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PubMed1988

[How reliable is microvascular surgery?].

Although in experimental microsurgery a patency rate of almost one hundred percent can be obtained, in clinical experience we now and then encounter a failure where circulation cannot be restored for reasons not known. Cold agglutinins, deficiency of Antithrombin III, and irreversible spasm of the vessel wall may be some of the factors which prevent the restoration of circulation in cases of replantation and free tissue transplantation. It is suggested that we collect such cases in a combined effort to find out more about possible causes.

Adult↗
PubMedDate not supplied

[Pathophysiology and clinical aspects of pre-eclampsia].

From the pre-natal follow-up it was remarkable that cases have been admitted relatively late. Hints to a possible development of preeclampsia could be seen from patients history or the routine check up, for example the registration of edema, fetal growth retardation and oligohydramnios. For early diagnosis of preeclampsia we recommend: Calculation of mean arterial blood pressure or its non-invasive measurement; determination of hematocrit, uric acid and total plasma protein (in particular hemorheologic measurements). Hypomagnesemia in preeclampsia, as described by some authors, was also seen in our cases. The complex symptomatology of preeclampsia could be attributed to a generalised disturbance of microcirculation, which leads to definite reactions of the organs concerned. The microcirculatory failure is caused by vasoconstriction, hemoconcentration, hyperviscosity and hypercoagulation (up to DIC and consumption coagulopathy). The resulting symptoms and syndromes can be: EPH, HELLP, hemolytic-uremic Syndrome, hepato-renal Syndrome, thrombocyte and antithrombin III deficiency etc. The drug of choice for treatment of preeclampsia is magnesium sulfate. Its application is based on long-term clinical experience and new aspects on the physiologic and pharmacologic role of magnesium. The recommendations of the German High Blood Pressure League to use calcium antagonists as a basis in the treatment of high blood pressure can be fulfilled particularly in pregnancy by the physiologic calcium antagonist Mg++. Magnesium sulfate should be given in a dosage of 24-72 g daily. The dose should also be made dependent from urinary output. Further treatment patterns of preeclampsia should be adjusted according to each case. The present results also support our hypothesis that magnesium deficiency (besides predisposing factors) could be responsible for the development of preeclampsia (present model shown in detail). Consequently, the early and long-term substitution of magnesium in pregnancy could help reduce preeclampsia.

Adult↗
PubMed1984

[Inferior caval syndromes. Apropos of 45 cases].

The authors report their experience with 45 cases of inferior vena cava thrombosis. Diagnosis was delayed for an average of 55 days. One-third of cases were revealed by an embolic complication. Inflammatory diseases were the most common causes (Behcet disease: seven cases, systemic lupus erythematosus: 5 cases). Malignancies accounted for 20% of cases. Abnormalities of coagulation were uncommon: antithrombin III deficiency in one patient and protein C deficiency in another. Estrogen-progestogen combinations could be incriminated in 4 cases. Outcome was fatal in 20% of cases, usually as a result of the underlying disease. Functional status was good in two-thirds of patients without malignancy followed up for an average of 27 months. In 14 patients a clip was inserted to ensure total (3 cases) or partial (11 cases) interruption of vena cava blood flow because of a free thrombus and/or recurrent pulmonary embolism. Three patients had thrombectomy. After clip insertion two embolisms were recorded, one of which occurred in the immediate post-operative period.

Adolescent↗
PubMed1994

[Cerebral infarction in patients aged 16 to 35 years. Prospective study of 52 cases].

OBJECTIVES: Aetiologies of cerebral ischemic events in young adults are various and often contraversial: atherosclerosis plays a role after age 35 years, cardioembolism is often recognized as the major cause, and numerous new predisposing conditions have been described. To evaluate very premature cerebral infarction, we studied prospectively 52 consecutive patients (34 women and 18 men) aged 16-35 years (mean 28.6 +/- 5.6 years) admitted over a 6-year period for an arterial cerebral infarct identified on CT, on MRI or both. METHODS: All patients were investigated using a standard protocol including cerebral angiography, transthoracic echocardiography, 24-hour Holter ECG monitoring, coagulation inhibitors levels, lupus anticoagulant testing, HIV and syphilitic serologies. Additional investigations were performed in a majority of patients: transesophageal echocardiography in 33, contrast echocardiography in 50, and anticardiolipin antibodies levels in 38. RESULTS: The arterial distribution of infarction did not differ from that of overall stroke patients. Sixteen cases were classified as certain aetiologies, including cardiac diseases with high embolic risk (n = 9), spontaneous arterial dissection (n = 4), moya-moya disease (n = 1), atherosclerosis (n = 1), and inherited antithrombine III deficiency (n = 1). Eighteen cases were classified as possible aetiologies including cardiac diseases with low embolic risk (n = 11) such as mitral valve prolapse or atrial septal aneurysm, miscellaneaous arteriopathies (n = 3) such as arterial dysplasia, antiphospholipid antibodies syndrome (n = 1) and migrainous stroke (n = 3). The 18 last cases were classified as unknown aetiologies, despite all patients but one had at least one vascular risk factor: this group significantly differed from the formers in that the patients were more frequently women using oral contraceptive associated with tobacco consumption, hypercholesterolemia, or both. Two patients died acutely and the survivors were followed 31 +/- 18 months. Three patients experienced recurrent stroke. Functional outcome was assessed using the Rankin scale on admission and 6 to 12 months after the onset of stroke: patients evaluated < or = 2 (no to mild handicap) were 31% on admission and 82% after recovery. Twenty six p. cent remained unable to resume a work. CONCLUSION: Despite extensive evaluation, identification and classification of aetiologies of cerebral infarction in young adults should be cautious because the risk as well as the pathogenesis of many potential causes still need to be defined.

Adolescent↗
PubMed1993

[The fibrinolytic system and total parenteral nutrition].

A study is presented which was carried out in twenty-five patients operated on in a surgical service, and who received total parenteral nutrition. Samples were taken to check modifications to the fibrinolytic system occurring after administration of hyperosmotic solutions, principally lipidic emulsions, from a contemporary standpoint. We performed three extractions: on day one, day five and following the withdrawal of the fat emulsion, with study of modifications to antithrombin III, alpha 2 antiplasmin, plasminogen, C protein, plasminogen tissular activator (t-PA), plasminogen tissular activator inhibitor (PAI 1), fibrinogen, and fibrinogen/fibrin degradation products. The laboratory techniques used are shown, along with the results obtained: increased for alpha 2 antiplasmin and antigenic t-PA with significant values (p < 0.05) on the day of withdrawal in comparison with pre-PN values, plus increased values for plasminogen, fibrinogen/fibrin degradation products and t-PA activity. In conclusion, it is suggested that antithrombin III deficiency, along with reduction in C protein levels and increased fibrinogen and PAI 1 concentration predispose patients with t-PA release defects to thrombo-embolic complication.

Aged↗
PubMed1994

Hypercoagulable states as an evolving risk for spontaneous venous and arterial thrombosis.

This study of 49 patients with spontaneous venous and arterial thrombosis identified 27 with hypercoagulable states: 13 had only venous thrombosis (VT), six had episodes of VT followed by arterial thrombosis (AT) and eight had AT only. All 27 patients were less than 42 years of age; 22 had specific natural anticoagulant or fibrinolytic deficiencies: antithrombin III (nine patients), protein C (eight patients), protein S (three patients), heparin cofactor II (two patients), tissue plasminogen activator release (one patient) and mixed antithrombin III and protein S (one patient). The remaining five patients had recurrent thrombotic events associated with resistance to heparin anticoagulation, but no established laboratory diagnosis. Clotting complications included recurrent VT, pulmonary embolism, multiple failed arterial procedures and lower extremity amputation. The remaining 22 patients (mean age of 53 years, range of 46 to 63 years), 12 with VT and ten with AT, did not have laboratory evidence of hypercoagulability and none had recurrent vascular occlusions. All these patients were successfully treated by conventional therapy without any additional thrombotic events during the follow-up period. Young adults with spontaneous thrombotic events should be screened for possible hypercoagulable states. Additionally, these young patients need further evaluation and treatment of cardiovascular risk factors. Those with premature atherosclerosis have an especially poor prognosis despite surgical intervention and anticoagulant therapy.

Adult↗
PubMed1993

[Etiology and pathogenesis of spontaneous venous thromboembolism].

Thromboembolic disease is a major clinical problem. This article reviews the etiology and pathogenesis of spontaneous venous thromboembolism. There is an association between thromboembolism and hereditary deficiency of antithrombin III, protein C or protein S. In young patients, impaired fibrinolytic activity may represent the most common haemostatic abnormality associated with thrombosis. Dysfibrinogenaemia has been related to thrombotic disease in some patients. Moreover, the presence of phospholipid directed antibodies is associated with thrombosis in patients with connective tissue disorders. Therefore, for young patients with spontaneous thromboembolic disease, a thorough laboratory investigation is recommended.

Adult↗
PubMed1996

[Risk of thrombosis with oral contraceptives: value of a thrombophilia screening test].

Oral contraceptives increase the natural incidence of venous thrombosis of 1-2/10,000 women per year 3- to 4-fold. Recent studies have shown that desogestrel or gestodene containing formulations bear twice the risk of older low-dose ovulation inhibitors. During pregnancy, the incidence of thrombosis rises to 10/10,000 women-years and post partum up to 40/ 10,000. For 60% of thromboses no causal explanation can be found. In approximately 40% of the patients an inherited thrombophilia can be presumed. Among the hereditary types of thrombophilia, a resistance to activated protein C (APC-resistance) represents nearly 50%, while in 15 to 20% a deficiency of antithrombin III, protein C or protein S is found. APC-resistance, with a prevalence of 3-5% in the general population, increases the risk of thrombosis 8-fold and in users of oral contraceptives 35-fold. Antithrombin III-deficiency carries a comparable risk. Protein C-deficiency increases the risk of thrombosis 9-fold and in users of oral contraceptives 15-fold. Ovulation inhibitors do not influence the risk of thrombosis in women with protein S-deficiency. Anti-phospholipid-antibodies increase during treatment with oral contraceptives and represent a considerably enhanced risk of thrombosis. Inherent thrombophilia is suspected in a patient with a positive history or family history of thrombosis, especially with thrombosis before the age of 40 or with atypical localisation. Even in these risk groups, the cost-benefit ratio of selective screening is unfavorable, as today at most 70% of the hereditary thrombophilias can be diagnosed by laboratory analysis, and only very few of the patients will actually experience a thrombotic event: only 3 of 1000 carriers of APC-resistance will suffer from thrombosis during oral contraception. On the other hand, a negative result of laboratory tests does not exclude a hereditary thrombophilic disorder. At present, it is unclear whether a selective screening process is superior to a careful assessment of individual and family history. A general screening, however, cannot be justified because of the unfavorable cost/benefit ratio. If the individual or family history or pathological laboratory parameters indicate an increased risk of thrombosis, this risk has to be carefully weighed against the consequences of discontinuation of pill use. Those few individuals with risk factors who will experience a thrombo-embolic event, cannot be identified in advance. If oral contraceptives represent a particularly high risk in patients with thrombophilic disorders and/or other risk factors, other contraceptive methods should be considered. If a patient with risk factors decides on the use of oral contraceptives, she must be informed that in the case of symptoms indicating a thrombosis, a physician should be consulted immediately. The earlier an appropriate therapy is initiated, the more effectively pulmonary thrombo-embolism and permanent damage, such as the post-phlebitic syndrome, can be prevented.

Adult↗
PubMed1994

Thrombotic risk of women with hereditary antithrombin III-, protein C- and protein S-deficiency taking oral contraceptive medication. The GTH Study Group on Natural Inhibitors.

The thrombotic risk of women with a heterozygous natural clotting inhibitor deficiency taking oral contraceptives (OC) has not been evaluated. Therefore, a retrospective collaborative controlled cohort-study was carried out in 8 coagulation laboratories and thrombosis units in Austria, Germany and Switzerland. The incidence of thromboembolism in 48 females heterozygous for hereditary type I deficiency of antithrombin III (n = 15), protein C (n = 16) or protein S (n = 17), who had taken OC at least once in their life were compared with that of 48 deficient women, who had never taken OC (controls). Diagnosis of the deficiency state was made in the participating centers. Data on the onset and duration of OC intake and the date and site of thrombotic events were obtained from a questionnaire filled in by the patient or a physician during a visit at a participating center. The observation period in the OC patients was started with onset of OC intake and was terminated when a thromboembolic event had occurred or when OC medication were discontinued. In the patients without OC, the observation period began at an age matched to that of the OC patient and ended when a thromboembolic event had occurred or was continued as long as the corresponding OC patient was on treatment. In AT III-deficient females the probability for thrombosis was significantly higher for patients taking OC compared to the non-OC-patients (Wilcoxon test p = 0.004, Log Rank test p = 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

Antithrombin III↗
PubMed2002

Bacteroides fragilis bacteremia associated with portal vein and superior mesentery vein thrombosis secondary to antithrombin III and protein C deficiency: a case report.

Hypercoagulability is one of the causes of portal vein and superior mesentery vein thrombosis. We report a case of Bacteroides fragilis bacteremia associated with portal vein and superior mesentery vein thrombosis secondary to antithrombin III and protein C deficiency. The patient presented with high fever for more than 3 weeks. Abdominal sonography revealed a liver cyst of 1.7 cm in diameter over segment 4 and a renal stone of 0.7 cm in size over the lower portion of the right kidney but no evidence of hydronephrosis. Elevation of liver enzymes was also noted. Intermittent fever was noted despite treatment with ceftriaxone and doxycycline. On Day 15 of hospitalization, blood culture revealed B. fragilis, which prompted further investigation of the source of intraabdominal and pelvic infection. Abdominal computed tomography revealed portal vein and superior mesentery vein thrombosis. Endoscopic studies of the gastrointestinal tract showed no tumor or diverticulum. Study of coagulation factors disclosed deficiency of antithrombin III and protein C. Clinicians should remain aware of the need to promptly search for a portal or mesentery vein thrombosis in cases of Bacteroides bacteremia of unknown origin.

Adult↗
PubMed1984

Different forms of AT-III congenital defect: a study by crossed immunoelectrofocusing.

Antithrombin III (AT-III) deficiency may be due to quantitative or qualitative AT-III reduction. The diagnosis of qualitative disorder is suspected when a discrepancy is found between immunological and functional levels of AT-III. Heterogeneity has been hypothesized in both quantitative and qualitative deficiency of AT-III. A technique based on crossed immunoelectrofocusing (CIEF) was applied to investigate molecular variants of AT-III. 3 families with low functional and immunological levels of AT-III and 1 family with only a low functional AT-III level were investigated. An abnormal AT-III pattern was found with CIEF in the family with suspected qualitative disorder and in 1 of the families with quantitative disorder. The 2 abnormal patterns were different. Thus the use of CIEF AT-III patterns could help to define congenital AT-III deficiencies and could serve as a basis for classification of different forms of AT-III deficiency.

Adult↗
PubMed1988

Treatment with AT III concentrates in hereditary and acquired AT III deficiency.

41 patients with hereditary and acquired antithrombin II deficiency received a substitution therapy with human plasma fraction of antithrombin III from the GDR blood-transfusion service. In 6 patients a hereditary AT III deficiency was substituted and in this context the substitution in case of thromboses during pregnancy was explained. 35 patients with acquired AT III deficiency were substituted with AT III concentrate because of thromboembolic complication in the macro-circulatory system in case of AT III deficiency due to reduced synthesis, loss, increased consumption or a combination of these conditions or because of DIC. The substitution effect was good. Dosage and injection intervals depend on the clinical condition. Side-effects have not been observed.

Adolescent↗
PubMed1992

Cerebral infarction in a heterozygote with variant antithrombin III.

BACKGROUND: We report a heterozygous case of familial qualitative deficiency of antithrombin III associated with cerebral infarction. CASE DESCRIPTION: A 33-year-old man had a history of recurrent transient ischemic attacks from the age of 28. Cerebral computed tomography at age 29 disclosed a low-density area in the left frontal lobe, and an internal carotid angiogram showed branch occlusion of the right anterior cerebral artery and stenosis of the left middle cerebral artery. Occlusion of the right middle cerebral artery developed thereafter. The plasma antithrombin III antigen concentration and progressive antithrombin activity were normal, but plasma heparin cofactor activity was low in the patient and his father. Nucleotide sequence analysis of the proband's deoxyribonucleic acid showed no mutation in exons II and VI of antithrombin III. CONCLUSIONS: We conclude that abnormal antithrombin III with defective heparin binding, even though heterozygous, may cause ischemic stroke in young adults. We named this antithrombin III variant "Antithrombin III Nagasaki."

Adult↗
PubMed1990

A frameshift mutation leading to type 1 antithrombin deficiency and thrombosis.

Type 1 antithrombin III (ATIII) deficiency, which is the commonest form of inherited ATIII defect, is characterized by a quantitative reduction in both immunologically and functionally detectable protein. This condition is associated with a high incidence of thromboembolic disorder. Previous investigations have shown that the ATIII genes in the majority of cases are grossly intact, but the precise underlying molecular defects remain unknown. We have investigated the molecular basis of a type 1 ATIII deficiency in an Italian kindred by enzymatic amplification of the ATIII gene sequences in affected family members and direct sequencing of the amplified genomic DNA. A novel mutation, the deletion of a single T in the second position of codon 119, was identified in each of the affected individuals. The resulting frameshift leads to a premature termination in codon 126, effectively resulting in a null allele.

Adult↗