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Biomedical subjects

J Schifferli

Publications and source records attributed to J Schifferli.

At least 19 recordsLinked to original sources

Interferon-alpha2a effects on complement activation and regulation in MS patients.

OBJECTIVES: To evaluate the treatment effect of recombinant interferon-alpha2a (rIFN-alpha2a) on complement activation and regulation in MS patients. MATERIAL AND METHODS: Plasma levels of the complement activation products C3bc and terminal complement complex (TCC) and serum levels of the complement regulatory proteins, complement receptor 1, CR1 (CD35) and the membrane inhibitor of reactive lysis, protectin (CD59), were determined by enzyme-linked immunosorbent assay (ELISA) in MS patients treated with IFN-alpha2a (14 patients) or placebo (7 patients). RESULTS: The level of soluble CD35 decreased while the level of TCC and to a lesser degree C3bc increased in the IFN-alpha2a treated patients during the initial part of the treatment. There was also a concomitant reduction of leukocytes in the same patients. CONCLUSIONS: The results indicate that complement is activated during the initial phase of rIFN-alpha2a treatment. This could partly be due to a concomitant reduction in soluble CD35.

Adult↗

Reference typing report for complement receptor 1 (CR1).

A total of 100 Chinese blood donors (50 from Shen-Zhen and 50 from Taiwan) were studied by the participants in addition to 9 reference samples. A new nomenclature for the CR1 structural alleles was recommended by the participants which would use a numbering system, e.g. CR1*1. The structural allele frequencies in the Chinese were: CR1*1 (190 kD) 0.96, CR1*2 (220 kD) 0.03, CR1*3 (160 kD) 0.01 and CR1*4 (250 kD) 0.00. The HindIII expression polymorphism was also studied and the high expressing allele had a gene frequency of 0.71 while the low expressor gene frequency was 0.28. Erythrocyte copy numbers were quantified and compared between laboratories with good correlation (R = 0.55-0.88). The mean (+/- SD) erythrocyte copy number was 463 (+/- 229) in the Taiwan donors and 446 (+/- 207) in the Mainland Chinese.

Alleles↗

Soluble complement receptor type 1 in serum and cerebrospinal fluid of patients with Guillain-Barré syndrome and multiple sclerosis.

Activation of complement is critically involved in inflammatory reactions in both Guillain-Barré syndrome (GBS) and multiple sclerosis (MS). Soluble human complement receptor 1 (sCR1) blocks complement activation by both classical and alternative pathways. We studied serum and cerebrospinal fluid (CSF) concentrations of sCR1 in 23 patients with GBS, 27 patients with MS and 30 controls. No significant differences were found between patients and controls. Transient liver affection probably caused high serum sCR1 levels in two patients with GBS. The serum and CSF sCR1 levels were not correlated to the disease activity of GBS and MS, nor to the relapsing-remitting or chronic-progressive forms of MS. In GBS the CSF sCR1 levels correlated with the CSF total protein concentrations (r = 0.9, P < 0.01), suggesting that sCR1 leaks from serum into CSF via a damaged blood-nerve barrier. The serum sCR1 levels in GBS were slightly higher than in MS (P < 0.05). Whether this reflects changes in the release or consumption of sCR1 in these patients is at present unknown.

Adult↗

Takayasu arteritis preceded by cardiac and cutaneous lesions. A case report.

A diagnosis of aortic insufficiency and mitral stenosis was made in a 24-year-old woman after an episode of heart failure following delivery. A double valvular replacement was performed 4 years later. At that time she suffered from recurrent episodes of erythema nodosum like lesions with an histological diagnosis of cutaneous polyarteritis nodosa. After another 4-year interval she presented with severe arterial hypertension and a biological inflammatory syndrome. An arteriography disclosed stenoses of the abdominal aorta, renal, and iliac arteries as well as occlusion of the right subclavian artery, diagnostic of Takayasu arteritis. We postulate that this woman presented a rare combination of cardiac and skin involvement of Takayasu's disease which preceded the classical arteritis of the aortic branches by 8 and 4 years, respectively.

Adult↗

Effects of dialysate composition during hemodialysis on left ventricular function.

To determine the effects of dialysate composition during hemodialysis on left ventricular systolic and diastolic function, 12 patients treated by chronic hemodialysis (mean age 40.8 +/- 2.7 years), without overt heart disease, were studied by Doppler-echocardiography successively before and after acetate hemodialysis (AHD), bicarbonate hemodialysis (BHD), and acetate-free biofiltration (AFB). The three types of hemodialysis resulted in a comparable decrease of the body weight. Mean arterial blood pressure decreased by 5 mm Hg (NS), 8 mm Hg (NS) and 10 mm Hg (P < 0.05) during AHD, BHD and AFB, respectively. There was a significant increase of the heart rate and the shortening fraction of the left ventricular diameter after AHD, but not after BHD and AFB. Mean total systemic resistance increased by 20% after AHD, 18% after BHD and by 7% after AFB (all changes NS). During each type of hemodialysis there was a significant reduction of the peak velocity of the early diastolic rapid filling wave (peak E) without change of the peak filling velocity during atrial contraction (peak A). During AHD and BHD the pressure half-time of the early filling phase (TP/2) increased, and the velocity-integral of the early diastolic filling phase (E-area) decreased. However, TP/2 and E-area did not change significantly after AFB.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetates↗

High-dose intravenous IgG treatment and renal function.

In an open trial of high dose intravenous IgG (IVIG) treatment in nephrotic patients with glomerulonephritis, the first six patients so far studied showed a transient rise in plasma creatinine. This increase was not associated with any symptoms and the urinary deposit remained unchanged. Two other patients with pre-existing renal impairment but without nephrotic syndrome had a transient and reversible rise in plasma creatinine immediately after IVIG. These observations suggest that high-dose IVIG infusion can produce short-lived disturbances in renal function in patients with kidney diseases.

Adult↗

Anti-interleukin-1 alpha autoantibodies in humans: characterization, isotype distribution, and receptor-binding inhibition--higher frequency in Schnitzler's syndrome (urticaria and macroglobulinemia).

Since autoantibodies (Abs) to cytokines may modify their biologic activities, high-affinity binding factors for interleukin-1 alpha (IL-1 alpha BF) were characterized in human sera. IL-1 alpha BF was identified as IgG (1) by sucrose density-gradient centrifugation followed by immunodiffusion autoradiography, (2) by ligand-blotting method, (3) by ligand binding to affinity-immobilized serum IgG, and (4) by IgG affinity purification followed by sucrose density-gradient centrifugation. IL-1 alpha binding activity resided in the F(ab)2 fragment. The apparent equilibrium constant was in the range of IgG found after immunization with conventional antigens (i.e., 10(-9) to 10(-10) mol/L). Anti-IL-1 alpha IgG auto-Abs represented only an extremely small fraction of total IgG (less than 1/10(-5)). Some sera with IL-1 alpha BF and purified IgG thereof were able to inhibit by 96% to 98% the binding of human recombinant IL-1 alpha to its receptor on murine thymoma EL4-6.1 cells, whereas other sera did not. When 125I-labeled anti-IL-1 alpha IgG complexes were injected into rats, they prolonged the plasma half-life of 125I-labeled IL-1 alpha several fold and altered its tissue distribution. The predominant class was IgG (12/19), mainly IgG4 (9/19), but in five of the sera, anti-IL-1 alpha IgA was also detected. In a screening of 271 sera, IL-1 alpha BF was detected in 17/98 normal subjects and was not more frequent in several control groups of patients, except in patients with Schnitzler's syndrome (fever, chronic urticaria, bone pain, and monoclonal IgM paraprotein) (6/9; p less than 0.005). The pathologic significance of these auto-Abs remains to be determined.

Aged↗

Complement consumption in a patient with necrobiotic xanthogranuloma and paraproteinemia.

Necrobiotic xanthogranuloma and paraproteinemia have been reported to be associated with acquired deficiency of the early components of the classic pathway of complement. We describe a patient in whom activation of complement was suggested by a high level of C3d, an accelerated catabolism of C1q, and a rapid consumption of C4 after an intravenous infusion of fresh-frozen plasma. This infusion was followed by an episode of pulmonary edema. Patients with complement deficiency or depletion should be monitored closely while receiving plasma, to avoid this potential complication.

Complement C1q↗

Comparison of two hemodialysis membranes, polyacrylonitrile and cellulose acetate, on complement and coagulation systems.

Two hemodialysis membranes, polyacrilonitrile (AN 69) and cellulose acetate (CA), were compared for their effects on complement and hemostasis. Two groups of 5 patients, in dialysis for more than 5 years, were successively dialysed for 4 weeks periods with each type of membrane. We measured C3a (complement activation), platelets and beta-thromboglobulin (platelet activation), thrombin-antithrombin III complexes and fibrinopeptide A (coagulation activation), using C-Reactive Protein as a control for dilution effects. As previously shown, activation of complement was more important with CA than with AN 69 (p less than 0.01). In contrast, activation of coagulation (increase in fibrinopeptide A and thrombin-antithrombin III complexes) was more pronounced with AN 69 than with CA. This study emphasizes the need to consider different biological systems when the bioincompatibility of a hemodialysis membrane is evaluated.

Acrylic Resins↗

[Acquired hemophilia. Apropos of a case report with a fatal course].

We report the case of an 86-year-old female with an unremarkable past history who suddenly developed severe and generalized bleeding. Laboratory work-up revealed prolonged aPTT, a factor VIII level of 7% and the presence of a factor VIII inhibitor. In spite of whole blood, factor VIII concentrates and immunoglobulin administration, the patient died of multiple hemorrhages on the third day after admission. At autopsy no causal or associated pathology could be found. Chromatographic separation of plasma immunoglobulins showed that the anti-factor VIII activity was associated with the patient's IgG. This inhibition was partially corrected in vitro by injectable immunoglobulin preparation. The case report stresses the discrepancy between the measured levels of inhibitor and the severity of the bleeding; our in vitro results may shed light on the mode of action of intravenous immunoglobulins.

Aged↗

Dysfunctional C8 beta chain in patients with C8 deficiency.

Two sera from unrelated individuals, each lacking C8 activity, were examined by Western blot analysis. Using antisera raised against whole C8, the two sera are shown to lack the C8 beta chain, indicating a C8 beta deficiency, which is frequently observed in cases of dysfunctional C8. In contrast, by means of a specific anti-C8-beta antiserum, a C8 beta-like polypeptide chain of apparently identical molecular weight compared to normal C8 beta was detected. Digestion of normal and dysfunctional C8 beta with Staphylococcus aureus V8 protease revealed distinct differences in the enzymatic digestion pattern. We conclude that the dysfunction in the C8 protein in these two patients resides in the dysfunctional C8 beta chain, and that this form of C8 deficiency is distinct from C8 deficiencies previously reported, in which one or both C8 subunits are lacking.

Adult↗

[Invasive pulmonary aspergillosis associated with an acute influenza virus infection].

We report a fatal case of invasive pulmonary aspergilloma (API) in association with an influenza A virus infection, occurring in a patient without any factor generally associated with a lowering of immunocompetence. There are five cases in the literature associating these two infections: the immunosuppressive effect of the influenza virus is possibly responsible for the mycotic invasive mass often encountered in the immunosuppressed.

Acute Disease↗