Acceptability and tolerability of a new phe-free protein substitute for the dietary management of patients with phenylketonuria, aged ≥16 years

SummaryDeficiency of protein S has been associated with an increased risk of thrombotic disease as already shown for protein C deficiency. Deficiencies of any of these two proteins predispose to venous thrombosis but have been only rarely associated with arterial thrombosis.In this study we describe a case of severe cerebral arterial thrombosis in a 44-year old woman with protein S deficiency. The defect was characterized by moderately reduced levels of total and markedly reduced levels of free protein S. C4b-bp level was normal. Protein C, AT III and routine coagulation tests were within the normal limits.In her family two other members showed the same defect. All the affected members had venous thrombotic manifestations, two of them at a relatively young age. No other risk factors for thrombotic episodes were present in the family members. The patient reported was treated with ASA and dipyridamole and so far there were no relapses.

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The Relationship between Plasma Microparticles, Protein S and Anticardiolipin Antibodies in Patients with Human Immunodeficiency Virus Infection

Thrombosis and Haemostasis ◽

10.1055/s-0038-1650519 ◽

1996 ◽

Vol 76 (01) ◽

pp. 038-045 ◽

Cited By ~ 24

Author(s):

Jean-Christophe Gris ◽

Pierre Toulon ◽

Sophie Brun ◽

Claude Maugard ◽

Christian Sarlat ◽

...

Keyword(s):

Poor Prognosis ◽

Anticardiolipin Antibody ◽

Protein S ◽

Anticardiolipin Antibodies ◽

Protein S Deficiency ◽

Free Protein ◽

Precipitation Technique ◽

S Deficiency ◽

Immunodeficiency Virus ◽

Antibody Titers

SummaryThe high prevalence of free protein S deficiency in human immunodeficiency virus (HlV)-infected patients is poorly understood. We studied 38 HIV seropositive patients. Free protein S antigen values assayed using the polyethylene-glycol precipitation technique (PEG-fS) were statistically lower in patients than in controls. These values using a specific monoclonal antibody-based ELISA (MoAb-fS) and the values of protein S activity (S-act) were not statistically different between patients and controls. C4b-binding protein values were not different from control values. In patients, PEG-fS values were lower than MoAb-fS values. Ten patients had a PEG-fS deficiency, 4 patients had a MoAb-fS deficiency and 8 had a S-act deficiency. Protein S activity and MoAb-fS were lower in clinical groups with poor prognosis and in patients with AIDS but PEG-fS was not. A trend for reduced S-act/MoAb-fS ratios was observed in patients. PEG-fS was negatively correlated with anticardiolipin antibody titers whereas MoAb-fS was not. The plasma of PEG-fS deficient HIV-patients contained high amounts of flow cytometry detectable microparticles which were depleted from plasma by PEG precipitation. The microparticles were partly CD42b and CD4 positive but CD8 negative. These microparticles were labelled by an anti free protein S monoclonal antibody. The observed differences between MoAb-fS and PEG-fS values were correlated with the amount of detectable plasma microparticles, just like the differences between MoAb-fS and S-act. Plasma microparticles correlated with anticardiolipin antibody titers.In summary, free protein S antigen in HIV infected patients is underestimated when the PEG precipitation technique is used due to the presence of elevated levels of microparticles that bind protein S. The activity of free protein S is also impaired by high levels of microparticles. The prevalence of free protein S deficiency in HIV positive patients is lower than previously published (4/38, -10%) and is correlated with poor prognosis. By implication, use of a PEG precipitation technique might give artefactually low free protein S antigen values in other patient groups if high numbers of microparticles are present. In HIV patients, high titers of anticardiolipin antibodies are associated with high concentrations of cell-derived plasma microparticles.

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Free Protein S Deficiency Is a Risk Factor for Venous Thrombosis

Thrombosis and Haemostasis ◽

10.1055/s-0038-1665408 ◽

1997 ◽

Vol 78 (05) ◽

pp. 1343-1346 ◽

Cited By ~ 54

Author(s):

Elena M Faioni ◽

Carla Valsecchi ◽

Alessandra Palla ◽

Emanuela Taioli ◽

Cristina Razzari ◽

...

Keyword(s):

Risk Factor ◽

Relative Risk ◽

Venous Thrombosis ◽

Protein S ◽

Unexpected Finding ◽

Reference Ranges ◽

Protein S Deficiency ◽

Men And Women ◽

Free Protein ◽

S Deficiency

SummaryA recent study suggests that protein S deficiency is not a risk factor for venous thrombosis. Since this unexpected finding would have important clinical implications if confirmed, we performed a case-control study with the aim to determine the prevalence of protein S deficiency in patients with thrombosis and in healthy individuals taken from the general population and the relative risk of thrombosis in protein S-deficient patients. Free protein S concentration was measured in 327 consecutive patients with at least one venous thrombotic episode and in 317 age- and sex-matched control individuals. Different normal reference ranges were obtained and adopted for men and women. Protein S deficiency was found in 3.1% (95% Cl: 1.5-5.2) of patients and in 1.3% of controls (95% Cl: 0.3-2.8). Ten patients and 4 control subjects had protein S deficiency, which determined a relative risk of thrombosis (sex- and age-adjusted odds ratio) of 2.4 (95% Cl: 0.8-7.9). When men and women were analyzed separately, the risk was 5.0 (95% CI: 0.6-43.6) and 1.6 (95% Cl: 0.4-6.7) respectively. PS-deficient men had more thrombotic episodes than women and later in life. Multivariate analysis established that sex was an independent determinant of the number of episodes, as was age, while PS deficiency was not. However sex and PS deficiency status were both determinants of age at first thrombotic episode.

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MATHEMATICAL MODELING OF OPTIMIZED RECEPTURAL OF LOW-LACTOSE SYNBIOTIC YOGHOURT ICE CREAM

Animal Science and Food Technology ◽

10.31548/animal2020.02.083 ◽

2020 ◽

Vol 11 (2) ◽

pp. 73-82

Author(s):

А. Trubnikova ◽

О. Chabanova ◽

S. Bondar ◽

Т. Sharakhmatova ◽

Т. Nedobijchuk

Keyword(s):

Concentration Factor ◽

Raw Materials ◽

Ice Cream ◽

Test Sample ◽

Protein Concentrate ◽

Important Indicator ◽

Free Protein ◽

Mass Fractions ◽

New Type ◽

Main Components

Optimization of the formulation of synbiotic yogurt ice cream low-lactose using lactose-free protein concentrate of buttermilk and yogurt with low lactose content is the goal of expanding the range of low-lactose dairy products and improving the functional and health properties of ice cream. Low-lactose ice cream formulation optimization was performed using a gradient numerical method, namely conjugated gradients (Conjugate Gradient). The optimization algorithm is implemented in Mathcad. An array of data with a set of indicators for the choice of a rational ratio of lactose-free protein concentrate of buttermilk and yogurt base and inulin content for ice cream mixtures is presented. The influence of the ratio of the main components of the mixtures on the foaming ability, which determines the quality of the finished product, has been studied. An important indicator is taken into account - the concentration factor of buttermilk, which is additionally purified from lactose by diafiltration. The graphic material presented in the work clearly demonstrates that the rational ratio of yogurt base and lactose-free protein concentrate of buttermilk, obtained by ultrafiltration with diafiltration purification at a concentration factor of FC = 5 is 40.6: 59.4. The content of additional components included in the recipe of a new type of ice cream is optimized in the work, the mass fractions of which were: inulin - 3.69 %; lactulose – 1 %; ginger - 0.3 %; citric acid - 0.15 %; stabilization system - 0.2 %. The chemical composition and quality indicators of the mixture for ice cream low-lactose synbiotic yogurt, consisting of raw materials in the optimal ratio, were determined. The lactose content in the test sample of the ice cream mixture was 0.99%, the antioxidant activity was 3.1 times higher than in the mixture for traditional yogurt ice cream. The most likely number of lactic acid microorganisms, CFU / cm3 is (2.8 ± 0.9) · 108, the number of bifidobacteria, CFU / cm3 is (2.5 ± 0.2) · 109. The results of the research will be implemented in dairy companies in the production of ice cream.

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