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Blood Reviews Jun 2004Thromboembolic disease (TE) has been described as the new epidemic of tertiary paediatrics, and no where is this more evident than in the neonatal population. As... (Review)
Review
Thromboembolic disease (TE) has been described as the new epidemic of tertiary paediatrics, and no where is this more evident than in the neonatal population. As survival of premature and sick newborns has improved, the frequency of complications associated with intensive supportive therapy and monitoring has increased. Clinically significant thrombosis is emerging as one of the more common complications associated with improved neonatal outcome. The long-term implications of neonatal thrombosis are only just being realised. This systematic review will consider the epidemiology, diagnostic strategies, and outcome for both arterial and venous TE in neonates. The role of inherited thrombophilic abnormalities, and the evidence for anticoagulation therapy will also be considered. The lack of high level evidence in determining optimum therapy is obvious. Further research regarding diagnostic strategies, and optimal therapies is urgently needed.
Topics: Catheterization, Peripheral; Catheters, Indwelling; Child; Factor V; Female; Genetic Markers; Genetic Predisposition to Disease; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Protein C Deficiency; Protein S Deficiency; Thromboembolism; Thrombolytic Therapy; Venous Thrombosis
PubMed: 15010146
DOI: 10.1016/S0268-960X(03)00042-0 -
Thrombosis and Haemostasis Jul 2003Clinical equipoise exists regarding whether relatives of individuals with venous thromboembolism (VTE) and thrombophilia should be screened for thrombophilia. There have... (Review)
Review
Clinical equipoise exists regarding whether relatives of individuals with venous thromboembolism (VTE) and thrombophilia should be screened for thrombophilia. There have been no systematic attempts to summarize studies that have assessed the incidence of VTE in relatives. The purpose of this review was to systematically identify and review observational studies with thrombophilic relatives and to summarize their findings with respect to their risk of VTE. We conducted a systematic literature review and included nine observational studies meeting a priori inclusion criteria. Potentially eligible studies evaluated VTE incidence in relatives of index patients (probands) with symptomatic thrombophilia. In the four prospective studies, the incidence of VTE for asymptomatic family members with factor V Leiden ranged from 0.58-0.67% per year, 1.0-2.5% for protein C deficiency, 0.7-2.2% for protein S deficiency, and 4% for antithrombin deficiency. About half of all VTEs occurred during well-known risk periods but incidence rates were decreased by use of prophylaxis. No deaths from pulmonary embolism or fatal hemorrhages from anticoagulants were reported. The incidence of VTE was generally lower in the retrospective studies. The pooled relative risk from four retrospective studies for factor V Leiden carriers was 3.69 (CI 2.27, 6.00) and from two studies the pooled relative risk for deficiencies of protein C, protein S, and antithrombin was 10.58 (CI 5.38, 20.81). In conclusion, the risk of VTE events in asymptomatic relatives is low, but this may be an underestimate. Anticoagulant prophylaxis during risk periods appears to be of benefit but further research in this area is required.
Topics: Adolescent; Adult; Aged; Anticoagulants; Blood Coagulation Factors; Cohort Studies; Family Health; Female; Humans; Incidence; Male; Middle Aged; Prospective Studies; Retrospective Studies; Risk; Thromboembolism; Thrombophilia; Venous Thrombosis
PubMed: 12876621
DOI: No ID Found -
Archives of Pathology & Laboratory... Nov 2002To review the state of the art relating to protein S deficiency as a risk factor for thrombosis and to make recommendations regarding the use of protein S measurements... (Review)
Review
OBJECTIVES
To review the state of the art relating to protein S deficiency as a risk factor for thrombosis and to make recommendations regarding the use of protein S measurements in the assessment of thrombotic risk in individual patients and families.
DATA SOURCES, EXTRACTION, AND SYNTHESIS
Selection criteria were developed for the inclusion of publications from 1985 to 2001 based on the relevant literature concerned with the systematic review of diagnostic tests. Minimal selection criteria were agreed on and the articles stratified into level 1 if they met these criteria and level 2 if they did not meet these criteria. The included articles were reviewed by the authors and abstracted onto predetermined data collection forms. These forms were then scored and recommendations based on level 1 studies. As described elsewhere, results of discussions at the College of American Pathologists Conference XXXVI on Diagnostic Issues in Thrombophilia were used to revise the manuscript into its final form.
CONCLUSIONS
Consensus was reached on 16 recommendations for the use of protein S assays in the assessment of thrombotic risk in individuals and families. Two themes run through the conclusions. First, protein S assays are the most technically problematic of the assays reviewed at this conference. Second, only 2 papers evaluating the diagnostic use of protein S assays met our level 1 inclusion criteria. These 2 problems point out the need for better standardized assays and rigorous studies of the diagnostic utility of these assays.
Topics: Blood Coagulation Tests; Humans; Immunoassay; Practice Guidelines as Topic; Protein S; Protein S Deficiency; Risk Assessment; Risk Factors; Thrombophilia
PubMed: 12421142
DOI: 10.5858/2002-126-1349-AROTTD -
European Journal of Obstetrics,... Feb 2002To determine whether inherited and acquired thrombophilias are associated with adverse obstetric complications. (Review)
Review
OBJECTIVE
To determine whether inherited and acquired thrombophilias are associated with adverse obstetric complications.
STUDY DESIGN
A systematic review; studies where women with adverse obstetric complications were tested for one or more acquired and inherited thrombophilias were included.
MAIN OUTCOME MEASURES
Prevalence of thrombophilia in women with severe pre-eclampsia/eclampsia, severe placental abruption, intrauterine growth restriction or unexplained stillbirth.
RESULTS
Compared with controls, placental abruption was more often associated with homozygous and heterozygous factor V Leiden mutation, heterozygous G20210A prothrombin gene mutation, homocysteinaemia, activated protein C resistance or anticardiolipin IgG antibodies. Women with pre-eclampsia/eclampsia were more likely to have heterozygous factor V Leiden mutation, heterozygous G20210A prothrombin gene mutation, homozygous MTHFR C677T mutation, protein C deficiency, protein S deficiency or activated protein C resistance compared with controls. Unexplained stillbirth, when compared with controls, was more often associated with heterozygous factor V Leiden mutation, protein S deficiency, activated protein C resistance, anticardiolipin IgG antibodies or lupus anticoagulant. Women with intrauterine growth restriction had a higher prevalence of heterozygous G20210A prothrombin gene mutation, homozygous MTHFR C677T gene mutation, protein S deficiency or anticardiolipin IgG antibodies than controls. There was wide heterogeneity in the prevalence of thrombophilia between the studies.
CONCLUSIONS
Women with adverse pregnancy outcome are more likely to have a positive thrombophilia screen but studies published so far are too small to adequately assess the true size of this association. Screening for thrombophilia should not become standard practice until clear evidence emerges that thromboprophylaxis during pregnancy improves perinatal outcome. Further research into the link between the observed association, causality and heterogeneity is required.
Topics: Abruptio Placentae; Eclampsia; Female; Fetal Death; Fetal Growth Retardation; Humans; MEDLINE; Pre-Eclampsia; Pregnancy; Pregnancy Complications, Hematologic; Pregnancy Outcome; Thrombophilia
PubMed: 11803092
DOI: 10.1016/s0301-2115(01)00496-1 -
Stroke Dec 2000Hypercoagulable states are a recognized, albeit uncommon, etiology of ischemic stroke. It is unclear how often the results of specialized coagulation tests affect... (Review)
Review
BACKGROUND
Hypercoagulable states are a recognized, albeit uncommon, etiology of ischemic stroke. It is unclear how often the results of specialized coagulation tests affect management. Using data compiled from a systematic review of available studies, we employed quantitative methodology to assess the diagnostic yield of coagulation tests for identification of coagulopathies in ischemic stroke patients.
SUMMARY OF REVIEW
We performed a MEDLINE search to identify controlled studies published during 1966-1999 that reported the prevalence of deficiencies of protein C, protein S, antithrombin III, plasminogen, activated protein C resistance (APCR)/factor V Leiden mutation (FVL), anticardiolipin antibodies (ACL), or lupus anticoagulant (LA) in patients with ischemic stroke. The cumulative prevalence rates (pretest probabilities) and positive likelihood ratios for all studies and for those including only patients aged =50 years were used to calculate posttest probabilities for each coagulopathy, reflecting diagnostic yield. The cumulative pretest probabilities of coagulation defects in ischemic stroke patients are as follows: LA, 3% (8% for those aged =50 years); ACL, 17% (21% for those aged =50 years); APCR/FVL, 7% (11% for those aged =50 years); and prothrombin mutation, 4.5% (5.7% for those aged =50 years). The posttest probabilities of ACL, LA, and APCR increased with increasing pretest probability, the specificity of the tests, and features of the patients' history and clinical presentation.
CONCLUSIONS
The pretest probabilities of coagulation defects in ischemic stroke patients are low. The diagnostic yield of coagulation tests may be increased by using tests with the highest specificities and by targeting patients with clinical or historical features that increase pretest probability. Consideration of these data might lead to more rational ordering of tests and an associated cost savings.
Topics: Adolescent; Adult; Aged; Blood Coagulation Disorders; Blood Coagulation Tests; Case-Control Studies; Cerebral Infarction; Comorbidity; Female; Humans; Male; Middle Aged; Prevalence; Stroke
PubMed: 11108774
DOI: 10.1161/01.str.31.12.3067