Hepatic paraganglioma and multifocal gastrointestinal stromal tumor in a female: Incomplete Carney triad

The Carney triad (CT) describes the coexistence of multiple neoplasms including gastrointestinal stromal tumors (GISTs), extra-adrenal paraganglioma and pulmonary chondroma. At least two neoplastic tumors are required for diagnosis. In most cases, however, CT is incomplete. We report a case of an incomplete CT in a 34-year-old woman with a multifocal GIST and non-functional paraganglioma of the liver. Preoperative evaluation with a gastrofiberscope and abdominal computed tomography revealed multiple gastric tumors resembling GISTs and a single liver lesion which was assumed to have metastasized from the gastric tumors. The patient underwent total gastrectomy and partial hepatectomy. Histologic findings confirmed multiple gastric GISTs and paraganglioma of the liver. We report a case of a patient with incomplete expression of CT.

Chiari三联征(Chiari's triad)

指各种原因所致的主动脉食管瘘发病时的一组特异性临床表现，首先是胸痛（一般为胸中部疼痛），随即出现前兆性动脉出血，一段时间间隔后出现致命性大出血，这一时间间隔可以是数小时至数天不等。Dubrueil于1818年第一次描述了主动脉食管瘘这一病症[1]。Chiari于1914年首先描述了主动脉食管瘘三联征并因之得名[2]，当时Chiari的描述是胸中部疼痛或吞咽困难，前兆性出血和致命性出血。Carter等于1978年对24例主动脉食管瘘的尸检研究发现[3]：80%的患者在致命性大出血前有前兆性出血。有关Chiari三联征的发生机制， Amin等认为[4]，胸痛可能是由于主动脉壁的扩张、腐蚀或局部切割，纵隔炎，食管穿孔，或肿瘤侵犯主动脉、胸膜等所致。前兆出血暂时停止可能是由于动脉壁痉挛，血管内压力下降或主动脉周围血肿等因素而致瘘口暂时闭塞。随后闭塞的瘘口被感染或胃肠道消化液再次腐蚀掉，出现致命性大出血。Chiari三联征可发生于任何原因所致的主动脉食管瘘患者，包括主动脉夹层动脉瘤，食管及气管的恶性肿瘤，手术后并发症，食管异物等。Chiari三联征的提出对我们进一步认识主动脉食管瘘这一高度致命性疾病的发病机制及发病规律具有重大的理论意义和临床价值。在前兆性出血后的数小时至数天的时间内，外科医生有了较充足的诊断及处理时间，使得主动脉食管瘘的手术治愈成为可能[5]。

Loss of fragile histidine triad protein expression in inflammatory bowel disease

AIM： To investigate the expression of fragile histidine triad （FHIT） protein in 64 patients with ulcerative colitis （UC） and Crohn＇s disease （CD）, and its relation with clinicopathological data. METHODS： Rabbit-anti-FHIT antibody was used to detect FHIT protein expression in 64 formalin-fixed, paraffin-embedded tissue specimens of inflammatory bowel disease （IBD） by citrate-microwave-streptavidin （SP）-HRP immunohistochemical method. RESULTS： The positive FHIT protein expression was 22.79% ± 16.16%, 42.14% ± 16.82% in active and remittent phases of UC, 36.07% ± 19.23% in CD, and 57.05% ±8.86% in normal colon mucosa. Statistically significant differences in FHIT protein expression were observed between the active and remittent phases of UC, between the active phase of UC and normal colon mucosa, as well as between the remittent phase of UC and normal colon mucosa, and between CD and normal colon mucosa. CONCLUSION： Our results show that FHIT protein expression is completely absent or reduced in IBD, suggesting that the FHIT gene might be associated with the oncogenesis and progression of IBD, an early event from inflammatory conditions to carcinoma in IBD.

中性粒细胞胞外诱捕网与Virchow′s triad的联系及其在静脉血栓栓塞症中的研究进展

静脉血栓栓塞症(VTE)包含深静脉血栓形成(DVT)与肺血栓栓塞症,是常见的一种心血管疾病。其中,DVT的Virchow三要素包括血管内皮损伤、血液高凝状态和静脉血流滞缓。此外,激活的中性粒细胞释放中性粒细胞外诱捕网(NETs)也参与VTE的发病过程。NETs由解聚的染色质和各种蛋白质组成,它可以参与血管内皮损伤、促进血液达到高凝状态并导致静脉血流滞缓,从而影响VTE的进展。因此,更好地理解NETs在VTE病理生理过程中的作用对于其早期诊断、预防和治疗具有重要意义。

NUMERICAL STUDY FOR RESONANT TRIAD INTERACTION AND SECONDARY INSTABILITY IN TWO-DIMENSIONAL SUPERSONIC BOUNDARY LAYER

By direct numerical simulation, a supersonic boundary layer was studied to see whether the mechanism for the generation of sub-harmonic waves, similar to those for the incompressible flows, existed in the process of laminar-turbulent transition. The results showed that mechanisms of both resonant triad and secondary instability did exist. Discussions were made on whether these two mechanisms are really important in laminarturbulent transition.

Familial primary pigmented nodular adrenocortical disease without Carney complex (CNC): A case report and review of literature

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of familial Cushing's syndrome. It is characterized by bilateral adrenocortical hyperplasia with small to normal-sized adrenal glands containing multiple small adrenal cortical pigmented nodules [1,2]. PPNAD may occur in an isolated form or as familial PPNAD. Familial cases of PPNAD are usually associated with Carney complex in which Cushing’s syndrome is the most common endocrine manifestation [3]. Familial cases of PPNAD without associated Carney complex are very rare. Only a few cases of familial isolated PPNAD have been reported in the literature, mostly in females [4]. Isolated familial PPNAD has got a better prognosis than familial PPNAD associated with Carney Complex. This observation has important consequences for clinical management, follow-up and genetic counselling of such patients. Familial cases of PPNAD are rare and mostly present in females with associated Carney complex. We herein report a case of familial Cushing’s syndrome in male siblings due to PPNAD without associated Carney complex.

Fazari’s Sign in Ultrasound Scan of Morbid Adherent Placenta/Placenta Accreta Spectrum (MAP/PAS) and Fazari’s Triad during (MAP/PAS) Surgery

Morbid Adherent Placenta (MAP)/Placenta Accreta Spectrum (PAS) is a serious diagnosis which has a risk of complications. Ultrasound scan helps in early diagnosis and has great value in further confirmation and follow up. Observed new clinical sign and associated clinical triad are discussed here.

Carnoy's预固定剂量对中期染色体分散度的影响

目的定量研究Carnoy's预固定剂量变化对外周血淋巴细胞中期染色体分散度的影响。方法用0．075 mol／L KCl对经培养的外周血淋巴细胞于37℃低渗处理35 min;在预固定环节,Carnoy's液的量分别控制在1．25％、2．50％、3．75％、6．25％和12．25％(终体积浓度);常规制备中期染色体标本,Giemsa染色后观察不同预固定剂量下淋巴细胞及中期染色体的分散质量,计算中期染色体铺展的面积及染色体的分散质量。结果当预固定剂量占总体积的1．25％、2．50％或3．75％时,染色体的分散质量较好,可用核型多,当预固定剂比例≥16．25％后,细胞间相互黏连的程度和染色体间相互积聚的倾向愈发明显。对染色体铺展面积的定量结果显示,预固定剂比例为1．25％、2．50％和3．75％时,分裂相核型的平均分布面积分别为1 246±236 mm2、1 143±178 mm2和1 017±163 mm2,均显著高于预固定剂用量为6．25％和12．25％时中期分裂相核型的平均分布面积:876±81 mm2和518±65 mm2,双侧t检验显示组间比较差异显著(P<0．05)。结论在染色体标本制备过程中,预固定剂的用量是最终影响染色体分散质量的重要因素之一。

散发性结直肠癌组织中FHIT、MSH2蛋白的异常表达及其临床意义

背景与目的:脆性组氨酸三联体(fragilehistidinetriad,FHIT)蛋白表达缺失是胃肠道肿瘤的频发事件,但在大肠癌却有争议;最近研究表明FHIT蛋白表达失活可能是错配修复蛋白,尤其是mutS同种组织蛋白2(mutShomolog2,MSH2)改变的结果。本研究旨在探讨FHIT、MSH2蛋白在散发性结直肠癌(sporadiccolorectalcarcinoma,SCC)组织中的表达情况及其临床意义。方法:采用免疫组化SP法检测手术切除的84例SCC及其对应癌旁正常结直肠组织和23例肠腺瘤组织标本中FHIT、MSH2蛋白的表达。结果:FHIT蛋白在SCC组织、结直肠腺瘤组织、癌旁正常结直肠组织中阳性率分别为48.81%、73.91%和100%,三者的阳性率差异有显著性(P<0.05)。FHIT蛋白表达水平与SCC患者的年龄、性别及肿瘤部位、组织学类型无关(P>0.05),而与肿瘤浸润深度、分化程度、Dukes分期和淋巴结转移有关(P<0.05),在浸润深度越深、分化程度越低、Dukes分期越晚和有淋巴结转移的癌组织中,FHIT蛋白低表达就越明显;而MSH2蛋白表达水平仅与Dukes分期有关(P<0.05)。SCC中FHIT蛋白表达与MSH2蛋白表达呈正相关(r=0.3728,P<0.01)。结论:(1)FHIT蛋白表达水平与SCC的恶性程度有关,可作为预测SCC浸润转移潜能的一项有意义的生物学指标;(2)SCC中FHIT、MSH2蛋白表达二者之间呈正相关。

Carney三联征临床病理分析1例

目的:探讨Carney三联征的临床病理特征、生物学行为及预后.方法:收集1例Carney三联征临床资料,光镜下观察其组织形态学特征并行免疫组织化学分析,对相关文献资料进行回顾分析与总结.结果:患者年轻女性,先后出现肺多发性软骨瘤及胃肠道间质瘤.镜下肺软骨瘤由境界清楚的软骨小叶构成,小叶间被纤维血管分隔;胃肠道间质瘤表现为胃黏膜下多发结节,镜下肿瘤细胞呈巢团状在肌壁间浸润性生长,瘤细胞呈上皮样,圆形或多角形,胞质丰富红染,显中度异型性,核分裂像易见;免疫组织化学染色肿瘤细胞CD34、CD117、Vimentin和PDGFRA阳性.结论:Carney三联征好发于年轻女性,包括肺多发性软骨瘤、胃肠道间质瘤和肾上腺外副神经节瘤,可同时出现,也可仅存在二联征.

胃肠间质瘤并发肺软骨瘤的不全性Carney三联征

Carney三联征是非家族遗传性综合征,包括胃部的胃肠间质瘤、肺软骨瘤及肾上腺外副神经节瘤。患者可表现为完全性或不完全性的三联征表达,且肿瘤往往为多发性。在此,我们报道1例37岁的不全性Carney三联征患者,其表现为多发的胃GISTs及肺软骨瘤。患者接受了胃部分切除及伊马替尼辅助治疗2年。在随后的5年随访时间里,患者未出现病情进展。通过对本例病例的报道及相关文献复习,我们认为当发现Carney三联征以肿瘤类型表达时,应注意三联征存在的可能性,尤其是对于年轻女性患者,长期随访有助于三联征的发现。

Anaphylaxis and Undiagnosed Aspirin Exacerbated Respiratory Disease in the Ambulatory Surgery Center: A Case Report

Severe bronchospasm and anaphylaxis are unanticipated emergencies that may occur in the ambulatory surgery setting. I present a case in which an asthmatic male with nasal congestion has anaphylaxis after induction, with severe bronchospasm as the primary manifestation. During the course of hospitalization, he was exposed to aspirin and a second episode of severe bronchospasm occurred. He was diagnosed with both anaphylaxis to an anesthetic medication and Aspirin Exacerbated Respiratory Disease, or Samter’s Triad.

Rare Case of Spontaneous Perinephric Hematoma with Two-Year Follow-Up

Background: Spontaneous perinephric hematoma with no associated pathology or provocation is a rare clinical phenomenon. The hematoma requires a two-year interval for a favorable hematoma resolution, and no associated hypertension or renal scarring. Aims: Evidence of the efficacy of conservative management for spontaneous perinephric hematoma with a 2-year follow up. Case Presentation: A previously healthy 38-year-old woman, presented with a sudden onset of left flank pain, associated with fatigue and pallor. The patient remained hemodynamically stable with no significant history or associated provocations identified. Conclusion: The acute management strategy is favorable in such condition, as the hematoma remains under tamponade in the retroperitoneal space, regardless of the size and organ displacement. Closed observation, serial blood investigation and repeated CT scans are vital to assist in the decision to intervene.

An unusual case of Carney triad with high level catecholamine- secreting but no existence of extra-adrenal paraganglioma

In 1977, Carney originally described the association of .gastric epithelioid leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma, and this unusual syndrome was subsequently called ＂Carney triad＂. At present, the diagnosis of Carney triad, requires at least two of three components-gastrointestinal stromal tumor （GIST）, pulmonary chondroma and extra-adrenal paraganglioma. Up to now, fewer than 80 cases of Carney triad have been reported worldwide.1 ^We described a case of incomplete Carney triad with multiple pulmonary chondromas and GIST. Although the patient was detected in high level secreting of catecholamine, no extra-adrenal paraganglioma has been found.

“女运动员三联征”的研究现状及进展

"女运动员三联征"是女运动员参加剧烈运动所遇到的特殊医学问题,由于其具有隐蔽性,可能给女运动员机体带来一系列潜在和远期的危害,因而成为近年来相关领域研究的新热点。就"三联征"对女运动员健康的影响进行了综述,对其病因及发病机理进行了分析,并对其预防和治疗提出了相应的措施及方法。

胰岛素瘤的诊断与治疗(附25例报告)

胰岛素瘤在临床上较为少见，作者通过对我院收治２５ 例患者的总结，进而探讨该病的定性、定位诊断及手术治疗。其中１０ 例患者具有 Ｗｈｉｐｐｌｅ 三联征表现并因此而得到早期诊断；１２ 例行选择性腹腔动脉造影，１０ 例明确肿瘤的部位，准确率为８３ ％ ，而Ｂ 超、ＣＴ 检查分别为１６ ％ （４／２５） ，４０ ％ （８／２０） 。２５ 例患者均实施手术治疗，５ 例行胰体尾切除，２０ 例行肿瘤摘除。１５ 例行术中血糖监测，切除肿瘤１ 小时后，血糖值均达到正常水平。因此我们认为，Ｗｈｉｐｐｌｅ 三联征是胰岛素瘤定性诊断的主要依据； 选择性腹腔动脉造影对定位诊断有重要价值；诊断一旦成立，应及时手术切除肿瘤，术中血糖监测是判断肿瘤是否完全切除的有效方法。

如何让囚徒走不出困境——三人博弈理论及其应用

以囚徒困境为代表二人博弈模型在学术界已经被广泛研究并加以应用。囚徒困境理论往往容易形成一种简单化和两极化的固定思维模式。囚徒困境的二人博弈模型实际上是三人博弈模型的简化。而三人博弈模型在现实生活中比二人博弈模型更加贴近现实,更具有解释力。分析三人博弈模型的结构及其理论意义,并以刑事诉讼法为例对三人博弈模型的应用进行探讨很有必要。

Aspirin-Exacerbated Respiratory Disease: The Dentist’s Role in Recognition, Referral, and Management

Aspirin-exacerbated respiratory disease (AERD), also known as Samter’s triad, is characterized by aspirin intolerance, nasal polyps with recurrent rhinitis, and asthma. The components of AERD are frequently encountered in dental offices. This article reviews the screening and appropriate referral of patients who may have AERD. The implications of AERD on a patient’s daily life, as well as a brief overview of potential treatments, are followed by a case report of a 36-year-old female patient. In the case report, a 36-year-old female patient with a history of chronic sinusitis, asthma, and aspirin allergy presents to a dental office with no evidence of oral disease. The case then describes the referral process to Otorhinolaryngology (ENT) for further evaluation, the diagnosis of Samter’s triad, and subsequent treatment through endoscopic sinus (ESS) surgery to remove nasal polyps. The patient experienced relief of symptoms at the one-year postoperative follow-up. As dental providers often serve as a frequent point of contact for many patients—some of whom may see their dentists more regularly than their primary care providers—we are uniquely positioned to recognize signs and symptoms in the head and neck region that may indicate systemic disease.

原发性肺软骨瘤的外科诊治分析

目的探讨原发性肺软骨瘤的临床特点及外科诊治方法。方法回顾性分析四川省人民医院2005年1月至2017年11月手术治疗且病理确诊的20例肺软骨瘤的临床资料,其中开胸手术5例,腔镜手术15例,6例行肺叶切除术,14例行肺楔形切除术。结果全组无术前确诊病例,手术均顺利完成,无围手术期死亡,2例发生并发症。手术时间(112.5±50.2)min,术中失血(58.5±31.0)m L,术后留置胸腔引流管时间(3.9±2.5)天,术后住院天数(5.4±2.3)天。随访16例,平均73.8月,1例死于非肿瘤性病变,余均无复发、转移、并发Carney综合征等。结论肺软骨瘤临床表现缺乏特异性,容易误诊,外科手术切除是诊断和治疗肺软骨瘤的有效措施。手术效果良好,但需终身随访。

二维超音速边界层中三波共振和二次失稳机制的数值模拟研究

通过直接数值模拟的方法,探讨在超音速边界层的转捩问题中,是否存在和不可压缩流情况相似的产生亚谐波的机制.结果表明,三波共振和二次失稳这两种机制都存在.讨论了这两种机制在层流至湍流的转捩中的重要性是否的确很大的问题.