bluuebunny/crossref_metadata_2025_split huggingface dataset:
Personality Characteristics of Adult Hemophiliacs
Authors: Charney D.S. , Caldwell A. , Ackerman L.L. , Strickler E. 
Published: May 2019

The psychological characteristics of 36 adult hemophiliacs, mean age 29.1 years, were analyzed by means of the Minnesota Multiphasic Personality Inventory (MMPI).The MMPI profile patterns were compared between various groups of the hemophiliac sample. By the clinical criterion used in our clinic, the sample was divided into severe and mild-moderate groups. The mild-moderate group appeared to be mildly anxious-hysterical, frightened, and health preoccupied with a profile of '317-2 9 5684 0/. The severe group appeared to be more estranged and alienated than the mild-moderate group, and has a significantly higher psychopathic deviate scale (P<.01). The mean profile of the severe group was '458 23-9167 0/.The most striking differences were found in comparing subgroups of high (N=9) vs low bleeders (N=9) within the group of severe hemophiliacs with factor levels of 3% or less. The high bleeders (>243 units/lb/year, mean Factor VIII level 1.6%) were much more disturbed, had all mean clinical scales above 60 (8'25137496-0/) and significantly higher scores for Depression, Psychasthenia, and Schizophrenia (P <.05). The low bleeders (< 243 units/lb/year, mean Factor VIII level 1.8%) had no mean scale scores above 60 ('34519687 2/0).The results indicated a clear difference in the psychological adjustment of the high and low bleeders to their illness. The high bleeders had a marked increase in self negativity i.e. a sense of being defective, of hopelessness and of pessimism. Surprisingly, the low bleeders has adjusted auite well to their serious vulnerability.
Hemophilia: A Moral Dilemma
Authors: VanHarlingen David 
Published: May 2019

Hemophilia places stresses of a financial, social and psychological nature on both the victim’s family and the community. Decisions about having children, or paying for rehabilitative or preventive therapy with public funds, involve the resolution of value conflicts at a personal and at a societal level.Moral/ethical dilemmas based on these and similar questions were constructed and administered to a group of non-hemophiliac classroom teachers. Our work indicates that these dilemmas are effective in presenting content knowledge about hemophilia problems and that they are able to increase one’s level of moral reasoning. This increases the probability that all possible consequences of an action will be carefully considered, although it does not lead automatically to a predetermined resolution of the dilemma.The applicability of these dilemmas for use in genetic counseling of hemophilia families or to develop knowledge among parents and friends of new hemophiliacs will be explored.
Analysis of Bleeding Frequency in Hemophilia.
Authors: Lovrien E.W. , Underwood S. , Hammerling J. , Rivas M. , Elder J. 
Published: May 2019

Patients receiving AHF by home infusion therapy completed a form at the time of each infusion which allowed an accurate appraisal of bleeding frequency by site. A simple data management system was used to analyze the pattern of bleeding in 86 patients who averaged 28 months on the program with an age range from 2 to 52 years. The mean age was 17.1 years. The number of bleeds divided by the number of months = bleed per month index (BMI). The composite BMI for all 86 patients = 4.765 with a total of 11,504 recorded bleeds. 20% occurred in the head and neck, chest and abdomen = 16.66%, buttock and G.U. = 11.65%, upper extremity = 22.02%, lower extremity = 26.37% and miscellaneous = 3.30%. Extremities accounted for 48.39%. The most common bleeding sight was the abdomen 14.7166%, 2nd = nose 12.03%, 3rd = R lower leg = 10.68%. In the upper extremity, 15.76% were right sided compared to 6.25% left; lower = 23.34% right, 3.03% left. The knees accounted for only 1.113% of all the bleeds. The highest BMI on a patient = 12.4 avg. bleeds per month over 4 years.The patients were divided into age categories of 3 years. The highest BMI for R. lower leg was age 6-9; R. knee age 12-15; abdomen 15-18, but high in all age ranges. Urinary bleeding was highest in early childhood in 0-3, 3-6, 6-9, and 9-12 year olds. By careful observation of the BMI, the management of early bleeding to prevent deformity should be enhanced. A high BMI may be used to guide prophylactic infusion. This study has demonstrated that a simple data processing system is useful in directing infusion therapy to minimize deformity.
Activated Prothrombin Concentrates in Management of Bleeding in a Classical Hemophiliac with Inhibitor
Authors: Whaun J.M. , Kaegi A. 
Published: May 2019

Bleeding in hemophiliacs with circulating anticoagulants is still a serious management problem. We would like to report our efforts at achieving hemostasis in a 31 year old AHF deficient severe hemophiliac with inhibitor detected in Feb. 1972. He presented with right forearm flexor compartment bleed which did not respond to conservative management with Konyne (30 u/kg q3h) and necessitated a fasciotomy to relieve compression. No hemostasis occurred until 4 hrs. post-operatively when he received his first infusion of Auto-Factor IX*, 6 vials (60 u/kg). At this time he also received blood for his shocky state. With the institution of regular infusions of Auto-Factor IX alternating with Konyne, hemorrhage was controlled. A week later a split thickness skin graft (from R. thigh) was applied. With continued infusions of mainly Auto-Factor IX (60 u/kg q6h) the grafted and donor sites healed. The patient was subsequently discharged with full range of movement in all limbs. His inhibitor levels which ranged between 1 - 3 Bethesda units/ml shortly after admission over a period of 3 weeks rose to over 100 units/ml. Six months after the episode his inhibitor levels are still over 100 units/ml. Activated prothrombin concentrates are effective in hemostatic control of life-threatening bleeding in hemophiliac patients with inhibitors.* Kindly supplied by Dr. Aaron Josephson, Travenol Laboratories, Chicago, 111. (Hyland Division).
Carrier Detection in Hemophilia B.
Authors: Briët E. , Bertina R.M. , Tilburg N.H. van , Veltkamp J.J. 
Published: May 2019

We examined 37 obligatory carriers of hemophilia B and 40 normal women. The levels of both factor IX activity and factor IX antigen were determined. The factor IX antigen levels were assayed in an inhibitor neutralization test in which a rabbit antiserum was used. 12 out of the 37 carriers had oral contraceptives and so did 20 out of the 40 normals.Higher levels of both factor IX activity and factor IX antigen were found in the women, carriers as well as normals, using oral contraceptives. In carrier detection programs, therefore, a distinction should be made between women using the pill and those wo do not. The attempt to discriminate between carriers and normals on basis of factor IX activity and antigen levels proved to be as unsatisfactory as it was on the basis of factor IX activity levels only. The overlapping area of normals and carriers was smaller in the group of women on oral contraceptive medication. On the basis of our findings we expect that we can detect with a 90% confidence 17 carriers and 17 normals in a group of 100 potential carriers with an a priori chance on carriership of 50%. The detection rate is somewhat higher for women using oral contraception. The assay of factor IX antigen by means of the inhibitor neutralization assay does not appear to improve carrier detection.Results with an immuno-electrophoretic assay of factor IX antigen will be discussed.
Hemophilia in Norway
Authors: Egeberg O. 
Published: May 2019

Studies of all hemophilic families in Norway have resulted in the following distributions as to type and degree of factor defects.Of the hemophilia B families, 3 were of the type BM. In connection with multiple transfusions to patients with severe hemophilia A, 13 patients developed severe abnormal inhibitor to f.VIII, 3 of them died. In several other such transfused hemophilia A patients, weaker coagulation inhibitory activities have been demonstrated.
Fibrinogen, Fibrinogen Heterogeneity and Fibrinolytic Activity in Diabetes Mellitus
Authors: Gurewich Victor , Lipinska Izabella , Pulini Maria , Gordon Edwin , Lipinski Boguslaw 
Published: May 2019

Fibrinogen (F) concentration, fibrinogen heterogeneity on 3.5% Polyacrylamide gels, fibrinolytic activity (FA) measured by euglobulin fraction on fibrin plates and fibrin degradation products (FDP) were measured in 66 patients with well documented diabetes mellitus (DM) and in 50 healthy subjects of comparable age. A high molecular weight and 2 lower molecular weight (LMW and LMW1) fibrinogen fractions were identified. The mean values and statistical evaluation of their differences were as follows:The clinical duration of DM, degree of control or type of medication did not appear to influence these findings. However, within the patient group, those with clinical evidence of microvascular disease had significantly (p<0.02) higher LMW1 fibrinogen and lower FDP (p<0.01) than the remainder. These findings suggest that DM is associated with fibrin deposition, and accelerated F degradation to LMW and LMW1 fractions and that these processes may be associated with the development of vascular lesions.
Biological Properties of an Heparin-Poly (Methyl Methacrylate) Copolymer
Authors: Labarre D. , Boffa G.A. , Jozefowicz M. , Boffa M.C. 
Published: May 2019

The heparin-poly(methyl methacrylate) copolymer was prepared by polymerization of methyl methacrylate on an heparin radical. It was initiated by cerium IV ions and heparin in nitric aqueous solution.An heparin effect was present in the plasma in contact with the copolymer. It was not due to a release of heparin in the plasma, but to a contact effect. The anticoagulant effect was depending upon the concentration of the copolymer. The activity of clotting factors in plasma, after a contact with the copolymer was not decreased except for factor V. Antithrombin III was selectively adsorbed on the surface of the copolymer.The copolymer lost progressively its anticoagulant properties after each contact with the plasma. This might be due to the adsorption of fibrinogen on the copolymer. Labelled 125I albumin, transferrin, Ig G and fibrinogen were not similarly desorbed in the presence of serum and of plasma.This copolymer seems to offer interesting properties for blood devices.
Antithrombin III and its Interactions with Heparin
Authors: Rosenberg R.D. , Jordan I.R. , Armand G. , Lam L.H. , Beeler D.L. 
Published: May 2019

Heparin functions as an anticoagulant by binding to antithrombin and accelerating the rate at which this protein inhibitor inactivates the serine proteases of the hemostatic system. Despite considerable effort, the precise relationship between the structure of this mucopolysaccharide and its biologic properties has remained elusive.We have provided the first evidence that only a small portion of a given heparin preparation binds tightly to antithrombin and is responsible for the bulk of anticoagulant activity. The major fraction of these products has very little anticoagulant effect.In our presentation, we shall discuss optimal techniques for fractionating heparin into “highly active” and “relatively inactive” molecular forms.Furthermore, we shall consider the distinctive interactions of these two types of mucopolysaccharides with antithrombin, thrombin and other hemostatis system components.Lastly, we shall provide evidence that several structural differences exist between these two forms of heparin.
Relationship Between Platelet Density and Number of Dense Bodies
Authors: Rendu F. , Lebret M. , Caen J.P. 
Published: May 2019

Dense bodies are the storage site of 5-HT and their number might correlate with the different platelet populations. Mepacrine, a basic fluorescent dye, is a suitable tool for studying abnormalities in platelet 5-HT storage.The content of platelet DB can be readily estimated by examination under fluorescence microscope of platelets charged with mepacrine. In human platelets, a mean number of 6.3 DB/ platelet was found. This estimation has been performed in platelets of different densities separated on sucrose gradient : heavy platelets, in which 5-HT uptake is strongly inhibited by reserpine, had a mean number of 7 DB/platelet and none was empty of DB ; light platelets which are less active in regard to 5-HT and insensitive to reserpine, had less than 3 DB/platelet and most of them had a cytoplasmatic fluorescence. Thus the proportion of light and heavy platelets can be directly established by staining platelets with mepacrine.In view of this, platelets of two patients with an abnormal density distribution were studied for 5-HT and charged with mepacrine. The first presented an Hermansky-Pudlak syndrome (HPS), an increased proportion, of light platelets, a low 5-HT uptake insensitive to reserpine and a great reduction of mepacrine-stained DB. The other patient with a Bernard-Soulier syndrome had only heavy platelets ; 5-HT uptake in these large platelets was markedly increased and all platelets had more than 20 DB stained with mepacrine.These results confirm that there is a good relationship between platelet density and number of dense bodies.
Normalization of Platelet Survival in an Experimental Model after Platelet Exposure of Thrombin in Vitro or adp in Vivo
Authors: Meuleman D.G. , Buchanan M.R. , Giles A.R. , Hirsh J. 
Published: May 2019

It is generally assumed that when platelets are damaged during preparation, their subsequent survival in the circulation is decreased. We have examined the effect of exposure of platelets to stimuli such as thrombin and ADP prior to studying their interaction with either damaged vessel wall or in vivo aggregation by infused collagen. Catheters inserted in the abdominal aorta of rabbits produced marked vascular damage. This was associated with a significant shortening of platelet half life (T½) of homologous 51Cr-labelled platelets (T½-18hrs: Control animals T½-34hrs). In contrast, the platelet half lives of platelets degranulated by thrombin in vitro and those harvested from donors treated with ADP in vivo were 23 and 30 hrs respectively. In vivo collagen-induced platelet aggregation of the thrombin degranulated and the ADP exposed platelets was also found to be significantly diminished when compared with those obtained from untreated control animals.These results suggest that exposure of platelets to either exogenous or endogenous stimuli could possibly mask a reduction in platelet survival in thrombovascular disorders and its modification by drugs.
The Conversion of Bovine Abnormal Prothrombin into Prothrombin
Authors: Vermeer C. , Soute B.A. M. , Hemker H.C. 
Published: May 2019

In order to characterize the enzyme system which converts abnormal prothrombin (PIVKA-II) in prothrombin we prepared liver cell-free extracts from normal healthy cows. The enzyme system devoid of either precursor or prothrombin was isolated by differential centrifugation and partly purified with the aid of column chromatography.The carboxylation of purified PIVKA-II to prothrombin was detected by means of the one-stage coagulation assay and with 14CO2 incorporation. The reaction was dependent upon microsomal supernatant, vitamin K, ATP, Mg++ ions, O2, pH and temperature. Inhibitors were: warfarin, 2.4-dinitrophenol and factor II. From our experiments we concluded that PIVKA-II can act as a precursor of prothrombin and that PIVKA-II can be converted into the normal clotting factor in an in vitro cell-free system. In contrast to the systems published by others, here the converting enzymes are added to separately purified precursor proteins, so the system provides a way to study the nature of this reaction in more detail.
The Production of Heart Attacks and Cerebral Stroke by Thromboxane A2 Mixtures and their Prevention with Eg626 (Phthalazinol)
Authors: Shimamoto T. , Kobayashi M. , Takahashi T. , Takashima Y. , Motomiya T. , Morooka S. , Numano F. 
Published: May 2019

Thromboxane A2 (TXA2) has been implicated as the causative agent in variant angina, myocardial infarction, sudden cardiac death and stroke. In order to verify experimentally such hypothesis, the TXA2-mixture was produced by washed rabbit or human platelets stimulated by thrombin (3U for 1.2 × 109 platelets) and the dose of TXA2 was measured by rabbit aortic strips.The intracoronary (by our aortic balloon technique) or intracarotid injection of TXA2-mixture successfully produced variant angina-like response, fatal or non-fatal myocardial infarction and cerebral infarction resp. in 102 rabbits. The responses were recorded by a movie, polygraphic method including ECG, EEG and histological technique. The effective doses ranged from 1~2 μg of TXA2 in terms of angiotensin II for the intracoronary and 3 to 6 μg for the intracarotid injection resp. The mixture, kept at 37°C for 5 min, lost TXA2 and remained without effect, despite the presence of other vasocontrictors, showing the leading role of TXA2 for the occlusion of arterioles. The variant angina-like response, myocardial infarction and stroke were statistically significantly prevented by pretreatment of rabbits with therapeutic doses (0.1~1.0 mg/kg, i.v.) of EG626, an orally available potent TXA2-antagonistic and cAMPPDE-inhibiting substance, among drugs tested. The occlusion of arterioles by TXA2 by its time-matched vasoconstrictive activities after production of platelets aggregates as the plug was prevented by EG626 by reducing the grade of vasocontriction and platelet aggregation resulting into sliping away of the aggregates and the maintenance of the blood flow. Such facts may show the importance of EG626-type drugs in prevention of heart attacks and stroke as evidenced by its preliminary clinical results.
Inhibition of Platelet Reactivity by Endothelial Cells
Authors: Harker L.A. , Joy N. , Wall R.T. , Quadracci L. , Striker G. 
Published: May 2019

The process of endothelial thromboresistance has been studied by measuring platelet reactivity to cultured human endothelial cells (EC), vascular smooth muscle cells (SMC), and fibroblasts (FB). Human C-serotonin labeled platelets in a concentration of 300,000 cells/ul and washed cultured cells (brought into monocellular suspension by EDTA buffer) were reacted in stirred plasma suspensions at 37°C while assessing light transmission, C-serotonin release and electron microscopy. SMC from adult arteries and veins induced aggregation and release (A-R) in a dose response fashion with maximal C-serotonin release of 62% ± 7 by 106 SMC/ml, whereas comparable reactivity was induced by 3 × 106 /ml umbilical vein SMC and 5 × 10 FB/ml. Trypsinization at 0.05% solution for 2 minutes destroyed the capacity of SMC and FB to induce A-R.EC failed to induce aggregation and release in concentrations as high as 10 × 106 cells/ml. Moreover, the addition of EC to suspensions of SMC or FB in ratios as low as 1:5 prevented platelet A-R. The inhibitory effect of EC was not destroyed by trypsinization. Platelet A-R induced by ADP, epinephrine or collagen were similarly impaired by the addition of EC (106 cells/ml) while control studies using comparable numbers of SMC and FB produced no change. EC grown in the presence of indomethacin failed to inhibit platelet reactivity. ADP, epinephrine and collagen induced A-R were also significantly reduced by supernatant media from EC cultures. Supernatant media from SMC and FB failed to affect A-R. These results indicate that the lack of platelet reactivity to endothelial cells is mediated through endothelial cell synthesis of a soluble platelet prostaglandin inhibitor, probably prostacyclin.
Platelet Survival and Platelet Serotonin Release in vivo in Various Animal Models
Authors: Cargill D.I. , Ryan R.J. 
Published: May 2019

Decreased platelet survival is a clinically accepted indicator of increased platelet consumption, and in some cases may reveal “hyperthrombotic” states. Decreased platelet survival has also been demonstrated in the homocystinuric baboon and the hypercholesterolemic rhesus monkey (Ross and Harker). Treatment with antiplatelet drugs does increase platelet survival in patients as well as in experimental animals.We have found platelet survival to be decreased in both uricemic and hypercholesterolemic guinea pigs.However, platelet survival in the atherosclerotic rabbit and the streptozotocin diabetic rat was normal. When atherosclerotic rabbits or diabetic rats were given platelets labelled with 51Cr and 14C-serotonin, they were found, five days after receiving the platelets, to have higher 51Cr/14C ratios than did their corresponding controls. This suggests a higher serotonin release from circulating platelets in these models.The 51Cr/14C ratio in platelets may be a more sensitive measure of platelet activity in vivo than is platelet survival alone.
Serotonin Release from Filtered and Unfiltered Human Platelets Induced by Antigen/Antibody Complexes of Different Valencies
Authors: Kazatchkine M. , Caen J. , Johnson Anthea H. , Mowbray J.F. 
Published: May 2019

In order to study the nature of receptors for immune complexes (IC) on the surface of platelets, the release of radiolabelled serotonin produced by exposure of platelets to IC of known composition was studied. Free plasma components might alter this reaction, so platelets were used both in platelet rich plasma (PRP) and after gel filtration. As it has been thought that the aggregation of the immunoglobulin molecules through the antigen was neccessary for the release reaction, we have studied complexes made with antigens of different valencies. The antigen was albumen substituted with different numbers of dinitrophenol (DNP) groups, and the antibody rabbit-anti DNP. Polyvalent antigen complexes were also made from bovine serum albumen (BSA) and rabbit anti-BSA antibody. Soluble and insoluble IC made in a variety of ratios with polyvalent antigen induced release both in PRP and in gel filtered platelets. Soluble complexes in antigen excess were the most effective. Complexes of monovalent antigen prepared, using two methods of monovalent substitution, were also capable of inducing release in PRP and in gel filtered platelets, although the complex could not exist in an aggregated form. Release was less than that produced by polyvalent complex, but could be increased by the addition of CI.These results show that, at least with monovalent IC, the simultaneous involvement of more than one receptor is not an absolute requirement for platelet release. These receptors for monovalent complexes could be different from the Fc receptors for aggregated immunoglobulins or polyvalent complexes.
The Passovoy Defect: Further Case Reports and Preliminary Characterization
Authors: Hougie C. , Brown J.E. , Lakin-Thomas P. , McPherson R.A. , Aronson L. , Baugh R.F. 
Published: May 2019

Since the first report of a patient with a deficiency of the Passovoy factor (Lancet 11:220, 1975) we have encountered 10 other cases from six kindred. The patients have a small but significant prolongation of the activated partial thromboplastin time which is corrected by normal plasma. They have normal levels of all the hitherto reported clotting factors, including Fletcher and Fitzgerald, no evidence of an inhibitor, and no mutual correction. The condition is transmitted as an autosomal dominant. Most of the patients have had a moderate to mild bleeding diathesis, thus the proband in the original kindred recently bled profusely into the neck three days following the removal of a small cervical node and was given 6 units of blood to control the bleeding. Attempts to characterize the factor have been hampered by the relative insensitivity of the assay method; however a fraction has been found which shortens the clotting time of Passovoy deficient plasma, but not plasmas deficient in other clotting factors; the biochemical properties of this factor are currently under investigation.This work was supported by USPHS research grant HL 19272.