妊娠期肝内胆汁淤积症诊疗指南(2015)

<正>妊娠期肝内胆汁淤积症(intrahepatic cholestasis of pregnancy,ICP)是一种重要的妊娠期并发症,主要导致围产儿死亡率增加。其发病有明显的地域和种族差异,迄今国际上尚无有关ICP的统一诊治意见。2011年,中华医学会妇产科学分会产科学组组织国内有关专家,制订了第1版《妊娠期肝内胆汁淤积症诊疗指南》,该指南的发表对我国ICP诊治的规范起到了重要的指导作用。近年来,在ICP诊治方面又有了新的认识,为此,对其进行了重新修订。ICP曾有过许多命名,也引起了诊断和治疗的混乱,自第1版ICP诊疗指南发表后,国内教科书     

妊娠肝内胆汁淤积症与血清25-羟基维生素D3和TNF-α相关性分析

目的观察妊娠肝内胆汁淤积症与血清25-羟基维生素D_3和TNF-α相关性。方法选取32例妊娠肝内胆汁淤积症(ICP)患者及30例正常孕妇,检测血清中胆汁酸、25-羟基维生素D_3[25-(OH)D_3]、TNF-α浓度,并分析三者之间相关性。结果年龄、既往胆石症病史、双胎妊娠、总胆红素、胆汁酸、25-(OH)D_3、TNF-α等指标在两组的差异具有统计学意义(P0.05);年龄、25-(OH)D_3及TNF-α水平是妊娠期妇女发生ICP独立危险因素;ICP胆汁酸与25-(OH)D_3水平呈负相关(β=-0.74,P0.01),与TNF-α水平呈正相关(β=0.56,P0.05),与年龄无关(β=-0.16,P0.05)。结论 ICP患者普遍存在血清25-(OH)D_3严重不足和TNF-α大量释放。     

熊脱氧胆酸治疗妊娠期肝内胆汁淤积症48例临床观察

妊娠期肝内胆汁淤积症（ICP）多发于妊娠中、晚期,以肝内胆汁淤积为特征。ICP的主要危害是导致早产、死胎和胎儿宫内窘迫。目前尚无有效治疗方法。1998年1月至2003年12月,我们对24例ICP患者采用熊脱氧胆酸治疗,并进行了临床观察。现报告如下。     

中西医结合治疗妊娠期肝内胆汁淤积症的疗效观察

正妊娠期肝内胆汁淤积症(ICP)是妊娠中晚期特发性疾病,主要临床表现为皮肤瘙痒和胆汁酸升高,主要危害胎儿,使围生儿的早产率和死亡率显著升高~([1])。ICP的发病率在0.8%~12.0%,发病机制尚不清楚,可能与女性激素、某些药物、遗传和环境等因素相关,可以在本次妊娠分娩后迅速消失,当再次妊娠或者口服雌激素避孕药等时,常可复发。ICP的出现对妊娠期预后的影响,主要表现在胎儿宫内窘迫、早产、死胎、产后     

肝内胆汁淤积症诊治专家共识(2021年版)

肝内胆汁淤积症是指由于肝实质细胞和/或肝内胆管疾病引起胆汁合成缺陷、排泌异常, 以及胆管中胆汁流动的机械性或功能性障碍, 继而使得胆汁成分入血所致的临床症候群, 常见于胆汁淤积性肝病、妊娠期肝内胆汁淤积症以及遗传代谢相关胆汁淤积等。近年来肝内胆汁淤积症的诊治资料有了进一步积累, 为将更新的诊疗依据提供给广大肝病医务工作者, 特组织专家在2015年《肝内胆汁淤积症诊治专家共识》基础上进行共识更新, 旨在为临床肝内胆汁淤积症诊治提供参考和指导。     

妊娠肝内胆汁淤积症患者胎儿缺氧的影响因素

妊娠肝内胆汁淤积症(intrahepatic cholestasis of pregnancy,ICP)是一种特发于妊娠期的疾病.ICP孕妇预后尚好,但胎儿宫内窘迫、早产、死胎却是ICP的主要危害[1].     

妊娠期肝内胆汁淤积症病因及发病机制

妊娠期肝内胆汁淤积症(ICP)是以妊娠期发生皮肤瘙痒、黄疸，产后症状迅速消失为特点的妊娠期并发症。因其容易引起胎儿窘迫、早产、死胎、死产，故被列为高危妊娠之一。现对ICP病因和发病机制的研究进展综述如下。     

妊娠期肝内胆汁淤积症母胎血清胆汁酸谱研究进展

<正>妊娠期肝内胆汁淤积症(Intrahepatic Cholestasis of Pregnancy,ICP)以妊娠中晚期发生皮肤瘙痒、黄疸为主要症状~[1]。ICP孕妇的预后良好,但是对胎儿危害很大,容易发生早产、胎儿窘迫,死胎、死产等不良结局。ICP的重要特点是孕妇及胎儿的血循环中的胆汁酸水平明显增高。多种不同类型的胆酸存在于血液中,共同构成胆汁酸谱。研究表明,不同疾     

妊娠期肝内胆汁淤积症134例护理分析

妊娠期肝内胆汁淤积症（intrahepatic cholestasis of pregnancy,ICP）是妊娠中、晚期特有的并发症,临床上以皮肤瘙痒和黄疸为特征,ICP孕妇预后良好,主要危害胎儿,使围生儿发病率和死亡率增高,本文对134例ICP孕妇的护理进行回顾性分析,现报道如下。     

ABCB11缺陷病

遗传性婴儿胆汁淤积症是儿童期肝病死亡或致残的重要原因.随着分子医学的发展,ABCB11缺陷病等一系列遗传因素引起的婴儿肝内胆汁淤积症被发现和认识.ABCB11缺陷可引起几种临床表现不同的胆汁淤积,包括进行性家族性肝内胆汁淤积症2型(PFIC2)、良性复发性肝内胆汁淤积症2型(BRIC2)、妊娠期肝内胆汁淤积症(ICP2)以及药物性胆汁淤积症(DIC).     

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。     

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。     

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。     

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.