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Biomedical subjects

I Alberca

Publications and source records attributed to I Alberca.

At least 55 records · Page 3Linked to original sources

Congenital deficiency of vitamin K-dependent coagulation factors and protein C.

A 15-month-old girl from Coimbra (Portugal) had a history of numerous hemorrhagic episodes with multiple bruises, hematomas but not hemarthroses. On serial testing she showed deficiency of factors II, VII, IX, X and protein C. Malabsorption-induced vitamin K deficiency, liver disease or ingestion of a coumarin compound were excluded. An absence of detectable abnormalities was found among her relatives. Consanguinity was not present. The immunologic assay, immunoelectrophoresis or antibody neutralization, revealed much higher levels of these factors than the clotting assay. The non-physiological activator (Echis carinatus venom) produced higher levels of prothrombin activation than those detected by physiological activation. Two-dimensional immunoelectrophoresis of the patient's plasma in calcium showed that prothrombin had the same mobility as acarboxyprothrombin. No significant response to large doses of intravenous vitamin K3 (6 mg) was observed. Transfusion of 120 ml of frozen fresh plasma led to an immediate increase in the procoagulant activities of vitamin K dependent protein, similar to that found after perfusion of plasma plus vitamin K3. The results obtained from this patient suggest a defect in the gammacarboxylation mechanism inside the hepatocyte.

Blood Coagulation Disorders↗

DDAVP in uremia.

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Adolescent↗

Coagulation proteins showing abnormal electrophoretic mobility in commercial concentrates of factor VIII and prothrombin complex.

Five commercial factor VIII (FVIII) concentrates and three prothrombin complex concentrates (PCC) were studied with reference to the qualitative evaluation of factors II, IX, fibronectin, alpha 2-antiplasmin (alpha 2-AP), antithrombin III (AT-III) and subunits A and S of FXIII by crossed-immunoelectrophoresis (CIE) and von Willebrand factor antigen (vWF:Ag) by radio-CIE. This latter protein had a different pattern with the absence or a decrease of larger forms and the presence of a fast-moving precipitating peak, suggesting degradation of the vWF:Ag in FVIII concentrates. In contrast, the electrophoretic mobility of fibronectin, alpha 2-AP and AT-III was normal. All PCC showed a more anodic mobility of factor IX. alpha 2-AP also exhibited a different electrophoretic pattern to that of normal plasma. Abnormality of AT-III was also found in heparin-binding studies. The techniques used in the purification procedures are probably the mechanism responsible for the partial denaturing of these proteins.

Antithrombin III↗

Factor VIII-related antigen in cerebrospinal fluid.

A hundred and one samples of cerebrospinal fluid (CSF) were obtained from patients with bacterial meningitis (18), viral meningitis (9), lymphoproliferative disorders (33), 15 with meningeal infiltrations, multiple sclerosis (8), stroke (8) and 25 subjects with normal CSF. All samples were studied for VIIIR:Ag with specific and sensitive immunoradiometric assay (IRMA) and Laurell's technique. Prothrombin and factor IX antigenic activities were investigated by Laurell's technique. Simultaneously, plasma specimens from ten patients with bacterial meningitis were evaluated. Only a selective increase of VIIIR:Ag was demonstrated in CSF from bacterial meningitis whereas prothrombin and factor IX were not detected. VIIIR:Ag plasma and CSF levels were uncorrelated. Similarly, no relationship could be established between the degree of elevation of VIIR:Ag in the CSF and their protein concentration. These findings suggest that VIIIR:Ag elevation in CSF has diagnostic value for bacterial meningitis and that disruption of the blood-brain barrier is not responsible for their elevated levels. Accordingly, the presence of VIIIR:Ag in CSF may be an indication of endothelial damage in the choroid plexi.

Antigens↗

Acute phase reactant proteins in differential diagnosis of monoclonal gammopathy.

The serum levels of five individual serum proteins, i.e. ceruloplasmin, alpha 1-antitrypsin, orosomucoid, haptoglobin and transferrin, were studied in 55 multiple myeloma (MM) patients, in 34 essential monoclonal gammopathy (EMG) patients, in 14 EMG patients excluded for active inflammatory process and in 14 healthy control subjects. Transferrin--negative acute phase reactant (APR)--was significantly decreased and the remaining proteins under study--positive APR--slightly increased in the EMG group compared with healthy controls. Transferrin and ceruloplasmin levels were significantly different when the MM was compared to the EMG group so that these parameters might be useful in differential diagnosis between both groups. In the MM clinical stage III, the differences are even more significant. In the IgG3 MM the APR levels seem to be more significantly changed than in the IgG1 and IgG2 MM. In EMG patients with active inflammatory process who were excluded ceruloplasmin, alpha 1-antitrypsin, orosomucoid and haptoglobin were significantly increased when compared with the EMG group.

Blood Proteins↗