Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0009443 (cold)
 92,137 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired deficiency in C1 esterase inhibitor (C1 INH) was first described by Caldwell in 1972. Since that date, about 30 cases have been reported, in most cases during proliferative lymphocyte B syndromes. The acquired C1 INH deficiency can provoke episodes of angioneurotic edema as in hereditary AE. The complement profile differs, notably by the usual sudden fall in C1. Documented data suggest consumption of complement and therefore of C1 INH. The present case is the first reported of an acquired C1 INH deficiency during a cold hemagglutinin disease. The activation of complement by the classical pathway appears provoked by tumoral cells rather than a humoral factor, as suggested by the efficacy of anti-tumoral therapy in contrast to plasmapheresis in the present case. A possible mechanism for the C1 INH deficiency is discussed.
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PMID:[The course of acquired C 1 esterase inhibitor deficiency in lymphoproliferative syndrome]. 307 95

During the last few years, the structure and function of human C1-inhibitor have been elucidated. Chromogenic substrate assays for determination of C1-inhibitor activity in plasma are available, and have proved to be of value not only for the diagnosis of hereditary angioedema but also in acquired diseases involving C1-inhibitor, such as cold urticaria and autoimmune disorders as well as acute-phase types of disease states.
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PMID:Aspects of C1-inhibitor biochemistry and pathophysiology. 332 41

Since a protease inhibitor or anaphylatoxin inactivator deficiency might explain why certain individuals are prone to develop chronic urticaria/angioedema or anaphylactoid reactions to radiographic contrast media, serum alpha 1-protease inhibitor, alpha 1-antichymotrypsin, alpha 2-macroglobulin, inter-alpha-inhibitor, antithrombin III, alpha 2-plasmin inhibitor, C1 inhibitor, and serum carboxypeptidase N were assessed by immunologic or functional methods. These values all were within normal limits in both groups of patients except for a low mean alpha 1-protease inhibitor level in chronic idiopathic urticaria/angioedema and cold urticaria patients and marginal decreases of alpha 1-protease and inter-alpha-inhibitor levels in radiographic contrast medium reactors. However, these abnormalities were not thought to be of pathogenetic significance.
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PMID:Plasma protease inhibitor and anaphylatoxin inactivator levels in chronic urticaria/angioedema and in patients experiencing anaphylactoid reactions to radiographic contrast media. 394 18

Coagulation contact factors were studied in 9 patients with hereditary angioneurotic oedema. After storage of the plasma at O degrees C for 20 hours, activation of prekallikrein and factor VII, together with prekallikrein consumption, were observed. The cold activation, due to deficiency of C1 esterase inhibitor, was not suppressed by polybren in vitro (factor XII activation inhibitor) but was prevented by aprotinins (kallikrein inhibitors). These findings would suggest that patients with hereditary angioneurotic oedema should be investigated for prekallikrein consumption and should be treated with aprotinin as soon as the attack begins.
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PMID:[A study of coagulation contact factors in hereditary angioneurotic oedema (author's transl)]. 616 18

Acid-pretreated normal human plasma generates renin activity at 0 degree C and neutral pH by the activation of prorenin. The activation is caused by kallikrein generated from prekallikrein by activated factor XII. Nonacidified plasma also generates renin at 0 degree C, but at a lower rate (cold-promoted activation). In normal plasma, 14% +/- 1% of prorenin (mean +/- SEM, n = 30) was activated during incubation at 0 degree C for 7 days (range 6% to 26%). Cold-promoted activation of prorenin was within the normal range in plasma deficient in factor XI, X, IX, VIIIC, VII, V, prothrombin, or high mol wt kininogen. Cold-promoted activation of prorenin was less than or equal to 1% in plasma deficient in factor XII or prekallikrein. Reconstitution of these plasmas with highly purified factor XII or prekallikrein restored normal prorenin activation. Correction of high mol wt kininogen deficiency had no effect. Thus cold-promoted activation of prorenin depends on the presence of factor XII and prekallikrein, whereas the other clotting factors are not essential. The influence of the inhibitors C1 esterase-inhibitor, alpha 2-macroglobulin, antithrombin III, and alpha 1-antitrypsin on the activation of prorenin was studied in factor XII-deficient plasma from which one or more of these inhibitors had been selectively removed by immunoadsorption. Factor XII was subsequently added, and the generation of renin at 37 degrees C was observed after complete factor XII-high mol wt kininogen-mediated activation of prekallikrein induced by dextran sulfate. No activation of prorenin was observed at 37 degrees C after depletion of C1 esterase inhibitor, alpha 2-macroglobulin, antithrombin III, or alpha 1-antitrypsin. When prekallikrein was activated in plasma depleted of both C1 esterase-inhibitor and alpha 2-macroglobulin, 6% of prorenin was activated in 2 hours at 37 degrees C. After additional depletion of antithrombin III, the activation increased to 47%. These results indicate that the contact activation system is capable of activating prorenin in plasma at physiologic pH and temperature when the three most important kallikrein inhibitors, C1 esterase-inhibitor, alpha 2-macroglobulin, and antithrombin III, are absent.
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PMID:Prorenin-renin conversion by the contact activation system in human plasma: role of plasma protease inhibitors. 636 96

Plasma levels of six protease inhibitors have been measured in patients with chronic urticaria, chronic urticaria with angio-oedema, cold and cholinergic urticaria. In chronic urticaria C1 esterase inhibitor activity was increased compared with a reference control population but there was no detectable abnormality of any other protease inhibitor. Patients with chronic urticaria/angio-oedema showed a reduction in inter-alpha trypsin inhibitor. They also manifested a rise in C1 esterase inhibitor. In cold urticaria there was a significant lowering of alpha 1 antichymotrypsin. The reduction in alpha 1 antitrypsin in this group probably reflects a genetic difference compared with the control population. Patients with cholinergic urticaria also showed a reduction of alpha 1 antichymotrypsin. The elevated levels of alpha 2 macroglobulin in the three groups are probably due to differences in the mean age of these groups compared with the reference population. Comparison of levels of subgroups of patients with and without active lesions suggest that a consumptive effect may contribute to the reduced values, although it seems unlikely to account for them entirely. The results suggest that involvement of pharmacologically active products of protein digestion may be involved in the pathogenesis of urticaria and should prompt attempts to identify these agents and encourage trial of medications which lead to inhibition of proteolytic activity in urticaria.
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PMID:Protease inhibitor profiles in urticaria and angio-oedema. 696 79

In hereditary angioedema (HAE), normal C1-inhibitor (C1-INH) is low and the contact system activated. Recently, the findings of a tissue factor mutant selectively deficient in promoting the conversion of FVII to FVIIa, but with retained cofactor for FVIIa, made it possible to examine reliably the pre-existing content of FVIIa in HAE patients. This was of interest as FXIIa (mainly inhibited by C1-INH) is able to activate FVII directly. FVIIa in 21 remission HAE patients were within normal limits but nearly doubled as compared to their 23 normal siblings (p = 0.0017). Cold promoted activation of FVII (CPA) (common clot assay) was displayed in plasma of all 5 untreated patients (C1-INH function < 35%), but not in plasma of 2 patients treated prophylactically with danazol (C1-INH function about 40%). These results suggest that there is a minute, yet significant activation of FVII in patients with C1-INH deficiency.
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PMID:Factor VIIa in patients with C1-inhibitor deficiency. 856 Apr 20

Excessive activation of the protein cascade systems has been associated with post-transplantation inflammatory disorders. There is increasing evidence that complement not only significantly contributes to ischemia/reperfusion injury upon cold storage of the organ but also, although to a different degree, to allograft rejection. Complement activation is most fulminant in hyperacute rejection but seems also to contribute to acute transplant rejection. Therapeutic substitution of appropriate regulators, therefore, appears to be a reasonable approach to reduce undesirable inflammatory reactions in the grafted organ. C1-inhibitor, a multifunctional regulator of the various kinin-generating cascade systems (for review see: E. Hack, chapter in this issue), is frequently reduced in patients suffering from severe inflammatory disorders. Studies applying pathophysiologically relevant animal models of allo- and xenotransplantation as well as promising first clinical results from successful allotransplantation now provide evidence that C1-inhibitor may also serve as an effective means to protect the grafted organ against inflammatory tissue injury. In xenotransplantation, complement inhibition by specific regulators such as C1-inhibitor may help to overcome hyperacute graft rejection. After a brief introduction on the significance of complement to allo- and xenotransplantation the following review will focus on the impact of C1-inhibitor treatment on transplantation-associated inflammatory disorders, where complement contributes to the pathogenesis.
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PMID:C1-inhibitor and transplantation. 1239 13

The use of plasma in Sweden is relatively high compared to other countries in the European Union. An analysis of all transfusion recipients in Orebro county during the whole year 2000 was performed. There were 3159 transfusion recipients of whom 96% had a registered diagnosis and 50% had undergone a "true" operation. Seven hundred and eleven patients (23%) had received plasma. Significantly more operated than nonoperated and more men than women received plasma. The typical plasma recipient was a man undergoing cardiovascular surgery. In Sweden there are two main types of plasma components: fresh frozen (FFP) and nonfrozen liquid plasma stored for up to 14 days, both considered to be clinically equal for most indications. The quality of these components as well as stored thawed FFP has been studied. The major storage effect was cold-induced contact activation and thereby consumption of C1 esterase inhibitor (C1INH) by day 14 in 22%. The citrate content in plasma sustained the overall coagulation function over 14 days. Other studies have shown that the levels of FV and ADAMTS 13 after 14 days remain at 70% or more compared to those for FFP. Since it is immediately available, liquid, nonfrozen or thawed, plasma is of great value in emergencies. Quality criteria for plasma components need to be assessed against evidence based indications and published in guidelines.
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PMID:Use of plasma: clinical indications and types of plasma components in Sweden. 1840 74

Alternanthera tenella Colla extracts are used in Brazilian traditional folk medicine to treat a variety of infectious diseases as well as inflammation and fever. In this work, the immunomodulatory, anti-inflammatory and potential toxic effects of cold (CAE) and hot (HAE) aqueous extracts of A. tenella were investigated in vivo. In addition, we analyzed the phytochemical properties of both extracts. BALB/c mice were immunized in vivo with sheep red blood cells and concomitantly inoculated intraperitoneally (i.p.) with each extract (50, 100 or 200 mg/kg). Specific antibody-producing cells were enumerated using plaque-forming cell assays (PFC) and anti-SRBC IgG and IgM serum levels were measured via enzyme-linked immunosorbent assay. Body and lymphoid organ weights were determined after treatments in order to evaluate toxic effects. Carrageenan-induced paw edema was employed to investigate anti-inflammatory activity in mice inoculated i.p. with CAE or HAE (200 or 400 mg/kg). Phytochemical screening was performed using spectrometric and chromatographic approaches and revealed that CAE possessed higher tannin and flavonoid levels than HAE. PFC numbers were increased after treatment with CAE (100 mg/kg) four days after immunization, as were the serum antibody titers after four and seven days, suggesting immunostimulatory activity through modulation of B lymphocyte functions. Body and organ weights did not show major changes, suggesting that extracts administered to mice did not induce significant toxicity. Both extracts had significant anti-inflammatory activity in the paw edema assay. These results suggested that aqueous extracts from A. tenella contained several chemical compounds that possess positive and/or negative modulator effects on the immune system, which appeared to correlate with tannin and flavonoid levels in those extracts. In summary, these studies provide important insight into the biological activities of A. tenella.
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PMID:Evaluation of immunomodulatory and anti-inflammatory effects and phytochemical screening of Alternanthera tenella Colla (Amaranthaceae) aqueous extracts. 1894 27


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