Behçet’s disease in Egypt: a multicenter nationwide study on 1526 adult patients and review of the literature

Objectives

The present work was conducted to estimate the prevalence of adult Behçet’s disease (BD) in adult Egyptian and to study the clinical pattern and influence of age at-onset and sex on disease phenotype. Also, we investigated the spectrum of presentation and frequencies along the north-to-south gradient of the country.

Patients and method

The population-based, multicenter, cross-sectional study included 1526 adult BD patients from 26 specialized Egyptian rheumatology centers. Demographic, clinical, and therapeutic data are assessed for all patients.

Results

The mean age of patients was 35.7 ± 9.84 years, disease duration 6.58 ± 5.25 years, and age at onset 29.37 ± 8.6 years; 91 were juvenile-onset (JoBD). There were 1102 males and 424 females (M:F 2.6:1). Regarding co-morbidities, 19.92% were diabetic, and 26.05% were hypertensive. The mean body mass index was 27.57 ± 5.24 (43.1% overweight; 25.9% obese). The mean BD current activity form was 4.48 ± 4.28. Regarding the medications use, systemic steroid and colchicine were the most common drugs used (947 (90.2%) and 611 (82.7%), respectively). The overall estimated prevalence of BD in Egypt was 3.6/100,000 population being highest in the two main cities: Alexandria (15.27) and Cairo (8.72). Pathergy test was positive in 43.4%. 90.2% were receiving systemic steroids and 8.3%, biologics. Disease characteristics were comparable between JoBD and adult-onset BD cases. Central nervous system (CNS), deep venous thrombosis (DVT), and gastrointestinal (GIT) involvement were significantly higher in males (p = 0.01, p = 0.001, and p = 0.001 respectively) while joint affection (p = 0.001) and disease activity (p = 0.011) were increased in females.

Conclusions

This study provides current prevalence of BD in Egypt; 3.6/100,000 with no remarkable north-to-south gradient. The sex influences the disease phenotype with the CNS, DVT, and GIT involvement are higher in males, while the joint affection and disease activity were increased in females.

Key Points

• The prevalence and phenotype of Behçet’s disease across Egypt is presented in a multicenter nationwide study.

• The potential influence of the age at onset and sex on disease phenotype is highlightened.

• A review of the literature worldwide is presented allowing comparisons with studies from other nations.

     

The spectrum of BRCA1 and BRCA2 pathogenic sequence variants in Middle Eastern,North African,and South European countries

BRCA1 BRCA2 mutational spectrum in the Middle East, North Africa, and Southern Europe is not well characterized. The unique history and cultural practices characterizing these regions, often involving consanguinity and inbreeding, plausibly led to the accumulation of population‐specific founder pathogenic sequence variants (PSVs). To determine recurring BRCA PSVs in these locales, a search in PUBMED, EMBASE, BIC, and CIMBA was carried out combined with outreach to researchers from the relevant countries for unpublished data. We identified 232 PSVs in BRCA1 and 239 in BRCA2 in 25 of 33 countries surveyed. Common PSVs that were detected in four or more countries were c.5266dup (p.Gln1756Profs), c.181T>G (p.Cys61Gly), c.68_69del (p.Glu23Valfs), c.5030_5033del (p.Thr1677Ilefs), c.4327C>T (p.Arg1443Ter), c.5251C>T (p.Arg1751Ter), c.1016dup (p.Val340Glyfs), c.3700_3704del (p.Val1234Glnfs), c.4065_4068del (p.Asn1355Lysfs), c.1504_1508del (p.Leu502Alafs), c.843_846del (p.Ser282Tyrfs), c.798_799del (p.Ser267Lysfs), and c.3607C>T (p.Arg1203Ter) in BRCA1 and c.2808_2811del (p.Ala938Profs), c.5722_5723del (p.Leu1908Argfs), c.9097dup (p.Thr3033Asnfs), c.1310_1313del (p. p.Lys437Ilefs), and c.5946del (p.Ser1982Argfs) for BRCA2. Notably, some mutations (e.g., p.Asn257Lysfs (c.771_775del)) were observed in unrelated populations. Thus, seemingly genotyping recurring BRCA PSVs in specific populations may provide first pass BRCA genotyping platform.     

影响医院中心注射室护理工作满意度的相关因素调查

目的对中心注射室护理工作的满意度进行调查,了解影响医院护理工作满意度的因素。方法选择2011年1～12月在中心注射室接受治疗的患者252例为研究对象,采用问卷法对患者进行调查,并分析影响注射室护理工作满意度的因素。结果接受调查的住院患者中,患者的满意度得分与患者的年龄、受教育程度、收入水平、住院科室、住院天数以及是否手术存在相关性。结论护理人员应重视和加强护理工作质量和效率,提高临床患者对护理工作的满意程度。     

二尖瓣环收缩期最大位移达峰时间评价扩张型心肌病患者左心室长轴收缩功能的超声心动图研究

Objective To evaluate the value of peak time(PT) and corrected peak time(PTc) in the assessment of left ventricular long axis systolic function in patients with dilated cardiomyopathy(DCM).Methods Thirty-one DCM cases and thirty-six healthy cases were investigated using echocardiography.To obtain image of apical four-chamber view and apical two-chamber view, PT was measured by the technique of tissue motion annular displacement.The value of PTc was corrected by R-R interval.The critical value of PT and PTc was obtained by ROC curves.Results Compared with the PT of control group, the PT of DCM group at the site of septal and lateral were significantly increased ( P ＜0.05), but the PT at the site of anterior and inferior were no significantly increased ( P ＞0.05).The PTc at the four sites were significantly increased compared with control group ( P ＜0.05).The areas of under the ROC curve of PTc was 0.849 (95 % CI 0.699～0.929, P = 0.000), sensitivity and specificity of diagnosis cardiac dysfunction were 80.6 % and 66.7% respectively.Conclusions The PTc was significantly increased in patients with DCM.PTc was useful to evaluate left ventricular dysfunction in DCM patients.     

牛羊猪种布鲁氏菌快速鉴别PCR方法的建立及评价

目的 建立一种可在一个反应体系中同时鉴别布鲁氏菌及牛羊猪种布鲁氏菌的快速PCR鉴别方法。方法 将布鲁氏菌及牛羊猪种布鲁氏菌的扩增引物进行优化组合,建立全新的BAMS-PCR方法;随后对该方法的特异性和灵敏度进行评价,并对现场分离的219株布鲁氏菌进行鉴别,评价其在布鲁氏菌鉴别中的应用价值。最后,用该方法对临床标本中布鲁氏菌的DNA进行扩增,评价其在临床诊断中的实用性。结果 BAMS-PCR方法可在同一反应中同时鉴别布鲁氏菌及牛种(1,2,4型),羊种(1,2和3型)和猪种(1型)布鲁氏菌,并有较好的特异性和敏感性。布鲁氏菌属,牛种,猪种和羊种布鲁氏菌引物的检测灵敏度分别为10 pg/μL,100 pg/μL, 10 pg/μL和100 pg/μL。BAMS-PCR对219株临床分离菌株的鉴定结果和常规生物分型方法的鉴定符合率为100%。经BAMS-PCR检测,97份临床血清样本仅6份为阳性,全血和组织(羊脾)样本全部为阴性。结论 BAMS-PCR是一种简便、快速、高效、准确的布鲁氏菌鉴别方法,可作为临床分离菌株的首选鉴别方法。     

Acute appendicitis in the setting of Clostridium difficile colitis: case report and review of the literature

A 72-year-old man was hospitalized for exacerbation of chronic obstructive pulmonary disease and was treated with oral prednisone and 7 days of moxifloxacin. Five days after completing the antibiotic course, he developed watery diarrhea and diffuse, crampy abdominal pain. On presentation he was afebrile, and abdominal examination revealed diffuse tenderness without peritoneal signs. Stool tested positive for Clostridium difficile toxin A by enzyme-linked immunosorbent assay. Despite starting oral metronidazole, the patient developed a fever of 101.2 degrees F 36 hours after his initial episode of diarrhea, 12 hours after admission. His abdominal pain intensified and became localized to the right and left lower quadrants. Computed tomography scan revealed both a thickened cecal wall and an edematous appendix with ileocecal stranding consistent with appendicitis. Appendectomy was performed, and the appendix was found to be suppurative in appearance and nonperforated. The cecum had mild edema and erythema, whereas the colon and rectum were grossly unaffected. Pathology examination revealed exudative material in the appendiceal lumen and a diffuse transmural inflammatory cell infiltrate. The patient had an uneventful recovery and continued to improve on oral metronidazole. Although Clostridium difficile colitis and appendicitis are each very common independently, C. difficile as an etiology of appendicitis is exceedingly rare. A review of the literature revealed 2 prior cases. We speculate that this association is underdiagnosed, because milder cases might respond to antibiotic therapy alone, and severe cases might involve the entire colon and require total colectomy. In each scenario, the involvement of the appendix might be overlooked.     

Adipocytokine profile of type 2 diabetics in metabolic syndrome as defined by various criteria

BACKGROUND: This study aims to identify which among the metabolic syndrome (MS) definitions are closely associated with pathological levels of leptin, adiponectin, resistin, tumour necrosis alpha (TNF-alpha) and C-reactive protein (CRP) among type 2 diabetics. MATERIALS AND METHODS: Three hundred and five (160 males; 145 females) adult type 2 diabetic Saudis participated in this cross-sectional study. Leptin, adiponectin, resistin, TNF-alpha and CRP were analysed, using enzyme-linked immunosorbent assays (ELISA). Each participant was screened for MS based on the definitions of WHO, AHA/NHLBI and IDF. RESULTS: IDF holds the most identified patients [190 (62.3%)] in both, males [107 (66.9%)], and females [83 (57.2%)]. In males, hyperleptinemia, hypoadiponectinemia and hyperresistinemia were strongest in the AHA/NHLBI-defined MS [odds ratio (95% confidence interval 'CI') of 2.03 (1.05-3.93); 1.31 (0.55-3.1); 1.63 (0.42-6.4) respectively]. The risk of elevated CRP was highest on the WHO definition [odds ratio (95% CI) of 2.04 (0.46-9.04)]. In females, the IDF-defined MS has the strongest association in all four parameters: odds ratio (95% CI), as follows: leptin [2.09 (0.14-30.71)]; adiponectin [6.00 (0.47-76.17)]; resistin [0.47 (0.18-1.23)] and CRP [3.07 (0.21-45.10)]. CONCLUSION: Gender differences exist in assessing the risk of various adipocytokine abnormalities in relation to the various criteria. This study supports the use of IDF definition among females and AHA/NHLBI in males in studies involving MS and obesity, since these definitions hold stronger predicting powers in detecting pathological levels of key adipocytokines.     

Clinical significance of serum B cell chemokine (CXCL13) in early rheumatoid arthritis patients

Background

The diagnosis of early rheumatoid arthritis (RA) is challenging. B-cell chemokine (CXCL13) plays a critical role in the disease pathogenesis.

MCP-1在小型猪慢性阻塞性胰腺炎组织中的表达及其意义

目的 检测慢性阻塞性胰腺炎小型猪血单核细胞趋化蛋白-1(MCP-1)含量及胰腺组织MCP-1蛋白表达,探讨其在胰腺纤维化中的作用.方法 采用不全结扎主胰管方法建立小型猪慢性阻塞性胰腺炎模型,术后4、6、8 周分批处死动物.观察胰腺病理变化;胶原(VG)染色评估胰腺纤维化程度;ELISA法检测血MCP-1浓度;免疫组化法检测胰腺组织MCP-1、α-SMA、PDGF、TGF-β1和NF-κB表达.结果 造模成功14头(58.3%).术后4周起胰腺体尾部呈萎缩性改变,间质纤维组织增生,炎细胞浸润,第8周改变最明显.胰腺纤维化Ⅰ期5头(35.7%),Ⅱ期4头(28.6%),Ⅲ期5头(35.7%).术后4、6、8周造模成功组血MCP-1含量分别为(102.44±36.25)pg/m1、(97.84±28.67)pg/ml、(94.32±28.42)pg/ml,显著高于对照组的(10.42±5.86)pg/ml、(8.58±4.86)pg/ml、(8.22±4.58)pg/ml(P值均＜0.01).对照组胰腺无MCP-1蛋白表达,造模成功组胰腺组织内见MCP-1蛋白表达,且MCP-1表达与α-SMA、PDGF、TGF-β1和NF-κB表达呈正相关.结论 MCP-1在慢性阻塞性胰腺炎胰腺纤维化过程中起重要作用.     

耳后进路夹层法与内植法鼓膜修补术疗效比较

【目的】探讨并比较耳后进路夹层法与内植法鼓膜修补术疗效。【方法】收集需行鼓膜修补术患者80例（92耳）,按手术方法分为夹层法组42例（46耳）与内植法组38例（46耳）。分别观察两组术后听力恢复情况、术前术后言语频率平均听阈、术后3个月鼓膜情况、术后6个月愈合率。【结果】术后听力恢复情况夹层法优于内植法（P ＜0．05）;夹层法术后言语频率平均听阈明显高于内植法（P ＜0．01）;术后随访3个月后两组引起再穿孔、鼓膜内陷的发生有统计学差异（ P ＜0．05）;术后6个月愈合率比较,夹层法优于内植法,两组有统计学差异（ P ＜0．05）。【结论】耳后进路夹层法与内植法鼓膜修补术短期疗效相当,但前者更有利于听力恢复,是治疗鼓膜穿孔的较为理想的方法。