Intraabdominal panniculitis. Report of three cases and review of the literature

Intraabdominal panniculitis is a rare, benign idiopathic disorder of the mesentery. Patients usually present with abdominal pain and a palpable mass. The cross-sectional imaging findings are characteristic and consist of a fibrofatty central mesenteric mass lesion encapsulating the mesenteric vessels with displacement of the bowel loops, that can suggest the diagnosis. Imaging is also important to establish a definitive diagnosis by an image-guided percutaneous biopsy, assess extent of the disease for selection of appropriate therapy, exclude associated abnormalities namely malignancies, and for follow-up.     

Castelman's disease. Report of two cases with review of the literature

Castelman's disease, an unusual condition of unknown cause, consisting of a massive proliferation of lymphoid tissue. Three histologic variants (hyaline vascular, plasma-cell, and mixed) and two clinical types (localized and multicentric) of Castelman's disease have been described. Localized disease can be cured with surgery or radiotherapy, but complete remission in patients with multicentric disease have been achieved only with prednisone or chemotherapy given at the time of diagnosis. The aim of this study is to report two cases of retroperitoneal Castelman's disease with review of the literature. The first patient, of 36 years old, presented for abdominal pain with anorexia and weight loss. The abdomino-pelvic CT scan showed a 6 cm retroperitoneal mass. The biopsy of this lesion suspected a lymphoproliferative disease. At laparotomy total excision of mass was made and the final histology revealed a hyalino-vascular type of Castelman's disease. The second patient, of 26 years old, presented for left lombar pain with weight loss. The abdominopelvic CT Scan showed a 6.5 cm retroperitoneal mass. The biopsy of this lesion showed a Castelman's disease. At laparotomy total excision of mass was made and the final histology confirmed a hyalino-vascular type of Castelman's disease.     

Neurological manifestations of angioedema. Report of two cases and review of the literature

Two children had recurrent neurological symptoms associated with angioedema. Extensive clinical and laboratory investigations failed to reveal a separate cause for the neurological changes. Only 19 prior cases of this association have been reported. Seizures and headaches are the most common symptoms, but a wide variety of focal and generalized deficits may occur. The release of vasoactive substances such as histamine, serotonin, and prostaglandins, which are also putative neurotransmitters, is postulated to be the cause of the cerebral symptoms.     

重症病毒性心肌炎--附48例分析并文献复习

目的分析重症病毒性心肌炎的诊断和治疗,旨在避免误诊,提高抢救成功率.方法对1999年5月～2003年5月收治的48例重症病毒性心肌炎患者的临床特点、心电图及诊治经过进行分析.结果本研究48例重症病毒性心肌炎患者中,39例(81.3%)首发临床表现均为胃肠道症状,4例(8.3%)为呼吸道症状,仅5例(10.4%)为心脏症状,呈现不典型性.心电图呈频发室早22例(45.8%),酷似广泛性急性心肌梗死早期图形10例(20.8%),短阵室速8例(16.7%),房室传导阻滞5例(10.4%),窦速伴T波或ST段改变3例(6.3%).病初误诊率85.4%.甲基泼尼松龙冲击治疗组存活率(96.7%)显著大于未使用甲基泼尼松龙的对照组(55.6%),P＜0.05.结论对临床上首发表现为非心脏症状而血流动力学异常的患者,应立即行心电图检查,任何形式的心电图异常均应立即考虑重症病毒性心肌炎的可能.甲基泼尼松龙冲击治疗可阻遏病情的恶化.     

无色素性恶性黑色素瘤7例报道并文献复习

目的:探讨无色素性恶性黑色素瘤(AMM)病理形态学特征、免疫表型特点及鉴别诊断。方法:应用HE染色、免疫组化标记对7例无色素性恶性黑色素瘤(AMM),进行临床资料分析、形态学和组织结构特点的观察。结果:肿瘤组织结构上出现束状、巢状、腺泡状、腺样,形态学上出现梭形细胞、上皮样细胞、横纹肌样细胞、巨细胞、小细胞;免疫组化标记:Vimentin、S-100、HMB45、Melan-A阳性,Syn阳性1例,CgA阳性1例,其中1例Ki-67阳性细胞数达10%,CK、LCA、CD68、SMA、CD21、CD34均阴性。结论:恶性黑色素瘤的组织结构和形态学复杂多样,无色素性恶性黑色素瘤更是增加了诊断的难度,免疫组织化学对其诊断和鉴别诊断有重要价值。     

继发性贲门失弛缓症(5例报告并文献复习)

目的 总结继发性贲门失弛缓症临床特征及误诊原因.方法 经计算机选取2000-2012年本院确诊的贲门失弛缓症患者149例,其中5例为继发性贲门失弛缓症,观察症状持续时间;钡餐下食管形态学改变,包括食管最大宽度及狭窄长度变化;胃镜下贲门黏膜改变;食管下段压力变化,CT检查结果.结果 本组继发性贲门失驰缓症平均年龄(54.0±23.7)岁,症状持续时间平均(4.6±0.7)个月,检查显示食管贲门狭窄长度平均(3.80±0.26)cm,食管最大径平均(4.32±0.64)cm,食管下端括约肌测压(LES)检查平均(40.50±0.70)cmH2O,CT检查提示食管下段贲门及纵隔占位,手术探查病理证实胃黏液腺癌1例,贲门腺癌2例,食管下段鳞状细胞癌1例,纵隔型肺腺癌1例.结论 继发性贲门失弛缓症少见,临床诊断中多存在误诊,延误治疗,应根据患者资料全面认真分析,做出正确判断.     

肺硬化性血管瘤3例报道及文献复习

目的：探讨肺硬化性血管瘤(Sclerosing hernangioma,SH)的形态学特征。方法：复习3例肺SH，均作细胞角蛋白(CK)、上皮性膜抗原(EMA)、嗜铬索A(CgA)、间皮细胞(MC)、突触素(Syn)等免疫组化(SP法)观察。结果：肺SH的组织学形态主要表现为实性区、乳头区、血管瘤样区和硬化区。肿瘤成分主要为多角形细胞和乳头表面的立方细胞。3例立方细胞CK阳性。3例多角形细胞和立方细胞EMA均为阳性。2例部分表达CgA。结论：肺SH的组织结构、组织成分、细胞形态等均有别于炎性增生，应分类于肺的良性上皮性肿瘤。     

丘脑神经节胶质细胞瘤6例并文献复习

目的探讨丘脑神经节胶质细胞瘤(ganglioglioma,GG)的临床特点、治疗和预后。方法对6例经手术和病理证实的丘脑神经节胶质细胞瘤患者的临床资料进行回顾性分析并复习文献。结果丘脑神经节胶质细胞瘤主要临床表现为颅内压增高和偏身运动感觉障碍,患者平均发病年龄12.5岁。6例肿瘤镜下全切除2例,近全切除3例,大部切除1例。术后3例出现对侧肢体偏身运动感觉障碍加重。6例患者平均随访29个月,2例恢复正常工作,3例生活可自理,1例间变性神经节胶质细胞瘤患者复发后死亡。结论丘脑神经节胶质细胞瘤由于缺乏特异的临床症状和神经影像学表现,术前诊断困难,明确诊断需要组织病理学检查。手术治疗是主要的治疗方法,间变性神经节胶质细胞瘤预后差,术后应长期严密随访。     

Shoulder enlargement as the presenting sign in syringomyelia. Report of two cases and review of the literature.

Two adult patients had painful enlargement of the shoulder and complete destruction of the humeral head. Angiograms of the shoulder showed highly vascular lesions in both patients. Both patients had syringomyelia, for which the swollen shoulder was the first manifestation.     

成人Bartter综合征2例报道并文献复习

目的 分析成人Bamer综合征的临床特点。方法 回顾性分析2例本病。结果 2例均为青年女性，均有不同程度的多饮、多尿、肌无力和抽搐；实验室检查均表现为低血钾、代谢性碱中毒，血肾素活性、血管紧张素Ⅱ及醛固酮明显升高，而血压正常；补钾、补镁、安体舒通等治疗后症状缓解，血钾水平升高。结论 成人出现双下肢乏力，低血钾碱中毒，而血压正常时需考虑本病，本病行立-卧位肾素-血管紧张素-醛固酮测定可基本诊断，必要时行肾穿刺活检；治疗以补钾为主，辅助治疗包括补镁、前列腺素合成酶抑制剂、醛固酮拮抗剂等。