46例急性多灶性脑出血的临床研究

目的研究急性多灶性脑出血（AMCH）的病因、发病机制及临床特点。方法对本院46例急性多灶性脑出血患者的临床诊疗资料进行回顾性分析。结果AMCH占同期脑出血患者的3．3％,主要病因为高血压（33例,占71．7％）,脑血管畸形（2例,占4.3％）,白血病（3例,占6．5％）,脑淀粉样血管病（4例,占8．7％）,原因不明（4例,占8．7％）。出血好发部位依次为基底节（25例,占543％）,脑叶（17例,占37．0％）,脑干（3例,6．5％）和小脑（1例,2．2％）。临床表现为不同程度的意识障碍和肢体活动障碍。结论AMCH病因多为高血压,其虽发病率低,但起病急,病情危重,病死率高,需要临床医生掌握其临床特点,及时给予相应的治疗。     

儿童盆腔及泌尿生殖系横纹肌肉瘤的尿路造影和CT表现及诊断价值分析

目的探讨儿童盆腔及泌尿生殖系RMS的尿路造影和CT表现及诊断价值。方法对2012年3月至2014年3月我院收治的40例盆腔及泌尿生殖系RMS患儿的影像学资料进行回顾性分析。结果 16例膀胱RMS患儿的IVP、CT均表现为有葡萄球状充盈缺损存在于膀胱内,VCUG可见肿物向后尿道延伸;8例前列腺RMS患儿表现为膀胱颈部受到尿道肿物压迫抬高,环绕尿道的盆腔底部有包块;12例盆腔RMS患儿的IVP、CT均表现为盆腔有包块,膀胱受压迫移位变形;2例尿道RMS患儿表现为有充盈缺损存在于尿道内;2例阴道RMS患儿的IVP表现为有充盈缺损存在于膀胱颈部。结论儿童盆腔及泌尿生殖系RMS的尿路造影和CT具有较高的诊断价值。     

炎症性肠病的胰腺表现

临床上,炎症性肠病(IBD)除了病变本身引起的胃肠道症状外,还可能出现全身多个器官、系统的症状,称为IBD的肠外表现(EIM)。IBD治疗药物也可引起EIM。EIM可发生于IBD诊断之前或之后,或同时出现,可显著影响患者的功能状态和生活质量。本文对IBD患者可能出现的胰腺表现进行概述,并简要阐明其发生机制。鉴于EIM的错综复杂,临床工作者应予足够重视,从而有利于及早诊断和治疗。     

不同前纵隔病变的CT特征性表现特点及CT诊断

目的探讨不同前纵隔病变的CT特征性表现特点及CT诊断价值。方法选取我院于2012年3月-2014年2月收治的39例病理诊断确诊前纵隔病变患者为研究对象,均予以病理检查及CT影像学检查,记录相关检查结果,以病理检查结果为"金标准"评估CT诊断的准确性、特异性、敏感性,记录漏诊率及误诊率。结果此次入组的39例患者病理检测结果显示确诊良性病变共33例(84.6%),其中胸腺瘤19例(48.7%),畸胎瘤11例(28.2%),纵膈淋巴瘤3例(7.7%);确诊恶性病变共6例(15.4%),其中胸腺瘤3例(7.7%),畸胎瘤2例(5.1%),纵膈淋巴瘤1例(2.6%);以病理检测结果为"金标准"评估CT检测准确性,显示CT正确诊断恶性病变4例,良性病变32例,其中假阴性2例,假阳性0例,诊断准确性为92.3%(36/39),特异性为97.0%(32/33),敏感性为66.7%(4/6),漏诊率为0.0%(0/3 9),误诊率为7.7%(3/39)。结论 CT检查能有效提升前纵隔病变患者术前诊断准确性及有效性,可作为重要辅助诊断手段应用于临床诊疗中,以此缩短诊疗时间,提高诊疗效率。     

母细胞性浆细胞样树突细胞肿瘤41例临床分析

目的总结母细胞性浆细胞样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm,BPDCN)的诊治要点及预后,为临床进一步认识和诊治该病提供依据。方法以"母细胞性浆细胞样树突细胞肿瘤"、"母细胞性NK细胞淋巴瘤/白血病"或"无颗粒型CD4+CD56+血液皮肤肿瘤"及"BPDCN"为检索词,检索2009年1月—2013年12月万方数据库、中国知网(CNKI)和Pub Med发表的相关病例报道并对其资料进行回顾性分析。结果经检索共筛选出26篇文献BPDCN 41例,男29例,女12例;平均63.7岁。临床主要表现为皮肤受损伴淋巴结和骨髓受累,肿瘤细胞表达CD4、CD56、CD123,不表达髓系、T细胞及B细胞特异性标志。本组41例采用淋巴瘤、急性髓系白血病、急性淋巴细胞白血病方案治疗或单药化疗,其中33例达血液学缓解,有20例完全缓解;15例在化疗后予异体移植,其中14例达血液学缓解。41例平均生存期为12.3个月。结论 BPDCN以老年人好发,诊断依赖于临床资料、组织病理学和免疫组织化学标记,病程呈侵袭性,预后多较差。     

Incidence des troubles digestifs sur l’état respiratoire des enfants porteurs de handicap

Gastrointestinal manifestations are common in children with disability and concern more than 60% of them. They are involved in many complications, especially in respiratory disorders (persistent congestion, aspiration pneumonia). It is mainly the oromotor dysfunction (OMD) and the gastroesophageal reflux disease (GERD) sometimes associated to delayed gastric emptying, since they are source of oropharyngeal aspirations or pneumonia in case of massive GERD. The OMD is diagnosed during clinical examination and may require in some cases an exploration of swallowing. Its management mainly includes the modification of food texture. In severe forms prohibiting oral feeding, a gastrostomy is indicated. In case of DOM associated to severe GERD, antireflux surgery such as Nissen fundoplicature is coupled with gastrostomy. GERD is explored by 24 h pH monitoring if non-externalized. The upper digestive tract endoscopy is only indicated in case of suspected esophagitis. Only proton pump inhibitors had demonstrated efficacy in GERD and should be prescribed in combination with other measures such as right lateral decubitus positioning in some patients. Antireflux surgery is indicated in case of severe complicated GERD resistant to medical treatment. Due to their central role in respiratory complications, digestive disorders should be systematically investigated and managed to improve the quality of life of children with disabilities.     

Syndrome d’Evans : attention aux diagnostics par excès

Introduction

Evans syndrome (ES) is characterized by the coexistence of an autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Despite being frequently evocated in the simultaneous presence of anemia and thrombocytopenia, this rare disease only accounts for 0.8 to 3.7% of patients with ITP or AIHA.

Case reports

We report three suspected cases of ES, diagnosed in the presence of thrombocytopenia and hemolytic anemia association, with a positive direct Coombs test in two patients. Standard ES treatment failure and occurrence of aditional features subsequently led to correct diagnosis to thrombotic thrombocytopenic purpura, myelodysplastic syndrome with AIHA, and ITP with hemorrhagic anemia, respectively.

Conclusion

Bicytopenias, even in an immunological context, are not sufficient to ascertain ES diagnosis. Our cases illustrate the diagnostic difficulties that may arise in daily practice, and induce over-diagnosis of this rare disease.