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[Portal vein thrombosis in HIV-infected patients: report of four cases].

Portal vein thrombosis (PVT) seems rare among HIV infected patients. Even though, the report of such cases is of great interest because it may help to determine the factors of occurrence. We describe cases of PVT in 4 HIV-infected men, aged 32 - 64. Two of them were co-infected with hepatitis C virus (HCV). The four patients had a history of disseminated mycobacterial infection (one case of tuberculosis, 3 cases of mycobacterium avium complex infection) with abdominal lymphadenitis. Despite HAART, their immunodeficiency was profound (CD4: 65 to 216/mm(3)). At the time of diagnosis, two patients were treated with protease-inhibitor containing regimen: indinavir (one case), ritonavir-saquinavir (one case). PVT was revealed by haematemesis (one case), abdominal pain (ome case), anasarca (2 cases). In three patients, the diagnosis of PVT was confirmed by imagery (echo-doppler or angio- RMI), and for the last patient, PVT was found during the transjugular intrahepatic portosystemic shunt setup. A low level of C protein was diagnosed in one case. Cirrhosis was not found in HIV-HCV co-infected patients. Two patients died early after diagnosis, one patient died 3 years after the onset of symptoms. Various factors may cause the development of a PVT in HIV infected patient. Serious immunodeficiency, opportunistic infections such as tuberculosis and mycobacterium avium complex related infection with abdominal lymphadenitis can further the development of PVT. Protease-inhibitor might have facilitated the process. Due to the severe prognosis of advanced cases, early evocation of diagnosis is needed.

AIDS-Related Opportunistic Infections↗

Extreme right lobar atrophy of the liver: a rare complication of autoimmune hepatitis.

Lobar atrophy is a rare morphologic change of the liver. We describe a 73-year-old woman with mild liver dysfunction and history of Sjögren's syndrome who had right hepatic lobar atrophy. Serum biochemistry levels were as follows: albumin, 4.5 g/dl; total bilirubin, 1.0 mg/dl; alanine aminotransferase, 25 international units/l; aspartate aminotransferase, 27 international units/l; alkaline phosphatase, 333 international units/l; and gamma-glutamyl transpeptidase, 332 international units/l. Serological data were as follows: rheumatoid factor, 27.9; anti-nuclear antibody, 1:640; and antismooth muscle antibody, 1:80. Viral markers for hepatitis B were all negative. Anti-hepatitis C virus (anti-c-100) was negative. Portal hypertension developed thereafter, and the patient died of hepatic failure at age 76. Postmortem examination revealed autoimmune hepatitis with moderate fibrosis, portal vein thrombus, and complete obstruction of the right hepatic duct due to hepatolithiasis. Terminal hepatic failure resulted from combination of decreased hepatic volume due to the right lobar atrophy, exacerbation of autoimmune hepatitis in the remnant left hepatic lobe, decreased portal venous blood flow due to thrombosis, portal hypertension, and cholangitis with hepatolithiasis. This is the first reported case of hepatic lobar atrophy due to autoimmune hepatitis. From a clinical standpoint, patients with hepatic lobar atrophy, even if asymptomatic, should be followed up with careful attention to progression of liver diseases, portal hypertension, and biliary complications.

Aged↗

Liver imaging in the diagnosis of hepatic venous thrombosis in paroxysmal nocturnal hemoglobinuria.

Eleven patients with paroxysmal nocturnal hemoglobinuria were studied by serial liver imaging. This disease has a generalized thrombotic tendency, and progressive diffuse hepatic venous thrombosis has been emphasized in recent studies. Radionuclide imaging proved to be of great help in establishing the diagnosis of hepatic venous thrombosis and following the progress of the disease process. Abnormal patterns in hepatic venous occlusion include (a) enlargement of the liver (especially the right lobe), (b) extrahepatic localization of radionuclide, and (c) areas of decreased uptake in the distribution of the involved veins. These findings develop rapidly and are very characteristic in the clinical setting.

Adolescent↗

Post-radiation survival time in hepatocellular carcinoma based on predictors for CT-determined, transarterial embolization and various other parameters.

AIM: In this retrospective study of unresectable hepatocellular carcinoma (HCC), we have investigated the efficacy of CT-derived parameters, laboratory measurements, clinical assessment and associated transarterial embolization (TAE) as predictors of post-radiotherapy survival time. METHODS: Sixty-six patients diagnosed with unresectable HCC that had undergone radiotherapy at two medical university hospitals in Taipei were enrolled in the study. Using multivariant analysis, pre-treatment parameters including tumor number and CT confirmation of PVT and ascites were compared. Multivariant analysis was also used for comparison of the mean pretreatment values for laboratory measurements, including alpha-fetoprotein, direct/total bilirubin and GOT/GPT levels, and clinical history of chronic hepatitis across the three survival-time categories. The chi2 was used to test the significance of the relationship between survival time and TAE procedure. The P values for the above tests were deemed statistically significant where P<0.05. RESULTS: Portal vein thrombosis (P = 0.032) and ascites (P<0.05) were negative predictors of post-radiation survival time. Low-grade liver cirrhosis (A or B), lower tumor volume and low levels of AFT, GOT/GPT, and total bilirubin were predictors of longer post-radiation survival time (P<0.05). CONCLUSION: The CT and clinical and laboratory assessment provide a reference for, and enable estimation of, probable survival times in HCC patients after radiotherapy. Tumor volume, severity of liver cirrhosis, status with respect to portal vein thrombosis and ascites and AFT, GOT/GPT and total bilirubin values were significant predictors of survival in this study.

Ascites↗

Vena cava 3D contrast-enhanced MR venography: a pictorial review.

Three-dimensional contrast-enhanced magnetic resonance venography (CE MRV) is a sensitive and accurate method for diagnosing vena cava pathologies. The commonly used indirect approach involves a nondiluted gadolinium contrast agent injected into an upper limb vein or, occasionally, a pedal vein for assessment of the superior or inferior vena cava. In our studies, a coronal 3D fast multi-planar spoiled gradient-echo acquisition was used. A pre-contrast scan was obtained to ensure correct coverage of the region of interest. We initiated contrast-enhanced acquisition 15 sec after the start of contrast agent injection and performed the procedure twice. The image sets were obtained during two 20-30-sec breath hold, with a breathing rest of 5-6 sec, to obtain the first-pass and delayed arteriovenous phases. For patients with Budd-Chiari syndrome, a third acquisition coinciding with late venous phase was collected to visualize the hepatic veins, which was carried out by one additional acquisition after a 5-6-sec breathing time. This review describes the clinical application of 3D CE MRV in vena cava congenital anomalies, superior and inferior vena cava syndrome, Budd-Chiari syndrome, peripheral vein thrombosis extending to the vena cava, pre-operational evaluation in portosystemic shunting and post-surgical follow-up, and road-mapping for the placement and evaluation of complications of central venous devices.

Adult↗

Primary deep venous septic thrombophlebitis in a child.

We describe a 12-year-old boy in whom primary Staphylococcus aureus thrombophlebitis of the femoral and iliac veins developed without history of trauma and with no association with acute osteomyelitis. The patient responded to therapy with intravenously administered antibiotics for 6 weeks, although oxacillin-associated hepatitis developed during the course of treatment.

Child↗

Morphological and hemodynamic changes in the portal venous system after distal splenorenal shunt: an ultrasound and pulsed Doppler study.

We investigated a group of patients who underwent distal splenorenal shunt using high-resolution real-time equipment and a duplex scanner with the aims: (i) to evaluate the rate of visualization of shunt; (ii) to assess change in size in the portal vein, and (iii) to characterize the flow pattern in the splenic vein and to study flow direction and velocity in the portal vein, thus adding new data on the efficacy of this operation in maintaining hepatic perfusion. Real-time ultrasonography was performed in 29 patients before surgery, after 7 to 30 days and after 4 to 12 months. Direct visualization of the shunt was achieved in 53.5% of the patients. Mean caliber of the portal vein significantly decreased after the operation: preoperative = 1.52 +/- 0.32; after 7 to 30 days = 1.32 +/- 0.16 (p less than 0.001), and after 4 to 12 months = 0.99 +/- 0.19 (p less than 0.001). The overall postoperative incidence of portal thrombosis was 22.2%. Thirteen of these patients also underwent a postoperative (4 to 36 months) pulsed Doppler investigation. Flow towards anastomosis was demonstrated in the splenic vein in 11 patients, and in 7 cases, typical caval pulsatility was observed. Decreased hepatopetal flow in the portal vein was found in 10 of 13 patients. In two patients, no flow was detectable and in the other the flow was hepatofugal. On the basis of our data, we can affirm that pulsed Doppler investigation may provide useful data for the evaluation of shunt patency and preservation of a decreased portal hepatic perfusion in the majority of patients.

Adult↗

Management of portal venous thrombosis in hepatic transplant recipients.

Portal venous thrombosis in hepatic transplant candidates is considered a relative contraindication to transplantation. In addition to thrombectomy, which is often technically impossible, donor portal venous arterialization or extra-anatomic venous bypass have been described. Two patients who underwent portal venous resection and subsequent anatomic reconstruction are presented herein. In the first patient, a graft with donor common iliac vein was interposed, and in the second, the donor portal vein was long enough to be anastomosed to the mesentericosplenic venous confluens. One patient is well 12 months after transplantation with patent portal vein and the other died of fungal sepsis after rejection treatment (the portal vein being open and unobstructed at autopsy).

Anastomosis, Surgical↗

Hemodynamic changes in patients with portal venous obstruction.

Splanchnic and systemic hemodynamics were evaluated in five adult patients with portal hypertension due to portal venous obstruction and were compared with those of patients with cirrhosis and those of a control group. In patients with portal venous obstruction, the gradient between wedged and free hepatic venous pressures ranged from 2 to 3 mm Hg, as in the control group, and was significantly lower than in patients with cirrhosis. Hepatic blood flow averaged 0.521 +/- 0.127 liter per min per m2 (mean +/- S.D.) and was significantly lower than that measured in cirrhotic and normal patients. Cardiac index averaged 3.788 +/- 0.629 liters per min per m2; this high value for cardiac index was not significantly different from that measured in cirrhotic patients, but was significantly higher than in patients of the control group. It is suggested that a hyperkinetic circulatory state may result from the development of portal-systemic venous collateral circulation in patients with portal venous obstruction having normal liver and hepatic venous pressures.

Adult↗

Cardiac catheterization through the internal jugular vein in pediatric patients. An alternative to the usual femoral vein access.

The percutaneous femoral vein approach is used routinely for cardiac catheterization in the pediatric age but in some children, it may be impossible as in the case of iliac vein or inferior vena cava thrombosis due to previous cardiac catheterization, or inconvenient as for right ventricular endomyocardial biopsies. In the period between 1982 and 1990, 160 cardiac catheterizations or right ventricular endomyocardial biopsies were performed in 102 children. Patients ranged in age between 2 months and 17 years (mean, 3.8 years) and in weight from 3.2 to 57.3 kg (mean, 14.4 kg). Indications for the internal jugular vein approach were as follows: (1) thrombosis of the inferior vena cava due to previous cardiac catheterization in 42 patients (41 percent); (2) right ventricular endomyocardial biopsy after cardiac transplant in 19 patients (19 percent); (3) control catheterization of the pulmonary arteries following classic or bidirectional cavopulmonary anastomosis in 16 patients (16 percent); (4) superior vena cava obstruction following Mustard's procedure in 14 patients (14 percent); (5) failed percutaneous femoral venous approach in six patients (6 percent); and (6) absence of the hepatic segment of the inferior vena cava in four patients (4 percent). The right or left internal jugular vein could be entered in all but three procedures (98 percent). Seventeen patients had more than one procedure through the same internal jugular vein and the vein was found patent in all. A complete right heart cardiac catheterization was performed using this route. Right ventricular endomyocardial biopsy and interventional procedure were performed through this route. Two major complications occurred. A patient developed a central transient ischemic attack and another patient developed a persistent Horner syndrome. Accidental carotid puncture occurred in five patients without consequences. Our data indicate that cardiac catheterization in infants and children can be performed safely through the internal jugular vein, with a high success rate and a low incidence of major complications.

Adolescent↗

[Liver transplantation in low portal vein flow: separation of portal vein areas with divided portal-venous and arterialized caval-venous liver perfusion. 1. Clinical case report].

A new method for the performance of a hepatic transplantation in spite of a low portal blood flow situation is described casuistically. In a 36-year-old-patient suffering from liver cirrhosis due to hepatitis B, the portal blood system of the right and left liver parts were divided, the left part was perfused with a low flow of portal blood, the right one with arterialized caval blood. The function of the transplanted liver and the early postoperative course were excellent. During the further postoperative course portal perfusion presumably diminished or stopped on the left side from three weeks and on the right side from two months postoperatively. Nevertheless the general condition of the patient improved continuously; transient elevations of transaminases may reflect the disturbance of portal perfusion. The technique of this arterialized caval blood perfusion of the portal system is presumably applicable also for situations, in which there is no portal blood flow available for perfusion of a liver graft. Thus, the absence of possibility for reconstruction of portal blood inflow or a situation with a hypoplastic portal vein may no longer be considered as a technical contraindication for liver grafting.

Adult↗

Small-for-size grafts in living-related liver transplantation.

BACKGROUND: The problems associated with small-for-size grafts in living-related liver transplantation are not fully understood. STUDY DESIGN: A consecutive series of 79 patients underwent 80 living-related liver transplantation procedures, including one retransplant, at the University of Tokyo from January 1996 to January 2000. They were divided into two groups by graft size: graft weight/recipient standard liver volume ratios of 40% or less (n = 24), and more than 40% (n = 56). Preoperative status, mortality, morbidity, duration of hospital stay, and postoperative graft function were examined and compared between the groups. RESULTS: The rate of patients who were restricted to the intensive care unit preoperatively was comparable between the groups (33% versus 21%, p = 0.27). The mean standard liver volume ratios were 37% in the small graft group and 84% in the large group. Survival rates were 80% (5 of 24) for the small graft group, which was significantly lower than that for the large group (96%, 54 of 56, p = 0.02). The rate of acute rejection was comparable between the groups (33% versus 43%, p = 0.47). Vascular complication was observed in 17% of the small graft group patients and 23% of the large group (p = 0.77). No difference was observed in the frequency of bile leakage or bile duct stenosis (25% versus 21%, p=0.77). Hyper-bilirubinemia and elongation of prothrombin time persisted longer in the small graft group than in the large group (p < 0.0001 for both). CONCLUSIONS: Our surgical results may suggest that a graft weight ratio of 40% or less provides a lower chance of survival after living-related liver transplantation.

Adolescent↗

[Good response in case of hepatocellular carcinoma with portal tumor thrombs--a case report of interdisciplinary local therapy].

A 56-year-old male patient with chronic C type hepatitis had HCC which invaded right portal vein trunk (Vp3). In August 2000, we performed intrahepatic artery infusion chemotherapy with CDDP and 5-FU under subcutaneous interferon alpha treatment. In addition, we used chemoradiation therapy for portal tumor thrombus in HCC. As the result of such therapy, the size of HCC and portal tumor thrombus reduced and the level of PIVKA-II decreased. There were no side effects except fever due to interferon alpha treatment. In February 2001, we performed devascularization and RFA therapy for HCC in S7 of liver under laparoscope. The level of PIVKA-II was within the normal range. It is important to perform interdisciplinary therapy appropriate for the HCC status.

Antineoplastic Agents↗

[Diarrhea as first clinical manifestation of hepatocellular carcinoma].

Paraneoplastic syndromes are frequently associated with various types of malignant tumors but are fairly rare in the course of hepatocellular carcinoma (HCC). We describe the clinical case of a 76 year old man with chronic hepatitis C infection related to liver disease who had suffered for several months from chronic runny but blood and mucus-free diarrhea, together with progressive weight loss and flushing of the face. Serological tests made on admission confirmed the chronic liver disease and showed an increase of serum levels of some neuroendocrine hormones, i.e. 5-hydroxytryptamine and vasoattive intestinal peptide. Ultrasound and CT scans led to the diagnosis of HCC. The diarrhea and the increase in some neuroendocrine hormones were therefore interpreted as expression of a paraneoplastic-like neuroendocrine syndrome that had preceded the onset of HCC by some months. The patient died a few months after the diagnosis of HCC, from total portal vein thrombosis and consequent liver and renal failure. This clinical report draws the attention to the possibility of paraneoplastic syndrome expression before the clinical onset of HCC and to the role that neuroendocrine hormones may have on the growth and spread of HCC.

Aged↗

Adjuvant arterial infusion chemotherapy after resection of hepatocellular carcinoma with portal thrombosis: a pilot study.

BACKGROUND/PURPOSE: The prognosis of hepatocellular carcinoma (HCC) with tumor thrombosis of the main trunk or major branches of the portal vein (mPVTT) is extremely poor, even if it is curatively resected. Uncontrollable multiple metastases to the residual liver are often observed within several months after the operation. We report here the results of a pilot study, showing the efficacy of adjuvant arterial infusion chemotherapy after the resection of HCC with mPVTT. METHODS: Twelve patients had curative resection of HCC with mPVTT. Six of the patients were treated by the arterial infusion of a chemotherapeutic agent via a subcutaneously implanted injection port after curative resection of HCC with mPVTT. The initial course consisted of the daily administration of cisplatin (CDDP) and continuous infusion of 5-fluorouracil (5-FU). This was followed by the weekly or biweekly administration of CDDP and subsequent infusion of 5-FU until the cumulative dose of 5-FU reached 15 g. RESULTS: The median overall survival time was 58.0 months with adjuvant chemotherapy and 8.0 months without adjuvant chemotherapy. The median disease-free interval was 15.0 months with adjuvant chemotherapy and 4.0 months without adjuvant chemotherapy. Adverse reactions were tolerable nausea and loss of appetite. CONCLUSIONS: This chemotherapeutic regimen achieved favorable results and may be useful as adjuvant chemotherapy in treating patients after curative resection of HCC with mPVTT.

Adult↗

Insulin, C-peptide and glucagon levels during OGTT in hepatic cirrhosis and in patients with prehepatic block.

In order to investigate pancreatic B-cell function in hepatic cirrhosis and to elucidate the role of porto-caval shunt-circulation in the development of hyperinsulinism and hyperglucagonemia in cirrhotic patients, blood glucose, plasma insulin and glucagon, and serum C-peptide concentrations were measured during OGTT in 11 control and 16 cirrhotic subjects as well as in 7 patients with prehepatic block secondary to thrombosis of the portal vein. Insulin and glucagon levels were significantly higher in the cirrhotic than in the control group (for insulin: p less than 0.01, less than 0.001, less than 0.01 and less than 0.05 at 0, 60, 90 and 120 min, respectively; for glucagon: p less than 0.01, less than 0.01, and less than 0.05 at 0, 30 and 60 min, respectively). Serum C-peptide levels were, however, similar in the two groups with the exception of the 30-min value, which was significantly lower in the cirrhotic group (p less than 0.05). Plasma insulin and glucagon concentrations in patients with prehepatic block were similar to those of the controls but significantly lower than the values found in cirrhotic patients (for insulin: p less than 0.05 at 0, 30, 60 min, respectively). Serum C-peptide levels of these patients were not significantly different either from the control values or from those obtained in the cirrhotic group. Accordingly, pancreatic B-cell secretion is not increased in hepatic cirrhosis. Hence, the hyperinsulinism is due to decreased heptic degradation of the hormone. Decreased degradation of both insulin and glucagon should be attributed mainly to parenchymal liver damage, rather than porto-systemic shunting.

Adolescent↗