特发性门静脉高压症3例

本院2009年11月-2010年4月收治特发性门静脉高压症(IPH)患者3例,现报道如下. 一、病例资料 A患者,男性,13岁,因发现脾大2个月于2009年入院.既往病史提示:患者于家中接生,剪断脐带时未严格消毒;B患者,男性,19岁,因发现脾大3年于2009年11月入院,2008年2月及2009年9月曾发生上消化道出血,表现为呕血、便血.     

Portal biliopathy--a lesser known complication of portal hypertension. Case report and review of the literature

Portal biliopathy is a newly introduced term, describing the changes observed in the biliary ducts of portal hypertension patients. We present here the case of a patient diagnosed with portal vein thrombosis secondary to a chronic pancreatitis; the abnormalities in the bile ducts were observed during the endoscopic retrograde cholangiopancreatography. We also review the existing information on this subject in the literature.     

Multiple duodenal polyps in uraemia: a little known clinical entity

Multiple duodenal polyps were found by endoscopy in five out of 33 patients with chronic renal failure and in one of 300 controls. In the uraemic cases the polyps were caused by circumscribed nodular hyperplasia of Brunner's glands. One of these five patients later underwent surgery for duodenal obstruction caused by the polyps; the other patients were asymptomatic. One of the controls had multiple duodenal polyps. The recorded incidence of multiple duodenal polyps was significantly higher among patients with chronic renal failure than in patients without renal disease. Patients with chronic renal failure and polyps did not differ significantly from the other renal patients in the comparison of variables such as gastric acid secretion, serum concentrations of gastrin and group I pepsinogens or mean length of dialysis treatment. The mean pH of gastric resting juice was significantly higher, however, in uraemic patients with polyps than in those without. Uraemic patients displayed a gastric acid secretion capacity within normal range, and significantly raised serum gastrin and group I pepsinogen concentrations.     

Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a review of literature

Idiopathic portal hypertension (IPH) is characterized by dense fibrosis of portal tracts and portal venous obliteration. Little is known about the etiopathogenesis of IPH. Association of various autoimmune diseases such as systemic lupus erythematosus in IPH suggests that IPH may share immunological disturbances with such autoimmune diseases. We recently experienced two autopsy cases presenting with both diffuse scleroderma and IPH. Dense fibrosis was found in both the dermis and intrahepatic portal tract of these cases. In addition, small vascular damages were commonly observed to various degrees in these fibrotic areas of both organs. The activation of fibroblasts and vascular damages mediated by various growth factors and cytokines reportedly involved in the dermis in scleroderma might have also been operative in portal tracts in these two cases of IPH. A review of literature disclosed eight overlapping cases of IPH and scleroderma (middle- to old-aged females), and scleroderma was diagnosed earlier than IPH. These findings suggest that similar pathogenetic processes are operative in the dermis as well as in the portal tracts of the liver in these cases.     

采用覆膜支架经颈静脉肝内门体分流对改善术后分流道狭窄及门静脉高压的系统评价

目的 比较采用覆膜支架和裸支架的经颈静脉肝内门体分流术(TIPS)对改善术后分流道狭窄及门静脉高压的临床疗效的影响.方法 检索各大数据库分别采用覆膜支架和裸支架行TIPS后关于临床疗效的对照研究,评价文章质量.运用Revman4.2软件对TIPS分流道狭窄率、门静脉高压相关临床症状复发率和病死率进行分析.结果 共获得1340例相关病例,随机对照研究(12.8%对43.9%,P=0.004)与多项非随机对照试验研究(11.6%对57.6%,P＜0.01)均表明,覆膜支架较裸支架能明显降低TIPS术后1年分流道狭窄率,两类研究报道的覆膜支架组门静脉高压相关症状的复发率也较裸支架组显著降低(7.7%对29.3%,6.4%对14.5%,P＜0.05).结论 覆膜支架在降低TIPS术后分流道狭窄、减少门静脉高压相关临床症状复发方面优于裸支架,这对于改善TIPS术后患者生存时间及质量具有重要意义.     

Adrenal myelolipoma. 6 cases and a review of the literature

Adrenal myelolipoma is an uncommon, benign and hormonally inactive tumor. Most lesions are asymptomatic and usually are discovered incidentally at autopsy studies. Authors report on 6 patients (5 women, 1 man) with adrenal myelolipomas (5 right, 1 left), analyze their morphological findings and association with an adrenal hormonal overproduction. Five of the patients underwent surgery because of tumor size, in 3 of them histological evaluation confirmed myelolipoma and in 2 cases an adrenocortical adenoma with foci of myelolipoma. All the patients were asymptomatic and in 4 cases hormonal overproduction was not found. One female patient has oveproduction of dehydroepiandrosteron-sulphate (DHEAS) indicating a 3beta hydroxylase deficiency in this tumor and 1 patient has primary aldosteronism with a histological finding of an association of adrenocortical adenoma with foci of myelolipoma. Neither Cushings syndrome nor congenital adrenal hyperplasia were present in our group of patients.     

Transjugular intrahepatic portosystemic shunt in the treatment of portal vein thrombosis: a critical review of literature

Reports of successful transjugular intrahepatic portosystemic shunt (TIPS) surgery in patients with portal vein thrombosis (PVT) are considered anecdotal owing to the technical difficulty of the procedure and potential procedure-related complications. A literature review was undertaken to determine the feasibility and safety of TIPS in the treatment of PVT. All studies in which TIPS was attempted in patients with PVT were identified by searching through the PUBMED and MEDLINE databases. A total of 424 PVT patients undergoing TIPS were reported in 54 articles. The success rate of TIPS insertion was 67–100% in 19 case series. Further, 85 patients with portal cavernoma underwent successful TIPS insertions. Three therapeutic strategies of TIPS placement were used: (1) TIPS placement followed by portal vein recanalization via the shunt, (2) portal vein recanalization via percutaneous approaches followed by TIPS placement, and (3) TIPS insertion between a hepatic vein and a large collateral vessel without portal vein recanalization. Four approaches were used to access the portal vein: transjugular, transhepatic, transsplenic, and transmesenteric. Intra-abdominal hemorrhage secondary to hepatic capsule perforation was lethal in only three patients. No episode of pulmonary embolism was reported. Other procedure-related complications were reversible. The overall incidence of shunt dysfunction and hepatic encephalopathy was 8–33% and 0–50%, respectively. In conclusion, the reviewed studies uniformly support the feasibility and safety of TIPS for PVT even in the presence of portal cavernoma. Further, several major issues that remain unresolved are discussed.     

Noncirrhotic portal hypertension associated with didanosine: a case report and literature review

Noncirrhotic portal hypertension (NCPH) has recently been reported as a liver complication in human immunodeficiency virus (HIV)-infected patients and has been found to be associated with exposure to didanosine. Here, we describe the case of an HIV-infected patient with portal hypertension who initially presented with massive ascites and portal vein thrombosis. The patient's HIV-1 infection was well-controlled with highly active antiretroviral therapy (lamivudine/didanosine plus nevirapine) for 3 years since its diagnosis in 2007. He had no history of alcoholism, drug abuse, or liver diseases. An extensive work-up for other possible causes of liver disease was performed, but the results were inconclusive. In addition to reporting this case, we have reviewed the literature on didanosine-related NCPH and analyzed the findings of 61 similar previously reported cases.     

IgM myeloma: 6 cases and a review of the literature

IgM myeloma is a rare plasma cell neoplasia, with an estimated incidence of 0.5% in patients with myeloma. Approximately, between 2 and 3.3% of IgM monoclonal gammopathy are IgM myeloma. Six unpublished cases of IgM myeloma, association of an IgM monoclonal gammopathy and an exclusive plasma cell neoplasia, are reported. Forty-six other cases have been found in the literature. The initial clinical characteristics of these patients are: sex-ratio of 1.1, mean age of 62 years, fatigue in 95% of the cases, bone pain in 80%, osteolytic lesions in 78%, fever in 13%, hepatomegaly and splenomegaly in 8%, lymphadenopathy in 10%, hemorrhagic diathesis in 35% and neurologic involvement in 18%. Initial biological features are: anemia in 62% of the cases, creatininemia greater than 20 mg/l in 10%, calcemia greater than 120 mg/l in 24%. Mean serum IgM level is 33 g/l, mean medullary plasmocytosis is 52%. 80% of the patients presented with IgM kappa and only 20% with IgM lambda. Proteinuria with light chains are found in 65%. One-year survival is estimated at 82%, 2-year at 62%, 3-year at 46% with a median of 30 months. No prognostic factor is found. IgM myeloma with characteristics of both myeloma and macroglobulinemia appears well individualized among B-cell neoplasia. However, the distinction between Waldenstr?m's macroglobulinemia and IgM myeloma can be difficult in case of lympho-plasmocytic bone marrow proliferation with osteolytic lesions.     

Noncirrhotic portal hypertension in HIV-infected patients: a case control evaluation and review of the literature

Idiopathic noncirrhotic portal hypertension (NCPH) is an infrequent but possibly underestimated cryptogenetic liver disease recently described in small series of HIV-infected patients. The exposure to antiretroviral drugs, a direct role of HIV itself, microbial translocation from the gut, or a thrombophilic propensity have been suggested as possible pathogenic mechanisms. In this case control study, we describe 11 HIV-infected patients with idiopathic NCPH and compare the activity of protein C and S, and soluble CD14 levels (a surrogate marker of the translocation of intestinal bacterial products) with 10 age- and gender-matched HIV-infected controls with no liver disease. The clinical presentation of the 11 patients with NCPH was characterised by acute variceal bleeding (2/11), ascites (2/11), portal thrombosis (2/11), and ultrasonographic and endoscopic signs of portal hypertension (11/11), with slightly high alanine transaminase (ALT) and γglutamyl transpeptidase (γ-GT) levels. The FibroScan median liver stiffness was 8.1?kPa, which is inconsistent with significant fibrosis, and nodular regenerative hyperplasia was diagnosed in the 5 patients who underwent liver biopsy. The NCPH patients showed no impairment of hepatic synthesis, but had lower serum albumin levels and a higher international normalized ratio (INR) than the controls (p?=?0.01), and lower protein C and S activity, although within the normal range (p?=?0.02 and 0.3, respectively). No significant difference in soluble CD14 was seen between the two groups. In conclusion, the etiology of NCHP is not still established, but in order to prevent the dramatic complications of portal hypertension, all HIV-infected patients with unexplained liver enzyme abnormalities or thrombocytopenia should be considered for further investigations by means of thrombophilic screening, Doppler ultrasound evaluation, and in the presence of portal hypertension, endoscopy and liver biopsy.     

特发性非肝硬化性门脉高压1例并文献复习

<正>特发性非肝硬化性门脉高压(idiopathic non-cirrhotic portal hypertension,INCPH)是一种血管性疾病,临床表现为门脉高压的一系列症候,但缺乏肝硬化的病理学表现,约占门脉高压症患者的10%。因缺乏相对特异的诊断标准,易误诊。现报道1例我科诊治的以脾功能亢进症为首发症状的、经13年就医路终确诊的INCPH患者,以期提高临床医师对该病的诊治水平。1病例摘要患者女,68岁。主因“发现脾肿大13年,间断腹泻8月”于2020年9月9日入院。患者于13年前因“急性胆囊炎、胆囊结石”于我院普外科行手术治疗,     

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.     

Pulmonary hypertension in pregnancy: two cases and review of the literature

Pulmonary arterial hypertension (PAH) in pregnancy carries a mortality of 30–56%. There are few published data to guide clinicians in its management. Two pregnant women with severe PAH have been treated at Royal Perth Hospital with a successful result in both. Their presentation and management are described. We review the physiological changes in pregnancy, pathophysiology in PAH, and review the literature describing treatment of PAH in pregnancy.     

门静脉闭锁致门脉高压相关性肺动脉高压1例并文献复习

目的:复习罕见门静脉解剖异常导致门脉高压性肺动脉高压(PPHTN)的相关文献,了解这类患者临床特点。方法:回顾分析1例成人门静脉闭锁导致PPHTN患者的临床经过和特点,并以"门静脉闭锁"、"门脉高压性肺动脉高压"为检索词,在万方数据库和中国全文期刊数据库中进行检索,以"atresia of portal vein"、"pulmonary hypertension"为检索词在pubmed全文数据库中进行检索。结果:患者女性,71岁,3年前因反复"肝性脑病"发现门静脉主干闭锁,脾静脉纤细;肠系膜上静脉-左肾静脉门体分流形成。胃底周围见静脉曲张,门腔静脉自发分流形成可能。2年6个月前出现双下肢水肿,1月前活动耐力明显下降,伴夜间阵发性呼吸困难,伴腹胀。UCG示下腔静脉增宽,心室呈"D"型影,s PAP84mm Hg(1mm Hg=0.133kPa)。因患者入院后出现院内感染及急性肾损伤,未能行右心导管检查,结合病史及检查结果,除外肺实质疾病、肺血栓栓塞症、结缔组织疾病及其他可能导致肺动脉高压(PAH)的药物使用或毒物接触史,同时确定患者存在门脉高压导致的胃底静脉曲张,诊断患者为非肝病性门静脉解剖异常导致的PPHTN。共检索中文文献2篇,外文文献24篇,保留较为详细病例资料文献15篇,涉及病例25例。结论:非肝病性门脉高压所形成的门体分流可以导致高动力性PAH,先天性门体分流者多见,继发于成人门静脉闭锁者罕见,早期识别随访,及时针对PAH进行干预治疗,维护右心功能,可能会延迟右心衰竭发生的时间,改善预后。     

Two cases of esophageal cancer with portal hypertension: esophagectomy with venous shunt procedure

We performed venous shunt procedure in the reconstruction of the esophagus after esophagectomy using the gastric tube in two cases of esophageal cancer with portal hypertension due to liver cirrhosis. In both cases, the short-term postoperative course was uneventful, without congestion in the gastric tube. In Case 1 where the short gastric vein had been used as the shunt vein, the long-term postoperative course was also uneventful, without hepatic encephalopathy or hemorrhage from deterioration of the varices of the gastric tube. However, in Case 2 where the left gastroepiploic vein had been used, hepatic encephalopathy developed due to excessive shunt flow. These results suggested that appropriate shunt flow could be expected by using short gastric vein.     

Recurrent malignant hypertension: a report of two cases and review of the literature

Malignant hypertension (MHT) is a rare and life-threatening condition which is defined clinically as severe hypertension accompanied by bilateral retinal haemorrhages and/or hard exudates, with or without papilloedema. If untreated, the prognosis of MHT is poor. With MHT being a relatively rare condition, it would be unusual to see it on more than one occasion in the same patient. We describe in detail two cases from a disease register of 400 cases of MHT seen in one medical centre over 33 years.     

肝硬化门静脉高压伴自发性门体分流患者的诊治现状

自发性门体分流(SPSS)在临床上并未得到足够重视,其在肝硬化患者中的发生率达38%~40%,并且在46%~70%的顽固性脑病患者中可见。SPSS封堵可能是治疗肝硬化顽固性脑病的一种安全且有效的手段。经颈静脉肝内门体分流术(TIPS)在预防和治疗门静脉高压并发症中的作用已经得到证实,对于肝硬化门静脉高压伴食管胃底静脉曲张破裂出血且可见SPSS形成的患者,行TIPS降低门静脉压力预防曲张静脉再出血是必要的,同时联合SPSS封堵可能会降低TIPS的术后并发症,更大程度发挥TIPS优势使患者最大程度获益。目前,仍需要大量的随机对照试验来进一步证实。