先天性肝内胆管囊性扩张症1例

先天性肝内胆管囊性扩张症即Caroli’s病,1958年由Jaequc Caroli首次详细描述并提出。此病是一种少见病,我院于2011年3月18日收治1例,现报告如下。1病例资料患儿,女,10岁,因＂面色苍白2年,呕血、黑便3个月,腹痛2 d＂入院。2年前患儿无明显诱因出现面色苍白,伴有头晕和全身乏力,无发热,家长未予重视。2010年12月患儿面色苍白明显加重,并出现呕咖啡色样物质1次,量不多,非喷射性,     

The rate of cholangiocarcinoma in Caroli Disease A German multicenter study

BackgroundCaroli Disease (CD) and Caroli Syndrome (CS) are rare disorders presenting with dilation of the intrahepatic bile ducts. CD/CS are associated with cholangiocarcinoma (CCA). However, the true incidence of CCA is still unclear, although it may serve as an indication for surgery. In this paper, we analyzed (I) the incidence of CCA in German centers, (II) reviewed our single center population together with its clinical presentation and (III) performed a thorough literature review.Methods17 large HPB-centers across Germany were contacted and their patients after surgical treatment due to CD/CS with histopathology were included. Medline search for all studies published in English or German literature was performed. Patients who underwent surgery at our department between 2012 and 2020 due to CD or CS were analyzed.ResultsIn the multicenter study, 79 patients suffered from CD and 119 patients from CS, with a total number of 198 patients. In 14 patients, CCA was found (Overall: 7,1%; CD: 6,3%, CS 7,6%). Between 2012 and 2020, 1661 liver resections were performed at our department. 14 patients underwent surgery due to CD or CS. Histological examination showed synchronous cholangiocarcinoma in one patient.The literature review revealed a CCA-rate of 7,3% in large series, whereas in case reports a rate of 6,8% was found.ConclusionThere is risk of malignant transformation and patients with CD might also benefit from resection due to improvement of symptoms. Therefore, resection is strongly advised.As certain patients with CS require transplantation, treatment should not be guided by the relatively low rate of CCA but by the concomitant diseases that come along with hepatic failure.     

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.     

Caroli病Ⅱ型伴门脉高压2例

Caroli病是一种临床少见的先天性疾病,临床表现多样,易漏诊、误诊。本文通过2例Caroli病Ⅱ型伴门脉高压病例分析并复习相关文献,以提高临床医师对本病的认识,及时作出正确的诊断治疗。     

Bile duct cyst in adults:Interventional treatment,resection,or transplantation?

Cystic dilatations of the bile ducts may be found along the extrahepatic biliary tree,within the liver,or in both of these locations simultaneously.Presentation in adults is often associated with complications.The therapeutic possibilities have changed considerably over the last few decades.If possible,complete resection of the cyst(s)can cure the symptoms and avoid the risk of malignancy.According to the type of bile duct cyst,surgical procedures include the Roux-en-Y hepaticojejunostomy and variable types of hepatic resection.However,the diffuse forms of Todani type Ⅴ cysts(Caroli disease and Caroli syndrome)in particular remain a therapeutic problem,and liver transplantation has become an important option.The mainstay of interventional treatment for Todani typeⅢbile duct cysts is via endoscopic retrograde cholangiopancreatography.The diagnostic term"bile duct cyst"comprises quite different pathological and clinical entities.Interventional therapy,hepatic resection,and liver transplantation all have their place in the treatment of this heterogeneous disease group.They should not be seen as competitive treatment modalities,but as complementary options.Each patient should receive individualized treatment after all of the clinical findings have been considered by an interdisciplinary team.     

Caroli病引发上消化道出血1例报告

1病例资料患者女性,18岁,回族,主因“间断呕血、黑便7年,加重3 d”于2018年10月18日收住本院。7年前患者因呕血、黑便于当地医院就诊,诊断为“上消化道出血”,予以输血、止血等内科保守治疗后好转。5年前因上腹胀痛伴黑便于宁夏某医院就诊,完善相关检查诊断为“多囊肝”,行胃镜检查无食管胃底静脉曲张,予以对症处理。期间患者间断有呕血、黑便症状,伴恶心,平均每年发作1次,多次予以输血等内科保守治疗。2年前患者因“食管胃底静脉曲张破裂出血、门静脉高压症”行经颈静脉肝内门体分流术,术后仍有反复呕血、黑便,平均每半年发作1次,均予以抑酸、止血、输血等内科保守治疗。     

Caroli病24例临床分析

目的提高临床医师对Caroli病的认识。方法回顾性总结解放军总医院2005年1月至2010年10月24例Caroli病患者的一般资料、临床特点以及治疗方法。结果本组患者男女比例1∶2.4;平均发病年龄20.1岁;临床表现以上腹痛、发热、黄疸、消化道出血为主;胆管结石是最常见的合并症;影像学检查发现21例(87.5%)及胎儿1例;20例行外科手术治疗,其中肝移植2例,随访4个月至5年一般情况良好;2例行内镜下食管胃底静脉曲张硬化剂、组织胶治疗,分别随访3个月、1年无再发出血。结论Caroli病患者起病早,临床表现多样,诊断主要依靠临床表现、影像学检查,外科手术及内镜下治疗是有效的治疗方式。     

Caroli综合征患者18例的临床诊治特点分析

目的:对Caroli综合征(CS)患者的临床、实验室检查结果和影像学特点进行总结和分析,以期加深对本病的认识,并探讨提高早期诊断可能性的方法。方法:收集2008年1月至2021年6月在北京协和医院住院治疗并经病理或临床、影像学诊断为CS的18例患者,回顾性分析患者的一般资料,临床表现,实验室检查结果(白细胞计数、血红蛋...     

Caroli病的影像学表现

目的 探讨Caroli病的影像表现及各种影像方法对其应用价值.方法 回顾性分析经MRCP、ERCP、PTC或手术病理证实的7例Caroli病患者的影像资料,并复习相关文献.结果 ①超声（6例）与CT（5例）平扫检查均显示肝内囊柱形或梭形病变.②超声与CT共显示特异性征象5例：超声与CT同时显示“囊尾征”2例,CT显示“中心点征”并“索条征”1例,CT分别显示“中心点征”与“索条征”各1例.“中心点征”与“索条征”均系64排CT容积平扫结合MPR薄层重建显示.③超声与CT共显示胆管结石5例、胆囊结石1例与门脉高压症2例,并显示右肾旋转不良1例、右肾多囊肾伴结石1例.④MRCP（4例）、ERCP（2例）或PTC（1例）均直观地显示扩张胆管的部位、大小、形态、范围、扩张程度及其伴发的胆管结石和胆管炎,其特征性征象系“悬挂征”,MRCP结合原始资料或常规MRI显示门脉高压性肝硬化并腹水1例、右肾旋转不良1例、右侧多囊肾伴结石1例.结论 超声与CT能部分显示本病的特异征象,而ERCP、PTC与MRCP对其有确诊价值;MRCP结合原始资料或常规MRI有利于本病的分型及合并症的检出,且简便无创,应列为首选检查.     

Diagnosis and management of choledochal cyst: 20 years of single center experience

We report the first case series from Africa and the Middle East on choledochal cyst,a disease which shows significant geographical distribution with high incidence in the Asian population.In this study,the epidemiological data of the patients are presented and analyzed.Attention was paid to diagnostic imaging and its accuracy in the diagnosis and classification of choledochal cyst.Most cases of choledochal cyst disease have type?ⅠandⅣ-A cysts according to the Todani classification system,which support the etiological theories of choledochal cyst,especially Babbitt’s theory of the anomalous pancreaticobiliary duct junction,which are clearly stated.The difficulties and hazards of surgical management and methods used to avoid operative complications are clarified.Early and late postoperative complications are also included.This study should be followed by multicenter studies throughout Egypt to help assess the incidence of choledochal cysts in one of the largest populations in Africa and the Middle East.     

Ⅱ型Caroli病1例

病例：患者男,21岁,学生,因“呕血、黑便”于2008年11月26日入院。患者11年前曾有类似发作史,无明显诱因出现恶心、呕吐。呕吐物如咖啡样,内含少量食物,量约500ml,解黑色稀便2次（量不详）,伴头晕、心悸。于当地医院就诊．经B超等影像学检查诊断为“特发性门静脉高压合并上消化道出血”,行“门奇静脉断流＋脾切除术”,     

Caroli病合并多囊肾、胆总管狭窄1例报道

本文通过报道1例典型的Caroli病(Caroli’s disease,CD)合并多囊肾、胆总管狭窄(癌变可能)患者的病史资料及治疗回顾,并对比分析腹部CT、MRI、MRCP、ERCP等影像学检查,进一步探索该病的临床症状、诊断、鉴别以及治疗情况。     

Rare cystic liver lesions: A diagnostic and managing challenge

Cystic formations within the liver are a frequent finding among populations.Besides the common cystic lesions,like simple liver cysts,rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis.Thorough knowledge of each entity’s nature and course are key elements to successful treatment.Detailed search in PubMed,Cochrane Database,and international published literature regarding rare cystic liver lesions was carried out.In our research are included not only primary rare lesions like cystadenoma,hydatid cyst,and polycystic liver disease,but also secondary ones like metastasis from gastrointestinal stromal tumors lesions.Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided.A diagnostic and therapeutic algorithm is also proposed.The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities,diagnostic tools,and treatment modalities is stressed.Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team,in order to receive the most appropriate treatment,since many cystic liver lesions have a malignant potential and evolution.     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.