Efficacy of Cyclophosphamide in the Treatment of Interstitial Lung Disease Associated with Systemic Sclerosis

BackgroundCyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with systemic sclerosis (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months.Patients and methodRetrospective study of 37 patients with ILD associated with scleroderma treated with intravenous CYC for 24 months and regularly assessed by LFT (at baseline, 6, 12 and 24 months) including forced vital capacity (FVC) and transfer capacity of carbon monoxide (DL_CO).ResultsThe differences between FVC and DL_CO values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized functional parameters. There were no differences in FVC or DL_CO when patients treated for 6 months were evaluated according to the type of SSc skin involvement of (diffuse or limited) or according to the evolution time of ILD before the start of treatment. Although patients with severe restriction (FVC<70%) showed more improvement, it was less than 10% in all casesConclusionIn this series of patients with ILD associated with SSc, intravenous CYC was effective in stabilizing lung function parameters in long-term treatment.     

Menkes' Kinky Hair Disease Associated with a Large Bladder Diverticulum: A Case Report

We report a 2 year-old boy with Menkes' kinky hair disease associated with a solitary huge bladder diverticulum. To our knowledge this is the first reported case treated successfully by surgical excision under general anesthesia, which has been previously considered hazardous due to the poor general condition of the patient.     

ASO Visual Abstract: High BRCA2 Gene Expression is Associated with Aggressive and Highly Proliferative Breast Cancer

Annals of Surgical Oncology -     

锂中毒肾损害-附9例报道

目的 分析总结接受碳酸锂治疗导致的锂肾病并发肾病综合征患者的临床特点和危险因素,初步探讨其治疗方法及疗效.方法 对9例碳酸锂治疗导致的锂肾病患者临床表现进行分析总结,在对症治疗同时,对其中并发肾病综合征的5例患者给予0.5～1mg·(kg·d)-1强的松口服治疗,其中3例并发肾病综合征患者为排除原发肾小球疾病接受了肾穿刺活检.结果 并发肾病综合征的患者平均接受锂制剂治疗时间为10.2±3.2年,显著长于无肾病综合征的患者(4.3±2.2年);两组患者血清锂浓度无显著意义;非肾病综合征组脱水病例多于肾病综合征组.并发肾病综合征患者肾穿刺活检病理结果显示间质白细胞浸润和纤维化、小管变性和局灶坏死,光镜下肾小球无明显病理改变.全部患者临床痊愈,肾病综合征均获得缓解.未观察到强的松治疗的副作用.结论 碳酸锂治疗导致的锂肾病并发肾病综合征并不少见,与血清锂浓度无明显关系,长期接受锂制剂治疗是其危险因素,对于强的松口服治疗有效,而腹泻和脱水是无肾病综合征锂肾病患者的危险因素.     

肾嗜酸性细胞瘤12例临床分析

目的：总结分析肾嗜酸性细胞瘤临床诊治及预后特点,提高肾嗜酸性细胞瘤的诊治水平。方法：回顾性分析2003年1月～2010年9月诊治的12例经手术及病理检查证实为肾嗜酸性细胞瘤患者的临床及预后资料：12例患者中,男7例,女5例,平均年龄56．4（39～73）岁。肿瘤最大径平均4．3（3．0～8．0）cm。8例行肾癌根治术,4例行肾部分切除术。结果：在平均33．8（8～92）个月的随访中,所有患者未发生死亡或者复发（疾病相关生存率为100％）。结论：肾嗜酸性细胞瘤为一种临床少见的良性肿瘤,在临床上无特异性,确诊需依据病理组织学、免疫组织化学及电镜检查结果综合判断,影像学检查对于诊断有部分提示作用,对治疗选择起一定指导意义。保留。肾单位手术是推荐的治疗术式,尤其是对于肿瘤体积较小的患者。     

膀胱软斑病6例临床病理分析及预后

目的探讨膀胱软斑病的临床病理特征、发病机制、鉴别诊断及预后。方法对6例膀胱软斑病患者的临床特征、影像学及组织病理学表现进行分析,并复习相关文献。结果组织学形态均可见成片组织细胞样细胞分布,散在淋巴细胞,浆细胞浸润。组织细胞内外部分可见靶环或枭眼状的Michaelis-Gutmann小体(M-G小体即软斑小体)。PAS和普鲁士蓝铁染色阳性。免疫组化CD68弥漫(+),CK(-),Ki-67增殖指数为10%。随访3~18个月,均预后良好。结论软斑病是一种少见的慢性肉芽肿性炎症疾病,全身均可受累,且以泌尿系统(膀胱)最常见。因影像学多表现为实质性占位,而易误诊为肿瘤。病理诊断为金标准,可采用抗生素、手术及免疫治疗相结合的综合治疗方案进行治疗。     

髋臼加盖成形术治疗儿童股骨头缺血性坏死12例报告

目的探讨髋臼加盖成形术治疗儿童股骨头缺血性坏死的疗效。方法12例股骨头缺血性坏死患儿,平均年龄9.8(8～12)岁,Harris髋关节功能评分平均72.9±9.6,均行髋臼加盖成形术。在髋臼上方2.5cm处横行劈开髂骨外板,植入楔形同种异体骨,加深髋臼窝,增加股骨头包容,术后髋人字石膏完全制动3月。结果12例患者均获随访,平均时间12(7～18)个月。术后Harris髋关节功能评分平均92.2±6.7。采用功能改善率评定疗效,治愈9例,显效2例,有效1例。结论髋臼加盖成形术治疗儿童股骨头缺血性坏死具有术式简单,创伤小,治疗周期相对短,疗效良好等优点。     

经尿道双极等离子电切术治疗女性膀胱颈梗阻(附18例临床分析)

目的探讨经尿道双极等离子电切术治疗女性膀胱颈梗阻的疗效。方法采用经尿道双极等离子膀胱颈电切术治疗18例女性膀胱颈梗阻患者。结果平均手术时间16(11～48)min,失血<10ml,无水中毒及尿失禁发生。术后病理报告为膀胱颈粘膜下纤维组织增生伴玻璃样变性,部分平滑肌变性增生及粘膜下炎性细胞浸润。本组18例患者均获随访,平均12(4～23)个月,治疗后生活质量评分由4.2±1.1降至1.7±0.5(P<0.01),最大尿流率由(3.8±2.9)ml/s升至(16.0±6.4)ml/s(P<0.01),残余尿量由(355.5±196.5)ml降至(34.5±18.9)ml(P<0.01)。结论经尿道双极等离子电切术是治疗女性膀胱颈梗阻有效的腔内治疗方法。     

Invasive Micropapillary Carcinoma of the Breast: A Clinicopathologic Study of 103 Cases of an Unusual and Highly Aggressive Variant of Breast Carcinoma

Invasive micropapillary carcinoma (IMPC) of the breast is an uncommon, highly aggressive breast cancer that may occur in pure and mixed forms. Our aim in this study is to investigate the relationship between clinical, histopathologic, and immunohistochemical features of pure and mixed IMPC cases diagnosed and treated at our institution. One hundred and three IMPC cases diagnosed at our institution over a period of 19 years have been selected. Clinical, histopathologic features, as well as hormone status and c‐erb‐B2 overexpression of tumors were re‐evaluated. Mann–Whitney U, chi‐squared, Kaplan–Meier, and Fisher's exact tests were used for statistical analyses. Results were considered to be significant at p < 0.05. Twenty cases (19.4%) were pure, and 83 cases (80.6%) were mixed IMPC. The most common nonmicropapillary invasive carcinoma component in mixed cases was invasive ductal carcinoma (IDC; 78.3%). Progesterone receptor was significantly less positive in pure IMPC cases (p = 0.031). There was no statistically significant difference between the two groups, in terms of mean age of the patients (53.0 versus 52.8), mean tumor size (26.6 mm versus 27.7 mm), presence of high‐grade tumor (p = 0.631), presence of sentinel lymph node (SN) metastasis (p = 1.000), axillary lymph node metastasis (p = 1.000), lymphatic invasion (p = 1.000) and blood vessel invasion (p = 0.475), c‐erbB‐2 overexpression of tumor cells (p = 0.616), distant metastasis (p = 0.549), or overall survival (p = 0.759). The local recurrence rate of the two groups was not statistically significant either (16.7% versus 4.3%). However, local recurrence was detected 12% more commonly (p = 0.100), and ~8 months earlier (p = 0.967) in pure IMPC cases, compared to mixed cases. In addition, presence of local recurrence was found to be statistically significantly associated with estrogen receptor (ER) status (p = 0.004), progesterone receptor (PR) status (p = 0.001), and c‐erb‐B2 overexpression (p = 0.016) in all patients. Overall survival rate was significantly associated with ER staining of the tumor (log‐rank = 0.028). Our findings suggest that hormone receptor negativity may explain the more aggressive behavior of pure IMPC compared to mixed cases. Besides, longer survival period of patients with ER positivity, and the relationship of hormone status and c‐erb‐B2 overexpression and local recurrence further support favorable prognostic value of hormone receptors in invasive breast cancer.     

先天性心脏病围术期急性肺出血12例

目的探讨先天性心脏病患者围术期急性肺出血的发生原因及处理措施,总结其治疗经验。方法2000年1月至2008年3月我中心共收治先天性心脏病围术期合并急性肺出血患者12例,男7例,女5例;年龄1～1460d(373±477d),体重2.9～15.0kg(6.73±3.63kg)。患者均在气体静脉全身麻醉下经胸骨正中入路行根治手术;发生急性肺出血后,应用左心减压、高频振荡通气(HFO)、气雾吸入伊洛前列素等作为主要治疗手段。结果12例急性肺出血患者中,抢救成功8例,共死亡4例;1例急性肺出血发生于术前,8例急性肺出血发生于体外循环结束后,3例发生于术后监护阶段;生存的8例患者术后随访3个月～1.5年,胸部X线片示:肺部体征良好,无明显渗出等表现。结论急性肺出血在先天性心脏病围术期是较为严重的并发症,应明确病因,及早干预,才能提高患者的生存率。     

膀胱重复畸形2例临床分析

目的：提高对膀胱重复畸形的认识和诊疗水平.方法：回顾性分析2例膀胱重复畸形患者的临床资料,并结合文献资料进行讨论.结果：例1经膀胱尿道镜检查后发现左侧膀胱前壁有一菜花状肿物,病理检查结果提示膀胱高-中分化鳞状上皮细胞癌,行全膀胱切除＋双侧输尿管皮肤造口术.例2行双膀胱融合和腹前壁修补术.结论：膀胱重复畸形极其罕见,并且很少单独存在,多合并尿路或其他器官畸形.手术是唯一能根治的方法,治疗应遵循个体化原则.     

Xanthogranulomatous Cholecystitis. Retrospective Analysis of 12 Cases

Xanthogranulomatous cholecystitis is a rare variant of chronic cholecystitis characterized by severe proliferative fibrosis and accumulation of lipid-laden macrophages in areas of destructive inflammation. The macroscopic appearance generally mimics a gallbladder carcinoma.

Twelve cases of xanthogranulomatous cholecystitis were identified from a retrospective analysis of the patient records of 770 cholecystectomy cases operated on in our department from January 1996 to October 2001. There were four men and eight women. Mean age of presentation was 52.5 years. Eleven patients had gallbladder stones. Seven patients had a history of acute cholecystitis and five patients of biliary colicky pain. Five cases were presented with obstructive jaundice and five with acute cholecystitis. Right upper quadrant mass was palpable in three patients. All patients underwent cholecystectomy. Open surgery was planned and performed in three patients. Laparoscopic cholecystectomy was planned in nine patients but converted to open surgery in three cases. Nine patients had an uneventful postoperative course. One patient developed wound infection and one patient a postoperative pulmonary infection. One patient developed acute abdomen in the 2^nd postoperative day and was re-operated for bile peritonitis. No mortality was seen in the series.     

胆源性肝脓肿的治疗(附12例报道)

目的 探讨胆源性肝脓肿的诊治方法.方法 对我院2000～2004年期间收治并确诊为胆源性肝脓肿的12例患者进行分析,在应用抗生素和全身支持的前提下,再依据其病变发生、发展的不同阶段采用不同手段治疗.结果 4例急性期患者中2例行急诊胆道引流手术后治愈,另2例转为亚急性期(脓肿融合期);6例亚急性期患者均经B超导向下行脓肿穿刺抽脓后注入抗生素治疗后治愈;4例慢性期患者行脓肿切开引流后治愈.结论 本病在应用广谱抗生素(二联抗生素)和全身支持治疗的前提下,再根据病变不同时期采用不同方法治疗,可获得良好效果.     

肥胖相关性肾病伴IgA肾病2例临床病理分析

目的：探讨肥胖相关性肾病（ORG）伴IgA肾病的组织形态学特征、诊断、鉴别诊断、治疗及预后。方法：回顾性分析2例ORG伴IgA肾病的临床病理资料,并结合文献进行复习。结果：2例均为成年人,男女各1例,中位年龄35.5岁;以尿检异常为主要症状,体型肥胖,BMI均超正常值。光镜示：肾小球显著肥大,系膜区及系膜旁区沉积物伴一定数量的肾小球硬化。免疫组化显示以IgA沉积为主的系膜区沉积。电镜显示系膜区少量沉积物伴足突融合及微绒毛变性。结论：ORG伴IgA肾病非常罕见,依赖病理形态学和免疫表型,结合患者有肥胖及BMI超标即可确诊。病理学上需与IgA沉积及肾小球硬化相关肾病加以鉴别。ORG伴IgA肾病治疗方法多种,其预后较难预测。     

腹腔镜处理膀胱平滑肌瘤3例报告

目的探讨腹腔镜处理膀胱平滑肌瘤的适应证和疗效。方法 2003年8月～2010年10月腹腔镜手术治疗3例膀胱平滑肌瘤。采用三孔法,气腹压力10～15 mm Hg。暴露膀胱后,经尿管注入400 ml无菌盐水充盈膀胱,距肿瘤0.3cm处切开膀胱壁,直视下将瘤体及周围0.3 cm完整切除。结果 3例手术均成功,手术时间分别为70、90、40 min。术中、术后无周围器官损伤和尿漏发生。3例术后分别随访96、72、12个月,无肿瘤复发。结论体积较大的膀胱平滑肌瘤或壁间型、浆膜下型需行肿瘤剜除或膀胱部分切除术者是腹腔镜手术的适应证,腹腔镜处理安全、有效。