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PubMed2000

Acute arterial thrombosis with antithrombin III deficiency in nephrotic syndrome: report of a case.

Nephrotic syndrome frequently causes venous thromboembolic complications. Arterial thrombosis has rarely been reported and is mainly observed in children. Only six cases of lower extremity arterial thrombosis in adults have been reported in the literature. The outcome in these cases was unsatisfactory because of the high rates of limb loss and recurrence of thrombosis. We report successful treatment of a 39-year-old man who suffered from right lower extremity arterial thrombosis associated with decreased levels of serum antithrombin III. He was admitted to our hospital with severe pain in his right foot. No pulse was palpable in his right dorsalis pedis or posterior tibial arteries. His right foot was cold and mottled, with a reduced sensation and motor activity. The laboratory data revealed a serum total protein concentration of 3.9g/dl and an albumin concentration of 1.5 g/dl. The coagulation profile showed a fibrinogen level of 879 mg/dl and antithrombin III value of 9.5%. Right lower extremity arteriography showed a complete occlusion of the right deep femoral artery and popliteal artery, and a filling defect in the common femoral artery. An emergency thrombectomy was performed under general anesthesia. The patient was treated successfully, and surgical treatment was followed by anticoagulant therapy with 1,000 units of antithrombin III. A renal biopsy revealed histologic evidence of minimal change of glomerulonephritis. He was discharged 3 months later, and no recurrence of thrombosis has yet been observed.

Acute Disease↗
PubMed1994

Increased endogenous thrombin generation in children with acute lymphoblastic leukemia: risk of thrombotic complications in L'Asparaginase-induced antithrombin III deficiency.

Pediatric patients with acute lymphoblastic leukemia (ALL) are at an increased risk of thromboembolic events. Potential responsible mechanisms include the disease process itself, treatment with chemotherapeutic agents (particularly L-Asparaginase [ASP]), or a combination of the disease and treatment. We studied thrombin regulation in 26 consecutive children with ALL and 14 healthy age-matched controls by: (1) plasma concentrations of prothrombin; (2) plasma inhibition of 125I-alpha-thrombin; and (3) four biochemical markers of in vivo thrombin activation (thrombin complexed to its inhibitor antithrombin III [ATIII; TAT], prothrombin fragment 1.2 (F1.2), activated protein C complexed to the inhibitors alpha 1 antitrypsin [APCAT]), and protein C inhibitor (APC-PCI). Measurements were made at presentation before treatment, after treatment with ASP alone, and during combination chemotherapy with and without ASP. At presentation, the capacity to generate thrombin (reflected by plasma prothrombin concentrations) and the capacity to inhibit thrombin (125I-alpha-thrombin--inhibitor complex formation) were similar in children with ALL compared with that for healthy children. After ASP alone or as part of combination chemotherapy, prothrombin levels were preserved, whereas plasma inhibition of 125I-alpha-thrombin decreased significantly because of a decrease in plasma concentrations of inhibitors, most importantly ATIII. After combination chemotherapy without ASP, plasma concentrations of ATIII and the capacity to inhibit 125I-alpha-thrombin returned to normal values, whereas prothrombin levels increased above control values. Thrombin generation in vivo also differed from healthy controls. At presentation, plasma concentrations of three of four markers of in vivo thrombin activity (TAT, F1.2, APCAT, but not APC-PCI) were increased in children with ALL. Neither ASP alone nor combination chemotherapy with or without ASP significantly altered values of these three markers. In summary, although the in vitro capacity to generate thrombin was preserved, the in vitro capacity to inhibit 125I-alpha-thrombin decreased after ASP therapy. Evidence for increased endogenous thrombin generation was documented in children with ALL at presentation and throughout treatment. We speculate that poor regulation of this thrombin may contribute to thrombotic complications in children with ALL.

Adolescent↗
PubMed1983

Severe acquired antithrombin III deficiency in relation to hepatic and renal insufficiency and intrauterine fetal death in late pregnancy.

Acute fatty liver (AFLP) is a rare complication of late pregnancy. The maternal and fetal mortality has earlier been reported to be about 75%, but during the last decade a reduced mortality to about 30 and 50%, respectively, has been reported in the literature. Disseminated intravascular coagulation (DIC) has been suggested as a contributing cause to the high mortality. The treatment of DIC has long been under debate, and recently the administration of antithrombin III (AT) concentrate in addition to other supportive treatment has been reported successful. This paper presents the survival of 1 patient with severe liver and renal failure indicating AFLP complicated by severe disturbances in blood coagulation and fibrinolysis. The patient was treated with AT concentrate and small doses of heparin, blood coagulation factors, large amounts of glucose intravenously and supportive intensive care. The pregnancy was terminated by cesarean section. The child was stillborn and 75% of the placental parenchyma was fibrosed.

Acute Kidney Injury↗
PubMed1990

A Scottish Hebridean antithrombin III deficient family--twelve years on.

The members of this family with ATIII deficiency have been followed for at least 12 years (1976-1989). All those with previous venous thrombo-embolism have been free from recurrence when on warfarin. During the half century 1931-1981, all 11 full term pregnancies in four affected patients were associated with venous thromboembolism; one patient was enigmatic having one full term pregnancy, without thrombotic event; between 1982 and 1989 three pregnancies have been actively managed with no clinical thrombosis. Management involved use of monitored, self administered, subcutaneous heparin before or very soon after conception and throughout pregnancy (warfarin having been stopped), planned delivery under cover of intravenous antithrombin III, reduction of heparin dosage at delivery and reintroduction of warfarin in the puerperium. The recognised hazards of heparin therapy in pregnancy did not occur. The involvement of the arterial system is reviewed. Clinical evidence provides tentative suggestions on (a) possible additional risk of cigarette smoking (b) avoidance of venography (c) avoidance of varicose vein surgery. A probe is now available for the defective antithrombin III gene in this family, but there has been no occasion yet to apply this in antenatal diagnosis.

Abortion, Spontaneous↗
PubMedDate not supplied

Mesenteric venous thrombosis in a patient with prothrombin 20210A mutation and antithrombin III deficiency: challenges to conventional anticoagulation--a case report.

Mesenteric venous thrombosis presents as vague abdominal pain in patients with a medical or family history suggestive of a hypercoagulable state. Classic computed tomography findings will often confirm the diagnosis, and the presence of persistent abdominal pain or tenderness will determine the need for surgical intervention. Expeditious anticoagulation is the treatment of choice. This case demonstrates the CT findings for mesenteric venous thrombosis and discusses the challenges of anticoagulation in a patient with 2 hypercoagulable disorders.

Abdominal Pain↗
PubMed1994

Antithrombin III deficiency in neonatal respiratory distress syndrome.

Neonatal respiratory distress syndrome (RDS) is an acute lung injury believed to result primarily from surfactant deficiency in the immature lung. Although surfactant replacement therapy has improved the outcome of this disease, RDS remains a major cause of neonatal mortality and morbidity. Preliminary experimental evidence suggests that unopposed intravascular thrombin activity may contribute to the progression of RDS by promoting high permeability pulmonary oedema and pulmonary hypertension. In the extravascular lung compartment, polymerizing fibrin may inhibit surfactant function. In addition, interstitial and alveolar thrombin formation and resulting fibrin deposition may contribute to the development of chronic lung disease through amplification of inflammation and fibrosis. There is good evidence that extravascular coagulation occurs during the course of RDS. Fibrin is a major component of the hyaline membranes, which are a hallmark of acute lung injury, and which can be regarded as locally produced clots. It has been less certain whether neonatal RDS is also associated with intravascular activation of the coagulation system. Although low levels of antithrombin III (AT III) have been reported in infants with RDS, direct evidence of increased intravascular thrombin formation has been lacking. However, recently, plasma concentrations of thrombin-antithrombin III (TAT) complexes have been measured in infants with RDS and correlated with RDS severity. TAT formation was significantly increased in severe neonatal RDS, while free AT III activity was decreased. These data are consistent with increased thrombin generation and resulting AT III consumption. Therefore, to regulate thrombin activity, infants with severe RDS may benefit from replacement therapy with AT III concentrate. This hypothesis has been strengthened by experiments that have demonstrated the efficacy of thrombin inhibition in several animal models of acute lung injury. However, controlled clinical trials will be required to determine whether thrombin is just a coincidental marker of neonatal RDS, or whether unopposed thrombin activity exacerbates the disease process.

Animals↗
PubMed1984

Hereditary antithrombin III deficiency: biochemical aspects.

Since the description of the first thrombophilic family with congenital AT-III deficiency, increasing numbers of different types of the condition have become evident. Initially the anomaly seemed to be homogeneous and simple: the three main characteristics of AT-III (thrombin inactivating and heparin cofactor activity, antigen concentration) were decreased. This type of AT-III deficiency (type 1) was later divided into type 1a and 1b on the basis of the heparin affinity of the AT-III molecule. The first family with a different qualitative AT-III disorder (type 2) was described by our group in 1974. In the members of this family the AT-III antigen concentration was normal, but the molecule had no functional activity (AT-III Budapest). In the last few years some new variants of type 2 hereditary AT-III disorders have been observed; they are characterized by a loss of one or more functional properties of the AT-III molecule.

Antigens↗
PubMed1985

Antithrombin III deficiency: a report of 14 cases belonging to three different kindreds.

A hereditary deficiency of AT III is described in 14 subjects belonging to three different kindreds. There is no consanguineity in any of the families investigated. The pattern of inheritance of defect appears autosomal dominant. Seven of the affected subjects presented thrombotic episodes (deep vein thrombosis, splanchnic thrombosis, pulmonary embolization). The main laboratory features were: normal routine clotting tests, decreased AT III activity in all assay systems and concomitantly reduced AT III antigen levels. Crossed immunoelectrophoresis showed only reduced peaks with respect to normal in both plasma and serum. No correlation was found between age of patients and AT III levels.

Adult↗
PubMed1983

Antithrombin III deficiency in ischaemic stroke.

AT III activity and concentration were measured in 36 patients (mean age 65.5 yrs, range 43-77 yrs) with ischaemic stroke within maximally 48 h of the acute event. In 12 patients (= 33%) AT III activity was reduced below 18.4 IU/ml: 50% of these patients showed normal and 50% reduced AT III concentration of less than 22 mg/dl. In 15 patients AT III activity and concentration were measured in the acute phase on admission to hospital and 12 months later. In the acute phase, AT III activity was reduced when compared with AT III concentration (y = 0.19 chi + 15.5) and did not correlate with the latter. 12 months later, however, AT III activity and concentration correlated significantly (r = 0.92; p less than 0.001) and the regression line was steeper (y = 0.8 chi). During the acute phase of ischaemic stroke, intravascular coagulation is evidently increased and inactive AT III-thrombin complexes are formed, whereby the concentration of active AT III decreases. A patient with progressive stroke and reduced AT III activity of 14.2 IU/ml was therefore substituted with AT III concentrate. The further neurological course was favourable.

Adult↗
PubMed1995

Massive deep vein thrombosis in a patient with antithrombin III deficiency undergoing ovarian stimulation for in vitro fertilization.

OBJECTIVE: To present the first report of a thromboembolic complication in early pregnancy after ovarian hyperstimulation for IVF in a patient with AT III deficiency who was treated successfully and subsequently delivered a healthy male infant at 32 weeks of gestation. DESIGN: Case report. SETTING: Hospital-based clinic for reproductive medicine. PATIENT: A 28-year-old woman who consulted our IVF clinic with a 3.5-year history of primary infertility. INTERVENTIONS: Intravenous heparin therapy. RESULTS: The patient responded adequately to heparin therapy and was discharged home on SC heparin. A primary cesarean section was performed at 32 weeks of gestation because of poor fetal growth and transverse lie. CONCLUSIONS: We stress the importance of obtaining a thorough personal and family history before initiation of ovarian hyperstimulation. Measuring activity of AT III, protein C, and protein S in patients with a suspicious history of thromboembolic episodes occurring at an early age may lead to the implementation of appropriate prophylactic measures, preventing potentially life-threatening complications.

Adult↗
PubMed1993

[A case of congenital antithrombin III deficiency with pulmonary thromboembolism and cerebellar infarction].

A 41-year old woman was admitted to a local hospital because of leg pain, chest pain and dyspnea after taking estrogen for two months for irregular menstruation. On admission to the hospital, her lung scintigram showed multiple segmental perfusion defects in the right lung. Pulmonary angiography showed several thrombi in the proximal pulmonary artery. A diagnosis of pulmonary thromboembolism was made. AT-III activity was found to be only 50% of normal value, indicating that the pulmonary thromboemboli were due to AT-III deficiency. The patient was then put on anticoagulants for two years. When she was 43-year-old, she was admitted to Keio University Hospital because of worsening of dyspnea. After admission her dyspnea had got better due to bed rest. Her brain CT, which was performed because of her gait disturbance, indicated past right cerebellar infarction. We gave her 2 mg of warfarin and maintained a thrombotest of about 30%. The investigation of her family revealed that her son also showed decrease AT-III activity, indicating a congenital abnormality. 26 families with this disease have been reported in Japan. Most had venous thromboemboli, but only a few cases had brain infarction as well. In addition, it is suggested that an estrogen therapy for irregular menstruation may have contributed, in this particular case, to the onset of thromboemboli.

Adult↗
PubMed1982

Antithrombin III deficiency: decreased synthesis of a biochemically normal molecule.

A 29-yr-old white female has suffered from recurrent venous thromboses over the last 12 yr. Plasma antithrombin III (AT-III) levels were 48% of normal by immunoelectrophoresis and 56% by chromogenic assay. Three of four siblings and the father had similar AT-III levels without associated venous thromboses. Heparin-Sepharose chromatography demonstrated normal behavior of the patient's AT-III. Her purified AT-III could not be distinguished from AT-III purified from a normal control either by SDS polyacrylamide gel electrophoresis or by crossed immunoelectrophoresis, and the heparin cofactor activity and the progressive antithrombin activity of both AT-III samples were identical. Turnover studies were made in the patient using her own purified AT-III labeled with 131I, (*I). The results did not differ significantly from studies made with autologous *I-AT-III in two normal control women. Her fractional breakdown rate of 0.54 total plasma AT-III per day compared with 0.45 and 0.52 in the controls. These studies indicate that the patient synthesizes a normal AT-III molecule at half normal rates.

Adult↗