Pseudomyxoma peritonei arising from intraductal papillary neoplasm after surgical pancreatectomy: report of 2 cases and review of the literature

We report 2 cases of pseudomyxoma peritonei (PMP) combined with an intraductal papillary mucinous neoplasm (IPMN) and a review of the literature. In both cases, PMP emerged after surgical resection of the IPMN. In one case, neoplastic foci were present in the surgical margin and PMP was found 1 year after the initial resection, while PMP emerged 5 years after surgical resection in the other case. Including these 2 cases, 8 cases of PMP arising from IPMN have been reported. This condition occurs more frequently in males (7 males, 1 female), while the age at diagnosis ranges from 49 to 82 years, with a mean of 63.3 years. IPMNs occur more commonly in the tail of the pancreas (62.5%). Two different patterns regarding the mechanism of PMP arising from IPMN have been indicated; a rupture of the pancreatic duct, after which the neoplasm spreads through the fistula into the peritoneal cavity, and post-surgical development of PMP after insufficient surgical treatment for an IPMN. Our findings indicate that attention must be given to avoid mucous leakage and obtain a negative surgical margin during surgical treatment of an IPMN.     

肝紫癜病2例报道及分析

1病例资料病例1:患者,女,54岁,因"右上腹痛1周伴皮肤巩膜黄染"入院。既往2型糖尿病病史10余年,长期口服格列吡嗪、二甲双胍,血糖控制可。否认慢性肝炎肝硬化等其他慢性疾病史。入院查体:皮肤巩膜黄染,腹软,上腹部轻度压痛,无反跳痛及肌卫,肝脾肋下未及,肝区叩痛(+),移动性浊音阴性。实验室检查:血常规:WBC 13.67×109/L,RBC 4.66×1012/L,     

甲状旁腺癌(附二例报告及文献复习)

甲状旁腺癌(Parathyroid carcinoma，PTC)发病率相对较低，占原发性甲状旁腺功能亢进症(PHPT)的0．1％～5％^[1,3]，国内文献已报道56例．本院在收治的33例PHPT中有2例PTC。现报道如下，并结合文献进行讨论。     

Pneumococcal pyomyositis: report of 2 cases and review of the literature

Streptococcus pneumoniae is an uncommon cause of pyomyositis. It is unclear whether the clinical presentation and outcome of pneumococcal pyomyositis differ depending on the host's underlying immune status. We describe 2 patients with pneumococcal pyomyositis, review all published cases, and compare characteristics between apparently healthy hosts and at-risk hosts. A total of 35 cases of pneumococcal pyomyositis were identified, 11 in apparently healthy hosts and 24 in at-risk hosts. Two-thirds of the patients had an antecedent respiratory illness or meningitis. At-risk hosts tended to have a longer interval between the development of symptomatic muscle infection and the diagnosis of pyomyositis and a significantly higher risk of disseminated disease at presentation, as manifested by involvement of multiple noncontiguous muscles or presence of meningitis. Overall, other than 1 death, all patients recovered with antibiotics and surgical drainage, but as might be expected there was a significantly higher rate of complications among at-risk hosts.     

Kartagener 综合征2例及文献复习

目的：分析 Kartagener 综合征的临床特点,以提高临床医师对 Kartagener 综合征的认识。方法回顾性分析2例 Kartagener 综合征患者的临床资料并结合文献复习,分析 Kartagener 综合征的临床特点和治疗方法。结果2例患者为姐弟关系,其父母为近亲结婚。2例患者均有确诊 Kartagener综合征的支气管扩张、内脏转位、副鼻窦炎三联征等依据。结论 Kartagener 综合征是一种罕见疾病,近亲结婚可使后代 Kartagener 综合征的发病率增加,提高对 Kartagener 综合征的认识有利于其早期诊断和治疗。     

弥漫性肺动静脉瘘2例报道及文献复习

目的 探讨弥漫性肺动静脉瘘（PAVFs）的临床表现、诊断和治疗方法.方法 回顾性分析2例弥漫性PAVFs的病例资料,并结合国内外相关报道进行文献复习.结果 2例弥漫性PAVFs患者临床均表现为劳累性呼吸困难、紫绀和杵状指/趾等三联征.例1为女性,基础疾病为遗传性毛细血管扩张症,临床表现为反复鼻衄及面部毛细血管扩张等.例2为男性,基础疾病为肝硬化,临床表现为肝肺综合征.2例患者超声心动图均未见明显异常,超声心动图声学造影左心房显影.胸部CT显示双肺血管影增粗,胸膜下多发小结节影和磨玻璃影.肺动脉造影显示肺循环时间缩短,例2患者左下叶背段动脉分支与肺静脉直接连通,经导管行栓塞术,术后氧合无明显改善.随访5年,例1病情平稳,例2死于肝硬化,二者均无脑部并发症.结论 弥漫性PAVFs临床罕见,常表现为劳累性呼吸困难、紫绀和杵状指/趾等三联征.肺动脉造影和超声心动图声学造影有助于早期诊断.     

家族性肺泡微石症2例并文献复习

目的 探讨肺泡微石症(pulmonary alveolarmicrolithiasis,PAM)的病因、流行病学及临床特点,以提高对该病的临床认识.方法 对2例家族型肺泡微石症患者进行病例报道和文献复习.结果 PAM是一种具有家族聚集倾向的常染色体隐性遗传疾病,临床症状轻微,影像学改变较明显,表现为两肺弥漫性小结节影,以中下肺为主,病理表现为肺泡腔内不规则同心分布的钙盐沉着,无有效治疗手段,主要是以对症治疗为主,终末期可进行肺移植.结论 PAM是一种临床症状与影像学表现不相称的呼吸系统疾病,容易被误诊为肺结核,诊断不明时,可行病理检查进一步确定诊断.     

Ectopic pancreas in mediastinum: report of 2 cases and review of the literature

Ectopic pancreas in mediastinum is uncommon. In this paper 2 cases of patients who had large cysts or cystic-solid masses containing pancreatic tissue in mediastinum were reported. Although the clinical presentations of these 2 patients were nonspecific, some specific characteristics in the imaging findings were discovered, which will be helpful for the diagnosis. However, the final diagnosis depends on pathologic examination.     

Hematogenous enterococcal vertebral osteomyelitis: report of 2 cases and review of the literature

Enterococci are a major cause of bacteraemia and endocarditis and are increasingly being implicated in bone and joint infections. Hematogenous enterococcal vertebral osteomyelitis, however, has been only rarely reported. Here we present the first 2 patients from the United States and review the literature on 10 additional cases that have been published since 1967. The clinical presentation of enterococcal vertebral osteomyelitis was similar to cases due to other bacteria. Enterococcus faecalis caused most cases, consistent with its presumably increased virulence. All enterococcal cases in the literature were reported from Europe, which may be due to epidemiological differences related to antibiotic utilization and infection control practices between the US and Europe. Nine of all 12 cases were reported since 1995, which may be consistent with the increase in occurrence of enterococcal infections in general, in association with increasing patient co-morbidities, invasive procedures, and indwelling vascular devices.     

皮下脂膜炎样T细胞淋巴瘤2例报告并文献复习

目的：探讨皮下脂膜炎样T细胞淋巴瘤（SPTCL）的临床和病理特征。方法：对2例SPTCL的临床资料进行分析，并结合文献复习，为其诊断和鉴别诊断提供一条思路。结果：临床上首发症状均表现为皮下结节，单发或多发。全身表现多种多样，但均出现持续高热、消瘦、肝功能严重受损，累及骨髓，伴致死性嗜血细胞综合征，病程进展迅速，生存不超过1年；病理检查见皮下脂肪组织内有原发的小、中或大的多形性T细胞，围绕脂肪细胞呈花环状外观。周围区组织细胞反应性增生活跃，伴有吞噬红细胞现象，并见多核巨细胞和肉芽肿样反应。瘤细胞浸润在脂肪小叶内，而脂肪小叶间隙无累及。肿瘤表达细胞毒T细胞的免疫表现。结论：SPTCL是一种以累及皮下脂肪组织为主的特殊类型的淋巴瘤，伴嗜血细胞综合征者预后差。     

肺泡蛋白沉积症2例报告并文献回顾

目的总结肺泡蛋白沉积症（PAP）的临床特征、诊断及治疗。方法对2例患者的临床资料进行分析,并回顾复习有关文献。结果 PAP临床无特征性,以咳嗽、咳痰、气促多见,但体征较少。胸部CT可见＂地图样＂改变或＂铺路石＂征。结论 PAP发病率低,起病隐匿,误诊或漏诊率较高,支气管肺泡灌洗液及肺组织的病理检查可用于PAP的早期诊断,全肺灌洗是安全有效的治疗方法 。     

Osteopoikilosis: report of 3 cases and review of the literature

Osteopoikilosis, osteopathia condensans disseminata, is a rare hereditary autosomal dominant sclerosing bone dysplasia, more common in males. The diagnosis is usually made incidentally from radiographs which show multiple, small, well-defined, variably shaped and widely distributed (over the skeleton) sclerotic areas. The involvement is symmetrical, and the predilected locations are the phalanges of the hand, carpal bones, metacarpals, foot phalanges, metatarsals, tarsal bones, ilium, femur, radio and sacrum. It must be distinguished from melorheostosis, osteopathia striata and fundamentally from osteoblastic bone metastases, on the basis of the clinical, radiological (roentgenographs, computed tomography and magnetic resonance) and radionuclide scanning characteristics. Histologically, there are focal condensations of compact lamellar bone within the spongiosa. We report three cases of osteopoikilosis and review the literature. Two cases didn't have affectation in phalanges of the hand, which had not been previously reported, to our knowledge.