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学科主题: 内科学
题名:
胶囊内镜下小肠淋巴管扩张症相关特征分析
作者: 司要然
答辩日期: 2016-05-09
导师: 吴静
专业: 内科学
授予单位: 北京大学
授予地点: 北京大学第九临床医学院
学位: 硕士
关键词: 胶囊内镜 ; 小肠淋巴管扩张症 ; 内镜下特征
其他题名: Analysis of the acteristics of intestinal lymphangiectasia in capsule endoscopy
分类号: R656.7
摘要:

 

目的:总结小肠淋巴管扩张症的临床特征,分析胶囊内镜下小肠淋巴管扩张症的镜下形态特点,提高对小肠淋巴管扩张症内镜下表现的认识,促进小肠淋巴管扩张症的早期诊断与治疗。

方法:回顾2010年1月至2015年12月于北京大学第九临床医学院消化内科明确诊断为小肠淋巴管扩张症,且行胶囊内镜检查的55例患者的临床资料。分析总结患者的临床表现、实验室检查及影像学检查,并总结胶囊内镜下形态特点等。

结果:最终共纳入55例患者,年龄为5~68岁,平均年龄为27.53±19.26岁;其中男性患者28例(50.9%),女性患者27例(49.1%),男女比例为1.04:1;未成年患者(<18岁)22例(40%),成年患者(≥18岁)33例(60%)。原发性小肠淋巴管扩张症46例(83.6%),继发性小肠淋巴管扩张症9例(16.4%),其中继发性小肠淋巴管扩张症的病因分别为乙肝后肝硬化5例(55.6%)、肾脏恶性肿瘤1例(11.1%)、缩窄性心包炎1例(11.1%)、系统性红斑狼疮及干燥综合症各1例(11.1%)。

主要临床表现包括:水肿,腹泻、腹痛、腹胀及恶心、呕吐等消化道症状,其中以水肿(74.5%)、腹泻(56.3%)最为常见;另外,还有腹腔积液、胸腔积液、心包积液及盆腔积液等多浆膜腔积液,其中以胸腹水(63%)最为常见。特殊临床表现包括:抽搐(10.9%)、骨折(1.8%)、生长发育迟滞(1.8%)等。非特异性临床表现包括:纳差、消瘦、乏力、黑便、便血、咳嗽、发热、胸闷、憋气等。

血常规检查显示:淋巴细胞绝对值降低(平均值为1.07±0.83×109/L,降低例数比例为67.3%)。血生化结果显示:低蛋白血症主要表现为白蛋白与球蛋白同时降低(平均值分别为21.42±5.56 g/L,16.03±7.05 g/L,降低例数比例分别为100%、83.6%),血脂降低以甘油三酯降低为主(平均值为1.05±0.66 mmol/L,降低例数比例为76.4%),同时伴有血清钙、血清铁的降低(平均值分别为1.82±0.43 mmol/L,10.07±6.94 mmol/L,降低例数比例分别为82.3%、52.7%)。

影像学检查提示:核素蛋白丢失显像、核素淋巴管显像及直接淋巴管造影三种检查方法中,核素蛋白丢失显像阳性率最高(93.3%),核素淋巴管显像次之(72.7%),而直接淋巴管造影的阳性率最低(17.3%)。

病理检查提示:在行病理检查的32例小肠淋巴管扩张症中呈阳性者仅19例(59.4%),其中活检标本阳性者17例(89.5%),手术标本阳性者2例(10.5%)。

小肠淋巴管扩张症累及病变部位提示:病变局限于十二指肠者10例(18.2%),局限于空肠者12例(21.8%),局限于回肠者2例(3.6%)。同时累及十二指肠、回肠者12例(21.8%),同时累及空肠、回肠者5例(9.1%),同时累及十二指肠、空肠者2例(3.6%)。同时累及三部分肠管者12例(21.8%)。综上,在55例患者中共有40例(72.7%)患者病变部位累及空肠,36例(65.5%)累及十二指肠,22例(40%)累及回肠。

IL在胶囊内镜下的典型表现包括:小肠绒毛增粗,呈颗粒样/鱼鳞样改变;淋巴液漏出,绒毛表面呈白点/白斑样改变,肠腔内可见乳白色液体;淋巴管呈囊状/柱状扩张;呈弥漫性或者散在性分布。非典型表现主要包括:小肠黏膜肿胀/增厚,结节/息肉样改变,糜烂、溃疡及肠腔狭窄、出血(具体图片见附录)。

结合胶囊内镜下形态特点及累及的病变部位,将IL分为Ⅰ型~Ⅳ型,共四种镜下类型,其中Ⅰ型为非典型病变,Ⅱ~Ⅳ型为典型病变。具体分型标准如下:Ⅰ型:非典型病变,小肠黏膜水肿/增厚、息肉样/结节样隆起、糜烂或溃疡、狭窄、出血(不考虑病变累及的范围);Ⅱ型:局限性典型病变,具有典型的内镜下表现,病变范围不超过1/3小肠(主要累及十二指肠、空肠及回肠等三部分小肠中的一部分者);Ⅲ型:节段性典型病变,具有典型的内镜下表现,病变范围不超过2/3小肠(主要累及十二指肠、空肠及回肠等三部分小肠中的两部分者);Ⅳ型:弥漫性典型病变,具有典型的内镜下表现,病变范围累及整个小肠(主要累及十二指肠、空肠及回肠等三部分小肠)。上述所有的病变类型在相应节段内既可以是散在性分布,也可以是弥漫性分布。

小肠淋巴管扩张症内镜下分型结果示:不典型组6例(10.9%);典型组49例(89.1%),典型组中Ⅱ型患者最多21例(38.2%),Ⅲ型次之16例(29.1%),Ⅳ型最少12例(21.8%)。

未成年组中原发小肠淋巴管扩张症的发生率明显高于成年组中原发性小肠淋巴管扩张症的发生率(100% vs 72.7%,P<0.01),成年组中84.8%(28/33)内镜下呈典型表现,低于未成年组95.5%(21/22),但两组差异没有统计学意义(P>0.05)。临床症状中未成年组呕吐发生率比成年组高(18.2% vs 0%,P<0.05),而成年组中胸腔积液的发生率更高(60.6% vs 31.8%,P<0.05)。成年组的血小板及红细胞计数明显低于未成年组[血小板计数,(351.3±96.2)x109/L vs (252.1±78.3)x109/L,P<0.01;红细胞计数,(4.9±0.4)x1012/L vs(4.2±1.0)x1012/L, P<0.01]。成年组的球蛋白浓度明显高于未成年组(17.6±8.7 g/L,13.7±1.8 g/L, P<0.05),成年组中的白/球比及血清磷的浓度均较未成年组明显降低(白/球比,1.3±0.4 vs 1.7±0.4, P<0.01;血清磷,1.2±0.2 mmol/L vs 1.5±0.4 mmol/L, P<0.01),但两组的白蛋白浓度没有统计学差异(P>0.05)。原发性小肠淋巴管扩张症患者的水肿发生率明显高于继发性小肠淋巴管扩张症患者(71.7% vs 33.3%,P<0.01)。原发性小肠淋巴管扩张症患者的白/球比及血小板计数比继发性小肠淋巴管扩张症患者高[白/球比,1.5±0.4 vs 1.1±0.4,P<0.01;血小板计数,(307.4±96.5)x109/L vs (212.1±64.2)x109/L,P<0.01],但两组的白蛋白及球蛋白浓度均没有统计学差异(P>0.05)。胶囊内镜下呈典型病变者与非典型病变者、内镜下呈典型病变的不同类型者及所有不同内镜下分型患者相比,在年龄、性别、病因及临床表现、血常规、血生化等方面,差异均没有统计学意义。

结论:

小肠淋巴管扩张症以水肿、腹泻及多浆膜腔积液积液为主要临床表现,血常规以淋巴细胞降低为主,血生化表现为白蛋白及球蛋白同时降低,血脂降低以甘油三酯降低为主。IL没有明显的性别差异。未成年患者多为原发性IL,继发性IL多见于成年人。原发性IL的临床症状更加严重,胶囊内镜下表现更为典型。

胶囊内镜是小肠淋巴管扩张症的主要检查方法之一,其镜下形态具有一定特征,与核素蛋白丢失显像等检查相结合,有助于小肠淋巴管扩张症的早期诊断与治疗。

 

英文摘要:

 

Objective: To summarize the clinical acteristics of intestinal lymphangiectasia, and analysis of the morphological acteristics of intestinal lymphangiectasia under the capsule endoscopy. To improve the clinical understanding of the endoscopic manifestations of intestinal lymphangiectasia, in order to promote the early diagnosis and treatment of intestinal lymphangiectasia.

Methods: We review January 2010 to December 2015 in the ninth hospital of Peking University, diagnosis for intestinal lymphangiectasia and the clinical data of 55 patients underwent capsule endoscopy. Analysis and summary of the clinical manifestations of patients, laboratory examination, imaging examination, and the morphological acteristics under capsule endoscope.

Results: A total of 55 patients are included in the final. The age ranged from 5 to 68 years with an average age of 19.26 ± 27.53 years. Including 28 males and 27 females, The ratio of male to female was 1.04:1. Non adult patients (< 18 years) are 22 cases (40%), adult patients (≥ 18 years) are 33 cases (60%). Primary intestinal lymphangiectasia in 46 cases (83.6%), secondary intestinal lymphangiectasia in 9 cases (16.4%). The etiology of secondary intestinal lymphangiectasia includes 5 (55.6%)cases of liver cirrhosis after hepatitis B, kidney malignant tumor in 1 case (11.1%) and constrictive pericarditis in 1 cases (11.1%), systemic lupus erythematosus and Sjogren's syndrome in 1 case (11.1%),respectively. The main clinical manifestations include: edema, diarrhea, abdominal pain, abdominal distension, nausea, vomiting and other gastrointestinal symptoms, in which edema (74.5%), diarrhea (56.3%) is the most common. In addition, there are abdominal effusion, pleural effusion, pericardial effusion and pelvic effusion,in which hydrothorax and ascites (63%) is the most common. The special clinical manifestation including convulsions (10.9%), fracture (1.8%), growth retardation (1.8%) and so on. The nonspecific clinical presentations include: anorexia, weight loss, fatigue, melena, hematochezia, cough, fever, chest tightness, shortness of breath and so on. Blood tests show: Absolute value of lymphocyte decreased (mean value is (1.07 ±0.83)x109/L, reduce cases percentage is 67.3%). Blood biochemical results show: Hypoalbuminemia is mainly of albumin and globulin also decrease (average values are 21.42±5.56 g/L, 16.03±7.05 g/L, the lowering ratio is 100% and 83.6%, respectively). Lowering blood lipids to triglycerides decreases (average value is 0.661.05 mmol/L, reduce cases percentage is 76.4%), and accompanied by serum calcium, serum iron decreases (average values are 1.82±0.43 mmol/ L, 10.07±6.94 mmol/L, reduce cases percentage is 82.3%, 52.7%). Imaging examinations show: In the three methods of radionuclide protein loss imaging, radionuclide lymphatic imaging and direct lymph node. The positive rate of radionuclide protein loss is highest (93.3%), followed by the radionuclide imaging (72.7%), direct lymph node is the least rare (17.3%). Pathological examinations show that the positive rate of 32 cases of intestinal lymphangiectasia is only 59.4% (19/32). Biopsy is positive in 17 cases (89.5%), and 2 cases(10.5%) are positive for surgical specimens. The involvement of the lesion in intestinal lymphangiectasia shows: Lesions confined to the duodenum in 10 cases (18.2%), localized in the jejunum in 12 cases (21.8%), localized in the ileum in 2 cases (3.6%). Also involving the two parts of the intestine, also involvement of the duodenum and ileum in 12 cases (21.8%), also involving the jejunum and ileum in 5 cases (9.1%), also involvement of the duodenum and jejunum in 2 cases (3.6%). While 12 cases involving the third part of the intestine in12 cases(21.8%). In summary, in patients with a total of 55 patients with 40 cases (72.7%) patients with lesions involving the jejunum, 36 cases (65.5%) involvement of the duodenum, 22 cases (40%) involving the ileum. The typical endoscopic findings under capsule endoscopy include: thickening the villi in the small intestine and is like particles / ichthyosiform change; lymph fluid leakage, villous surface is white / white like change, milky white liquid can be seen in enteric cavity; lymphatic is vesicula / rod expansion; diffuse or scattered in distribution. Atypical manifestations include: small intestinal mucosal swelling / thickening, nodular / polyp like changes, erosion, ulceration and intestinal lumen stenosis( specific see picture appendix). According to the morphological acteristics of capsule endoscopy and the involvement of the lesion site, IL can be divided into four types: type I to type IV. Among them, type I is atypical, type II to type IV are a typical lesion. Specific classification criteria are as follows: type I: atypical lesions, small intestinal mucosal edema / thickening, polyps / nodular uplift, erosion or ulcer, stenosis, bleeding (without considering the extent of lesion involvement); Type II: localized typical lesions, with typical endoscopic manifestations, the lesion is not more than 1/3 small intestine (mainly confined to the duodenum, jejunum and ileum, one part of the three part of the small intestine); Type III: segmental typical lesions, with typical endoscopic manifestations, the lesion is not more than 2/3 small intestine (mainly located in the duodenum, jejunum and ileum, and in two parts of the three part of the small intestine); Type IV: diffuse typical lesions, with typical endoscopic manifestations, the extent of the lesion involving the entire small intestine(mainly involved in the duodenum, jejunum and ileum, and so on the three part of the small intestine). All of the above types of lesions in the corresponding segment can be scattered in the distribution and can also be a diffuse distribution. Endoscopic classification of intestinal lymphangiectasia shows: atypical group accounted for 6 cases(10.9%); typical group accounted for 49 cases(89.1%),type II 21 cases (38.2%) is the highest proportion in the typical group, followed by type III 16 cases (29.1%), type IV 12 cases (21.8%) is the least rare. Incidence rate of primary intestinal lymphangiectasia in juvenile group is significantly higher than that in adult group (100% vs 72.7%, P <0.01). Typical manifestations of adult group is 84.8% (28/33) under endoscopy, the typical manifestations of minor group is 95.5% (21/22), but the difference is not statistically significant (P >0.05). The incidence of vomiting in the juvenile group is higher than that in the adult group (18.2% vs 0%, P <0.05). The incidence of pleural effusion is higher in the adult group (60.6% vs 31.8%, P <0.05). The platelet and red blood cell count of adult group is significantly lower than that of the minor group [platelet count, (351.3±96.2)x109/L vs (252.1±78.3)x109/L,P < 0.01; red blood cell count (4.9±0.4)x1012/L vs(4.2±1.0)x1012/L, P < 0.01]. The globulin concentration in the adult group increased significantly than the minor group (17.6±8.7 g/L,13.7±1.8 g/L, P <0.05), the albumin / globulin ratio and serum phosphorus concentrations of the adult group decrease significantly than the minor group (white ball ratio, 1.3±0.4 vs 1.7±0.4, P<0.01;serum phosphorus, 1.2±0.2 mmol/L vs 1.5±0.4 mmol/L, P <0.01), but there is no significant difference in albumin concentration between the two groups (P > 0.05). The incidence of edema of primary intestinal lymphangiectasiais higher than that of the secondary intestinal lymphangiectasia(71.7% vs 33.3%, P <0.01). The white / ball ratio and platele count of primary intestinal lymphangiectasia is higher than secondaryintestinal lymphangiectasia[white / ball ratio, 1.5±0.4 vs 1.1±0.4, P <0.01; platelet count (307.4± 96.5) x109/L vs (212.1±64.2)x109/L, P <0.01]. But there is no significant difference in albumin and globulin concentrations in the two groups (P > 0.05).Typical and atypical lesions under capsule endoscopy, typical of different types, all endoscopy under different type of patients in age, gender, etiology and clinical manifestations, blood routine, blood biochemistry, the differences are not statistically significant.

Conclusions: The main clinical manifestations of intestinal lymphangiectasia including edema, diarrhea and multiple serous effusion. Blood lymphocytes reduces mainly in blood routine examination, blood biochemical performances reduce of albumin and globulin at the same time,the decreases of blood lipid is mainly triglyceride. There is no obvious gender differences in IL, the primary ILmostly born in the minors, secondary IL are mostly in adults. The clinical symptoms of primary IL are more severe, and the performances under capsule endoscopy are more typical. Capsule endoscopy is one of the main methods of intestinal lymphangiectasia, it has some typical acteristics. Combined with radionuclide protein loss imaging and other examination , which is helpful to the early diagnosis and treatment of intestinal lymphangiectasia.

语种: 中文
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内容类型: 学位论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/124806
Appears in Collections:北京大学第九临床医学院_学位论文

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作者单位: 北京大学第九临床医学院

Recommended Citation:
司要然. 胶囊内镜下小肠淋巴管扩张症相关特征分析[D]. 北京大学第九临床医学院. 北京大学. 2016.
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