肺淋巴管肌瘤病9例临床分析

目的探讨肺淋巴管肌瘤(PLAM)的临床表现及诊治方法。方法报道并临床分析2005—2008年中南大学湘雅二院收治的具有较完整资料的9例肺淋巴管肌瘤病例,结合文献进行复习。结果 9例均为女性。均有活动后气促,1例有乳糜腹,2例有乳糜胸,3例有气胸,6例有咯血。9例高分辨CT(HRCT)均示肺部弥漫分布薄壁囊腔透光区的PLAM特征性表现而临床诊断。4例行病理组织检查确诊。国内首次报道予雷帕霉素治疗LAM且疗效满意的病例。结论 HRCT检查对PLAM具诊断价值,对育龄妇女发生的渐进性呼吸困难、气胸、乳糜胸、咯血应及时进行胸部HRCT检查,雷帕霉素给此病的治疗带来新希望。     

肺淋巴管肌瘤病的胸部影像学表现

目的探讨肺淋巴管肌瘤病（PLAM）的影像学表现。方法报告3例PLAM病例,结合文献资料对该病病因、临床特点及影像学表现进行讨论。结果 3例患者均为育龄期女性,首发症状均为活动后呼吸困难,其中1例合并气胸,1例合并气胸及乳糜胸,1例合并肺外淋巴管肌瘤。结合文献资料,本病常发于育龄期女性,临床表现依次为呼吸困难、咯血、气胸、乳糜胸等。本病X线片常呈非特异改变,CT较具特异性,主要表现为多发均匀分布囊状影,伴有间隔线,呈网格状改变,同时出现液气胸和淋巴结肿大,肺外也可有多发相关改变。肺功能测定常伴有阻塞性通气功能障碍。结论 CT检查对PLAM具有诊断价值。对育龄期妇女发生的渐进性呼吸困难、咯血、自发性气胸应及时进行胸部CT检查,并应常规进行腹部和盆腔影像学检查以了解患者是否合并肺外淋巴管平滑肌瘤。     

26例非创伤性乳糜胸临床分析

非创伤性乳糜胸发病率低，病死率高。我们收集了中山医院１９５７年４月～１９９８年１０月间，共２６例非创伤性乳糜胸病例，并随访２～１５年或至死亡，作回顾性分析。对象与方法　２６例患者中男１７例，女９例。年龄１７～７２岁，平均年龄３９岁。胸闷２４例，咳嗽９例，胸痛５例。胸液：右侧６例，左侧７例，双侧１３例。病因：大量骨溶解症、肝硬化失代偿各３例，肺结核、丝虫病各２例，纵隔肿瘤、胃癌、肺癌、肺炎、先天性淋巴管发育不良、硬皮病多发性肌炎重叠综合征各１例，原因不明１０例。胸液乳白色１１例，黄混色８例，粉红…     

老年人非霍奇金淋巴瘤合并乳糜胸一例并文献复习

目的 探讨乳糜胸的诊断及非霍奇金淋巴瘤乳糜胸的治疗,提高临床对真、假性乳糜胸的认识. 方法 报告并分析我院诊治的经淋巴结活检确诊为非霍奇金淋巴瘤(NHL)的1例老年非创伤性乳糜胸患者临床资料,并复习国内外相关文献,分析NHL乳糜胸的可能机制并总结其诊治与预后. 结果 83岁老年男性患者1例,始为单侧乳糜胸,后进展为双侧,约1年病史.明确病因困难,正电子发射断层成像术/计算机断层成像术(PET/CT)检查示胸内及右侧颈部淋巴结代谢活性增高并肿大,行颈部淋巴结活检确诊为弥漫大B细胞型NHL.检索国内外文献中19例NHL乳糜胸患者,采取的治疗方法有6例行放射治疗放疗均有效;11例行化学治疗(化疗),其中6例有效;1例行胸导管结扎术有效;1例行胸导管结扎加药物胸膜固定有效.该例患者拒绝化疗或放疗,行饮食疗法19个月后死亡. 结论 PET/CT有助于早期发现乳糜胸的隐匿病因.目前尚无标准的NHL乳糜胸治疗方法,应采取个体化治疗.NHL乳糜胸整体预后与非霍奇金淋巴瘤预后相似,要重视早期诊断、综合治疗以延长患者生存时间.     

肺淋巴管平滑肌瘤病

目的　提高对罕见病肺淋巴管平滑肌瘤病 (PL AM)的认识。方法　对我院诊治的患者进行分析 ,并结合文献复习。结果　 PL AM是一种罕见的弥漫性肺部疾病 ,临床反复发作自发性气胸或 /和乳糜胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障碍 ,低氧血症。胸部 X线表现为两肺弥漫分布网格状阴影 ,高分辨 CT(HRCT)示弥漫分布囊状改变。病理学检查示 :肺组织淋巴管增生和扩张 ,管外平滑肌明显增生。结论　育龄期妇女如反复发生气胸及出现原因不明的呼吸困难、咯血或乳糜胸 ,应疑诊 PL AM,及时行 HRCT及肺功能检查 ,必要时肺活检 ,以明确病理诊断。     

自发性乳糜胸6例临床分析

乳糜胸是临床较为少见的疾病 ,约占胸腔积液的 2 % ,病死率高。本文收集自 1994年至 2 0 0 0年我院收治的 6例自发性乳糜胸进行分析。并结合文献进行讨论。1　临床资料6例患者中男性 3例 ,女性 3例 ,年龄 46～ 73岁 ,平均 6 7.8岁。胸闷 6例 ,咳嗽 3例 ,胸痛 1例 ,乏力 1例 ,体重减轻 1例。胸腔积液 :右侧 3例 ,左侧 1例 ,双侧 2例。病因 :肺癌、淋巴瘤、肝硬化失代偿各 1例 ,原因不明 3例。胸液乳白色 3例 ,混浊黄色 2例 ,粉红色 1例 ,离心后均未见清亮。乳糜定性阳性。蛋白定量 14.8g/ L～ 49.0 g/ L,平均 42 .1g/ L。其中 3例测定胸水…     

后腹腔镜肾蒂淋巴管结扎术治疗乳糜尿

目的观察后腹腔镜下肾蒂淋巴管结扎术治疗乳糜尿的疗效。方法采用后腹腔镜下肾蒂淋巴管结扎术治疗乳糜尿11例（均为乳糜尿试验阳性及膀胱镜检确诊）。结果手术时间100～180 min,平均120 min;术中出血35～80 ml,平均60 ml。无中转开放手术。术后3～5 d拔出腹膜后引流管,切口均一期愈合,术后乳糜尿消失。随访6～24个月,乳糜尿均无复发。结论后腹腔镜肾蒂淋巴管结扎术治疗乳糜尿疗效确切,在手术创伤、住院时间、术后恢复等方面优于开放手术。     

肺淋巴管肌瘤病11例报告并文献复习

目的 探讨肺淋巴管肌瘤病( PLAM)的临床和预后.方法 分析经治的11例PLAM病人的资料,并复习文献.结果 11例均为女性;年龄31 ～55岁,平均(44±9)岁;均经肺组织活检明确病理;主要临床表现为进行性呼吸困难10例(发生率91％),咳嗽6例(55％),咯血2例(18％),乳糜胸3例(27％),气胸3例(27％)等;11例患者均进行肺功能检测,均有弥散功能下降,9例行动脉血气分析,其中7例表现为低氧血症,4例合并呼吸衰竭;11例行胸部高分辨率CT (HRCT),均显示两肺弥漫性薄壁囊状阴影.结论 PLAM以呼吸困难,咳嗽,气胸和乳糜胸为主要临床表现;双肺弥漫性薄壁囊状阴影为影像学特征表现;肺组织活检是确诊的主要依据.     

乳糜胸

【乳糜胸的病因学】乳糜胸是由胸导管阻塞或破裂引起的,可分为先天性、创伤性(医源性、非医源性或自发性)两类,以意外或医源性损伤最为常见。心脏、主动脉、食道和左锁骨下血管的手术有可能损伤胸导管。近十年来,随着胸部有创性操作和手术数量的增大,医源性乳糜胸的发生率也有所增加。胸骨切开术可损伤胸导管,多半与穿过胸腺组织的导管异常有关,据报道大多数病例发生在大血管畸形的外科矫正     

肺淋巴管平滑肌瘤病二例―附文献分析

目的提高对肺淋巴管平滑肌瘤病(PLAM)的认识并引起对该病的重视.方法对2例PLAM患者的临床资料进行分析,并结合文献进行复习.结果PLAM是一种持续发展的弥漫性肺间质疾病.临床表现为反复发作的自发性气胸、活动后呼吸困难、咯血和乳糜胸等.肺功能呈阻塞性肺通气功能障碍和弥散功能障碍,动脉血气分析显示低氧血症.胸部X线表现两肺弥漫性网格状,晚期呈蜂窝样变化.高分辨CT(HRCT)两肺广泛弥漫性分布的薄壁小囊状病变.病理学特点为肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生.免疫组化HMB45阳性.结论对育龄期妇女发生渐进性呼吸困难不能缓解,类似肺气肿的临床表现及X线胸片阴影,并出现反复气胸或乳糜胸水时即应考虑为PLAM可能,最好能以肺组织活检明确诊断.该病预后差,目前无有效的预防和治疗方法.     

免疫健全成人社区获得性巨细胞病毒肺炎一例并文献复习

目的:提高临床医师对免疫健全成人社区获得性巨细胞病毒肺炎(CMV-CAP)的认识。方法:报道清华大学第一附属医院呼吸与危重症医学科病房收治的1例青壮年CMV-CAP诊治经过,并以关键词"巨细胞病毒肺炎""免疫健全"和"Cytomegalovirus""CAP""Immunocompetent"在万方数据库及PubMed...     

A case of follicular lymphoma with onset of chylothorax]

We report a case of follicular lymphoma with chylothorax. A 45-year-old man visited our hospital complaining of shortness of breath and abdominal distension. Chest X-rays showed bilateral pleural effusion, and an abdominal CT scan revealed a large intraperitoneal tumor around the abdominal aorta and pancreas. Bilateral cervical and inguinal lymph nodes were swollen. Biochemical study of the pleural fluid revealed the presence of chylomicrons, and an inguinal lymph node biopsy led to a follicular lymphoma diagnosis. The patient achieved complete remission, with disappearance of pleural effusion, following 8 cycles of chemothreapy (R-CHOP). Cases of malignant lymphoma with chylothorax are rarely reported in Japan, but should be taken into account when examining cases of non-traumatic chylothorax.     

Thoracic duct injury during esophagectomy: 20 years experience at a tertiary care center in a developing country

Thoracic duct injury is an uncommon complication of esophagectomy. Experience in managing these cases is limited to large centers performing esophagectomies in good numbers. We analyzed the prospectively maintained esophageal diseases database of patients presenting to a surgical unit between 1982 and 2002. Among 552 esophagectomies during this period we had encountered 14 cases of chylothorax (2.54%). We analyzed the type and site of lesion and the impact of neoadjuvant therapy on the incidence of thoracic duct injury. Among 459 patients of transhiatal esophagectomy, 11 developed postoperative chylothorax (2.40%). In 93 transthoracic resections, there were three cases of chylothorax (3.23%; (P = 0.9185)). The incidence following preoperative radiotherapy was 2.17%. None of the 31 patients, who had undergone esophagectomy for benign diseases had developed chylothorax. In the carcinoma group the incidence in middle third lesions was 5.85% and in lower third lesions was 0.80% (P = 0.0018). Seven patients were managed conservatively. Two of these patients, for whom surgery had been planned, died before they could be taken up for surgery. In the remaining seven patients transthoracic ligation of the thoracic duct was performed. Two patients in this group died. The average hospital stay was 20 days in the conservative group and 12 days in the surgery group. Among the factors studied, patients with middle third lesions were at increased risk of developing postoperative chylothorax, when compared to upper or lower third lesions.     

Chylothorax and pseudochylothorax

Both chylothorax and pseudochylothorax are characterized by milky or turbid pleural effusions. The importance of distinguishing these two conditions cannot be overemphasized because the prognosis and management are different.     

Colonic lymphangiomatosis associated with anemia

Lymphangioma is an uncommon malformation of lymphatic system. Multiple colonic lymphangioma named as lymphangiomatosis is considered an extremely rare disease. Although lymphangioma is a benign tumor and most colonic lymphangiomas do not cause symptoms and do not require treatment, resection of lymphangioma is necessary in the presence of symptoms such as abdominal pain, bleeding, intussusceptions. We report a case of colonic lymphangiomatosis in a man who presented with abdominal discomfort and anemia, which was diagnosed and treated with endoscopic snare polypectomy.     

乳糜胸10例诊治分析

目的总结乳糜胸病因和诊治的临床经验。方法对10例乳糜胸患者的病史临床特点、诊断、治疗以及随访结果进行回顾性分析。结果10例乳糜胸，其中特发性乳糜胸5例，继发于胸部手术后2例，服用药物后继发2例，淋巴瘤1例。分别针对病因治疗（3例）或内科保守治疗（6例），行胸导管结扎手术（1例）所有病例均获得有效控制。结论早期病因诊断，个体化的治疗方案，有助于降低乳糜胸的死亡率。     

端粒酶在良恶性胸水中的表达及临床意义

目的 探讨端粒酶在良恶性胸腔积液中的表达及其与良恶性胸腔积液临床表现的关系.方法 利用荧光原位杂交方法对23例恶性胸腔积液和25例良性胸腔积液的端粒酶活性进行检测,并结合临床资料分析.结果 23例恶性胸水中,端粒酶表达阳性19例(82.6%),而25例良性胸水中,端粒酶表达阳性2例(8.0%),二者比较有统计学差异(P＜0.05);早期恶性胸腔积液和晚期恶性胸腔积液的端粒酶表达阳性率分别为81.8%和83.3%,二者比较无统计学差异(P＞0.05).结论 端粒酶阳性表达与恶性胸腔积液的发生有关,是一个重要的肿瘤标记物,在恶性胸水脱落细胞中检测到端粒酶表达可作为早期病变的辅助指标.     

Relapse of idiopathic chylothorax 17 years after remission of congenital chylothorax]

We report a case of chylothorax in a 17-year-old male. As a neonate, had had congenital chylothorax, and was successfully treated with medium-chain triglycerides; but recently presented with dyspnea after an episode of severe coughing. Radiographic examination disclosed abundant effusion in the right chest. The effusion was milky brown, had a creamy supernatant and a high triglyceride level (3085 mg/dl). This condition was diagnosed as idiopathic chylothorax. Lymphangiography showed a rupture of the right thoracic duct. Since the effusion was resistant to conservative therapy, we performed thoracoscopic clipping of the thoracic duct, which reduced the amount of pleural effusion. We speculated that coughing may have caused the rupture of a congenitally weakened thoracic duct.     

Chylothorax and cholesterol pleural effusion

A chylothorax and a cholesterol pleural effusion represent the two forms of lipid effusions encountered. Traditionally, a lipid pleural effusion is characterized by the presence of milky fluid. Although these two effusions often share a similar pleural fluid appearance due to the high lipid concentration, they have major differences in the pathogenesis, clinical presentation, diagnosis, predisposing conditions, and management of these effusions. A chylothorax is defined by the presence of chyle in the pleural space resulting from obstruction or disruption of the thoracic duct or one of its major tributaries. A triglyceride concentration > 110 mg/dL is virtually diagnostic, but the presence of chylomicrons confirms the diagnosis. However, a chylothorax defined by these criteria represents a heterogeneous group of clinical entities. The presence of chylomicrons or triglyceride levels > 110 mg/dL in a pleural effusion should be considered evidence of chyle leakage of indeterminate clinical significance. Many cases of a chylous effusion may be associated with other causes of pleural fluid formation. In the case of an acute or chronic chylothorax due to recent or remote thoracic duct injury, this assessment is essential, as surgical ligation of the thoracic duct is often entertained. In other cases, especially lymphoma or chylous ascites, treatment of the underlying condition is indicated regardless, and the assessment of the response to treatment is a reasonable initial approach. In contrast, a cholesterol effusion is typically the result of long-standing pleurisy with elevated cholesterol levels in the pleural space; however, this paradigm has been challenged. Lung entrapment with thickened parietal and visceral pleural membranes is the typical radiographic findings of a cholesterol effusion. Most cases of cholesterol pleural effusions are attributed to tuberculous or rheumatoid pleurisy. Decortication is the mainstay of treatment for a cholesterol effusion in symptomatic patients with restrictive lung function.     

A case of gallbladder cancer manifesting chylous ascites and chylothorax

A case of gallbladder cancer manifesting both chylous ascites and chylothorax was reported. A 66-year-old man was hospitalized with milky ascites. The patient was diagnosed as having gallbladder cancer based on findings of endoscopic retrograde cholangiopancreatography (ERCP) and celiac angiography. The diagnosis of chylous ascites was confirmed by the presence of microscopically visible free fat and the biochemical analysis of the fluid. The patient also gradually developed chylous thoracic effusion. Autopsy revealed lymphogenous metastasis in multiple retroperitoneal and mediastinal nodes. Chylothorax and chyloperitoneum are relatively rare. Only five cases have been reported in Japan that manifested both conditions.