病理证实的症状、影像不典型的Caroli病1例

Caroli病是一种以非阻塞性肝内胆管扩张为特征的罕见先天性疾病。大多认为是常染色体隐性遗传病,但许多病例无法追寻典型的遗传家族史。Caroli病包括两种类型,简单型（称为Caroli病）和以先天性肝纤维化和/或多囊肾病为特征的Caroli综合征。认为PKHD1基因是Caroli病、Caroli综合征的致病基因。     

先天性肝纤维化24例临床分析并文献复习

目的分析先天性肝纤维化的临床特征。方法对24例先天性肝纤维化患者临床症状、体征、实验室化验检查及病理学特点进行回顾性分析。结果 24例患者均有门脉高压表现,8例曾有消化道出血。化验肝功能正常或轻度异常。影像学检查提示7例存在肝肾囊肿。22例病理结果均提示先天性肝纤维化,其中9例合并先天性肝内胆管扩张（Caroli’s）病。结论对于病因不明门脉高压,尤其门脉高压与肝功损害程度不一致的患者应尽量行肝组织活检病理检查以协助诊断。先天性肝纤维化与肝肾囊肿、Caroli’s病常常伴发。     

17例先天性肝纤维化临床和病理分析

目的分析先天性肝纤维化(CHF)的临床和病理学特征。方法回顾性分析17例CHF患者的临床和病理学资料。结果 5例患者呕血、黑便(29%),8例患者肝、脾肿大(47%),15例患者肝功能正常或轻微异常(88%),16例患者经病理学诊断为CHF,7例(41.2%)合并Caroli病。结论 CHF患者临床表现以门脉高压型为主,肝功能指标多正常,可重叠Caroli病,确诊需经肝组织病理学检查。     

Caroli病Ⅰ、Ⅱ型的临床特征—78例分析

背景:Caroli病是一种以肝内大胆管节段性囊状扩张为特征的罕见先天性遗传性肝病。典型的Caroli病(Caroli病Ⅰ型)仅涉及胆管畸形,Caroli综合征(Caroli病Ⅱ型)指Caroli病合并先天性肝纤维化和门静脉高压。目的:探讨Caroli病Ⅰ、Ⅱ型的临床特征。方法:纳入2005年9月—2015年11月于首都医科大学附属北京世纪坛医院和解放军第302医院确诊Caroli病的患者78例,分为Ⅰ型组和Ⅱ型组,对两组性别、年龄、临床表现、体征、实验室指标、影像学和组织病理学表现等进行回顾性分析。结果:Ⅰ型组患者21例,中位年龄49.00(31.00,57.25)岁,食欲减退和细菌性胆管炎的发生率显著高于Ⅱ型组(P0.05),无呕血、黑便,血常规正常,ALT、AST、碱性磷酸酶(ALP)、γ谷氨酰转移酶(GGT)和亮氨酸氨肽酶(LAP)水平升高;Ⅱ型组患者57例,中位年龄29.00(14.50,40.00)岁,呕血、黑便、肝脾肿大、腹水和食管静脉曲张发生率显著高于Ⅰ型组(P0.05),WBC、HGB、血细胞比容(HCT)和PLT水平均低于正常值下限,肝功能基本正常。两组间年龄、凝血酶原时间(PT)、凝血酶原活动度(PTA)、国际标准化比率(INR)、纤维蛋白原(FIB)、WBC、中性粒细胞、RBC、HGB、HCT、PLT、ALT、ALP、GGT和LAP水平差异有统计学意义(P0.05)。Caroli病Ⅰ型病理以肝内胆管节段性囊状扩张伴炎性细胞浸润为主要特征,Caroli病Ⅱ型病理以肝内胆管扩张伴门静脉周围纤维化为主要特征。结论:Caroli病Ⅰ型以肝内胆管节段性囊状扩张为主要特征,主要表现为肝功能异常,Caroli病Ⅱ型以门静脉高压症为主要表现,肝功能基本正常。     

儿童Caroli＇s病1例报告

Caroli＇s病又称为肝内胆管囊性扩张症或交通肝内胆管囊状扩张症,也有人称其为先天性肝内胆管囊状扩张症。1958年法国学者Caroli报告本病,此后国内外学者称其为Caroli＇s病[1]。本病临床上较为少见,我们近期发现1例,分析其临床资料,并复习了相关文献。     

先天性肝内胆管囊状扩张症（Caroli氏病）及其临床

Caroli氏病也称为先天性肝内胆管囊状扩张症,Caroli等曾对该病的临床特征做了详细的描述:①该病患者的肝内胆管呈节段性的囊状或柱状扩张;②对胆石病、胆     

Caroli病伴肾消耗病1例报告并文献复习

<正>Caroli病是一种先天性多发节段性的肝内胆道扩张合并先天性肝纤维化的疾病,临床表现多样,主要通过影像学表现诊断。Caroli病常并发多囊肾,但并发青少年肾消耗病(nephronophthisis,NPHP)的病例较为少见。现将1例Caroli病并发NPHP的患者报道如下。1病例资料患者女性,18岁,因"反复水肿11个月,肝功能异常7个月"于2016年10月24日入本院。患者11个月前无明显诱因出现双下肢水肿,未予诊治。7个月前患者于发热、干咳后水肿加重,由颜面部逐渐蔓延     

Caroli病合并多囊肾1例

病例报告女，35岁，近IWk来自觉上腹部不适、隐痛，有尿急尿频症状，无血尿脓尿现象‘彩超检查：肝脏内胆管呈柱状扩张，且见实质内多个大小不一圆形液性无回声，边界较清晰．双侧肾脏增大，其实质内数个大小不等圆形无回声，边界清楚．CT平扫示：肝实质内见胆管多发条状、囊状扩张，后者分布于肝顶部、肝左叶及右叶后段近被膜下．肝左叶萎缩，尾叶增大，肝裂增宽．脾脏增大（占8个肋单元）．双肾外形增大，肾实质内见多个大小不等囊性低密度区，边界清楚．影像学诊断：Caroli病合并多囊肾．Caroli病合并多囊肾1例@吴家良$辽宁省司法厅凌…     

先天性肝纤维化14例临床病理特点

目的总结先天性肝纤维化的临床特点,描述先天性肝纤维化时产生门静脉高压的病理形态学特点。方法回顾性分析2000年～2009年9月,北京友谊医院门诊及住院诊断为先天性肝纤维化患者的临床特点,对活检肝组织标本进行特殊染色及免疫组织化学染色。结果先天性肝纤维化14例,男女比例1.33：1,平均年龄（14.78±8.52）岁,临床主要表现为门脉高压症型占57.1%,胆管炎型占14.29%,混合型占7.14%。肝脾肿大为最主要的体征占85.7%。常见的合并症为多囊肾和肝内胆管扩张,即Caroli’s病各占14.28%。病理形态学观察提示先天性肝纤维化导致门脉高压的机制包括：门静脉发育不良,门脉数量减少,纤维间隔挤压门静脉。结论先天性肝纤维化是导致非肝硬化性门脉高压症的常见原因之一,掌握其临床及病理特点,有助于提高诊治水平。     

先天性肝纤维化伴Caroli病3例

先天性肝纤维与先天性肝内胆管护张症(又称Caroli病)是一组临床少见的常染色体隐性遗传性疾病,以儿童、青少年多发,临床常易漏诊、误诊,其病变常累及整个肝脏,且多与先天性肾囊肿等伴发.     

胰岛素瘤的解剖分布特点分析

目的胰岛素瘤是最常见的胰腺神经内分泌肿瘤，因其临床表现多样，导致诊断困难。影像学诊断尤其是超声内镜(EUS)在胰岛素瘤的诊断中起着重要作用，拥有较高的敏感性和特异性。本研究拟通过明确胰岛素瘤的解剖分布特点，以期有助于提高影像学的诊断准确率和降低漏诊率，尤其是在教育和培训实践中对于EUS的学习者更具有指导价值。 方法回顾性分析解放军总医院第一医学中心病案资料数据库1993年1月至2019年11月经外科手术、病理确诊为胰岛素瘤的患者的临床资料，检索方法采取搜索术后病理诊断为"胰岛素瘤"的病例，通过查阅病例的方法，提取出胰岛素瘤的大小和解剖分布等数据，进一步分析其特点。 结果共检索到确诊为胰岛素瘤的患者116例，其中，男45例、女71例，年龄13～76岁，平均年龄(44.4±14.85)岁。胰岛素瘤单发110例(94.8%)、多发6例(5.2%)。位置分布：头颈部46例(39.7%)，单发45例、多发1例；体尾部68例(58.6%)，单发65例、多发3例；全胰腺多发2例(1.7%)。病变大小特点：最大径0.4～3.4 cm，平均大小(1.53±0.58)cm。≤1 cm 29例、>1 cm而≤1.5 cm41例、>1.5 cm而≤2.0 cm28例，≤3 cm 15例，>3 cm 3例。年龄与肿瘤的大小相关，≤44岁患者肿瘤平均大小为(1.36±0.51)cm、>44岁患者肿瘤平均大小为(1.70±0.60)cm，P<0.05。头颈部的肿瘤大于体尾部的肿瘤，头颈部肿瘤平均大小(1.66±0.63)cm，体尾部(1.42±0.52)cm，P<0.05。 结论胰岛素瘤在胰腺体尾部较头颈部更好发；绝大多数单发，但可以全胰腺多发；多数小于1.5 cm，肿瘤的大小与患者年龄和肿瘤的解剖分布相关。     

Pathogenesis of carcinoma of the papilla of Vater

Most adenomas and carcinomas of the small intestine and extrahepatic bile ducts arise in the region of the papilla of Vater. In familial adenomatous polyposis (FAP) it is the main location for carcinomas after proctocolectomy. In many cases symptoms due to stenosis lead to diagnosis at an early tumor stage. In about 80%, curative intended resection is possible. Operability is the most relevant prognostic factor. Most ampullary carcinomas resp. carcinomas of the papilla of Vater develop from adenomatous or flat dysplastic precursor lesions. They can be sited in the ampulloduodenal part of the papilla of Vater, which is lined by intestinal mucosa. They also can develop in deeper parts of the ampulla, which are lined by pancreaticobiliary duct mucosa. Intestinal-type adenocarcinoma and pancreaticobiliary-type adenocarcinoma represent the main histological types of ampullary carcinoma. Furthermore, there exist unusual types and undifferentiated carcinomas. Many carcinomas of intestinal type express the immunohistochemical marker profile of intestinal mucosa (keratin 7?, keratin 20+, MUC2+). Carcinomas of pancreaticobiliary type usually show the immunohistochemical profile of pancreaticobiliary duct mucosa (keratin 7+, keratin 20?, MUC2?). Even poorly differentiated carcinomas, as well as unusual histological types, may conserve the marker profile of the mucosa they developed from. These findings underline the concept of histogenetically different carcinomas of the papilla of Vater which develop either from intestinal- or from pancreaticobiliary-type mucosa of the papilla of Vater. Molecular alterations in ampullary carcinomas are similar to those of colorectal as well as pancreatic carcinomas, although they appear at different frequencies. In future studies, molecular alterations in ampullary carcinomas should be correlated closely with the different histologic tumor types. Consequently, the histologic classification should reflect the histogenesis of ampullary tumors from the two different types of papillary mucosa.     

Demonstration of the mechanism of action of biguanides

Summary Palmitic acid oxidation in rat diaphragm homogenate is depressed by biguanide concentrations that are still incapable of inhibiting oxidative phosphorylation. Glucose oxidation is not directly effected by the same biguanide concentrations: however, the inhibitory effect of palmitic acid on glucose oxidation is partly removed by biguanides. Inhibition of fatty acid oxidation, which accounts for most of the metabolic effects caused by these drugs, can be regarded as the fundamental mechanism of action of biguanides. There is some evidence suggesting that these drugs might interact with carnitine, thus preventing long-chain fatty acids from being transported across the mitochondrial membrane to the site of oxidation. Traduzione a cura degli AA.     

Lack of correlation of degree of villous atrophy with severity of clinical presentation of coeliac disease

BACKGROUND AND AIM: Both the clinical presentation and the degree of mucosal damage in coeliac disease vary greatly. In view of conflicting information as to whether the mode of presentation correlates with the degree of villous atrophy, we reviewed a large cohort of patients with coeliac disease. PATIENTS AND METHODS: We correlated mode of presentation (classical, diarrhoea predominant or atypical/silent) with histology of duodenal biopsies and examined their trends over time. RESULTS: The cohort consisted of 499 adults, mean age 44.1 years, 68% females. The majority had silent coeliac disease (56%) and total villous atrophy (65%). There was no correlation of mode of presentation with the degree of villous atrophy (p=0.25). Sixty-eight percent of females and 58% of males had a severe villous atrophy (p=0.052). There was a significant trend over time for a greater proportion of patients presenting as atypical/silent coeliac disease and having partial villous atrophy, though the majority still had total villous atrophy. CONCLUSIONS: Among our patients the degree of villous atrophy in duodenal biopsies did not correlate with the mode of presentation, indicating that factors other than the degree of villous atrophy must account for diarrhoea in coeliac disease.     

血吸虫童虫生长发育及其机制的研究进展

血吸虫童虫是宿主免疫系统攻击的重要靶标,包括皮肤型、肺型和肝门型童虫。宿主分子对童虫生长发育具有重要作用。童虫生长发育机制包括免疫调节、信号转导、性别发育及凋亡等。肌动蛋白、组织蛋白酶、烯醇化酶和葡萄糖基转移酶等分子为血吸虫童虫生长发育的重要分子。本文对血吸虫童虫生长发育及其机制的研究进展做一综述。     

124例耐多药结核分枝杆菌基因突变特征分析

目的对临床分离的耐多药结核分枝杆菌相关基因的突变特征进行分析。方法对124例耐多药结核分枝杆菌以及50株敏感株的耐药相关基因(包括异烟肼inh A、kat G、oxyR-ahp C间隔区以及利福平rpo B)进行序列测定,分析其基因突变情况。结果异烟肼耐药inh A基因突变率为14.5%;kat G基因突变率为70.2%(87/124),主要位于315位;oxyR-ahp C间隔区突变率为15.3%;inh A、kat G两种基因同时突变率75.0%,三种基因同时突变率为89.5%。利福平rpo B基因突变的检出率高达95.2%,突变主要发生在531、526、516位点。结论我省耐多药菌异烟肼耐药相关基因最常见突变为kat G 315、inh A C-T(-15)、axyR-ahp C间隔区(-10)C-T,利福平为rpo B531、526、516。结合MDR-TB耐药相关基因的特征分析,可以建立一种快速、准确、特异的适合于我省的检测结核菌耐多药性的新方法。     

氯硝柳胺悬浮剂的毒性评价

目的评价氯硝柳胺悬浮剂的毒性，为现场大规模应用灭螺提供依据。方法按照中华人民共和国国家标准GB 15670-1995《农药登记毒理学试验方法》和鱼类毒性试验方法进行。结果经口、经皮肤的LDso雌、雄性大鼠均>5 000 mg/kg，经呼吸道的LCso雌、雄性大鼠均>5 000mg/m3，该药经口、经皮肤、经呼吸道毒性均属微毒类药物；兔眼用药后，观察期内无不良反应，对眼无刺激性；皮肤用药后对皮肤无刺激性。与氯硝柳胺原药、氯硝柳胺乙醇胺盐原药和氯硝柳胺乙醇胺盐可湿性粉剂相比，氯硝柳胺悬浮剂对鱼急性毒性最低。结论氯硝柳胺悬浮剂属微毒类药物，对鱼的毒性低于其乙醇胺盐可湿性粉剂，适合于现场应用。     

Assessment of quality of life of parents of children with juvenile chronic arthritis

The aim of the study was to assess the quality of life (QOL) and the psychological status of parents of children with juvenile chronic arthritis (JCA). The QOL, anxiety and depression of the parents of 28 children with JCA were evaluated and compared to those of the parents of 28 healthy children. Mothers of JCA children and mothers of healthy children reported similar QOL. The reported anxiety and depression levels were similar for mothers and fathers in both groups. The parents of children with pauciarticular-type JCA reported lower QOL and higher levels of anxiety and depression than the parents of children with other types, namely polyarticular and systemic JCA. These findings may be explained by the fact that the pauciarticular patients had shorter disease duration and were less frequently seen in the outpatient clinic. The QOL of mothers of children with JCA was found to be slightly impaired in the group of children with pauciarticular JCA. Future larger studies are needed to confirm these results, as the number of subjects in the three groups was rather low. Received: 26 September 2001 / Accepted: 8 February 2002     

Numerical Simulation of the Effect of Rate of Change of Glucose on Measurement Error of Continuous Glucose Monitors

Background

A 5-day in-patient study designed to assess the accuracy of the FreeStyle Navigator® Continuous Glucose Monitoring System revealed that the level of accuracy of the continuous sensor measurements was dependent on the rate of glucose change. When the absolute rate of change was less than 1 mg•dl⁻¹•min⁻¹ (75% of the time), the median absolute relative difference (ARD) was 8.5%, with 85% of all points falling within the A zone of the Clarke error grid. When the absolute rate of change was greater than 2 mg•dl⁻¹•min⁻¹ (8% of the time), the median ARD was 17.5%, with 59% of all points falling within the Clarke A zone.

Method

Numerical simulations were performed to investigate effects of the rate of change of glucose on sensor measurement error. This approach enabled physiologically relevant distributions of glucose values to be reordered to explore the effect of different glucose rate-of-change distributions on apparent sensor accuracy.

Results

The physiological lag between blood and interstitial fluid glucose levels is sufficient to account for the observed difference in sensor accuracy between periods of stable glucose and periods of rapidly changing glucose.

Conclusions

The role of physiological lag on the apparent decrease in sensor accuracy at high glucose rates of change has implications for clinical study design, regulatory review of continuous glucose sensors, and development of performance standards for this new technology. This work demonstrates the difficulty in comparing accuracy measures between different clinical studies and highlights the need for studies to include both relevant glucose distributions and relevant glucose rate-of-change distributions.     

Angiographic diagnosis of the degree of serosal invasion of carcinoma of the colon

Angiography using Prostaglandin El^® was performed on 38 patients with carcinoma of the colon in order to diagnose the degree of serosal cancer invasion. The findings at angiography were classified into four groups:1) AG-S3, abnormal change (irregularity and/or encasement) up to marginal vessels; 2) AG-S2, abnormality up to vasa recta; 3) AG-S1, abnormality of penetrating branches of vasa recta within the wall of the colon; and 4) AG-S0, no distinct findings of abovementioned vessels. These angiographic findings were compared with both macroscopic and microscopic serosal cancer invasion. Angiographic diagnosis is in accord with the macroscopic findings in 84.2 percent of cases. Angiographic diagnosis is in accord with the microscopic findings in 32.4 percent of cases. Macroscopic findings confirm the angiographic diagnosis precisely but the conflict with microscopic findings should not be overlooked. This may be the result of inflammatory change, adhesion, and fibrosis around carcinoma of the colon.