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PubMed1996

Utilization of testing for activated protein C resistance in a reference laboratory.

Activated protein C (APC) resistance is a newly described thrombotic disorder accounting for the majority of patients with inherited thrombosis. The authors prospectively evaluated laboratory testing of this disorder over a 1-year period in their reference laboratory, which draws samples from a large number of community and academic hospitals throughout the United States. Testing for other inherited thrombotic disorders (antithrombin III deficiency, protein C and S deficiencies) occurred at a six-fold greater rate than that for APC resistance. Previously published studies have indicated a prevalence of up to 60% for APC resistance in populations with thrombosis; however, the prevalence rate in this study was only 12%. Of patient samples submitted for APC resistance assays, 37% were not evaluable because of concomitant anticoagulant therapy. Clinical pathologists and practitioners need to be made aware of APC resistance and of optimal sample collection to improve the efficiency of laboratory testing for thrombosis.

Adolescent↗
PubMed2003

Inherited and acquired thrombophilias and poor pregnancy outcome: should we be treating with heparin?

PURPOSE OF REVIEW: The most important acquired thrombophilia related to poor pregnancy outcome is probably antiphospholipid syndrome. Inherited thrombophilias that have been implicated in venous thromboembolism and poor pregnancy outcome and for which standard tests are generally available are antithrombin III deficiency, the factor V Leiden mutation, prothrombin G20210A mutation and the C677T polymorphism in the methylenetetrahydrofolate reductase system implicated in mild hyperhomocysteinaemia. The management of antiphospholipid syndrome with previous fetal losses is well documented and substantiated by small clinical trials. It is the purpose of this review to investigate new contributions to this field since June 2002. RECENT FINDINGS: Only one randomized trial was published during the review period, but a Cochrane review and several excellent review articles appeared detailing management. SUMMARY: There is a dire lack of randomized trials in the literature on the efficacy of heparin or other coagulation modulators on pregnancy outcome in patients with inherited thrombophilias. There is consensus on thrombo-prophylaxis for antiphospholipid syndrome. Protocols for the management of venous thromboembolism and pulmonary emboli related to pregnancy are well established.

Anticoagulants↗
PubMed1984

Computed tomography of visceral infarction due to coagulopathy.

The computed tomographic (CT) appearance of pancreatic infarction has not been described previously. We report a case in which pancreatic infarction associated with multiple visceral infarcts was detected by CT. In addition, CT revealed extensive, progressive arterial and venous thrombosis due to the patient's known deficiency of antithrombin III.

Antithrombin III Deficiency↗
PubMed2004

Extrahepatic portal vein thrombosis in children: etiology and long-term follow-up.

OBJECTIVE: Mortality of extrahepatic portal vein thrombosis depends on underlying causes other than gastrointestinal bleeding. The aim of this study was to evaluate the etiology, treatment, and prognosis of patients with extrahepatic portal vein thrombosis. METHODS: The records of 12 patients (age range: 1-9 years) diagnosed with extrahepatic portal vein thrombosis with a minimum follow-up of 2 years were analyzed retrospectively. Their diagnostic evaluations, treatment modalities, complications and long-term follow-ups were noted. RESULTS: Mean follow-up period was 7.4 +/- 3.9 years (2-14 years). Hemorrhage from esophageal varices was the prevalent symptom in 6 patients (50%). Six patients had signs of hypersplenism, 5 were found to have thrombophilia: 2 protein C, 1 protein S, 1 combined protein S, C, and antithrombin III deficiency, and 1 homozygous factor V Leiden mutation. Two patients had congenital cardiovascular abnormalities, and 1 patient developed portal thrombosis after splenectomy operation. None of the patients who started propranolol prophylaxis before first bleeding episode bled during their follow-up periods. Endoscopic sclerotherapy succeed in 66.6% variceal hemorrhages. Shunt surgery was performed in 1 patient. The patients neither faced a life-threatening variceal bleeding nor died during follow-up period. CONCLUSION: Prognosis of extrahepatic portal vein thrombosis is good in childhood. Thrombophilic states are the most frequent precipitating causes. Propranolol for prophylaxis of variceal bleeding and sclerotherapy might be the preferred modalities.

Child↗
PubMed1987

Recurrent primary mesenteric venous thrombosis.

We have reported a case of recurrent primary mesenteric venous thrombosis resulting in small bowel infarction. Resection of necrotic bowel, anastomosis, and postoperative anticoagulation remain the cornerstone of management. Delay in diagnosis and treatment contributes to the high mortality. A history of peripheral thrombosis, antithrombin III deficiency, hypovolemia, or carcinoma in susceptible patients with abdominal pain should arouse suspicion of ischemic bowel.

Humans↗
PubMed2001

Hypercoagulable states in renal transplant candidates: impact of anticoagulation upon incidence of renal allograft thrombosis.

INTRODUCTION: Although multiple studies of demographic variables have been associated with allograft thrombosis, these results are not routinely reproducible. Are ESRD patients with hypercoagulable states (HCS) (antithrombin III deficiency, protein S or C deficiency, activated protein C resistance, and anticardiolipin antibodies) at predictably greater risk for allograft thrombosis? METHODS: Between 1996 and 1999, all renal transplant candidates were screened for hypercoagulability risk factors [HRF] (multiple arteriovenous access thromboses, prior deep vein thrombosis, prior allograft thrombosis, collagen vascular disease, multiple miscarriages, diabetes, autoimmune disease, and Fabry's disease). HRF(+) candidates were then tested for HCS status. We administered preemptive posttransplant i.v. Heparin in HCS(+) patients and observed the impact of this intervention upon the incidence of allograft thrombosis. We compared demographic data and incidence of allograft thrombosis in an historic control (346 patients transplanted between June 31, 1992, and March 5, 1996) not tested for HCS and a study cohort (502 patients transplanted between March 6, 1996, and June 31, 1999) prospectively screened for HRF. HRF(+) patients who were HCS(+) in the study cohort received i.v. heparin immediately after transplant and p.o. warfarin as outpatients. RESULTS: Demographic characteristics previously implicated in allograft thrombosis were equivalently distributed in both cohorts with the exceptions that more living-donor transplants (33.1% vs. 15.3%) were performed in study cohort, CIT>24 hr occurred in more control patients (37.3% vs. 22.1%) and more study patients (16.7% vs. 0%) received tacrolimus. Hypercoagulable states were found upon reevaluating five of seven controls (71.4%), who lost prior allografts to thrombosis. Hypercoagulable states were prospectively detected in 10 study patients with hypercoagulability risk factors. Most (9 of 10) study patients receiving anticoagulation have achieved long-term allograft function. Study group allograft thrombosis incidence was reduced (1.59% vs. 4.05%). Hypercoagulable states were demonstrated in most episodes of allograft thrombosis. Control patients who lost prior allografts to thrombosis were anticoagulated after retransplantation and 100% achieved long-term allograft function. CONCLUSIONS: Long-term allograft function has been achieved in 90% of study patients when prophylactically anticoagulating study patients with hypercoagulable states. A 2.6-fold reduction in the expected incidence of allograft thrombosis was observed in anticoagulated patients with hypercoagulable states.

Adult↗
PubMed2003

Coagulopathies and arterial stroke.

Although hypercoagulable states are most often associated with venous thrombosis, arterial thromboses are reported in protein S, protein C, and antithrombin III deficiencies, factor V Leiden and prothrombin gene mutations, hyperhomocysteinemia, dysfibrinogenemia, plasminogen deficiency, sickle cell disease, and antiphospholipid antibody syndrome.

Anemia, Sickle Cell↗
PubMedDate not supplied

Antiphospholipid antibodies and coagulation regulatory protein abnormalities in children with pulmonary emboli.

PURPOSE: To evaluate the demographics, presentation, family history, and laboratory findings in children with clinically recognized pulmonary emboli. METHODS: Data were collected about children with clinically recognized pulmonary emboli from 1987 to 1994 at two pediatric hematology referral centers. RESULTS: Sixteen children, mean age 11.8 years (standard deviation 4.69 years) including 11 boys were affected. Lower extremity thromboses were present in 7/14 children evaluated. Eight of the 16 children were apparently well before development of pulmonary emboli; seven were found to have antiphospholipid antibodies. None of the 15 children tested were antithrombin III deficient. One of 14 children tested was protein C deficient. Three of 13 children tested were protein S deficient or had a free protein S antigen at the fifth percentile. One of 10 children tested had an acquired dysfibrinogenemia. Two of nine children tested had the Factor V Leiden mutation. CONCLUSIONS: Our limited data suggest at least 70% of children with pulmonary emboli referred for hematology evaluation have antiphospholipid antibodies and coagulation regulatory protein abnormalities.

Adolescent↗
PubMedDate not supplied

Outcome of noncatheter-related thrombosis in children: influence of underlying or coexisting factors.

PURPOSE: To determine the hereditary and nonhereditary risk factors contributing to the development of noncatheter-related thrombosis in children. PATIENTS AND METHODS: Ninety-five children with noncatheter-related thrombosis from a single center were analyzed for clinical manifestations and outcome during a period of 3.5 years. Patients with thrombosis were evaluated for congenital and acquired risk factors, and patients were screened for factor V Leiden, prothrombin 20210A mutations, protein C, protein S, antithrombin III deficiencies, and antiphospholipid antibodies. RESULTS: Twelve patients (12.5%) died of thrombosis that was associated with a nonmalignant underlying disorder, and 19 patients (20%) had significant complications, including amputation of extremities, epilepsy, hemiplegia, portal hypertension, recurrence of deep vein thrombosis, and postphlebitic syndrome. Two common mutations (factor V Leiden and prothrombin 20210A) were found in 32.5% of the patients with thrombosis. The most frequent underlying disorder was infection (68%). Ninety-three patients (98%) had one or more risk factors. CONCLUSIONS: The coexistence of an underlying disorder and the presence of predisposing factors such as infection and the factor V Leiden mutation may cause high rates of death and complications in children with noncatheter-related thrombosis. The results of this study indicate that the underlying disorder and the site of thrombosis determine the rates of death and complications in children with thrombosis.

Adolescent↗
PubMed2000

Inherited thrombophilia and fetal loss.

Acquired thrombophilia is a well-established cause of pregnancy loss. Increasing numbers of recent observations suggest that inherited thrombophilia is not only associated with gestational thromboembolism but is also a major cause of fetal loss. This review focuses on association of fetal loss with inherited thrombophilias, including dysfibrinogenemia and protein C, protein S, and antithrombin III deficiencies. Activated protein C resistance and factor V Leiden mutation are frequent causes of pregnancy loss. Thrombophilic states such as factor V Leiden and hyperhomocysteinemia may also play a role in other gestational vascular complications, including intrauterine growth restriction, preeclampsia, and placental abruption. Preliminary reports suggest that antithrombotic therapy may be of value in this setting. The potential application of antithrombotic modalities to prevent fetal loss in women with thrombophilia is discussed.

Abortion, Habitual↗
PubMed1994

The family history and inherited thrombophilia.

The role of the family history as a tool for the diagnosis of inherited thrombophilia has not been established. Several authors have indicated that a positive family history is not a good predictor of inherited abnormalities such as antithrombin III deficiency, or deficiencies of protein C or protein S. We have tried to approach the family history in a quantitative way. To this end we used the cumulative incidence data of thrombosis in the general population and also in a population of protein C deficient families to estimate the expected number of symptomatic subjects in a family under both the hypothesis of inherited thrombophilia and the null-hypothesis. Although a number of assumptions underlying our calculations need to be verified and probably adjusted before any truly quantitative meaning can be assigned to this approach, we feel that the family history is a useful diagnostic test for inherited thrombophilia if it is used in a critical way.

Adolescent↗
PubMed2006

Outcome after kidney transplantation in children with thrombotic risk factors.

BACKGROUND: According to the data from the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS), vascular thrombosis accounts for 11.6% of graft losses in pediatric renal transplantation. In adults, inherited and acquired thrombophilic risk factors, e.g. factor V Leiden mutation, have been associated with early graft loss and increased rejection episodes. Data on the impact of these factors on the outcome of children after renal transplantation are rare. METHODS/PATIENTS: Sixty-six pediatric patients awaiting renal transplantation (mean age 10.1 yr) were screened for inherited and acquired risk factors for hypercoagulable disorders (protein C, S, and antithrombin III deficiency, antiphospholipid antibodies, factor V Leiden, prothrombin, and MTHFR mutation) in order to intensify anticoagulation in those with an increased risk for thrombophilia: intravenous heparin was administered with a partial prothrombin time (PTT) prolongation of 50 s for 14 days and switched to low-molecular-weight heparin for another 8 wk before aspirin was introduced for the first year. Patients without hypercoagulable risk factors were treated with heparin without PTT prolongation for 14 days and switched to aspirin immediately afterwards. The results on graft survival, incidence of acute rejection episodes, and long-term renal graft function were analyzed between recipients with and without hypercoagulable risk factors. RESULTS: Thrombophilic risk factors were identified in 27.3% of our patients. No thrombosis occurred. One serious bleeding complication led to a second surgical intervention. The rate of acute rejection episodes was not increased in patients with and without thrombotic risk factors after 90 days (16.7 vs. 25%), 1 yr (22.2 vs. 33.3%), and 3 yr (38.9 vs. 41.7%) of follow-up, respectively (p = n.s.). After a mean follow-up of 3 yr the kidney function was comparable in both groups, with 63.1 in recipients with and 69.8 mL/min/1.73 m(2) in recipients without hypercoagulable risk (p = n.s.). At latest follow-up, three graft losses were found not to be attributed to thrombotic risk factors. INTERPRETATION: Children with thrombophilic risk factors were identified and treated with an intensified anticoagulation regimen after renal transplantation. An increased risk for graft failure, acute rejection episodes, or impaired renal function for pediatric renal transplant recipients with hypercoagulable status was not found.

Anticoagulants↗
PubMed1982

Oral contraceptives and venous thromboembolism.

In 58 patients with phlebographically diagnosed deep vein thrombosis during oral contraception the extension and localization of the thrombotic process was analysed in relation to the coagulation and fibrinolytic system 6 months after thrombosis. Left-sided thrombi dominated and the thrombi were more extensive and proximal on the left side. This left-sided dominance was more apparent with higher oestrogen content in the pills. The right-sided thrombi more often were the source of pulmonary embolism. In 31% of the patients a defective fibrinolytic system was found; this defect was seen more often in patients with right-sided thrombi. Only very few defects were found in the coagulation system. No patients had an antithrombin III deficiency.

Adolescent↗
PubMed2003

Chorioamnionitis caused by Serratia marcescens in a non-immunocompromised host.

A 26 year old pregnant woman with antithrombin III deficiency developed recurrent septicaemia with Serratia marcescens. In spite of the administration of antibiotics, high grade fever persisted. She subsequently manifested lower abdominal pain, and spontaneous abortion occurred. After the abortion, she became completely afebrile. The amnion was turbid, and microscopic examination of the placenta showed haemorrhage and massive infiltration of neutrophils, suggestive of infectious chorioamnionitis. Pulsed field gel electrophoresis showed that isolates from the blood, urine, and vaginal discharge were genetically identical. Intravenous pyelography revealed that she had a bilateral completed double ureter. It was thought that a urinary tract anomaly caused infection with S marcescens, and the pathogen spread to the chorioamnion via the bloodstream. This is the first report of chorioamnionitis caused by S marcescens in a non-immunocompromised host. In addition, these findings indicate that the chorioamnion can serve as a site for persistent infection in normal pregnancies.

Abortion, Spontaneous↗
PubMed1985

Regulation of blood coagulation and thrombosis.

The syndrome of the hereditary tendency to venous thrombosis or thrombophilia has been recognized only after the discovery of regulatory mechanisms of the haemostatic system. At present, several distinct defects are known as causes of this syndrome: antithrombin III deficiency, protein C deficiency, protein S deficiency, dysfibrinogenaemia and dysplasminogenaemia. It is likely that several additional defects will be found in the near future. Consequently, it is important that a family history be taken in all cases of 'spontaneous' venous thrombosis and that laboratory studies be done to identify any underlying defect in the regulation of the blood coagulation system. We present preliminary data, which suggest that the incidence of the thrombophilia syndrome is higher than that of the haemophilias.

Blood Coagulation↗
PubMed1994

Cerebellar venous infarction in chronic suppurative otitis media. A case report with review of four other cases.

BACKGROUND: Cerebellar venous infarction is a rare condition. Thus far only four cases have been reported in the literature. We recently encountered a patient with chronic suppurative otitis media complicated by cerebellar venous infarction. The features of cerebellar venous infarction in the other four cases are also reviewed. CASE DESCRIPTION: A 20-year-old man presented with clinical features suggestive of chronic suppurative otitis media. Computed tomographic scan of the brain revealed left mastoiditis with cholesteatoma and moderate communicating hydrocephalus. The patient was subjected to left radical mastoidectomy, and an attico-antral cholesteatoma was removed. Subsequently the patient developed clinical features suggestive of cerebellar abscess. A repeat computed tomographic scan revealed normal posterior fossa. Four-vessel angiography revealed left sigmoid and lateral sinus thrombosis and nonopacification of the left-sided cerebellar veins. Magnetic resonance imaging showed a venous infarct in the left cerebellar hemisphere. The patient was treated with cerebral dehydration measures. The patient subsequently improved and had no neurological deficit 3 months after surgery. CONCLUSIONS: Although cerebellar venous infarction is rare, it can occur in chronic suppurative otitis media, pregnancy, antithrombin III deficiency, and diabetic osmolar coma. Sometimes no cause is found. Treatment includes correction of the underlying cause. The presence of a hemorrhagic lesion on computed tomographic scan and deep coma at presentation indicate poor prognosis.

Adult↗
PubMed2005

Coagulation management of a patient with factor V Leiden mutation, lupus anticoagulant, and activated protein C resistance: a case report.

Although patients undergoing cardiac surgery often present with diverse comorbidities, those with coagulation derangements are especially challenging. The present report describes the management of a patient who presented with a Factor V Leiden mutation, lupus anticoagulant, and acquired activated protein C resistance. A 42-year-old female presented with acute shortness of breath and chest pain. She was otherwise healthy 1 month prior to admission when she presented with dysfunctional uterine bleeding, resulting in the transfusion of three units of packed red blood cells. Coagulation evaluation revealed that the patient had lupus anticoagulant, factor V Leiden mutation and an activated protein C resistance. The patient presented with an acute myocardial infarction and was found to have 90% stenosis of her left main coronary artery, moderate mitral and tricuspid regurgitation, and a left ventricular ejection fraction of 25%. An emergent off-pump coronary artery bypass procedure with placement of a vein graft to the left anterior descending artery was completed. Intraoperative thrombophilia was encountered as evidenced by both an elevated thromboelastograph coagulation index (+3.6) and an acquired antithrombin-III deficiency. Postoperatively, the patient was placed on low molecular weight heparin, but developed heparin-induced thrombocytopenia and was switched to a direct thrombin inhibitor, argatroban. The following case report describes the coagulation management of this patient from the time of admission to discharge 43 days later, and the unique challenges this combination of hemostatic defects present to the clinicians.

Activated Protein C Resistance↗
PubMed1998

Inherited Thrombophilia due to Factor V Leiden Mutation.

Inherited thrombophilia due to activated protein C resistance is now recognized as one of the major genetic risk factors in the development of venous thromboembolic disease. Activated protein C resistance is secondary to a point mutation in the factor V gene, factor V Leiden. The high prevalence of this mutation in the general population, mainly in Caucasians of European descent, is a major contributing factor to the high incidence of venous thromboembolic disease in the United States, affecting one in 1000 individuals annually. Heterozygosity and homozygosity for factor V Leiden increase the risk for thrombosis 5- to 10-fold and 50- to 100-fold, respectively, compared with genotypically normal individuals. Factor V Leiden is more common than all other known genetic risk factors for thrombosis, and its presence results in a compounded risk in patients with simultaneous inherited abnormalities such as protein C, protein S, antithrombin III deficiencies, hyperhomocysteinemia, and/or acquired risk factors. Therefore, detection of activated protein C resistance and genotyping for factor V Leiden are important for establishing risk for thrombosis and ultimately for patient management.

Journal Article↗