阵发性睡眠性血红蛋白尿症继发缺血性肠病的临床特点分析

目的 提高临床医师对阵发性睡眠性血红蛋白尿症(paroxysmal nocturnal hemoglobinuria, PNH)继发缺血性肠病的认识。方法 对我院1例及文献报道的15例PNH合并缺血性肠病的患者临床资料进行综合分析。结果 16例患者中，男7例，女9例，确诊年龄(41.0±9.6)岁(27～64岁),中位诊断用时42个月。病变分布方面，十二指肠及空回肠13例(81.3%),结肠3例(18.8%),胃2例(12.5%),盲肠1例(6.3%)。临床表现方面，表现为程度不一的腹痛(16/16),多因急腹症就诊，伴呕吐(9/16)、发热(4/16)等。单纯PNH继发缺血性肠病14例；AA-PNH综合征继发缺血性肠病2例。诊断以内镜检查为主，影像学及手术病理为辅。治疗方面，9例行手术治疗，其中3例术后复发，1例死亡；7例保守支持治疗，手术治疗复发率较保守治疗低。结论 PNH继发缺血性肠病罕见，临床表现主要为腹痛，内镜检查及病理有助于诊断，治疗后易复发，建议预防性抗凝治疗。     

结肠镜检查后并发缺血性肠病2例报道

随着人口老龄化、动脉硬化相关疾病发病率增加,缺血性肠病的患病率也逐年递增,临床上医源性缺血性肠病较少见,对2例结肠镜检查后并发缺血性肠病病例进行分析,提高临床医生对该病的认识。     

阵发性睡眠性血红蛋白尿患者并发大脑上矢状窦血栓形成1例并文献复习

目的:提高对阵发性睡眠性血红蛋白尿(PNH)并发血栓形成的认识。方法:报告1例以癫痫发作为首发表现的并发大脑上矢状窦血栓形成的PNH患者。结果:该患者诊断PNH后长期口服糖皮质激素治疗,在甲泼尼龙冲击治疗期间出现癫痫;通过磁共振大脑静脉造影确定该患者为大脑上矢状窦静脉血栓形成。结论:PNH是一种高凝状态性疾病,静脉血栓形成是其主要并发症和首要致死原因。PNH患者并发血栓形成的概率与患者的PNH克隆负荷密切相关。在PNH的诊治过程中应密切监测患者的凝血功能,避免血栓事件的发生。     

15例高原红细胞增多症合并缺血性肠病临床分析

目的 总结高原红细胞增多症（Highaltitude polycthaemia,HAPC）合并缺血性肠病（ischemic bowel disease,IBD）的诊治体会.方法 回顾分析2003年1月至2005年12月高原红细胞增多症合并缺血性肠病的临床资料.结果 高原红细胞增多症合并缺血性肠病可累及全消化道,诊断有一定困难,常易误诊,延误病情.主要通过结肠镜及钡灌肠确诊,肠系膜血管造影可用于缺血性小肠病的早期诊断.治疗主要通过扩血管、活血化瘀及营养支持等治疗.早期诊断可明显减少该病的手术率及死亡率.结论 高原红细胞增多症患者合并缺血性肠病的机会增多,是诱发缺血性肠病的危险因素,应引起临床医师的重视.     

产气单胞菌溶素前体变异体技术诊断阵发性睡眠性血红蛋白尿症临床特点总结

目的总结以产气单胞菌溶素前体变异体(FLAER)技术诊断的阵发性睡眠性血红蛋白尿症(PNH)患者的临床特点,提高对PNH的认识。方法回顾性分析2011年9月至2015年3月在南通大学第三附属医院血液科和江苏省人民医院血液科经FLAER诊断的98例以流式细胞术FLAER技术诊断的PNH患者资料,对临床表现、实验室检查结果、并发症进行分析归纳。结果通过FLAER技术诊断经典型PNH 43例,PNH合并骨髓疾病45例,亚临床型PNH 10例。70例有疲乏表现,41例以血红蛋白尿为主要表现,6例合并血栓形成(多为非常见部位),17例发生肾功能损害,19例合并肝功能损害,肺动脉高压仅2例。经典型PNH更易发生溶血,PNH合并骨髓疾病者造血功能衰竭特征较明显。增高的乳酸脱氢酶(LDH)值与PNH粒细胞克隆数间存在线性关系,且PNH粒细胞克隆数较高者更易发生血栓。结论 PNH患者以溶血及骨髓衰竭为主要表现,易见肝肾损伤及少见部位血栓,肺动脉高压少见。     

重型再生障碍性贫血患者经抗淋巴细胞球蛋白治疗后的血细胞形态学

重型再生障碍性贫血(SAA)患者经抗淋巴细胞球蛋白(ALG)治疗缓解后,可并发阵发性夜间血红蛋白尿(PNH)和骨髓增生异常综合征(MDS)。作者回顾性分析经 ALG 治疗后的 SAA患者血细胞形态学特点及其与 PNH、MDS 的关系。病人和方法 1976～1990年经 ALG 治疗的 SAA 共117例。其中26例晚期并发克隆性疾病:PNH 15例,MDS 9例,PNH 和 MDS 同时存在2例。并发克隆性疾病患者14年生存率为36%±13%,显著低于无并发症者(81%±10%,P=0.001)。对全部患者治疗前、缓解后的外周血,以及治疗前、治疗后1和3个月、缓解后的骨髓涂片进行全面的重新评价,分析 ALG 治疗的 SAA     

阵发性睡眠性血红蛋白尿并发颅内静脉窦血栓形成一例

颅内静脉窦血栓形成(CVST)是一种相对少见的特殊类型脑血管病,是指患者颅内静脉窦或静脉出现血栓和闭塞,其病因不同且临床表现缺乏特异性,因此容易出现漏诊、误诊。作者报道了1例以头痛起病,阵发性睡眠性血红蛋白尿(PNH)并发CVST的患者,结合国内外文献进行了分析,探讨了CVST的发病机制、治疗方法及如何获得更好的预后,以期提高临床医师对PNH并发CVST的认识。     

肠脂肪酸结合蛋白在缺血性肠病中的研究进展

近年来随着社会人口的日益老龄化,动脉硬化所致的疾病发病率增加,缺血性肠病的患病率也有所增加.由于临床症状不典型,缺血性肠病的误诊率、漏诊率较高,关于缺血性肠病的早期诊断手段尚在研究中.肠脂肪酸结合蛋白富含于小肠粘膜组织中,具有以下特点：（1）存在于胞浆中的可溶性蛋白;（2）具有高组织特异性;（3）组织含量丰富;（4）分子质量低,易于检测.目前肠脂肪酸结合蛋白广泛用于缺血性肠病诊断的研究.本文综述了肠脂肪酸结合蛋白的定义以及作用机制,并且从动物实验和临床实验两方面总结了肠脂肪酸结合蛋白在缺血性肠病诊断中的研究进展.     

上消化道出血并脑梗死16例分析

上消化道出血是临床常见急症之一，缺血性脑梗死在临床上比较多见，但急性上消化道出血并发脑梗死却少见。本文就我院自1999年1月至2009年1月收治的急性上消化道出血并发缺血性脑梗死患者16例的临床资料分析报告如下。     

探讨两种无创方法——肠道血管64排CT和结肠镜检查对急性缺血性肠病的诊断价值

目的 分析缺血性肠病的无创性诊断方法,并进行比较.方法 回顾性分析我院2007～ 2011年经临床证实的70例缺血性肠病患者的临床资料.结果 70例患者中,结肠镜检查结果提示缺血性肠病的有69例,64排CT检查结果提示缺血性肠病的有67例.两种方法诊断率均大于95％,且无统计学差异.结论 电子结肠镜和肠道血管64排C...     

12例缺血性肠病的临床分析

目的分析缺血性肠病（ischemic bowel disease,IBD）的临床特点、诊断及治疗。方法回顾性分析12例患者临床表现、合并症、腹部血管CT成像（CT angiography,CTA）或腹部血管彩超、电子结肠镜、治疗等。结果急性缺血性肠病3例,2例经手术治疗,1例发病72h内死亡。慢性缺血性肠病9例,8例经内科治疗好转,1例死亡。结论急性缺血性肠病多见于肠系膜上动脉闭塞,早诊断、早治疗较重要,出现肠坏死预后差。慢性性缺血性肠病多见于肠系膜上、下动脉狭窄及灌注不足,内科治疗疗效良好。肠道排空障碍可能是IBD的早期表现。     

Muscoloskeletal manifestations in inflammatory bowel disease.

Muscoloskeletal manifestations are the most common extraintestinal complications of inflammatory bowel disease. Wide ranges in prevalence have been reported, depending on the criteria used to define spondylarthropathy. In 1991, the European Spondylarthropathy Study Group developed classification criteria that included previously neglected cases of undifferentiated spondylarthropathies, which had been ignored in most of the oldest epidemiological studies on inflammatory bowel disease. The spectrum of muscoloskeletal manifestations in inflammatory bowel disease patients includes all of the clinical features of spondylarthropathies: peripheral arthritis, inflammatory spinal pain, dactylitis, enthesitis (Achilles tendinitis and plantar fasciitis), buttock pain and anterior chest wall pain. Radiological evidence of sacroiliitis is common but not obligatory. The articular manifestations begin either concomitantly or subsequent to the bowel disease; however, the onset of spinal disease often precedes the diagnosis of inflammatory bowel disease. The prevalence of the different muscoloskeletal manifestations is similar in ulcerative colitis and Crohn's disease. Symptoms usually disappear after proctocolectomy. The pathogenetic mechanisms that produce the muscoloskeletal manifestations in inflammatory bowel disease are unclear. Several arguments favour an important role of the intestinal mucosa in the development of spondylarthropathy. The natural history is characterized by periods of flares and remission; therefore, the efficacy of treatment is difficult to establish. Most patients respond to rest, physical therapy and nonsteroidal anti-inflammatory drugs, but these drugs may activate bowel disease. Sulphasalazine may be recommended in some patients. There is no indication for the systemic use of steroids.     

Recurrent symptomatic ischemic stroke in a 46-year-old African male revealing Angio-Behçet with severe cardiovascular involvement

Behçet’sdisease (BD) is a chronic, multisystem vasculitis. It is categorized under variable vessel vasculitis in the new Chapel Hill nomenclature as it involves blood vessels of any type and size. It is characterized by relapsing aphthous ulcers commonly occurring in the oral mucosa and genitalia with ocular involvement. Other organ systems may be involved any time throughout the course of the disease. The exact cause is unknown. However, combination of genetic and environmental factors is likely to play a role.Cardiac involvement may occur in the form of intracardiac thrombus, endocarditis, myocarditis, pericarditis, endomyocardial fibrosis, coronary arteritis, myocardial infarction, and valvular disease.We present a case of Angio-Behçet in a 46-year-old African male with severe cardiovascular involvement including pulmonary artery hypertension (PAH), right ventricular failure and left ventricular diastolic dysfunction diagnosed after 2 episodes of symptomatic ischemic stroke resulting from complete occlusion of the right internal carotid artery (ICA) up to its intracranial portion.Immunosuppressive and anticoagulant therapies have induced improvement in cardiac manifestations. Nevertheless, prompt recognition of the primarily vascular manifestation of BD without mucocutaneous manifestations was responsible for considerable delay that did not afford surgical therapy for the carotid occlusion.     

A case of Behcet's disease with intestinal involvement due to Crohn's disease

A 15-yr-old, Arabic male presented with painful, recurrent, self-resolving oral and genital ulcers, erythema nodosum and uveitis. Behcet's disease was diagnosed. A few months later, he experienced anorexia, abdominal pain, diarrhea, and weight loss. Although all routine laboratory and radiologic investigations were negative, colonoscopy revealed the presence of serpiginous ulcers with pseudopolyps and inflamed intervening mucosa in the proximal half of the colon. Therapy with oral steroids was helpful, but the disease exacerbated a few months after prednisone was discontinued. Repeat evaluation showed similar endoscopic findings and, on colonic biopsy, noncaseating granulomas compatible with Crohn's disease were seen. Again, the patient responded well to oral steroids and sulfasalazine. We believe that gastrointestinal involvement in our patient is compatible with Crohn's disease and that screening tests to rule out chronic inflammatory bowel disease should be performed in the presence of gastrointestinal involvement in Behcet's disease. Behcet's disease may be a part of the spectrum of chronic inflammatory bowel disease.     

Intramural gastrointestinal hemorrhage. Clinical and radiographic manifestations.

The clinical and radiographic manifestations of intramural gastrointestinal hemorrhage are presented together with a review of the literature. The observations described are based on the analysis of 20 cases affecting different segments of the gastrointestinal tract. The pathologic process is induced principally by anticoagulant therapy, bleeding diathesis, or abdominal trauma and occurs as a localized, well-defined, intramural mass or as a more diffuse segmental involvement. The clinical features are nonspecific and can vary from mild crampy abdominal pain to severe shock. Other manifestations include rebound tenderness, muscle guarding, and small bowel obstruction. The duodenum and small bowel are the most frequent sites of involvement. The intestinal mucosal folds may be prominent, stretched, and sharply defined or completely affaced, accompanied by a smooth narrowing. There is rapid clinical and radiographic remission with conservative therapy. Intestinal perforations and fibrotic strictures resulting in small bowel obstruction are a rarity, observed only in the posttraumatic variety.     

青黛相关缺血性结肠黏膜损伤的内镜及临床特点分析

目的 分析青黛所致缺血性结肠黏膜损伤的临床特点,提高对该疾病的认识及防治效果.方法 收集北京大学第三医院2005年12月至2010年12月因服用含青黛成分中成药后出现缺血性结肠黏膜损伤的13例患者资料,对其临床及内镜表现进行分析,总结该型结肠黏膜损伤的特点.结果 患者的年龄为(60.6±14.1)岁,男女比例为1:1.6.患者因银屑病或特发性血小板减少性紫癜(11P)服用复方青黛丸(胶囊)或升血小板胶囊,平均服药时间为(3.6±2.3)个月.临床表现为腹痛及血便,13例患者中8例便血前有排水样便的表现.肠镜下病变形态、病理活检符合缺血性损伤的表现,病变较重,并呈慢性炎表现.扩血管治疗疗效明确,平均止血时间(1.7±0.8)d,预后良好.随访3个月均无复发.结论 银屑病和ITP患者服用含青黛成分中成药可导致结肠黏膜损伤,严重者可致缺血性黏膜损伤,临床应用该类药物时应进行认真评估及观察.

Abstract：

Objective By analysing the clinical features of Indigo Naturalis-associated ischemic lesion of colon mucosa to improve the precautionary and therapeutic level of the disease.Methods Thirteen patients diagnosed as Indigo Naturalis-associated ischemic lesion of colon mucosa in Peking University Third Hospital from 2005 to 2010 were reviewed.The endoscopic and clinical features were analysed.Results The 13 patients with an average age of(60.6±14.1)years old were prescribed Chinese traditional medicine containing Indigo Naturalis for psoriasis or idiopathic thrombocytopenic purpura(ITP).The ratio of males to females was 1:1.6.The typical manifestations were abdominal pain and bloody stool with watering diarrhea before bloody stool in 61.5%patients.Endoscopic and pathological characteristics were coincident with ischemic lesion and more like a chronic index.Vasodilatic medicine was effective and the average hemostatic time was(1.7±0.8)days.The prognosis was well and no recurrence was found during 3 months follow-up.Conclusions Patients having psoriasis or ITP treated with Chinese traditional medicine containing Indigo Naturalis have an inclination to colon mucosa lesions, even ischemic lesion. Careful assessment and observation before prescribing arc necessary in these patients.     

SLCO2A1基因相关慢性肠病临床特点及遗传学特征分析

目的:总结分析SLCO2A1基因相关慢性肠病(CEAS)的临床特点及遗传学特征。方法:回顾性分析2012年1月至2019年12月北京协和医院收治的5例CEAS的病例,分析其临床表现、实验室检查、影像学及内镜检查、治疗转归以及家系特点和基因检测结果。结果:5例患者均为男性,胃肠道症状在青春期后出现。主要表现为腹痛、腹泻、间歇性黑便或便血、不全肠梗阻,以及贫血、低白蛋白血症、低钾血症。除食管外的全胃肠道均可受累,以胃及回肠为最常见。小肠病变特征为多发浅溃疡并肠腔狭窄,累及黏膜层及黏膜下层。5例患者均伴发原发性肥厚性骨关节病,1例伴发骨髓纤维化及胸导管发育异常。基因检测提示5例患者均为SLCO2A1基因纯合突变或复合杂合突变。炎症性肠病的常规治疗方法及COX-2抑制剂对该病无效。结论:CEAS是一种可广泛累及胃肠道的常染色体隐性遗传病,可伴发皮肤及骨骼受累,目前尚无特效治疗药物,临床上需注意与其他炎症性胃肠道疾病相鉴别。     

Mesenteric involvement in giant cell arteritis. An underrecognized complication? Analysis of a case series with clinicoanatomic correlation

We reviewed the clinical manifestations of mesenteric vasculitis due to giant cell arteritis (GCA) and considered features of the mesenteric anatomy in relationship to disease expression. We compiled and reviewed a case series by systematic identification of patients previously reported in the English-language literature to have mesenteric involvement from known GCA. Included in the analysis was a detailed case review of a patient with GCA and small bowel infarction seen at our institution. Twelve patients were identified with mesenteric ischemia attributed to GCA. Concomitant cranial and abdominal symptoms were present in 7 of the 12 patients, and cranial symptoms were absent in 5 patients who presented with abdominal complaints. The abdominal symptoms fell within a spectrum ranging from chronic postprandial symptoms to acute abdominal pain. Survival was observed in only 6 of the 12 cases, 3 of whom required bowel resection and were treated with high-dose corticosteroids. Review of the anatomic features of the specialized splanchnic circulation reveals an extensive collateral network that may protect against early disease expression from ischemia, despite mesenteric arteritic involvement. Mesenteric vasculitis resulting in small bowel infarction has only rarely been described in GCA but represents a serious and potentially treatable complication. We propose an explanation, based on mesenteric vascular anatomy, for the infrequency of symptomatic expression of this entity and suggest that occult mesenteric GCA may be present far more often than recognized.     

Clinical and serological spectrum of systemic lupus erythematosus in greek children

Summary In the present study 19 Greek Caucasian children with systemic lupus erythematosus (SLE), onset before the age of 16, were followed up for 1–12 years (mean 5.6 yrs.). Diagnosis was determined early in 14 patients and delayed by 2 to 6 years in 5. The clinical manifestations and laboratory findings did not differ significantly from those reported in adults with lupus. The major organ system involvement at onset and early course were skin and joints (80%) followed by kidneys (42%). During the course of the disease 26% of the children developed central nervous system (CNS) involvement. All the patients were treated with steroids and/or cytotoxic drugs in severe uncontrolled progressive disease. At the mean 5.6 years follow-up most patients were in remission on small doses of steroids; one patient still presents signs of active lupus nephritis and one patient died from sepsis. All the patients with CNS involvement recovered without permanent CNS residue.     

Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

AIM To investigate the characteristic radiologic findings of cryptogenic multifocal ulcerous stenosing enteritis(CMUSE) which can be differentiated from other similar bowel disease and to assess their clinical behavior.METHODS Twenty pathologically and clinically confirmed CMUSE patients(males:females = 8:12; mean age: 40.4 years) between March 2002 and August 2015 from seven academic centers in South Korea were retrospectively reviewed. We evaluated small bowel series(SBS; n = 25), computed tomography(CT) enterography(n = 21), magnetic resonance(MR) enterography(n = 2), and abdominopelvic CT(n = 18) images, focusing on enteric and perienteric manifestations. Any change in radiologic features during followup period was recorded. We evaluated clinical data including presenting symptoms, laboratory finding and presence of relapse from electronic medical records. Histopathologic findings were also evaluated. RESULTS The main symptoms were abdominal pain(n = 12) and anemia(n = 10). All patients showed small bowel strictures(n = 52, mean: 2.6 per patient) on initial CT/MR, located in the ileum(n = 47) or jejunum(n = 5). Strictures showed short-length(mean: 10.44 mm) and circumferential bowel wall thickening(mean: 5.56 mm) with layered enhancement(n = 48) that were also noted on initial SBS(n = 36) with shallow ulcers(n = 10). Some ulcerative lesions or wall thickening progressed into strictures on follow-up SBS/CT, and some strictures revealed recurrent ulceration on followup SBS. There were no penetrating disease features like fistula or abscess and no gastrointestinal tract involvement except the small bowel. Nine patients experienced disease recurrence(median relapse-free period: 32 mo) even post-operatively. Histopathologic features of surgically resected specimens were characterized as multiple superficial ulcerations confined to mucosa or submucosa and multiple strictures.CONCLUSION Under characteristic radiologic findings with multiple short-segmental strictures and/or shallow ulcers of the small intestine, CMUSE should be considered when assessing patients with recurrent abdominal pain and anemia.