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V Brancaccio

Publications and source records attributed to V Brancaccio.

54 records · Page 3Linked to original sources

A rapid screen for lupus anticoagulant with good discrimination from oral anticoagulants, congenital factor deficiency and heparin, is provided by comparing a sensitive and an insensitive APTT reagent.

Lupus anticoagulants (LA) are associated with an increased risk of thrombosis and laboratory detection is of major importance. Various tests are available for LA screening and confirmation, but they differ in sensitivity and specificity, frequently lacking the ability to discriminate between the presence of LA, heparin and oral anticoagulants. We noticed that a patient with LA who had a prolonged activated partial thromboplastin time (APTT) by our routine method, gave a normal result with a different APTT reagent. This latter reagent, which contained soy bean phosphatides (SBP), was compared with a reagent containing rabbit brain phospholipids complexed with kaolin (RBK), for APTT measurement in a variety of patients. There was no significant difference in APTT ratio between the two reagents in plasma samples from healthy normal subjects. In LA samples, SBP gave consistently lower APTT ratios than RBK (mean +/- SEM, 1.04 +/- 0.05 and 2.08 +/- 0.19 for SBP and RBK respectively; P < 0.001). In LA patients receiving oral anticoagulants for antithrombotic prophylaxis or treatment, the APTT ratio was again significantly shorter with SBP (1.60 +/- 0.17 and 3.40 +/- 0.67; P < 0.05). In LA negative patients receiving oral anticoagulants, the relationship was reversed, and a higher APTT ratio was obtained with SBP than RBK (1.61 +/- 0.13 and 1.31 +/- 0.12; P < 0.001). In addition, there were no significant differences in APTT ratios for the two reagents when samples from patients receiving heparin therapy, or patients with acquired factor VIII deficiency or inherited deficiency of factor VIII or IX were studied. The use of the SBP reagent alongside a LA sensitive APTT reagent allows a rapid screening for LA, as well as a confirmation of the phospholipid dependency of the inhibitor.

Administration, Oral↗

[Primary antiphospholipid antibody syndrome with left atrial intracardiac thombosis].

The present report describes a case of right atrial thrombus in an active 49-year-old man with a primary antiphospholipid syndrome. In 1984, the patient was admitted for autoimmune hemolytic anemia; during the hospitalization it was diagnosed a chronic hepatitis B. In July 1991, the patient had fever, mild jaundice, splenomegaly and pancytopenia; a diagnosis of hairy cell leukemia was made but it was not subsequently confirmed. Interferon therapy was started and the patient's clinical course mildly improved. However, over the same year, he experienced again a clinical deterioration. Lumbar ischemic ulcers occurred. The patient underwent elective splenectomy. Bone marrow biopsy revealed mielodisplastic syndrome. Necrotizing vasculitis with granulomatosis was diagnosed. The patient's condition improved after splenectomy. Repeated laboratory tests showed positivity for antiphospholipid antibodies. Transthoracic and transesophageal echocardiography demonstrated the presence of a right atrial thrombus, confirmed by nuclear magnetic resonance. The patient was started on long-term anticoagulant therapy, that resulted effective in reducing thrombus size.

Antibodies, Antiphospholipid↗

Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry.

PURPOSE: To assess the natural history and risk factors for thrombosis in a large cohort of unselected patients with antiphospholipid antibodies. PATIENTS AND METHODS: Three hundred sixty consecutive patients (118 males, 242 females, median age 39 years [range 2 to 78]) fulfilling the currently accepted criteria for diagnosis of lupus anticoagulant (LAC) (n = 326) and/or raised immunoglobulin G anticardiolipin antibodies (IgG ACA) (n = 185) were collected from 16 Italian institutions and prospectively observed for a median of 3.9 years (range 0.5 to 5). Main endpoints were the occurrence of arterial or venous thrombosis, the outcome of pregnancies, and any severe complications leading to hospitalization or death. RESULTS: Thirty-four patients developed a thrombotic complication, with a total incidence of 2.5% patient-years. Multivariate logistic regression analysis identified two independent risk factors for thrombotic events: a previous thrombosis (RR 4.9; 95% CI, 1.76 to 13.7; P < 0.005) and IgG ACA titer above 40 units (RR 3.66; 95% CI, 1.24 to 10.8; P < 0.01). A total of 28 pregnancies were observed in 25 women and 11 (39%) were abortive. Adverse pregnancy outcomes were significantly more frequent in women with a history of miscarriage or vascular occlusion (9/16, 56%) than in asymptomatic women (2/12, 17%) (P = 0.035). Four patients developed non-Hodgkin's lymphoma during the follow-up. Eighteen patients died. Vascular events and hematological malignancies represented the most frequent causes of death (n = 5 for each). CONCLUSIONS: The present study shows that: (a) previous thrombosis and ACA titer > 40 U are independent predictors of thrombosis; (b) history of miscarriage or vascular disease is significantly associated with adverse pregnancy outcome; (c) hematological malignancies can develop during follow-up in patients with antiphospholipid antibodies.

Adolescent↗

Thrombosis in Churg-Strauss syndrome. Beyond vasculitis?

Thromboembolism is considered a rare occurrence in the course of Churg-Strauss syndrome. We report three patients who experienced vascular occlusions at relapse and at first diagnosis of Churg-Strauss syndrome. The pathogenesis of thrombosis in Churg-Strauss syndrome and the potential role played by the eosinophil are discussed.

Adult↗

Minimal trauma knee hemarthrosis.

Four patients who suffered from minimal trauma knee hemarthrosis are presented. In all patients, the cause of the hemarthrosis was not the traumatic event in itself, but an underlying clotting or collagen disorder. The necessity of interaction between the orthopaedic surgeon and a coagulation unit is stressed.

Aged↗

Coagulation activation and fibrinolytic imbalance in subjects with idiopathic antiphospholipid antibodies--a crucial role for acquired free protein S deficiency.

To explore the coagulation/fibrinolytic balance and its relation with free protein S (f-PS) in subjects with antiphospholipid antibodies (aPLs) outside the setting of autoimmune inflammatory disorders, we carried out a cross-sectional study on 18 thrombotic patients with primary antiphospholipid syndrome and 18 apparently healthy subjects with persistence of idiopathic aPLs. Prothrombin fragment 1 + 2 (F1 + 2), thrombin-antithrombin complex (TAT) and D-Dimer (D-D) were taken as markers of thrombin generation and fibrin turnover. Mean F1 + 2 levels were higher in thrombotic (p = 0.006) and non-thrombotic subjects (p = 0.0001) than in controls as were those of D-D (p < 0.0001 and p = 0.003 respectively). TAT levels did not differ. Lower mean levels of f-PS were found in thrombotic (p = 0.0006) and non-thrombotic subjects (p = 0.002) than in controls. Within both groups, mean F1 + 2 levels were higher in subjects who had low f-PS levels compared to those with normal f-PS levels (p = 0.01). Gender analysed data revealed blunted tPA release (venous occlusion test) in thrombotic females (from 16.80 +/- 0.79 to 21.3 +/- 3.9 ng/nl, NS) but not in thrombotic males (from 18.2 +/- 2.0 to 33.7 +/- 4.9 ng/ml, p=0.01) nor in asymptomatic subjects of either sex. Also, in both patient groups females had higher mean PAI than males (p < 0.0002) and than control females (p < 0.02). Low free protein S was found in 100% of non-thrombotic and in 90% of thrombotic patients with defective fibrinolysis. These data are consistent with increased thrombin generation, accelerated fibrin turnover and fibrinolysis abnormalities also in asymptomatic carriers of aPLs and highlight a central role for acquired f-PS deficiency in the thrombotic tendency of the antiphospholipid syndrome.

Adult↗

Benign intracranial hypertension: a non-thrombotic complication of the primary antiphospholipid syndrome?

Benign intracranial hypertension is a rare complication of systemic lupus erythematosus often attributed to cerebral sinus thrombosis which impairs venous drainage and cerebrospinal fluid outflow. We report the case of a woman with a primary antiphospholipid syndrome who developed benign intracranial hypertension with no actual evidence of venous cerebral thrombosis and with no other possible cause for this clinical manifestation than high titres of anticardiolipin antibodies and a lupus anticoagulant.

Adult↗

Antiphospholipid antibodies, haemostatic variables and thrombosis--a survey of 144 patients.

Several clotting abnormalities have been put forth to explain the thrombotic tendency of the antiphospholipid syndrome, but a possible role for fibrinogen and von Willebrand factor has been poorly investigated. The present cross-sectional retrospective study evaluated the relationship of IgG anticardiolipin antibodies, lupus anticoagulants, fibrinogen and von Willebrand factor with the occurrence of arterial and venous thromboses in patients with antiphospholipid antibodies. Among the clotting assays for the detection of lupus anticoagulant, dilute Russell's viper venom time correlated with a history of venous thrombosis more strongly than activated partial thromboplastin time (p < 0.0002 vs p < 0.009) and was the only test which correlated with a history of arterial thrombosis (p < 0.01), also at low levels of IgG anticardiolipin antibodies (p = 0.003). By regression analysis, and after correction for confounders, serum levels of IgG anticardiolipin antibodies were found to be positively associated with the number of venous events (p < 0.001). Plasma levels of fibrinogen and von Willebrand factor were associated with each other (p < 0.0001; r: 0.48) and with the occurrence of arterial and venous thromboses (p < 0.001). Moreover, plasma levels of fibrinogen and von Willebrand factor in thrombotic patients with antiphospholipid antibodies were significantly higher than those of a control group of thrombotic patients who suffered thrombosis for other reasons (p < 0.0001 and p = 0.0008 respectively). Titres of IgG anticardiolipin antibodies correlated with plasma levels of von Willebrand factor (p < 0.0001; r: 0.42).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Primary antiphospholipid syndrome with a familial element and myocardial infarct in an adolescent].

We describe the case of a patient with primary familiar antiphospholipid syndrome and acute myocardial infarction. A previously healthy 15-year-old adolescent was admitted with severe chest pain lasting from 1 hour associated with inferoposterolateral ST-segment elevation. The patient received intravenous thrombolysis. A 2-dimensional echocardiogram revealed an area localized in the basal posterolateral left ventricular myocardium, that was akinetic and abnormally thin throughout the cardiac cycle. Peak creatinine kinase level was 1461 U/I. Subsequent electrocardiogram revealed inferoposterior infarction. Plasma anticardiolipin (aCL) IgG antibodies resulted positive (24 U.GPL) in repeated determinations. A dypiridamole echocardiographic test resulted negative. The patient's parents refused cardiac catheterization. He continues to do well at home 28 months after discharge. The patient's sister is affected by primary antiphospholipid syndrome characterized by recurrent abortion, very low platelet count and lupus anticoagulant positivity. Plasma aCL antibodies resulted positive also in the mother who did not have clinical manifestations.

Adolescent↗

Flomoxef, a new oxacephem antibiotic, does not cause hemostatic defects.

Antibiotics of the beta-lactam class may cause coagulation defects and bleeding. It has been suggested that N-methyltetrazolethiol (NMTT), a common side chain group at the 3'-position of the cephem or 1-oxacephem frame, could be responsible for the hypoprothrombinemic effect of the antibiotics and that it could inhibit the liver vitamin K-epoxide reductase activity. Flomoxef (6315-S) is a new oxacephem antibiotic which differs from latamoxef because it has [1-(2-hydroxethyl)-1H-tetrazol-5-yl] thiomethyl (HTT) as a side chain at the 3'-position of cephem group instead of NMTT and an extensive modification of 7 beta-acylamino side chain. The present study was carried out to study its effects on vitamin K-dependent blood coagulation parameters in human volunteers. Ten adult patients (6 men and 4 women), suffering from chronic bronchitis, entered into the study. Each patient received ten 1 g i.m. injections of flomoxef at 12-hourly intervals. Apparently, the treatment with this oxacephem antibiotic had no significant effect. PT, PTT and fibrinogen remained in the normal range in all patients and factors II+VII+X, protein C, protein S and AT III were not depleted. The trend was similar both in men and women. Based on the results of the present study, we conclude that flomoxef is an antibiotic that does not exhibit an effect on blood coagulation, even in males.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Bilateral renal artery occlusion in a patient with primary antiphospholipid antibody syndrome: thrombosis, vasculitis or both?

A 43-year-old man presented with oliguria and hypertension. Renal arteriography showed bilateral renal artery occlusion. Circulating antiphospholipid antibodies were found together with a change in natural anticoagulant plasma levels. Immunofluorescence of examined vessels showed immune complex vasculitis. We discuss the pathogenetic mechanism leading to the development of this rare occlusive event.

Adult↗

Pyruvate kinase deficiency: characterization of two new genetic variants.

Pyruvate kinase (PK) has been purified from the red blood cells of two sisters who had suffered severe chronic non-spherocytic haemolytic anaemia since infancy, and of one patient who had haemolytic anaemia during pregnancy. The two sisters showed remarkable clinical improvement following splenectomy. The enzyme from their red cells was found to exhibit low activity (about 25% of normal) in crude haemolysates, low affinity for the substrate, phosphoenol pyruvate (PEP), and high sensitivity to fructose-1,6-diphosphate (FDP) activation. This PK differs from previously reported variants and it is provisionally designated PK 'Torre Annunziata'. The enzyme from the other patient had near-normal activity in crude haemolysates, slight changes in kinetics with respect to the substrate, PEP, and with respect to the effects of FDP, ATP and pH, and a markedly reduced thermostability. This PK also differs from previously reported variants and it is provisionally designated PK 'Torre del Greco'. During the course of this study an improved method for purification of PK using Cibacron blue sepharose has been developed.

Adult↗

Hb Lepore and (haemo-) blastomata.

The still increasing amount of carriers and anemics by thalassemia (Th) and other Hb-pathies (approximately 4,000 among approximately 48,000 investigated people) have shown that Campania is the most affected world area by all Hb Lepre conditions. Among 161 people with heterozygous Hb Lepore we have noticed 10 cases associated with (hemo-) blastomata as follows: 2 Chr. Lymphatic Leukemia, 2 Ac. Lymphoblastic Leukemia, 1 Lymphosarcom, 1 Colon Cancer, 1 Uterin Cancer, 1 Plasmocytom, 1 Hodkgin Disease, 1 Ac. Promyelocyte Leukemia (or fatal ac. agranulocytemia?). In the literature we recently found 2 other similar cases. The incidence of such malignancies in our Hb Lepore people reaches 6%. On the contrary in the heterozygous Th. group, among 3,150 carriers, we diagnosed only 20 people with (hemo-) blastomata as follows: 12 Ac. Leukemia (9Lymphoblastic) and 8 Chr. Myeloid Leukemia, with an incidence rate of 0.6% namely a little higher than in normal people. This highly significant discrepancy rate shows an elective predisposition to (haemo-) blastomata from Leporian people.

Colonic Neoplasms↗