Arsenicosis,possibly from contaminated groundwater,associated with noncirrhotic intrahepatic portal hypertension

Background and Aims

Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH), a chronic microangiopathy of the liver caused by arsenicosis from use of contaminated groundwater, was reported from Asia. This study aimed to see, if in the twenty-first century, arsenicosis was present in NCIPH patients at our hospital and, if present, to look for groundwater contamination by arsenic in their residential locality.

Methods

Twenty-seven liver biopsy proven NCIPH patients, 25 portal hypertensive controls with hepatitis B or C related cirrhosis and 25 healthy controls, matched for residential locality, were studied. Eighty-four percent to 96 % of study subjects belonged to middle or lower socioeconomic category. Arsenicosis was looked for by estimation of arsenic levels in finger/toe nails and by skin examination. Arsenic levels in nails and in ground water (in NCIPH patients with arsenicosis) was estimated by mass spectrometry.

Results

Nail arsenic levels were raised in five (10 %) portal hypertensive study subjects [two NCIPH patients (both had skin arsenicosis) and three portal hypertensive controls]. All of these five patients were residents of West Bengal or Bangladesh. Skin arsenicosis was noted in three NCIPH patients (11 %) compared to none of disease/healthy controls. Ground water from residential locality of one NCIPH patient with arsenicosis (from Bangladesh) showed extremely high level of arsenic (79.5 μg/L).

Conclusions

Arsenicosis and microangiopathy of liver, possibly caused by environmental contamination continues in parts of Asia. Further studies are needed to understand the mechanisms of such ‘poverty-linked thrombophilia’.

     

Reduction of portal vein pressure with the enlargement of portal-systemic shunts: observation made in one patient

Whether portal vein pressure is reduced as spontaneous portal-systemic shunts are enlarged has long been disputed. We measured portal vein pressure directly by percutaneous transhepatic catheterization in one patient with cirrhosis at a 4-year interval, and demonstrated a significant reduction in portal vein pressure that had occurred along with enlargement of a collateral paraumbilical vein and splenorenal shunt during this period.     

Hepatic encephalopathy due to a large intrahepatic communication between the portal vein and inferior vena cava

A 59 year old man with cirrhosis presented with encephalopathy and hyper-ammonaemia. T1 weighted magnetic resonance imaging demonstrated a large void tubular structure connecting the right posterior portal vein branch and the inferior vena cava through the right hepatic lobe inferiorly, and cine-mode imaging showed a flow within this channel. Clearly in this patient a significant portion of the portal venous blood was being shunted into the inferior vena cava, causing encephalopathy. The exact origin of this channel is not known, but several possibilities are discussed. It is also predicted that similar previously unknown large intrahepatic shunts will be discovered increasingly with the availability of modern imaging techniques.     

非肝硬化门脉高压患者临床特点分析

目的 总结非肝硬化门脉高压症(NCPH)患者的临床特点和肝静脉压力梯度(HVPG)的变化.方法 2017年1月～2019年12月南京市第二医院住院的28例NCPH患者,采用Seldinger法穿刺右侧颈内静脉,使用一次性球囊导管测定肝静脉压力,计算HVPG,接受肝活检检查.结果 在本组28例NCPH患者中,诊断特发性门...     

Bile duct stricture due to caused by portal biliopathy: Treatment with one-stage portal-systemic shunt and biliary bypass

Portal biliopathy is a rare complication of extrahepatic portal vein obstruction. Jaundice occurs in symptomatic patients with fibrotic strictures. Short-term improvement in such patients can be achieved with endoscopic retrograde cholangio-pancreatography with balloon dilatation and stent placement. Surgery in these patients is traditionally two staged. We report the results of a one-stage procedure combining non-selective portal-systemic shunt surgery with biliary bypass, performed successfully on a 24-year-old man with a tight biliary stricture resulting from portal biliopathy. At 18-month follow up, the patient shows he is doing well, with normal liver function tests.     

Subclinical encephalopathy after portosystemic shunts in patients with non-cirrhotic portal fibrosis

Cerebral function was studied in patients with non-cirrhotic portal fibrosis who had no clinically detectable neurological abnormality, 14 before and 29 after a proximal lienorenal shunt operation. In each patient electroencephalography (EEG) and psychometric tests (the number connection test, construction of a five-pointed star and the reverse counting test) were performed. Psychometric tests were also done in an equal number of matched, healthy controls. Subclinical encephalopathy was diagnosed when any of these tests was abnormal. In the unoperated group the EEG was normal in all patients and there was no difference in the psychometric test results between patients and healthy controls. After portosystemic shunt operations, the EEG was abnormal in 10%, the number connection test in 38%, construction of a five-pointed star in 19% and the reverse counting test in 30% of the patients. Detection of subclinical hepatic encephalopathy in such a high proportion of operated patients with non-cirrhotic portal fibrosis suggests that the surgical procedure and its haemodynamic consequences per se could have been responsible.     

门静脉高压患者门静脉压力与血流动力学的相关性研究

目的 探讨门静脉高压患者门静脉血流动力学的变化特点及其与门静脉压力的相互关系。方法 采用彩色多普勒超声对４１例肝硬化门静脉高压患者（Ｃｈｉｌｄ Ａ、Ｂ级３１例、Ｃ级１０例）于手术前检测门静脉（ＰＶ）、脾静脉（ＳＶ）和肠系膜上静脉（ＳＭＶ）的内径和血流速度，再计算出相关的面积和血流量；于手术时对３１例ＣｈｉｌｄＡ十Ｂ级患者直接测量门静脉压力。３２例健康人和２６例慢性乙型肝炎患者（慢肝组）作为对照。结累 门静脉高压两组患者ＰＶ、ＳＶ和ＳＭＶ内径（ｃｍ）分别为１．５１和１．５２、１．３２和１．３４及１．１５和１．１５较慢肝组和正常组明显增宽，ｒ分别为１．３１和１．１６、０．９６和０．７９及０．９１和０．８２（Ｐ＜０．０１）；血流速度较正常组和慢肝组明显减慢（Ｐ＜０．０１）；门静脉高压Ｃ级组门静脉血流速度（ｃｍ／ｓ）为４．６５较门静脉高压Ａ十Ｂ级组（６．４２）明显减慢（Ｐ＜０．０１），而两组 ＳＶ和 ＳＭＶ的血流速度则差异无显著意义（Ｐ＞０．０５）；门静脉高压 Ａ＋Ｂ级组三条静脉的血流量明显大于正常组和慢肝组（Ｐ＜０．０１或Ｐ＜０．０５）；门静脉高压Ｃ级组门静脉血流量明显小于Ａ十Ｂ级组（Ｐ＜０．０１）；而ＳＶ和ＳＭＶ的血流     

Influence of different portal vein branches on hepatic encephalopathy during intrahepatic portal shunt via jugular vein

This letter is regarding the study titled ‘Targeted puncture of left branch of intrahepatic portal vein in transjugular intrahepatic portosystemic shunt (TIPS) to reduce hepatic encephalopathy’. Prior to the approval of TIPS dedicated stents (Viatorr stents) in China in October 2015, Fluency covered stents were typically used. As Fluency covered stents have a strong support force and axial elastic tension, a ‘cap’ may form if the stent is located too low at the end of the hepatic vein or too short at the end of the portal vein during surgery, leading to stent dysfunction. Since the blood shunted by the stent is from the main trunk of the portal vein, the correlation between the incidence of postoperative hepatic encephalopathy and the location of the puncture target (left or right portal vein branch) is worth discussion. Notably, no studies in China or foreign countries have proven the occurrence of left and right blood stratification after the accumulation of splenic vein and mesenteric blood flow in the main trunk of the portal vein in patients with cirrhotic portal hypertension.     

Embolization of splenorenal shunt associated to portal vein thrombosis and hepatic encephalopathy

Hepatic encephalopathy (HE) is a cognitive disturbance characterized by neuropsychiatric alterations. It occurs in acute and chronic hepatic disease and also in patients with portosystemic shunts. The presence of these portosystemic shunts allows the passage of nitrogenous substances from the intestines through systemic veins without liver depuration. Therefore, the embolization of these shunts has been performed to control HE manifestations, but the presence of portal vein thrombosis is considered a contraindication. In this presentation we show a cirrhotic patient with severe HE and portal vein thrombosis who was submitted to embolization of a large portosystemic shunt. Case report: a 57 years-old cirrhotic patient who had been hospitalized many times for persistent HE and hepatic coma, even without precipitant factors. She had a wide portosystemic shunt and also portal vein thrombosis. The abdominal angiography confirmed the splenorenal shunt and showed other shunts. The larger shunt was embolized through placement of microcoils, and the patient had no recurrence of overt HE. There was a little increase of esophageal and gastric varices, but no endoscopic treatment was needed. Since portosystemic shunts are frequent causes of recurrent HE in cirrhotic patients, portal vein thrombosis should be considered a relative contraindication to perform a shunt embolization. However, in particular cases with many shunts and severe HE, we found that one of these shunts can be safely embolized and this procedure can be sufficient to obtain a good HE recovery. In conclusion, we reported a case of persistent HE due to a wide portosystemic shunt associated with portal vein thrombosis. As the patient had other shunts, she was successfully treated by embolization of the larger shunt.