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61.
SUMMARY A patient with polycythaemia rubra vera is described, presenting primarily with symptoms attributed to depression. While in the ward she developed a stroke, confirmed on CT scan of the head. Venesection alone produced marked improvement in her neurological and psychiatric symptoms. The clinical picture corresponds most accurately to the neurological syndrome of abulia, which is an important differential diagnosis of depression with psychomotor retardation. The pathophysiology of impaired cerebral blood flow causing neuropsychiatrie symptoms in polycythaemia is discussed. 相似文献
62.
Mapping of monoclonal antibodies to human factor IX 总被引:2,自引:1,他引:2
Frazier D; Smith KJ; Cheung WF; Ware J; Lin SW; Thompson AR; Reisner H; Bajaj SP; Stafford DW 《Blood》1989,74(3):971-977
We used recombinant DNA techniques to map a panel of six monoclonal antibodies (MoAbs) to regions of the human factor IX molecule. A-2 maps to 17 amino acids at the amino terminus of the heavy chain of IXa; 2D5, an inhibitor of clotting, is defined to 36 amino acids of the first EGF- like domain of human factor IX. A-4, A-5, C10D, and FXC008 all map to a region of the heavy chain containing amino acids 180 through 310, suggesting an immunodominant site. FXC008 has been reported to interfere with binding of factor IXa to factor VIII:Ca. 相似文献
63.
Lems WF; Jahangier ZN; Raymakers JA; Jacobs JW; Bijlsma JW 《Rheumatology (Oxford, England)》1997,36(2):220-224
64.
65.
Evidence that several high-frequency human blood group antigens reside on phosphatidylinositol-linked erythrocyte membrane proteins 总被引:1,自引:2,他引:1
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder associated with absence of expression of phosphatidylinositol (PI)- linked membrane proteins from circulating hematopoietic cells of multiple lineages. Recent work demonstrated that decay accelerating factor, one such PI-linked protein, bears the Cromer-related blood group antigens. This study demonstrated that other high incidence antigens, including Cartwright (Yta/Ytb), Holley-Gregory (Hy/Gya), John Milton Hagen (JMH), and Dombrock (Doa/Dob), are absent from the complement-sensitive (PNH III) erythrocytes of patients with PNH. The relatively normal, complement-insensitive erythrocytes from the same patients express these antigens normally. Therefore, these antigens most likely reside on PI-linked proteins absent from PNH III, but not PNH I, erythrocytes. 相似文献
66.
67.
Complement-induced vesiculation and exposure of membrane prothrombinase sites in platelets of paroxysmal nocturnal hemoglobinuria 总被引:4,自引:4,他引:4
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired stem-cell disorder in which the glycolipid-anchored membrane proteins, including the cell-surface complement inhibitors, CD55 and CD59, are partially or completely deleted from the plasma membranes of mature blood cells. To gain insight into the pathogenesis of thrombosis that is frequently observed in this disorder, the procoagulant responses of PNH platelets exposed to the human terminal complement proteins C5b-9 were investigated. C5b-9 complexes were assembled on gel-filtered platelets by incubation with purified C5b6, C7, C9, and limiting amounts of C8. Platelet microparticle formation and exposure of plasma membrane- binding sites for coagulation factor Va were then analyzed by flow cytometry. PNH platelets exhibiting undetectable levels of surface CD59 antigen showed an approximately 10-fold increase in sensitivity to C5b- 9-stimulated expression of membrane-binding sites for factor Va when compared with platelets from normal controls. Expression of catalytic surface for the prothrombinase complex (VaXa) paralleled the exposure of factor Va-binding sites; the rate of prothrombin conversion by C5b-9- treated PNH platelets exceeded that of C5b-9-treated normal controls by approximately 10-fold at the maximal input of C8 tested (500 ng/mL). These data indicate that PNH platelets deficient in plasma membrane CD59 antigen are exquisitely sensitive to C5b-9-induced expression of prothrombinase activity, and suggest that the tendency toward thrombosis in these patients may be due, at least in part, to the deletion of this complement inhibitor from the platelet plasma membrane. 相似文献
68.
We used both radiolabeled and fluorescein-labeled antiglobulins in assays to detect antibodies against platelets in multiply transfused patients to determine the value of these tests in predicting the outcome of platelet transfusion in such patients. In 15 allosensitized patients, we studied 68 single-donor platelet transfusions, 43 (63%) of which had a poor outcome, defined as a corrected count increment (CCI), less than 10,000. The results obtained with either test were significantly correlated with the CCI following transfusion (p less than 0.001), but the assay using the radiolabeled antiglobulin had slightly better sensitivity, specificity, and predictive value. When the assays were used in combination, there was again significant correlation with the CCI of the transfusion, p less than 0.001. When both assays predicted failure of the transfusions, 31/31 (100%) such transfusions resulted in a CCI of less than 10,000, and when both assays predicted success of the transfusions, 14/15 (93%) such transfusions resulted in a CCI of greater than 10,000. Both assays are useful in predicting the outcome of the platelet transfusions; when the assay results were concordant, almost total predictive accuracy was obtained. 相似文献
69.
70.
Effect of low-dose prednisone (with calcium and calcitriol supplementation) on calcium and bone metabolism in healthy volunteers 总被引:2,自引:0,他引:2
Lems WF; Van Veen GJ; Gerrits MI; Jacobs JW; Houben HH; Van Rijn HJ; Bijlsma JW 《Rheumatology (Oxford, England)》1998,37(1):27-33
The administration of moderate to high doses of corticosteroids is
associated with bone loss. This probably results from the uncoupling of
bone formation (decreased) and bone resorption (unchanged or increased). We
examined the effect of low-dose (10 mg/day) prednisone (LDP) and the
possible mitigating effects of calcium and 1.25 (OH)2 vitamin D
(calcitriol) on calcium and bone metabolism in eight healthy, young male
volunteers. The study consisted of four observation periods: in the first
period, LDP was prescribed during 1 week; in the second, third and fourth
periods, calcium (500 mg/day), calcitriol (0.5 micrograms b.i.d.) and
calcium in combination with calcitriol, respectively, were added to LDP.
Bone formation was measured by means of serum osteocalcin, carboxy-terminal
propeptide of type 1 procollagen (P1CP) and alkaline phosphatase, bone
resorption by means of urinary excretion of calcium, hydroxyproline, (free
and total) pyridinoline, (free and total) deoxypyridinoline and serum
carboxy-terminal cross- linked telopeptide of type 1 collagen (1CTP).
Dietary calcium and sodium intake were maintained at a stable level during
the entire study period. Treatment with LDP led to a decrease in
osteocalcin, P1CP and alkaline phosphatase (all P < 0.01). Urinary
excretion of pyridinolines, hydroxyproline and serum 1CTP did not increase,
but remained unchanged or slightly reduced (P < 0.05), depending on the
time of measurement and the marker of bone resorption. Parathyroid hormone
(PTH) (insignificantly) increased during LDP (+19%) and LDP plus calcium
(+14%), but decreased during supplementation with calcitriol (-16%) and
calcium/calcitriol (-44%; P < 0.01). Urinary excretion of calcium
increased during treatment with LDP and calcitriol (P < 0.05) and
calcium/calcitriol (P < 0.05). It is concluded that LDP has a negative
effect on bone metabolism, since bone formation decreased while bone
resorption remained unchanged or decreased slightly. The increase in PTH
during LDP could be prevented by calcitriol combined with calcium
supplementation.
相似文献