scholarly journals Clues to Disease Activity in Juvenile Dermatomyositis: Neopterin and Other Biomarkers

Diagnostics ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 8
Author(s):  
Amer Khojah ◽  
Gabrielle Morgan ◽  
Lauren M. Pachman
Keyword(s):  
Disease Activity ◽  
Macrophage Activation ◽  
Nk Cell ◽  
Juvenile Dermatomyositis ◽  
Rapid Assessment ◽  
Elevated Serum ◽  
Muscle Enzymes ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

Easily accessible biomarkers are urgently needed to evaluate immune activation in Juvenile Dermatomyositis (JDM). The goal of this retrospective study is to define immunological and clinical differences between untreated JDM patients with either normal or elevated (>10 mmol/L) levels of neopterin, a biomarker of macrophage activation. We included all JDM with neopterin data obtained before initiating medical therapy. We assessed T, B, NK cell populations, muscle enzymes, and disease activity scores for skin (sDAS), muscle (mDAS), total (tDAS), the duration of untreated disease, disease course, and myositis-specific antibody (MSA). Seventy-nine percent of 139 untreated JDM patients had elevated serum neopterin. The group with elevated neopterin had significantly more active disease: tDAS 11.9 vs. 8.1 (p < 0.0001), mDAS 5.8 vs. 3.1 (p < 0.0001), sDAS 6.1 vs. 4.9 (p = 0.0002), aldolase 24.0 vs. 7.6 U/L (p < 0.0001), von Willebrand factor antigen (p < 0.0001), and ESR 19.8 vs. 11.5 mm/hr (p = 0.01). The flow cytometry documented both reduced T cells (1494 vs. 2278/mm3, p = 0.008) and NK cells (145 vs. 240/mm3, p = 0.003). TNFα-308AA/AG polymorphism was more common in children with elevated neopterin than TNFα-308GG (p 0.05). We conclude that the availability of neopterin data will contribute to the rapid assessment of untreated JDM disease activity.

Significance of Platelet β-Adrenoceptors for Platelet Responses In Vivo and In Vitro

1992 ◽  
Vol 68 (06) ◽  
pp. 687-693 ◽  
Author(s):  
P T Larsson ◽  
N H Wallén ◽  
A Martinsson ◽  
N Egberg ◽  
P Hjemdahl
Keyword(s):  
Ex Vivo ◽  
High Dose ◽  
Platelet Aggregability ◽  
Adrenoceptor Agonist ◽  
Dose Infusion ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

SummaryThe significance of platelet β-adrenoceptors for platelet responses to adrenergic stimuli in vivo and in vitro was studied in healthy volunteers. Low dose infusion of the β-adrenoceptor agonist isoprenaline decreased platelet aggregability in vivo as measured by ex vivo filtragometry. Infusion of adrenaline, a mixed α- and β-adrenoceptor agonist, increased platelet aggregability in vivo markedly, as measured by ex vivo filtragometry and plasma β-thromboglobulin levels. Adrenaline levels were 3–4 nM in venous plasma during infusion. Both adrenaline and high dose isoprenaline elevated plasma von Willebrand factor antigen levels β-Blockade by propranolol did not alter our measures of platelet aggregability at rest or during adrenaline infusions, but inhibited adrenaline-induced increases in vWf:ag. In a model using filtragometry to assess platelet aggregability in whole blood in vitro, propranolol enhanced the proaggregatory actions of 5 nM, but not of 10 nM adrenaline. The present data suggest that β-adrenoceptor stimulation can inhibit platelet function in vivo but that effects of adrenaline at high physiological concentrations are dominated by an α-adrenoceptor mediated proaggregatory action.

The Effect of n-3 Fatty Acids on Lipids and Haemostasis in Patients with Type lla and Type IV Hyperlipidaemia

1989 ◽  
Vol 62 (02) ◽  
pp. 797-801 ◽  
Author(s):  
E Berg Schmidt ◽  
E Ernst ◽  
K Varming ◽  
J O Pedersen ◽  
J Dyerberg
Keyword(s):  
Fatty Acids ◽  
Plasma Lipids ◽  
Plasma Cholesterol ◽  
Hdl Cholesterol ◽  
Apolipoprotein A1 ◽  
Type Iv ◽  
Before And After ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

SummaryPlasma lipids and haemostasis were investigated in 17 patients with hyperlipidaemia before and after 6 weeks supplementation with 6 g n-3 fatty acids. Nine of the patients had type IIa and 8 had type IV hyperlipidaemia. No effect on plasma cholesterol, LDL- or HDL-cholesterol were seen, but plasma triglycerides decreased after n-3 supplementation. Apolipoprotein B increased and apolipoprotein A1 decreased after the oil supplement. The bleeding time was prolonged, but platelet aggregation was unaltered by n-3 fatty acids. Protein C activity increased in type II a and decreased in type IV after the supplement. Fibrinolysis was markedly depressed while von Willebrand factor antigen was reduced after intake of n-3 fatty acids.

Comparative Study of Intranasal, Subcutaneous and Intravenous Administration of Desamino-D-Arginine Vasopressin (DDAVP)

1986 ◽  
Vol 55 (01) ◽  
pp. 108-111 ◽  
Author(s):  
M Köhler ◽  
P Hellstern ◽  
C Miyashita ◽  
G von Blohn ◽  
E Wenzel
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
Factor Xii ◽  
Additional Advantage ◽  
Mean Values ◽  
Routes Of Administration ◽  
The Mean ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

SummaryThis study was performed to evaluate the influence of different routes of administration on the efficacy of DDAVP treatment. Ten healthy volunteers received DDAVP intranasally (i.n.), subcutaneously (s.c.) and intravenously (i.v.) in a randomized cross-over trial. Factor XII and high molecular weight (HMW)-kininogen levels increased only slightly after DDAVP administration. The mean increase of factor VIII: C was 3.1 (i. v.), 2.3 (s. c.), and 1.3 (i.n.) - fold over baseline. Ristocetin cofactor (von Willebrand factor antigen) increased 3.1 (2.5), 2.0 (2.3) and 1.2 (1.2) - fold over baseline mean values after i.v., s.c. and i.n. DDAVP, respectively. The half-disappearance time of factor VIII and von Willebrand factor (vWF) after DDAVP ranged from five (factor VIII: C) to eight hours (vWF). The mean increase of fibrinolytic activity was more pronounced after i.v. DDAVP. The antidiuretic effect was moderate with no apparent differences between the routes of application. This study provides further evidence that both i.v. and s.c. DDAVP administration result in an appropriate and reliable stimulation of haemostasis. An additional advantage of s. c. administration is its suitability for home treatment.

scholarly journals Studies of the pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies

Blood ◽  
1984 ◽  
Vol 64 (3) ◽  
pp. 614-621 ◽  
Author(s):  
PM Mannucci ◽  
R Lombardi ◽  
R Bader ◽  
MH Horellou ◽  
G Finazzi ◽  
...  
Keyword(s):  
Lymphoproliferative Disorders ◽  
Type I ◽  
Agarose Gel Electrophoresis ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Monoclonal Gammopathies ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen ◽  
Ristocetin Cofactor

Abstract In seven patients with acquired von Willebrand's disease (AvWD) associated with lymphoproliferative disorders or benign monoclonal gammopathies, the platelet contents of von Willebrand factor antigen and ristocetin cofactor (vWF:Ag and vWF:RiCof, respectively) were normal. All the multimers of vWF:Ag could be seen in the 1.6% SDS- agarose gel electrophoresis patterns of plasma and platelet lysates. Infusion of 1-deamino-8-D-arginine vasopressin (DDAVP) augmented plasma levels of vWF:Ag and vWF:RiCof of all patients and corrected prolonged bleeding times (BT). However, compared with patients with congenital vWD type I and comparable degrees of baseline abnormalities treated in the same way, vWF:Ag and vWF:RiCof were increased less and cleared more rapidly from plasma and the BT remained normal for a shorter period of time. These studies provide evidence that these AvWD patients have qualitatively normal vWF in plasma, but at lower concentrations, that vWF in platelets is normal both qualitatively and quantitatively, and that cellular vWF can be rapidly released into plasma by DDAVP to correct the hemostatic abnormalities. However, vWF is removed rapidly from plasma, making the correction more transient than in congenital vWD type I.

scholarly journals Plasma levels of von Willebrand factor antigen in acute bronchitis and in a normal population

1998 ◽  
Vol 92 (3) ◽  
pp. 395-400 ◽  
Author(s):  
D.A.R. Boldy ◽  
P.E. Short ◽  
P. Cowen ◽  
F.G.H. Hill ◽  
D.C. Chambers ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Plasma Levels ◽  
Normal Population ◽  
Acute Bronchitis ◽  
Von Willebrand Factor Antigen ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

scholarly journals Assessment of Plasma von Willebrand factor antigen in non-metastatic Prostate Cancerpatients: across-sectional study of the Sudanese Population

2019 ◽  
Author(s):  
Mohammed Othman Hashim ◽  
Gad Allah Modawe ◽  
Ibrahim Khider Ibrahim
Keyword(s):  
Prostate Cancer ◽  
Von Willebrand Factor ◽  
Enzyme Linked Immunosorbent Assay ◽  
Sectional Study ◽  
High Concentration ◽  
Cross Sectional ◽  
Von Willebrand Factor Antigen ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Factor Antigen

Backgrounds: VonWillebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders arise due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF)..High plasma vWF concentrations have been reported in patients with various types of cancer, such as prostatic cancer.Metastasization may be associated with activation of haemostatic processes resulting in increased levels of circulating factor VIII-related antigen (FVIIIRAg) (von Willebrand factor antigen). Objective: To evaluate the status of VWF AginSudanese patients with Prostate Cancer attending RICK Methodology: This is a cross-sectional study carried out in Khartoum state at Khartoum oncology (RICK) hospital, during the period from April to June 2018, 45 samples were collected from patients with non-metastatic CA prostate, their ages ranged between 51 to 82 years. The vWF level was measured using Enzyme-linked immunosorbent assay (ELISA). Data were analyzed by the statistical package for social science (SPSS).   Results: Serology for vWF antigen was done for 45 cases of prostate cancer. According to the age, 2(8%) of patients with age 51-66 had a high concentration of VWF while 24(92%) had normal vWF antigen concentration; of those with age 67-82 years, 4(21%) had high vWF antigen and 15(79%) had normal antigen. Conclusion: The study revealedthat more than 80% of Sudanese patients withnon-metastatic prostate cancer have anormal concentration of VWF. Keywords: vWF, Prostate cancer, Age, ELISA

Sign in / Sign up

Export Citation Format

Share Document