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PubMed2000

Etiology based prevalence of Budd-Chiari syndrome in eastern India.

BACKGROUND: Diagnosis of Budd-Chiari syndrome (BCS) is often missed unless its possibility has been kept in mind. Obstruction of inferior vena cava (IVC) is reportedly the most frequent cause of BCS in Afro-Asian variety. AIM: An attempt was made to classify BCS (in an eastern Indian population) etiopathologically. PATIENTS AND METHODS: Thirty consecutive cases of BCS presenting over a period of five years were included. Following a thorough physical examination, necessary investigations (including coagulation profile, ultrasonography (with Doppler study) of hepatobiliary tract, hepatic vein and IVC angiography (n = 22) and liver biopsy (n = 26, including autopsy in two cases) were performed. RESULTS: Mean age at presentation was 32.7 +/- 10.36 years (range 12-60 years) with M:F = 21:9. Clinical presentations included, hepatomegaly in 28 (93.3%), ascites in 27 (90%), splenomegaly in 15 (50%), pain in abdomen in 26 (86.6%), jaundice in 10 (33.3%), back veins in 20 (66.6%) and gastrointestinal bleeding in three (10%) cases. Amongst the total of 30 patients, four, six and 20 cases presented as fulminant, acute and chronic BCS respectively. Twenty four cases of BCS could be diagnosed by ultrasonography alone, while the remainder required angiography for diagnosis. IVC and hepatic vein angiography revealed membranous obstruction in nine, partial stricture of IVC in six, and IVC and/or hepatic vein block in others. The etiopathological nature in 30 cases were as follows: idiopathic membranous obstruction in nine (30%), hepatocellular carcinoma in six (20%), idiopathic stricture in six (20%) cases and one case (3.3%) each of the following: cholangiocarcinoma, renal cell carcinoma, chronic pancreatitis, hydatid cyst in liver, protein S deficiency, oral contraceptive use, nephrotic syndrome (with antithrombin III deficiency), polycythemia rubra vera and chronic lymphatic leukemia. CONCLUSION: Idiopathic membranous obstruction and stricture of IVC are the commonest cause of BCS in the eastern part of India. Hepatocellular carcinoma is also a common cause, presenting in the fulminant form. Ultrasonography may be a helpful screening test for BCS, but IVC and hepatic vein catheterisation is essential for a complete work up of these patients.

Adolescent↗
PubMed1980

[Problems in intensive care posed by imbalance in the protease--protease inhibitor system].

Proteases have a wide range of functions: digestion (pancreatic proteases), protein catabolism (lysosomal proteases), blood coagulation, immune defences (complement), cellular division and proliferation, generation of biologically active oligopeptides (kinins, hormones) from inactive polypeptide precursors, inactivation of these oligopeptides, etc. The body protects itself against its own proteases, either by confining them to a given compartment (lysosome), by synthesising them in the form of inactive precursors (trypsinogen, prothrombin, etc.), or by synthesising proteins with an antiprotease activity. Any disturbance in one of the elements of this protective system may lead to severe pathological consequences: acute hemorrhagic pancreatitis with shock, coagulation disturbances (deficient hepatic synthesis of coagulation factors, congenital antithrombin III deficiency), angioneurotic oedema (congenital deficiency of C'l esterase inhibitor) pulmonary emphysema (local secretion of leukocyte elastase, congenital deficiency of alpha a-antitrypsin).

Angioedema↗
PubMed1995

Underlying disorders associated with severe early-onset preeclampsia.

OBJECTIVE: Our purpose was to determine whether patients with severe early-onset preeclampsia have hemostatic or metabolic abnormalities that are associated with a tendency to vascular thrombosis. STUDY DESIGN: A total of 101 patients with a history of severe early-onset preeclampsia were tested at least 10 weeks post partum for the presence of hyperhomocysteinemia (methionine loading test), protein C, protein S, and antithrombin III deficiency, activated protein C resistance, lupus anticoagulant, and immunoglobulin G and/or M anticardiolipin antibodies. RESULTS: Of the 101 patients, 39 (38.6%) had chronic hypertension. Of the 85 patients tested for coagulation disturbances, 21 (24.7%) had protein S deficiency. Of the 50 patients tested for activated protein C resistance, 8 (16.0%) were positive. Of the 79 patients tested for hyperhomocysteinemia, 14 (17.7%) had a positive methionine loading test. Finally, 95 patients were tested for anticardiolipin antibodies; 27 (29.4%) had detectable immunoglobulin G and/or M anticardiolipin antibodies. CONCLUSION: Patients with a history of severe early-onset preeclampsia should be screened for protein S deficiency, activated protein C resistance, hyperhomocysteinemia, and anticardiolipin antibodies, since these results may have an impact on counseling for and pharmacologic management in future pregnancies.

Antibodies, Anticardiolipin↗
PubMed1985

The hypercoagulable states.

Patients are considered to have hypercoagulable states if they have laboratory abnormalities or clinical conditions that are associated with an increased risk of thrombosis (prethrombotic states) or if they have recurrent thrombosis without recognizable predisposing factors (thrombosis-prone). The number of specific primary hypercoagulable states that are recognized is growing. These disorders are generally inherited abnormalities of coagulation in which a physiologic anticoagulant mechanism is defective: for example, antithrombin III deficiency, protein C and protein S deficiency, abnormalities of the fibrinolytic system, and dysfibrinogenemias. Secondary hypercoagulable states are generally acquired disorders in patients with underlying systemic diseases or clinical conditions known to be associated with an increased risk of thrombosis: for example, malignancy, pregnancy, use of oral contraceptives, myeloproliferative disorders, hyperlipidemia, diabetes mellitus, and abnormalities of blood vessels and rheology. The complex pathophysiologic features of these secondary hypercoagulable states are discussed, and a framework is provided for the laboratory investigation and systematic clinical approach to the patient.

Antithrombin III Deficiency↗
PubMed2005

Mesenteric and portal vein thrombosis: treated with early initiation of anticoagulation.

OBJECTIVE: Superior mesenteric vein thrombosis (SMVT) is generally difficult to diagnose and can be fatal. Mesenteric and portal vein thrombosis is rare and can be presented as more serious conditions than that of SMVT. We report patients with combined SMVT and portal vein thrombosis (PVT) who were treated successfully with early initiation of anticoagulation. METHODS: The medical records of six patients (five male, one female) who presented with combined SMVT and PVT in our institute between January 1994 and September 2003 were reviewed retrospectively. All of the patients were treated with early initiation of anticoagulation using unfractionated heparin or low molecular weight heparin. RESULTS: The mean hospital stay was 31 days and the mean follow-up period was 32 months. Three patients had an antithrombin III deficiency. The most common symptom was diffuse abdominal pain and signs included abdominal distension and tenderness. During the follow-up period, there were two patients who developed stricture of the small bowel necessitating resection and anastomosis of the small bowel. There was no case of peritonitis due to bowel necrosis or mortality. CONCLUSION: The early initiation of anticoagulation in patients of SMVT combined with PVT could minimise the serious complication such as peritonitis due to bowel necrosis required immediate exploratory laparotomy.

Adult↗
PubMed2004

Increased rates of thrombophilia in women with repeated IVF failures.

BACKGROUND: We investigated whether hereditary thrombophilia is more prevalent in women with recurrent IVF-embryo transfer failures. METHODS: This case-control study was conducted in an academic tertiary care hospital and compared 45 women with a history of four or more failed IVF cycles (group A) with 44 apparently healthy women matched for age and ethnic origin (group B). All participants were tested for inherited thrombophilias: mutations of prothrombin, factor V Leiden and methylene tetrahydrofolate reductase (MTHFR), and protein C, protein S and antithrombin III deficiencies. RESULTS: Excluding homozygotic MTHFR, the incidence of thrombophilia in group A, was 26.7% compared with 9.1% in group B (P = 0.003; odds ratio 2.9; 95% confidence interval 1.02-8.4). The incidence of thrombophilia in women with unexplained infertility in group A was 42.9% (9/21), compared with 18.2% in group B (P < 0.002). CONCLUSIONS: These data suggest that inherited thrombophilia may play a role in the aetiology of repeated IVF failures, particularly in the subgroup with unexplained fertility.

Antithrombin III Deficiency↗
PubMed2001

Cerebral venous thrombosis in children.

Cerebral venous thrombosis is an important cause of stroke in children. Understanding the natural history of the disease is essential for rational application of new interventions. We retrospectively identified 31 children with cerebral venous thrombosis confirmed by head computed tomography (4 patients) or by magnetic resonance imaging (27 patients). Risk factors, clinical and radiographic features, and neurologic outcomes were analyzed. There were 21 males and 10 females aged 1 day to 13 years (median 14 days). Nineteen (61%) were neonates. The most common risk factors included mastoiditis, persistent pulmonary hypertension, cardiac malformation, and dehydration. The chief clinical features were seizures, fever, respiratory distress, and lethargy. Fifteen patients had infarctions (8 hemorrhagic, 7 ischemic). Protein C and antithrombin III deficiency were the most common coagulopathies among 14 tested patients. On discharge, 11 patients were normal, 17 had residual deficits, and 2 patients died. Twenty-seven patients were followed from 1 month to 12 years (mean 22 months). At follow-up, 11 patients were normal, and 13 patients had development delay. One had residual hemiparesis and cortical visual impairment. Two had other deficits. Neonatal cerebral venous thrombosis is probably more common than previously thought, and outcomes are worse in this group. All children with cerebral venous thrombosis should be tested for coagulation disorders.

Adolescent↗
PubMed1993

Fixed mini-dose warfarin for prophylaxis of thromboembolic disease in pregnancy: a safe alternative for the fetus?

BACKGROUND: Fixed mini-dose warfarin has been used for thromboprophylaxis in high-risk nonpregnant patients with encouraging results. The usefulness of this strategy in pregnant women requires documentation of fetal safety. CASE: A woman with antithrombin III deficiency suffered a venous thrombosis during early pregnancy and could not be successfully managed long term with heparin. One milligram of warfarin daily was used for prophylaxis, and serial fetal blood samples were used to monitor the fetal coagulation status. No apparent coagulation abnormalities were demonstrated in the fetus at 33, 36, and 38 weeks' gestation. The woman suffered no further thromboses throughout the remainder of her pregnancy and puerperium. CONCLUSIONS: The efficacy of fixed mini-dose warfarin for prophylaxis in pregnancies at risk for thromboembolic disease will require further investigation. This fetus did not appear vulnerable to coagulation abnormalities as a consequence of the warfarin regimen.

Adult↗
PubMed1989

The relative frequency of hereditary thrombotic disorders among 107 patients with thrombophilia in Israel.

Since most patients with thrombophilia in Israel are referred for diagnosis to our center, it was possible to estimate the relative frequency of the hereditary disorders leading to thrombophilia. 107 unrelated patients were evaluated over 4 years. Diagnoses were established in 23 patients (21.5%) while in 84 (78.5%) no abnormality was detected. Antithrombin III deficiency was found in 8 patients (7.5%), dominant protein C deficiency in 6 (5.6%), recessive homozygous protein C deficiency in 1, protein S deficiency in 3 (2.8%) and dysfibrinogenemia in 1. Four additional patients (3.7%) had a lupus anticoagulant. The frequency of deep vein thrombosis and pulmonary embolism was similar in patients with and without a definite diagnosis. Thrombosis of visceral or cerebral vessels and a positive family history were more frequent among patients in whom a definite diagnosis was made. In both groups there was a substantial lag between the time of presentation of the first thrombotic episode and the time of evaluation. Since the number of referred patients with thrombophilia has gradually increased over the period of the study, it is at present impossible to establish the prevalence of the various hereditary disorders leading to thrombophilia in the population.

Adolescent↗
PubMed1996

Guidelines for the management of thrombophilia. Department of Haematology, The Royal London Hospital, Whitechapel, London, UK.

Although there are numerous risk factors for venous thromboembolic disease, the term 'thrombophilia' refers only to those familial or acquired disorders of the haemostatic system that result in an increased risk of thrombosis. The inherited thrombophilias include antithrombin III deficiency, resistance to activated protein C (factor V Leiden), protein C and protein S deficiencies as well as some rare forms of dysfibrinogenaemia. It is possible that other inherited conditions might also predispose to thrombosis. In contrast, when using the above definition, the antiphospholipid syndrome is the only genuine acquired thrombophilic state. Patients who have thromboembolic disease at a young age with no provoking event or who have a positive family history or whose thrombosis involves an unusual site should be investigated for thrombophilia. The management of a patient identified as having a laboratory abnormality associated with thrombophilia will depend on a variety of factors such as the patient's individual and family thrombotic history, the site of the thrombosis and the presence of other prothrombotic risk factors. The use of prophylactic anticoagulation during pregnancy and the puerperium requires particularly careful consideration in thrombophilic women. As more becomes known about the thrombophilias it will become possible to formulate more exact guidelines as to the management of these conditions.

Adult↗
PubMed1986

Purified radiolabeled antithrombin III metabolism in three families with hereditary AT III deficiency: application of a three-compartment model.

Purified human radioiodinated antithrombin III (125I-AT III) was used to study its metabolism in six members from three different families with a known hereditary AT III deficiency. Six healthy volunteers served as a control group. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and crossed immunoelectrophoresis (CIE) showed the purified AT III to be homogeneous. Amino acid analysis of the protein revealed a composition identical to a highly purified internal standard. The specific activity was 5.6 U/mg. Analysis of plasma radioactivity data was performed, using a three-compartment model. Neither plasma disappearance half-times nor fractional catabolic rate constants differed significantly between patients and control subjects. The mean absolute catabolic rate in the patient group was significantly lower than that of the control group at 2.57 +/- 0.44 and 4.46 +/- 0.80 mg/kg/day, respectively. In addition, the mean patient alpha 1-phase, flux ratio (k1,2 and k2,1) of the second compartment alpha 2-phase and influx (k3,1) of the third compartment were significantly reduced as compared with control values. It has been tentatively concluded that the observed reduction in the second compartment may be caused by a decrease in endothelial cell surface binding.

Adult↗
PubMedDate not supplied

[Thrombosis of the cerebral venous sinuses: a description of its clinical features, risk factors and treatment in a hospital of Colombia].

INTRODUCTION: Thrombosis of the cerebral venous sinuses (TCVS) is an infrequent entity that still represents a challenge in health care. PATIENTS AND METHODS: A descriptive study involving the evaluation of the medical records of patients that met the following criteria: over 18 years of age with a diagnosis of TCVS confirmed by CAT scan or cranial MRI, the absence of a history of intracranial surgery in the previous six months and absence of a history of intracranial infection. RESULTS: A total of 15 patients (14 females and one male) were found with an average age of 28.6 years. The time required for the development of the most frequent symptoms was 1 10 days (60%). The usual presentation was a syndrome of intracranial hypertension with focalisation and encephalopathy. Risk factors were identified in 13 patients (87%). TCVS was diagnosed by cranial MRI in the case of 14 patients (93%), by CAT scanning in one (7%) and this was also used to orient diagnosis in 12 cases (80%). The most frequently affected sinuses were the superior longitudinal and transverse, in 10 cases each (66%), and venous infarctions were also detected in 10 patients (66%). In the patients in whom we were able to evaluate clotting disorders, it was found that the only individual who displayed activated protein C resistance was positive, three out of four patients had a protein C deficiency and four out of six had an antithrombin III deficiency. CONCLUSION: TCVS presents as an intracranial hypertension syndrome and it is possible to find risk factors in as many as 85% of the cases.

Adult↗
PubMed2006

Acute cytomegalovirus infection complicated by venous thrombosis in a renal transplant recipient.

We report a case of simultaneous acute cytomegalovirus infection and venous thrombosis in a renal transplant recipient. On posttransplant month 3, the patient started complaining of left leg pain and swelling. Tibiopopliteal and femoral deep venous thrombosis were confirmed by Doppler ultrasonography. A serological test for CMV ELISA was strongly positive for IgM antibodies. Acute CMV infection was diagnosed by serum quantitative DNA polymerase chain reaction. Genetic predisposing risk factors for thrombosis (eg, protein C and S deficiency, factor V Leiden and prothrombin G20210A mutations, and antithrombin III deficiency) were not present. Results of tests for anticardiolipin antibodies, lupus anticoagulant, and antinuclear antibodies were also negative. No other clinical or biologic risk factors for thrombosis were detected in the patient. The patient responded well to intravenous gancyclovir and low-molecular weight heparin therapy. He was discharged in good condition. Our observation suggests that acute CMV infection may be the cause of a thrombotic event in renal transplant recipients.

Acute Disease↗
PubMed1992

Structure and mechanism of action of serpins.

Serpins have a central role in regulating proteolysis in blood coagulation, fibrinolysis, and inflammation. Congenital serpin deficiencies cause specific clinical syndromes such as thrombosis with antithrombin III deficiency or emphysema with alpha 1-antitrypsin deficiency. Acquired serpin abnormalities have also been reported, for example, increased PAI-1, which is believed to represent a major risk factor for the development of thromboembolic complications. This article has reviewed the structure/function relationships of serpins and have presented ideas concerning their mechanism of action. The implications of this information for designing drugs that could interfere with serpin function has also been described, and mention has been made of the therapeutic potential of such compounds.

Humans↗
PubMed1999

Screening for activated protein C resistance before oral contraceptive treatment: a pilot study.

The feasibility and cost-effectiveness of screening women for congenital thrombophilic alterations before oral contraceptive (OC) treatment was investigated. A total of 525 women (mean age 21.9 years, 73% aged < 25 years) were examined before their first OC course. At first screening, completely normal results were recorded in 485 (92.4%) women, the remaining showing single (n = 34) or multiple (n = 6) alterations. At second examination (possible in 37 of 40), activated protein C resistance (APCR) was confirmed in 21 cases (4.0%, 18 with factor V Leiden), protein C, or protein S reduction in 8 (1.5%) and 2 (0.4%) cases, respectively. No cases with antithrombin III deficiency were detected. The global estimated cost ($US) to detect one altered case was: $7795 for protein S, $2696 for antithrombin III (no case found), $1374 for protein C and $433 for APCR. The present study confirms that extensive thrombophilic screening before OC treatment is not currently advisable. APCR assessment, however, seems to have a favorable cost-effectiveness ratio: the alteration is frequent and has a synergistic effect with OC; sensibility and specificity of some methods are good; family history is unreliable to single out possible carriers; finally, carriers can be fully informed of their increased thrombotic risk if treated with OC and can receive thromboprophylaxis during life situations associated with high thrombotic risk (e.g., pregnancy and puerperium).

Activated Protein C Resistance↗