Peripheral hepatolithiasis incidentally found at autopsy

Summary Hepatolithiasis is a common disease in East Asia though very rare in the West. Four cases of hepatolithiasis in which calculi were incidentally found in the peripheral branches of the intrahepatic biliary tree at autopsy are described and compared with hepatolithiasis involving the major branches of the intrahepatic biliary tree. These four cases were all elderly, three patients were male and one female. The calculi were brown pigment stones in each case, as seen in the major branch type. The stone-containing ducts showed mild fibrosis and glandular proliferation with inflammatory changes in three cases; these changes were marked in the fourth case. The hepatic parenchyma around the stone-containing ducts was atrophic or collapsed in all four cases. The major branches of the intrahepatic biliary tree as well as the extrahepatic tree failed to show findings suggestive of bacterial infections or biliary anomalies. These data suggest that brown pigment stones develop primarily in the peripheral ducts in the liver. It remains uncertain whether the peripheral type eventually progresses to the major type or not.     

Risk of obstetric cholestasis in sisters of index patients

The aim of the present study was to evaluate the rate of intrahepatic cholestasis of pregnancy in first-degree relatives of index patients. Index patients (n=65) with singleton pregnancies complicated by intrahepatic cholestasis were identified among the women (n=11 984) who gave birth at Kuopio University Hospital in 1994-1998. The pregnancy histories of relatives of 56 index patients were reviewed and the rate of cholestasis in first-degree relatives was compared with that in the general obstetric population. Obstetric cholestasis was experienced by 9% of the parous sisters and 11% of the mothers of the index patients. The risk per delivery was 6% in the first-degree relatives. The rate in the general obstetric population was 0.54%. The odds ratios and 95% confidence intervals were 12.6 (5.6-28.1) for the sisters and 12.2 (6.2-24.2) for the mothers. Obstetric cholestasis clusters within some families and is under strong genetic influence, although the precise genetic pattern remains obscure. The sisters of index patients are at an increased risk of the disorder and may benefit from close obstetric care.     

Drug-induced intrahepatic cholestasis: characterization of different pathomechanisms

The pathogenesis of intrahepatic cholestasis in rats was studied using isolated perfused livers as an experimental model. Three basic mechanisms were differentiated: 1. Permeabilization of the bilio-sinusoidal barrier associated with electron microscopic alterations of the tight junctional complexes was found in livers of rats treated with -naphthylisothiocyanate (ANIT, 250 mg/kg body weight). Consequences of these alterations were: reflux of bile constituents such as taurocholate and sulfobromophthalein and increased access to the biliary space of paracellular markers such as inulin and sucrose. The clear-cut mechanism of ANIT cholestasis was used to distinguish other mechanisms of intrahepatic cholestasis. 2. Inhibition of the basic process of fluid secretion was found to be the primary event in the development of cholestasis induced by estrogens. After 5 days of treating rats with ethinyl estradiol (5 mg/kg/day), bile flow was diminished in isolated livers while the permeability of the biliary tract to sucrose and inulin was not affected. Accordingly, the maximal concentration of taurocholate in bile was increased, indicating that its secretion was sustained. The same effect was observed after 1 week of treatment with the depot estrogen estradiol valerate (1 mg/kg/week). After 3 weeks of treatment, however, the taurocholate concentration in bile was lowered and the clearance of sucrose was increased. Bile flow remained at the same cholestatic level for 20 weeks. These results suggest that estrogens have the potency to increase tight junctional permeability only in a second step in the development of cholestasis, following the inhibition of bile flow. 3. An additional mode of secretory inhibition was induced by lowering the concentration of Ca²⁺ in the perfusate of isolated liver. Using ANIT-pretreated livers, i. e., livers with very low capacity to secrete foreign dyes, a high rate of efflux of sulfobromophthalein into the perfusate of preloaded livers suggests stimulation of the efflux of cholephilic solutes across the sinusoidal membrane of liver cells.The results demonstrate that the term intrahepatic cholestasis comprises a number of different sites of interference with the complex process of bile secretion.Dedicated to Professor Dr. med. Herbert Remmer on the occasion of his 65th birthday     

Intravenous Lipid Emulsions in the Prevention and Treatment of Liver Disease in Intestinal Failure

The development of intestinal failure-associated liver disease (IFALD) in pediatric and adult patients on parenteral nutrition is usually multifactorial in nature due to nutritional and non-nutritional causes. The role of lipid therapy as a contributing cause is well-established with the pathophysiological pathways now better understood. The review focuses on risk factors for IFALD development, biological effects of lipids, lipid emulsions and the mechanisms of lipid toxicity observed in laboratory animals followed by a synopsis of clinical studies in pediatric and adult patients. The introduction of fish oil-based lipid emulsions that provide partial or complete lipid replacement therapy has resulted in resolution of IFALD that had been associated with soybean oil-based therapy. Based on case reports and cohort studies in pediatric and adult patients who were at risk or developed overt liver disease, we now have more evidence that an early switch to partial or complete fish oil–based lipid therapy should be implemented in order to successfully halt and reverse IFALD.     

磁共振胰胆管成像技术的临床应用

目的　探讨磁共振胰胆管成像（Ｍａｇｎｅｔｉｃ　ｒｅｓｏｎａｎｃｅ　ｃｈｏｌａｎｇｉｏｐａｎｃｒｅａｔｏｇｒａｐｈｙ,ＭＲＣＰ）技术的优势及临床意义。方 法３４例疑有胆道梗阻的患者,在常规ＭＲＩ扫描后,再以ＭＲＣＰ术行快速自旋回波重Ｔ２ＷＩ序列扫描,图像采用最大 强度投影法（ＭＩＰ）重建。结果２９例阳性患者清晰地显示了梗阻的部位、形态及肝内胆管扩张情况。结论　采用ＭＲＣＰ 技术,可以在不注入造影剂的情况下,取得类似内窥镜逆行胰胆管造影（ＥＲＣＰ）和经皮肝穿胆道造影的造影效果。它对 胆道梗阻有很高的敏感性和特异性。最大优势是无创伤性、无并发症。对年高、体弱及胆肠吻合术后ＥＲＣＰ插管困难者 尤为适宜,有望取代部分创伤性胆道造影技术。     

Surgical resection for non-Asian intrahepatic lithiasis: The Brazilian experience

BackgroundIntrahepatic lithiasis (IHL) is a rare disease in the western world. Complications associated with IHL include acute cholangitis, liver atrophy, secondary biliary cirrhosis, and risk for intrahepatic cholangiocarcinoma. Liver resection is considered the treatment of choice for IHL. The objective of this study was to analyze patients who underwent liver resection for non-Asian hepatolithiasis.Methods127 patients with symptomatic non-Asian hepatolithiasis underwent resection in six institutions. Demographic data, clinical presentation, diagnosis, classification according to stone location, presence of atrophy, bile duct stricture, biliary cirrhosis, incidence of cholangiocarcinoma, treatment and postoperative course were evaluated.Results52 patients (40.9%) were male and the mean age was 46.1 years. Sixty-six patients (51.9%) presented with history of cholangitis. Stones were located in the left lobe in 63 (49.6%), and right lobe in 28 patients (22.0%). Atrophy was observed in 31 patients (24.4%) and biliary stenosis in 18 patients (14.1%). The most common procedure performed was left lateral sectionectomy in 63 (49.6%) patients, followed by left hepatectomy in 36 (28.3%), right hepatectomy in 19 (15.0%), and associated hepaticojejunostomy in 28 (22.0%). Forty-two patients (33.0%) presented postoperative complications and the most common were biliary fistula (13.3%) and surgical site infection (7.0%). Postoperative mortality was 0.7%. Intrahepatic cholangiocarcinoma was observed in 2 patients (1.5%). Recurrence was identified in 10 patients (7.8%), mostly with bilateral stones and/or hepaticojejunostomy.ConclusionLiver resection is the standard treatment for symptomatic unilateral or complicated IHL with good operative results. Risk of cholangiocarcinoma was low in non-Asian patients.     

Functional anatomical hepatectomy guided by indocyanine green fluorescence imaging in patients with localized cholestasis: Report of four cases

BACKGROUNDLiver cancer is a malignant tumor with a high incidence. At present, the most effective treatment is laparoscopic hepatectomy (LH). Indocyanine green fluorescence imaging (ICG-FI) has become an important tool in LH, and the most common fluorescent types of tumors are total fluorescence, partial fluorescence, and rim fluorescence.CASE SUMMARYWe presented four cases of LH guided by ICG-FI in which we also observed the fourth special fluorescent type. When the tumor or intrahepatic stone compresses the adjacent bile duct to cause local cholestasis, the liver segment or subsegment with obstructed bile drainage will show strong fluorescence. Complete removal of the lesion together with the fluorescent liver parenchyma may help reduce the risk of tumor or stone recurrence.CONCLUSIONThis type of partial fluorescence can indicate local biliary compression, and the resection method is related to bile drainage, which may be called functional anatomical hepatectomy and ensures radical resection of the lesion.     

孕妇外周血NKT细胞水平与妊娠期肝内胆汁淤积症发病及病情关系

目的:探究孕妇外周血自然杀伤T(NTK)细胞水平与妊娠期肝内胆汁淤积症(ICP)发病及病情严重程度相关性。方法:选择2016年3月1日-2018年3月1日本院就诊的ICP患者52例(ICP患者组),根据ICP严重程度分为轻度ICP组(n=28)和重度ICP组(n=24),同时随机选择孕周相当的健康产前检查孕妇52例(健康孕妇组),比较各组外周血单个核细胞(PBMC)中NKT细胞水平,分析NKT细胞水平与ICP发生发展相关性。结果:ICP患者组外周血PBMC中NKT细胞含量高于健康孕妇组(P<0.05)。对ICP患者组和健康孕妇组外周血PBMC给予不同刺激物培养后,均显示出NKT细胞含量在正常组与空白组无差异(P>0.05),而在植物血凝素(PHA)组、ICP组、正常组依次降低(P<0.05)。重度ICP组外周血PBMC中NKT细胞含量高于轻度ICP组(P<0.05),NKT细胞含量与ICP病情严重程度呈正相关关系(P<0.05)。结论:ICP发生及进展与孕妇外周血NKT细胞水平呈正相关关系,监测NKT细胞水平可为诊断ICP提供新思路。     

脐动脉多普勒血流测定在肝内胆汁淤积症的应用

目的：评价脐动脉多普勒血流测定（Ｓ／Ｄ）与无钢荷试验（ＮＳＴ）在妊娠期肝内胆汁淤积症（ＩＣＰ）中的应用价值。方法：应用Ｓ／Ｄ与ＮＳＴ对孕龄为３５～４０周１５７例ＩＣＰ患者进行动态监测,并对新生儿Ａｐｇａｒ评分和羊水粪染的情况进行比较。结果：脐动脉血流Ｓ／Ｄ〈３、ＮＳＴ≥７分,胎儿预后良好;脐动脉血流Ｓ／Ｄ≥３、ＮＳＴ〈７分,胎儿预后不佳,两者差异有显著性（Ｐ〈０．００５）。结论：脐动脉多普勒血流检测弥补单纯ＮＳＴ对ＩＣＰ患者胎儿预后判断的不足,提高临床诊断准确率。     

妊娠肝内胆汁淤积症孕妇血及脐血中一氧化氮水平与胎儿脐血流的关系

目的　了解妊娠肝内胆汁淤积症 (ICP)时 ,孕妇血及脐血中一氧化氮 (NO)水平与胎儿脐动脉血流的关系 ,以进一步探讨ICP时胎盘循环阻力增高的原因。方法　对 35例ICP孕妇 (ICP)组 )及 32例正常孕妇 (对照组 ) ,用镉还原显色法测定母血及脐血NO水平 ;超声多普勒测定胎儿脐血流S/D值。结果　ICP组母血、脐血NO水平均明显低于对照组 (P <0 .0 1,P <0 .0 1) ;两组母血、脐血NO水平均有相关关系 (r =0 .5 481,r =0 .82 0 6 ,P<0 .0 1,P <0 .0 1) ,ICP组脐动脉血流S/D值明显高于对照组 (P <0 .0 1) ,母血、脐血NO水平与S/D值呈负相关(r =- 0 .34 15 ,r=- 0 .42 6 1,P <0 .0 5 ,P <0 .0 1。结论　ICP时母血、脐血NO水平降低对胎儿—胎盘循坏阻力增加有重要的作用