Adrenal cortical carcinoma initially presented with overwhelming disseminated intravascular coagulation

We report a 54-year-old man who had adrenal cortical carcinoma initially manifested as features of overwhelming disseminated intravascular coagulation (DIC). In the initial diagnostic work-up, an adrenal mass was detected with venous thrombi in the abdominal imaging study, but the radiologic diagnosis was a hematoma arising from the adrenal gland and a biopsy was not possible due to a bleeding tendency. A lot of platelets and plasma products were transfused, but the bleeding tendency and other DIC features persisted. Finally, he expired because of newly developed massive pulmonary thromboembolism. To our knowledge, this is the first reported case of adrenal cortical carcinoma complicated with bleeding tendency caused by DIC as an initial manifestation. This suggests that adrenal cortical carcinoma should be considered in a patient with an adrenal mass and DIC features.     

Torsion dynamics in the evolution from acute to chronic mitral regurgitation

BACKGROUND AND AIM OF STUDY: Left ventricular (LV) torsion reduces transmural fiber strain gradients during systole, and torsional recoil in early diastole is thought to assist LV filling. To test the hypothesis that deterioration of torsional dynamics accompanied LV dysfunction during the evolution of mitral regurgitation (MR), torsion was measured during the progression from acute to chronic MR in a canine model. METHODS: Seven dogs underwent cardiopulmonary bypass for LV marker placement and creation of MR by disrupting the posterior leaflet. After 7-10 days, three-dimensional marker coordinates were measured with biplane videofluoroscopy to study LV geometry, size and function, plus maximal torsional deformation, time of maximal torsion relative to end-ejection, and early diastolic torsional recoil during the first 5% of filling. After three months, the animals were re-studied. RESULTS: Progression from acute to chronic MR was associated with a significant decrease in maximum LV dP/dt (1,574+/-213 to 1,300+/-252 mmHg/s, p <0.01) and an increase in LVEDP from 11+/-5 to 15+/-5 mmHg (p <0.01). After three months of MR, maximum torsional deformation decreased from 6.3+/-1.9 to 4.7+/-2.0 degrees (p = 0.04), as did early diastolic recoil (-3.8+/-1.0 to -1.5+/-1.7 degrees, p = 0.03). CONCLUSION: Progression from acute to chronic MR is accompanied by decreased and delayed systolic LV torsional deformation and a decline in early diastolic recoil, which may contribute to LV dysfunction by increasing transmural strain gradients during systole and impairing diastolic filling. As torsional deformation and recoil can be measured non-invasively with MRI in humans, such measurements might prove useful in patients with progressive MR as an adjunct to determine the timing of surgical repair.     

赖诺普利逆转高血压患者左室肥厚的相关性研究

目的探讨赖诺普利对高血压患者左室肥厚的逆转作用。方法分别口服赖诺普利与卡托普利治疗高血压伴左室肥厚患者8周,应用超声心动图监测治疗前、后左室重量指数（LVMI）,同时采用放射免疫法和（或）化学发光法检测血浆血管紧张素Ⅱ（AT-Ⅱ）醛固酮（ALD）,血清胰岛素样生长因子-1（IGF-1）的浓度。结果①高血压组患者血浆AT-Ⅱ、ALD,血清IGF-1浓度明显高于正常对照组（P值分别为0.023,0.018,0.032）,LVH组高于无LVH组（P值为0.0083）。②治疗后与治疗前比较,两组患者AT-Ⅱ、ALD、IGF-1和LVMI水平均显著降低（P值分别为0.036,0.028,0.031）,但赖诺普利组降低幅度明显高于卡托普利组,差异有统计学意义（P〈0.01）。结论赖诺普利与卡托普利均有降压及逆转左室肥厚的作用,但赖诺普利逆转LVH的强度更强,且不良反应少,依从性强,值得临床推广使用。     

缺血预处理对高原大鼠肝脏缺血/再灌注损伤早期的影响

目的:了解在高原缺血预处理(IPC)对肝脏缺血/再灌注损伤(I/R)早期保护作用及其机制.方法:SD大鼠45只随机分为3组:假手术组、缺血再灌注组、缺血预处理组.各组恢复血流后分别于1,3,6 h取血液标本,检测血清ALT(nkat/L)、丙二醛(MDA,mmol/L)、NO(μmol/L)的含量.结果:缺血预处理组NO的水平明显高于缺血再灌注组(1 h:95.8±10.1 vs 64.2±6.8,P＜0.01;3 h:91.2±9.7 vs 69.5±7.2,P＜0.01;6 h:77.4±8.6 vs 63.7±6.1,P＜0.01),低于假手术组;而ALT(1 h:2257.1±201.7 vs 2912.2±398.4,P＜0.01;3 h:2465.5±243.4 vs 3637.4±441.8,P＜0.01;2545.5±223.4 vs 4027.5±496.8,P＜0.01)、MDA(1 h:25.1±4.3 vs 38.7±7.6,P＜0.01;3 h:27.5±5.4 vs 45.3±8.8,P＜0.01;34.2±6.7 vs 53.2±10.5,P＜0.01)明显低于缺血再灌注组,高于假手术组.结论:在高原缺氧环境下,缺血预处理对大鼠肝脏缺血再灌注损伤有明显保护作用,其机制可能与缺血与预处理抑制肝脏脂质过氧化物的产生,提高内源性NO的产生,改善肝脏的微循环有关.     

Pretreatment with intestinal trefoil factor alleviates stress-induced gastric mucosal damage via Akt signaling

BACKGROUNDStress-related gastric mucosal damage or ulcer remains an unsolved issue for critically ill patients. Stress ulcer prophylaxis has been part of routine intensive care, but uncertainty and controversy still exist. Co-secreted with mucins, intestinal trefoil factor (ITF) is reported to promote restitution and regeneration of intestinal mucosal epithelium, although the mechanism remains unknown.AIMTo elucidate the protective effects of ITF on gastric mucosa and explore the possible mechanisms.METHODSWe used a rat model of gastric mucosal damage induced by water immersion restraint stress and lipopolysaccharide-treated human gastric epithelial cell line to investigate the potential effects of ITF on damaged gastric mucosa both in vivo and in vitro.RESULTSITF promoted the proliferation and migration and inhibited necrosis of gastric mucosal epithelia in vitro. It also preserved the integrity of gastric mucosa by upregulating expressions of occludin and zonula occludens-1. In the rat model, pretreatment with ITF ameliorated the gastric mucosal epithelial damage and facilitated mucosal repair. The protective effects of ITF were confirmed to be exerted via activation of Akt signaling, and the specific inhibitor of Akt signaling {"type":"entrez-nucleotide","attrs":{"text":"LY249002","term_id":"1257710161","term_text":"LY249002"}}LY249002 reversed the protective effects.CONCLUSIONITF might be a promising candidate for prevention and treatment of stress-induced gastric mucosal damage, and further studies should be undertaken to verify its clinical feasibility.     

按三级学科方向培养手术室专科护士可行性的调查

目的：了解不同等级医院对手术室专科护士的需求,探索能满足不同等级医院手术室护理工作实际需求的专科护士培训方案。方法自行设计调查表,选择在江苏省手术室专科护士培训基地学习的80名手术室护士进行调查。调查内容包括一般资料、所在医院三级学科和亚专业学科细化情况、参加专科护士培训后工作状况、手术室专科护士的培养模式和按照三级学科培养手术室专科护士的实施方案4个方面。结果80名护士均认为有必要开展手术室专科护士培训,80．00％的人认为目前模式属手术室全科护士的培训;72．22％的二级医院的护士认为参加培训学习的理论知识、参加的临床实践技能能满足工作需要,可继续采取目前培养模式;74．19％的三级医院的护士认为培训知识尚能满足工作需要,但针对自己的三级学科方向还不够强、不够细,应该按照三级学科培养手术室专科护士。结论根据不同等级医院手术室护理工作的实际需求,建立统一招生、同步培训、分层培养的统分结合培养模式,能培养适应不同等级医院手术室需求的专科护士。     

3.0TMRI对颈动脉粥样硬化斑块成分分析及分型的研究

目的：探讨3.0 T高分辨磁共振成像对颈动脉粥样硬化斑块内部成分检出及斑块稳定性判定的价值。材料与方法33例颈动脉粥样硬化斑块患者行MRI检查,扫描序列包括三维时间飞跃法(3D-TOF)成像、双翻转T1加权成像、T2加权成像、质子密度(PD)加权成像及增强颈部血管成像,研究、分析斑块内成分及其分型。结果33例患者中共检出颈动脉粥样硬化斑块63个,分型如下：Ⅰ-Ⅱ型占12.7%,Ⅲ型占27%,Ⅳ-Ⅴ型占19%,Ⅵ型占31.8%,Ⅷ型占9.5%,各型斑块在颈动脉3个部位的分布差异无统计学意义(Fisher确切概率P=0.106);所有检出的颈动脉斑块中稳定斑块共31个,不稳定斑块共32个,二者在是否出现脂质核心(LRNC)、出血(IPH)、血栓形成及纤维帽(FC)破裂方面均有统计学差异(P＜0.05),而钙化出现率无统计学差异(P＞0.05)。结论3.0 T高分辨率MRI能识别、区分颈动脉粥样斑块内成分,能鉴别稳定斑块和不稳定斑块,为临床选择治疗方案及疗效评价提供指导。     

Inherited IL-17RC deficiency in patients with chronic mucocutaneous candidiasis

Chronic mucocutaneous candidiasis (CMC) is characterized by recurrent or persistent infections of the skin, nail, oral, and genital mucosae with Candida species, mainly C. albicans. Autosomal-recessive (AR) IL-17RA and ACT1 deficiencies and autosomal-dominant IL-17F deficiency, each reported in a single kindred, underlie CMC in otherwise healthy patients. We report three patients from unrelated kindreds, aged 8, 12, and 37 yr with isolated CMC, who display AR IL-17RC deficiency. The patients are homozygous for different nonsense alleles that prevent the expression of IL-17RC on the cell surface. The defect is complete, abolishing cellular responses to IL-17A and IL-17F homo- and heterodimers. However, in contrast to what is observed for the IL-17RA– and ACT1-deficient patients tested, the response to IL-17E (IL-25) is maintained in these IL-17RC–deficient patients. These experiments of nature indicate that human IL-17RC is essential for mucocutaneous immunity to C. albicans but is otherwise largely redundant.In humans, chronic mucocutaneous candidiasis (CMC) is characterized by infections of the skin, nail, digestive, and genital mucosae with Candida species, mainly C. albicans, a commensal of the gastrointestinal tract in healthy individuals (Puel et al., 2012). CMC is frequent in acquired or inherited disorders involving profound T cell defects (Puel et al., 2010b; Vinh, 2011; Lionakis, 2012). Human IL-17 immunity has recently been shown to be essential for mucocutaneous protection against C. albicans (Puel et al., 2010b, 2012; Cypowyj et al., 2012; Engelhardt and Grimbacher, 2012; Huppler et al., 2012; Ling and Puel, 2014). Indeed, patients with primary immunodeficiencies and syndromic CMC have been shown to display impaired IL-17 immunity (Puel et al., 2010b). Most patients with autosomal-dominant (AD) hyper-IgE syndrome (AD-HIES) and STAT3 deficiency (de Beaucoudrey et al., 2008; Ma et al., 2008; Milner et al., 2008; Renner et al., 2008; Chandesris et al., 2012) and some patients with invasive fungal infection and autosomal-recessive (AR) CARD9 deficiency (Glocker et al., 2009; Lanternier et al., 2013) or Mendelian susceptibility to mycobacterial diseases (MSMD) and AR IL-12p40 or IL-12Rβ1 deficiency (de Beaucoudrey et al., 2008, 2010; Prando et al., 2013; Ouederni et al., 2014) have low proportions of IL-17A–producing T cells and CMC (Cypowyj et al., 2012; Puel et al., 2012). Patients with AR autoimmune polyendocrine syndrome type 1 (APS-1) and AIRE deficiency display CMC and high levels of neutralizing autoantibodies against IL-17A, IL-17F, and/or IL-22 (Browne and Holland, 2010; Husebye and Anderson, 2010; Kisand et al., 2010, 2011; Puel et al., 2010a).These findings paved the way for the discovery of the first genetic etiologies of CMC disease (CMCD), an inherited condition affecting individuals with none of the aforementioned primary immunodeficiencies (Puel et al., 2011; Casanova and Abel, 2013; Casanova et al., 2013, 2014). AR IL-17RA deficiency, AR ACT1 deficiency, and AD IL-17F deficiency were described, each in a single kindred (Puel et al., 2011; Boisson et al., 2013). A fourth genetic etiology of CMCD, which currently appears to be the most frequent, has also been reported: heterozygous gain-of-function (GOF) mutations of STAT1 impairing the development of IL-17–producing T cells (Liu et al., 2011; Smeekens et al., 2011; van de Veerdonk et al., 2011; Hori et al., 2012; Takezaki et al., 2012; Tóth et al., 2012; Al Rushood et al., 2013; Aldave et al., 2013; Romberg et al., 2013; Sampaio et al., 2013; Soltész et al., 2013; Uzel et al., 2013; Wildbaum et al., 2013; Frans et al., 2014; Kilic et al., 2014; Lee et al., 2014; Mekki et al., 2014; Mizoguchi et al., 2014; Sharfe et al., 2014; Yamazaki et al., 2014). We studied three unrelated patients with CMCD without mutations of IL17F, IL17RA, ACT1, or STAT1. We used a genome-wide approach based on whole-exome sequencing (WES). We found AR complete IL-17RC deficiency in all three patients.