study was to determine the Apolipoprotein E (APOE) 4 allele frequency of patients with late-onset Alzheimer's disease (AD) and to determine the effects of oxidant-antioxidant balance on AD. DESIGN AND METHODS: PCR-RFLP was undertaken in 62 cases with AD and 56 aged-matched controls. Activities of reduced glutathione (GSH) and malondialdehyde (MDA) concentration were measured in same groups. RESULTS: Patients with at least one E4 allele genotype were significantly different in patients with AD (21%) than controls (9%) (p=0.01). Serum MDA levels were significantly different between AD patients and Control group (p=0.0001). There was no significant difference in serum GSH levels between AD patients and C groups. CONCLUSION: These results confirmed that the APOE4 allele occurs frequently in late onset AD compared with normal controls. Also elevated MDA levels are likely an essential factor in the pathogenesis and neuronal damage of AD.     

Color and power Doppler sonography of extracranial and intracranial arteries in Moyamoya disease

PURPOSE: To study the hemodynamic characteristics of moyamoya disease with color Doppler (CD) and power Doppler (PD) sonography. METHODS: The hemodynamic parameters of intracranial and extracranial arteries from 17 patients with moyamoya disease confirmed via digital subtraction angiography and 30 healthy controls were studied with conventional and transcranial CD and PD. RESULTS: The moyamoya vessels were detected as scattered color Doppler signal with low velocity and a low resistance index (RI) at the base of the brain in 10 of the 17 patients. The RI of the common carotid arteries and the internal carotid arteries of the patients was significantly higher, whereas the peak velocity was lower than in controls. The posterior carotid arteries were more frequently involved in children (43.8%) than in adults (5.6%). CONCLUSIONS: Transcranial CD and PD can be used to demonstrate the stenosis and occlusion of intracranial arteries and the abnormal vascular network at the base of the brain in most cases. Combined with the hemodynamic characteristics of extracranial arteries and the symptoms of the patients, an accurate diagnosis of moyamoya disease could be made in the majority of cases using PD.     

高尿酸血症对非酒精性脂肪肝肝损害程度的影响

目的 观察高尿酸血症患者超声诊断非酒精性脂肪肝(NAFLD)的严重程度及肝酶学升高程度,以探索高尿酸血症对NAFLD肝脂肪变的影响.方法 采用1∶1匹配的病例对照研究方法.从我院查体中心2008年1~12月体检者中选取符合诊断标准的624例高尿酸血症患者作为病例组.以病例组为标准,选取性别、工作单位相同,年龄±2岁的体检者作为对照组.测量其身高、体重、血压,空腹取血行肝肾功能、血脂、血糖、肝炎相关检查,同时行心电图、腹部超声、胸部X线等检查.结果 病例组患者BMI、MAP、TC、TG、HDL-C和LDL-C水平及超重或肥胖、FBS增高、血压增高、高TG血症的检出率均显著高于对照组(P<0.05);病例组NAFLD总检出率及轻、中、重度脂肪肝检出率均显著高于对照组(44.9%,26.6%、16.5%和1.8% vs.22.8%,16.0%、6.4%和0.3%;P<0.001)且在NAFLD患者中,病例组中重度脂肪肝的分布比例显著增高(χ2=5.015,P<0.05);无论是病例组还是对照组,超声诊断NAFLD患者ALT、AST、GGT和ALP水平均显著高于超声诊断正常肝组(P<0.05),病例组超声诊断正常肝组患者ALT水平高于相应对照组(P<0.01);病例组NAFLD患者ALT、AST、GGT水平显著高于对照组NAFLD患者(t=33.615、20.971、31.281;P<0.01),在NAFLD患者中,病例组NAFLD患者ALT、AST和GGT升高的检出率显著高于对照组NAFLD患者(χ2=25.352、6.170和45.124;P<0.05).结论 高尿酸血症是NAFLD进展的重要高危因素,与超声诊断脂肪肝的严重程度及肝酶学异常密切相关.