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Biomedical subjects

E Zanon

Publications and source records attributed to E Zanon.

At least 37 records · Page 2Linked to original sources

Anti-beta2-glycoprotein I antibodies in patients with acute venous thromboembolism: prevalence and association with recurrent thromboembolism.

To establish the prevalence of antibodies against beta2-glycoprotein I (beta2GPI) in unselected patients with venous thromboembolism, as well as the association with antiphospholipid antibodies (aPL) and a history of previous thromboembolism, we investigated the presence of these antibodies in 227 consecutive patients with acute deep vein thrombosis or pulmonary embolism, of whom 63 were carriers of aPL with or without lupus anticoagulant (LA), and seven were carriers of LA alone. The presence of antibodies against beta2GPI was demonstrated in 19 patients [8.4%; 95% confidence interval (CI), 4.5-11.3%]. All of them belonged to the group of 63 patients with aPL (30.2%). A history of a previous thromboembolism was identified in 11 of the 19 patients with anti-beta2GPI antibodies (57.9%) and in 45 of the 208 patients without these antibodies [21.6%; odds ratio (OR)=4.98; 95% CI, 1.89-13.1; p<0.0005]. In the subgroup of patients with aPL and/or LA, the rate of recurrent thromboembolism among patients with anti-beta2GPI antibodies (11 of 19, 57.9%) was significantly higher than that observed in patients without these antibodies (15 of 51, 29.4%; OR=3.3; 95% CI, 1.1-9.83; p=0.28). We conclude that in patients with acute venous thromboembolism the prevalence of antibodies against beta2GPI is unexpectedly high. The presence of these antibodies seems to identify a subgroup of patients with antiphospholipid antibodies who have a peculiarly high risk of thrombotic recurrences. Further prospective studies are indicated to better define the role of anti-beta2GPI antibodies in the development of recurrent thromboembolism.

Acute Disease↗

Psychological aspects and coping of parents with a haemophilic child: a quantitative approach.

Although haemophilia is a relatively rare hereditary disease, and is curable by blood products, the psychological and social problems of haemophilic patients and of their families are always serious. Anxiety for the risks of bleedings and the fear for infections transmitted by blood products cause stress and difficulty in coping with the situation. The aim of this paper is to assess resources, stress, and coping in parents with a haemophilic child. In order to measure the dimensions related to family stress and resources, social desirability, tendency to depression, and anxiety, the subjects were administered the short-form of the Questionnaire on Resources and Stress by Friedrich et al. revised by Saviolo & Cristante, along with the Social Desirability Scale, the Beck Depression Inventory, and the Ipat Anxiety Scale. The subjects of this study were 20 couples who were the parents of haemophilic sons. We analysed how the parents perceived the behavioural characteristics of their child, the differences between mothers and fathers in the questionnaire scores, and the correlation among the considered dimensions. The results show that the parents paid great attention to the problems of their sons, but mothers were more depressed and anxious about the disease of their child.

Adaptation, Psychological↗

Frequent but low titre factor VIII inhibitors in haemophilia A patients treated with high purity concentrates.

The development of inhibitor antibodies is one of the more important complications in the management of haemophilia patients. In a previous study, the prevalence of inhibitor varies between 5 and 52%, seems to be different for different types of concentrates, and is less frequent in multitransfused patients. In our prospective study we followed for 3 years 62 multitransfused haemophilia patients without inhibitor or past history of inhibitor. Thirty-seven haemophilia patients were treated with intermediate purity factor VIII concentrates, whereas 25 were given high purity concentrates (from the eighth month of the study five of these patients were treated with recombinant products). Factor VIII inhibitor antibody developed in seven of 25 haemophilia patients treated with high purity concentrates or recombinant products, whereas none of the haemophilia patients treated with intermediate purity concentrates had inhibitors. The difference was statistically significant (P < 0.001; OR = 0.06, 95% CI 0.001-0.3). In all patients, the titre of the inhibitor was low and no problem occurred in their management. Since inhibitors appeared in multitransfused patients when transfused with high purity concentrates and/or when the patients were switched to recombinant FVIII product, the development of inhibitor seems to be due to the administration of a new concentrate. Therefore this potential complication must be considered every time a new concentrate is administered.

Antibodies↗

Venous thromboses of upper limbs are more frequently associated with occult cancer as compared with those of lower limbs.

Three hundred and forty-three consecutive patients with deep vein thrombosis (DVT) were investigated for the possible presence of occult or undiagnosed cancer, of whom 305 patients had DVT of the lower limbs whereas 38 had DVT of the upper limbs. Cancer was diagnosed during a 12-month follow-up in nine patients with DVT of the upper limbs (23.7%) and in 34 patients with DVT of the lower limbs (11.1%). The difference was statistically significant. Furthermore, it was shown that the majority of cancers (seven of nine) in the case of DVT of the upper limbs were discovered during the first week of hospital admission. In contrast, in the case of DVT of lower limbs, only eight of 34 cancers were discovered during the initial investigation. Lung cancer and lymphomas represented the majority of cancers associated with upper limb venous thrombosis (seven of nine). In the case of DVT of the lower limbs, cancers were heterogeneous; however, 12 of 34 were cancers of the colon or prostate.

Arm↗

Combined factor V and factor VII deficiency due to an independent segregation of the two defects.

A patient with combined factor V and factor VII deficiency is described together with a family study. The propositus appeared to be double heterozygous for factor V and factor VII deficiency. Since the patient showed a parallel decrease of activity and antigen, he appeared to be double heterozygous for a true deficiency. The patient had inherited the factor V defect from the mother and the factor VII defect from the father. The parents of the propositus were not consanguineous. Other family members were found to have isolated factor V or factor VII deficiency. This is the third family so far described with this peculiar combined defect but the first to be investigated by clotting and immunologic assays.

Adolescent↗

Do hemophilia A and von Willebrand disease protect against carotid atherosclerosis? A comparative study between coagulopathics and normal subjects by means of carotid echo-color Doppler scan.

Atherosclerosis is a multifactorial disease caused by genetic and environmental factors with important clinical sequelae. The aim of this study was to evaluate the degree of carotid atherosclerosis by echo-color Doppler scan in a group of patients affected by hemophilia A and von Willebrand disease versus a group of normal subjects apparently free of atherosclerotic risk factors. All coagulopathics and normal patients who came to our Internal Medicine Department (Padua Hospital) underwent physical exam, blood analysis, standard electrocardiogram, chest x-ray, echo-color Doppler scan, and a thorough history. We examined 156 subjects, 76 coagulopathics (46 men, 30 women) and 77 normals (37 men, 40 women). Coagulopathics were affected by hypertension in 28.9% of cases, diabetes mellitus in 6.5%, dislipidemia in 17.1%, smoke in 39.4%, and obesity in 36.8% (p < .05). Echo-color Doppler scan revealed carotid plaques in 27.2% of control patients versus 13.1% of coagulopathics (p < .05). Hemophilics and subjects with von Willebrand disease with a more serious illness had fewer plaques than those with lighter defects. Coagulopathics showed 23.6% of the plaques we revealed on the whole, versus 76.3% of control subjects (p < .01), with a lighter degree of stenosis (p < .01). Our data demonstrate that patients with hemophilia A and von Willebrand disease have fewer carotid plaques and a smaller degree of carotid stenosis than normal subjects of the same sex and age. These data seem to strengthen the hypothesis that blood coagulation defects may allow protection against carotid atherosclerosis and its sequelae.

Aged↗

Serological markers of autoimmunity in patients with hemophilia A: the role of hepatitis C virus infection, alpha-interferon and factor VIII treatment in skewing the immune system toward autoreactivity.

The aim of this study was to investigate whether the immune system of patients with hemophilia A is skewed toward an aspecific activation and to identify the causative factors. It is well known that an immune derangement does exist in patients with hemophilia A. At least three factors potentially play a role: hepatitis C virus (HCV) infection, alpha-interferon therapy and the administration of factor VIII (FVIII). Sixty human immunodeficiency virus (HIV)-negative patients with severe or moderate hemophilia A were studied retrospectively. The serological markers of autoimmunity were evaluated and the results correlated with anti-HCV antibodies, FVIII treatment and alpha-interferon therapy. The role of these factors in the development of the anti-FVIII antibody was estimated concomitantly. The prevalence of autoantibodies and anti-FVIII antibodies was higher in HCV-positive than in HCV-negative patients before any treatment, although the difference was not statistically significant. The administration of FVIII further influenced the development of autoantibodies both in HCV-negative and HCV-positive patients, with no difference being observed between the two groups. As expected, fewer HCV-negative than HCV-positive patients developed anti-FVIII antibodies after administration of FVIII (31.8% versus 38%, respectively). Therapy with alpha-interferon did not seem to enhance significantly the risk of developing autoantibodies nor anti-FVIII antibodies. We observed a high prevalence of humoral signs of autoimmunity among patients with hemophilia A. Treatment with FVIII concentrate is probably the most important triggering factor. Monitoring these patients for autoimmune manifestations is recommended.

Adolescent↗

Short-term effects of transjugular intrahepatic portosystemic shunt (TIPS) on functional liver plasma flow in patients with advanced cirrhosis.

AIMS/BACKGROUND: TIPS, an effective procedure applied for the treatment of complications of portal hypertension, is potentially followed by worsening of the hyperdynamic circulation of cirrhosis and the impairment of liver function. The aim of the present study was to evaluate short-term changes of functional liver plasma flow after application of TIPS, using the hepatic (extrarenal) clearance of D-sorbitol (S-HCl). METHODS: Twenty-five cirrhotic patients submitted to TIPS for prevention of variceal rebleeding entered the study. At steady-state, during constant infusion of a solution of D-sorbitol (25 mg/min), appropriate blood and urine samples were collected in order to calculate S-HCI before and 120 min after TIPS opening. In addition, the hepatic extraction ratio of D-sorbitol was directly measured at the level of the right (Er), where TIPS was applied, and of the left (El) hepatic veins; meanwhile the portocaval gradient (PCG) was registered, before and after stent dilation. A comparison of values obtained before and after TIPS application was performed by Student's t-test for paired data. RESULTS: After application of TIPS, a substantial reduction was observed in PCG (12.1+/-4.2 vs 24.8+/-4.3 mmHg; p<0.001) and Er values (20.6+/-14.8 vs 57.5+/-22.3 %; p<0.001) but not El values (47.4+/-22.0 vs 53.4+/-21.4 %; p=0.178). S-HCl measured 120 min after TIPS opening was not statistically different from pre-TIPS values (389.2+/-212.1 vs 394.6+/-152.7 ml/min; p=0.892), although S-HCl variations in Child-Pugh class B patients were positively correlated with portal pressure variations (r=0.63, p=0.016). CONCLUSION: Our results demonstrate that in patients with advanced cirrhosis, TIPS procedure, while effective in reducing portal hypertension, does not lead to alterations in the functional liver plasma flow within the first 2 h.

Aged↗

[Percutaneous transcatheter therapy of visceral pseudoaneurysms].

BACKGROUND: The purpose of this work is to show the effectiveness of percutaneous embolization therapy in the treatment of visceral arterial pseudoaneurysms and to propose the method as a valid alternative to surgery. METHODS: Twenty-eight patients with visceral artery pseudoaneurysms were treated: 10 of them were hepatic; 9 renal; 7 duodenal-pancreatic; 1 splenic and 1 of the celiac trunk. Materials employed were: Gianturco metallic coils, Spongostan and Poli-Vinyl-Alcohol. RESULTS: Immediate success was obtained in all cases; recurrences occurred in 5 patients (18%) and they have all been successfully retreated with percutaneous method; complications occurred in 3 patients (10%) and they were all directly related to the pre-existing pathology and not to the percutaneous treatment. CONCLUSIONS: To obtain the best results from embolization, it is necessary to respect some conditions regarding the correct choice of embolic materials, the superselective catheterization of the injured artery and the low injection pressure of the contrast medium in order to avoid rupturing the thin pseudoaneurysmatic wall. The advantages of the percutaneous treatment are: less trauma, local anesthesia, reduction of the time between diagnosis and therapy, reduction of hospitalization stay and limitation of parenchymal destruction, Complications are relatively not frequent and in no cases the percutaneous therapy compromised the surgical intervention when it became necessary.

Aneurysm, False↗

Hyperdynamic circulation in patients with cirrhosis: direct measurement of nitric oxide levels in hepatic and portal veins.

BACKGROUND/AIMS: Peripheral vasodilation represents the main vascular dysfunction associated with the hyperdynamic circulation of liver cirrhosis. This study was intended to measure directly regional and systemic levels of nitric oxide, a potent vasorelaxing mediator, in order to assess its role in the development of hemodynamic changes of cirrhosis. METHODS: We compared nitric oxide levels in the splanchinic and systemic circulation of 25 patients with cirrhosis undergoing transjugular intrahepatic portosystemic stent shunt and in the hepatic vein and peripheral blood of 10 patients without cirrhosis submitted to venous catheterization. Nitric oxide levels were measured through electron paramagnetic resonance spectroscopy as nitrosylhemoglobin complexes. RESULTS: Significantly higher nitric oxide levels were calculated in patients with cirrhosis with respect to controls, both in the peripheral and hepatic veins. In patients with cirrhosis, nitric oxide levels in the portal vein (3.44 +/- 2.17, expressed in arbitrary units) were higher than in the systemic circulation (1.89 +/- 1.15), but lower than in the hepatic vein (4.75 +/- 2.53; p < 0.001 by variance analysis). CONCLUSIONS: These data suggest that nitric oxide synthetic pathway activity as well as nitric oxide release are enhanced at the level of splanchnic vasculature and, more important, in the hepatic tissue, confirming evidence of the predominant role of nitric oxide in the pathogenesis of hemodynamic changes in patients with cirrhosis with portal hypertension.

Aged↗

Factor V antigen levels in APC resistance, in factor V deficiency and in combined APC resistance and factor V deficiency (pseudohomozygosis for APC resistance).

Factor V antigen levels were measured in 40 patients with factor V deficiency (11 homozygous and 29 heterozygous), in 38 patients with factor V Leiden mutation (16 homozygous and 22 heterozygous) and in three patients with combined heterozygous factor V deficiency and heterozygous factor V Leiden mutation (so-called pseudohomozygosis for APC resistance). Twenty normal subjects of both sexes served as controls. Factor V antigen levels compared well with factor V activity in normal subjects and in all groups of patients. They were normal both in homozygous and heterozygous APC resistance patients. Factor V antigen determination may be useful for the diagnosis of pseudohomozygosis for APC resistance. These patients have a phenotypic picture similar to homozygous APC resistance, but show a factor V antigen level about half the normal value since they are compound heterozygotes for factor V deficiency and APC resistance. In contrast, homozygous patients for APC resistance show normal factor V activity and antigen levels.

Drug Resistance↗

[The percutaneous placement of intra-arterial catheters with "reservoirs" for subcutaneous infusion. The technic and preliminary results].

INTRODUCTION: We report our personal technique and the preliminary results of percutaneous implantation of intraarterial catheters connected to a subcutaneous infusion reservoir (Port-a-cath) for the regional chemotherapy of hepatic and extrahepatic tumors. January, 1996, to February, 1997 fifty patients underwent the procedure: 44 had liver cancers (42 had metastases and 2 hepatocellular carcinomas), 4 pelvic tumors (2 bladder carcinomas, one uterine cancer and one vaginal cancer), one had inoperable pancreatic tumor and one breast cancer. MATERIALS AND METHODS: The access was the left axillary artery in 45 cases, the femoral artery in 4 and both the femoral and the axillary artery in one case. The infusion catheter was placed in the hepatic artery in 44 cases, in the splenic artery in one case of pancreatic cancer, in the hypogastric artery in 4 cases and in the internal mammary artery in one case. When the catheter was positioned in the hepatic artery, embolization of the gastroduodenal or accessory hepatic arteries was performed using metallic coils; when the catheter was positioned in the hypogastric artery, the contralateral hypogastric artery and the ipsilateral gluteal branches were embolized. The catheter was then tunnelled and connected to a subcutaneous reservoir, sutured to the pectoral fascia or to the inguinal ligament. After the injection of heparinated solution, infusion chemotherapy was started the day after the procedure. RESULTS: We obtained immediate technical success in all cases. Four major complications occurred: a pseudoaneurysm of the left axillary artery (percutaneously treated by placement of a covered stent), 2 thromboses of the hepatic artery and one case of gastritis. Among minor complications, the catheter was displaced in 9 cases and 7 catheters were percutaneously replaced. Side-effects, not related to the procedure, were pain, nausea, vomiting and mucositis. During the follow-up, 7 patients died (6 for tumor progression); median catheter patency was 5.14 months. Though the aim of this work is to present the technical aspects of the procedure, we report the preliminary clinical data: radiological examinations showed partial tumor regression in 15 of 33 patients with 3-month follow-up; no change was shown in 2 patients and disease progression was found in 4; the response could not be assessed in the other cases. CONCLUSIONS: In conclusion, the percutaneous placement of intraarterial catheters for continuous regional chemotherapy is a feasible, safe and tolerable procedure and can represent an alternative to the surgical implantation of catheters in the hepatic artery for the treatment of liver metastases from colorectal cancer. The technique opens new therapeutic possibilities for the local treatment of extrahepatic tumors (such as gynecologic, vesical, pancreatic and breast cancers), even though its clinical efficacy must be assessed in selected trials.

Aged↗