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Biomedical subjects

S Schulman

Publications and source records attributed to S Schulman.

At least 91 records · Page 5Linked to original sources
PubMed1995

Continuous infusion of factor concentrates: review of use in hemophilia A and demonstration of safety and efficacy in hemophilia B.

Continuous infusions of factor VIII have proved feasible in patients with hemophilia A, and the hemostatic efficacy has sometimes been superior to that of repeated bolus injections of factor VIII. Avoiding dangerously low trough levels by maintaining a constant therapeutic level not only promotes hemostasis but requires substantially less factor concentrate, which represents a considerable saving. Only limited data on continuous infusion of factor IX are available, but these data suggest similar benefits. An investigation of the stability of four prothrombin complex concentrates and three highly purified factor IX concentrates is described, as is the successful treatment of four hemophilia B patients with continuous infusions of Mononine.

Blood Coagulation Factors↗
PubMed1995

Fibrin sealant in surgery of patients with a hemorrhagic diathesis.

The utilization of fibrin sealants in patients with hemostatic disorders has hitherto been very limited. Our experiences from 40 major and 8 minor surgical procedures, 10 circumcisions and 118 tooth extractions in 106 patients with hemophilia A, B or von Willebrand's disease, almost exclusively of the severe form, respectively, demonstrate the benefits regarding reduction of blood loss and requirements for systemic replacement therapy. The literature on the use of fibrin sealants in this group of patients, which mainly concerns dental procedures, is reviewed and compared with our data. The importance of the composition of the glue is also discussed in detail.

Blood Loss, Surgical↗
PubMed1994

Partial expression of GP Ib measured by flow cytometry in two patients with Bernard-Soulier syndrome.

Bernard-Soulier syndrome is a rare, congenital bleeding disorder caused by absent or defective GP Ib platelet membrane receptor for the von Willebrand factor (vWF). We studied two brothers with moderate bleeding symptoms. Bleeding time was prolonged and ristocetin-induced platelet aggregation was absent. Flow cytometric analysis showed that both boys had a subnormal expression of GP Ib. One antibody used (AN51) was bound only to 30% of the platelets and at a subnormal density. A second antibody (SZ2) also bound at a subnormal density but a normal fraction of the platelets were immunoreactive. Ristocetin stimulation of the patients' platelets in the presence of plasma resulted in a low binding of vWF, about 30% of healthy controls. On the other hand the expression of GP IIb/IIIa on the platelet membrane appeared to be supernormal even when the increased platelet size was taken into account as shown by the ratio between the density of GP IIIa and CD 9 structures. We conclude that these brothers have a variant of the Bernard-Soulier syndrome with a low expression of a GP Ib receptor.

Bernard-Soulier Syndrome↗
PubMed1994

Stability of factor VIII concentrates after reconstitution.

Adjusted-dose continuous infusion of factor VIII (F VIII) has recently been shown to reduce the doses of the factor in patients undergoing surgery by 50-75%. The main limitation of this method has been the instability of factor concentrates. All manufacturers are recommending infusion of the concentrate within hours after reconstitution. We studied the stability of 15 different lyophilized F VIII products. Reconstituted samples were stored for periods of 4, 24, and 72 hr and 1, 2, 3, and 4 weeks at temperatures of 4-8 degrees C, 20-23 degrees C, and 37 degrees C in their original glass containers and in plastic tubes and then frozen. Assays were performed in duplicate, using a one-stage clotting method and a chromogenic assay for F VIII, with all samples from a single concentrate in the same run. Activation of the coagulation factor occurred in some concentrates, more often at 4-8 degrees C than at 20-23 degrees C or 37 degrees C. The stability of all products was substantially better than that declared by the manufacturers. Several concentrates maintained factor activities above 80% of baseline for the entire period of 4 weeks at 4-8 degrees C or at 20-23 degrees C. The results demonstrate that many of the F VIII concentrates may be used for continuous infusion.

Drug Packaging↗
PubMed1994

Quality of oral anticoagulant control and treatment in Sweden. Duration of Anticoagulation (DURAC) Trial Study Group.

OBJECTIVES: To define laboratory-dependent and clinical factors that negatively influence the precision and safety of oral anticoagulation and to determine whether this creates differences in clinical outcome between the participating hospitals. DESIGN: Laboratory. The clinical chemistry laboratories of participating hospitals performed prothrombin time tests on blinded, standardized plasma samples on six occasions. Clinical. Patients with a first or second episode of venous thromboembolism were randomized to different durations of oral anticoagulation; the target was an international normalized ratio (INR) of 2.0-2.85. SETTING: Multicentre study at the departments of medicine of 16 Swedish hospitals. SUBJECTS: In total, 1124 patients with venous thromboembolism were followed for 600 patient-years of oral anticoagulation. Main exclusion criteria were previously known malignancy or venous ulcer, known congenital deficiency of an inhibitor of coagulation and unwillingness to participate. MAIN OUTCOME MEASURES: Laboratory. Interlaboratory variation was measured with coefficient of variation. Clinical. End-points were recurrent venous thromboembolism and haemorrhages requiring hospitalization or treatment with blood products or vitamin K. RESULTS: Laboratory. The interlaboratory variation in prothrombin time analyses was 11.3% at a mean INR of 3.8. No difference was detected between laboratories using the two prevalent thromboplastin reagents in Sweden or between those using the Behnk Coagulator and ACL instruments. INR results. Seventy-five per cent of the INR values were > or = 2.0, and 58% were within the target range. The time spent within the target range was between 57 and 74% at the worst and best hospital, respectively. Referral of patients to satellite clinics and fear of treating patients living in distant villages too intensively were factors that decreased the number of effectively anticoagulated patients. The percentage of patients effectively anticoagulated was lower amongst those < 50 than those > or = 50 years of age and also lower during the 1st year than during the 2nd and 3rd. Clinical events. These were eight objectively verified events of recurrent venous thromboembolism [1.3 in 100 patient-years or 0.7%; 95% confidence limits (CL): 0.2 1.2]. Seventeen haemorrhagic events occurred, corresponding to 2.8 per 100 patient-years or 1.5% (CL: 0.8, 2.2); two fatal haemorrhages corresponding to 0.3 per 100 patient-years or 0.2% (CL: 0.0, 0.4). The difference in the incidence of these complications between hospitals with > 60 and < or = 60% of patients effectively anticoagulated did not reach statistical significance. Patients with haemorrhagic complications were not older than the rest. CONCLUSIONS: The performance of the laboratories was acceptable. Clinically important differences between the hospitals were not observed. The incidences of thromboembolic and haemorrhagic complications were low, even in comparison with other randomized trials concerning venous thromboembolism. However, it might be possible to reduce the risk of haemorrhage further with increased centralization and improved education of patients as well as medical staff, and perhaps to reduce the risk of recurrent venous thromboembolism by aiming at a more intensive range of anticoagulation during the 1st month.

Administration, Oral↗
PubMed1994

Coping style in relation to the consumption of factor concentrate in HIV-infected hemophiliacs during the years after their infection became known.

A psychosocial investigation offered to all HIV-infected men with moderately severe or severe hemophilia in Sweden was made in 1986. Most of these men had been infected in the years 1980-1984 and told about their own infection in 1985. A noninfected group of hemophiliacs was used as a reference group in the psychosocial investigation. A psychosocial prognosis was made on the basis of the coping style observed by means of the Coping Wheel. Among subjects who showed evidence of a passive-pessimistic copying style, there was a significant decrease in the number of factor concentrate units received in 1987 and 1988. Among subjects in the group with a more active-optimistic coping style, there was a tendency of increasing the use of factor concentrate during the years after the HIV-infection became known. This result indicates that awareness of HIV-infection may influence specific hemophilia behavior among subjects with passive-depressed copying style.

Adaptation, Psychological↗
PubMed1994

Psychosocial self-prognosis in relation to mortality and morbidity in hemophiliacs with HIV infection.

HIV-infected hemophiliacs participated in a psychosocial prognosis study. The Coping Wheel was filled out 1-2 years after the subjects had been told that they were HIV-infected and between 1 and 7 years after they had become infected. The number of significant signs of disease as well as mortality were recorded during the years following the psychosocial measurements. These measures were related to three measures of anticipated future activities derived from the Coping Wheel, namely 'number of activities for oneself', 'number of activities with others' and a combined measure 'number of activities for oneself in relation to number of activities with others'. The results indicated that the subject's own psychosocial prognosis added to the prediction of mortality. The most important psychosocial factor was the combined measure: those with few anticipated activities for oneself in relation to activities with others had a greater likelihood of dying soon during follow-up. The latter prediction was true even after adjustment for age and condition of the immune system (CD4 count) at the start of follow-up. The conclusion is that the Coping Wheel, applied as in the present examination, may be of help in prognosis and in identifying psychosocial needs in patients with HIV infection.

Adaptation, Psychological↗
PubMed1994

Effects of acute beta-adrenergic receptor blockade on age-associated changes in cardiovascular performance during dynamic exercise.

The cardiovascular response to beta-adrenergic stimulation is markedly blunted with advancing age, and this blunting may underlie some of the prominent age-associated changes in the hemodynamic profile during dynamic exercise. To examine this hypothesis, we administered the nonselective beta-adrenergic receptor blocker propranolol (0.15 mg/kg IV) to 25 healthy normotensive men ages 28 to 72 years from the Baltimore Longitudinal Study of Aging (BLSA) immediately before maximal upright cycle ergometry with 99mTc gated cardiac blood pool scintigraphy. Their hemodynamic responses to exercise were compared with those of 70 age-matched healthy unmedicated male BLSA control subjects. The maximal cycle work rate achieved was similar in propranolol-treated men (158 +/- 32 W) and control subjects (148 +/- 32 W) and declined similarly with age in both groups. Hemodynamics at seated rest were not age-related in either group; however, propranolol-treated men had lower heart rates (HR), systolic blood pressure (SBP), ejection fraction, and cardiac index than control subjects but higher end-diastolic volume index (EDVI) and end-systolic volume index (ESVI) by covariance analysis. At maximal effort, several striking age-drug interactions were evident: Propranolol caused a greater reduction in HR and greater increases in EDVI and stroke volume index (SVI) in younger than in older men. Hence, at maximal work rate, HR declined less with age in the propranolol group (0.46 versus 1.09 beats per minute per year, P < .05 by covariance analysis); EDVI and SVI decreased with age (0.27 and 0.48 mL/m2 per year, respectively) after propranolol compared with increases of 0.47 and 0.16 mL/m2 per year in control subjects, respectively, each P < or = .05 by covariance analysis. The left ventricular contractility index, SBP/ESVI, at exhaustion was reduced by propranolol to a greater extent in younger than older men. Thus, acute beta-adrenergic blockade reverses the age-associated ventricular dilation at end diastole and end systole observed during upright cycle exercise and blunts the decline in maximal HR and myocardial contractility. These data suggest that the age-associated declines in maximal HR and left ventricular contractility during vigorous exercise are manifestations of reduced beta-adrenergic responsivity with advancing age which is partially offset by exercise-induced ventricular dilation.

Adrenergic beta-Antagonists↗
PubMed1994

High versus ultra-high purity factor VIII concentrate therapy: prospective evaluation of immunological and clinical parameters in HIV seronegative and seropositive hemophiliacs.

This study aimed at evaluation of the immunological status and the clinical course of both HIV seronegative and seropositive hemophiliacs treated with either an ultra-pure factor VIII product (UP-F VIII), or a high-purity F VIII (HP-F VIII) concentrate. Eighteen HIV seronegative patients were divided into two groups of therapy and their immune status was followed for 2 years. During the second year of the study 8 patients of the HP-F VIII and 6 from the UP-F VIII therapy groups were switched to the alternative F VIII concentrates. Eighteen asymptomatic HIV seropositive patients were also divided into therapy groups and their immune status and any development of HIV-related symptoms were followed for 4 years. Evaluation of the HIV seronegative patients during the first year did not reveal any differences between the groups in the CD4 or CD8 cell counts, in natural killer cell (NK) activity, or in the mitogenic responses of T lymphocytes to Phytohemagglutinin (PHA), and of B lymphocytes to Pokeweed mitogen (PWM). The switch of 8 patients from the HP-F VIII and 6 from the UP-F VIII groups to the alternative concentrate did not yield any changes in their immune profile during the second year of the study. The HIV seropositive groups differed in the initial CD4 count, with a lower CD4 count (193 +/- 126 vs 437 +/- 142) and a higher F VIII consumption (63,000 +/- 17,000 vs 26,000 +/- 10,000) in the UP-F VIII group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Monoclonal purified F VIII for continuous infusion: stability, microbiological safety and clinical experience.

Replacement therapy for patients with hemophilia A postoperatively or for major hemorrhage, administered as a continuous infusion, is efficient and reduces the requirement for factor VIII (F VIII). The convenience of the method is increased by using a minipump and not diluting the concentrate further after reconstitution. A monoclonally purified F VIII concentrate (Monoclate-P), was evaluated for its stability after reconstitution in different infusion systems, for its microbiological safety as well as clinical safety and efficacy in continuous infusion. The F VIII activity was unaffected by 2 of the 3 infusion systems at room temperature during 15 days, whereas in the third (CADD-1) it decreased below 80% of initial value after 3-7 days. Addition of heparin (1 U/ml) or low molecular weight heparin (1 anti-Xa U/ml), which are used to prevent thrombophlebitis at the site of infusion, did not affect the stability. Nine out of 9 samples taken from the infusion systems after 3 days and again after 7 days were sterile. After inoculation with Staphylococcus aureus or Escherichia coli the bacterial growth in samples of the reconstituted concentrate was not different from that in lidocaine in saline or heparin in saline. F VIII was given in continuous infusion with a minipump (Infu-Med) to 12 patients undergoing major surgery and 8 patients with major hemorrhage for a total of 157 days. A progressive decrease of the clearance was seen during the first 5 days of infusion from 3.0 to 1.7 ml/kg/h. Hemostasis was effectively achieved, and no infectious complications were registered.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Maintenance of the placental circulation to evaluate and treat an infant with massive head and neck hemangioma.

A maternal ultrasound was obtained because of polyhydramnios at approximately 30 weeks gestation. The study identified a fetus with a large, solid mass involving the neck and left face. Because of the concern for airway obstruction, we sought an alternative to normal delivery or routine cesarean section. At 35.5 weeks gestation the mother underwent deep halothane anesthesia followed by cesarean section. Initially, only the head and neck of the fetus were delivered and a large hemangioma was noted to involve the anterior and left neck and all of the left face to the left eye. While maintaining placental and umbilical cord circulation, the infant was successfully nasotracheally intubated. The umbilical cord was divided and the delivery completed. At 1 week of age the infant underwent partial excision of that portion of the hemangioma overlying the trachea and the left external carotid artery was ligated. The infant was successfully extubated 12 days later. In the first 4 months of life she had three episodes of platelet trapping requiring high-dose systemic steroid therapy. Beginning at 8 months of age she was begun on intralesional injections of a 50/50 solution of triamcinolone and betamethasone with a marked reduction in the size of the lesion. Preservation of the placental circulation during delivery resulted in the avoidance of a potential hypoxic or fatal event. Intermittent intralesional steroid injections appear to have played a role in the marked reduction in the size of this large subcutaneous head and neck hemangioma.

Adult↗
PubMed1993

An analysis of the duration of fentanyl and its metabolites in urine and saliva.

This study was undertaken to determine if metabolites of fentanyl might be useful in the detection and monitoring of substance abuse. The presence of fentanyl and two of its metabolites in the urine and saliva of seven female patients receiving small doses (110 +/- 56 micrograms) of fentanyl was studied up to 96 h from the time of administration. Fentanyl and its two metabolites (norfentanyl and despropionylfentanyl) were extracted from samples and analyzed by gas chromatography/mass spectrometry. Unchanged fentanyl was detectable in urine in all patients immediately postoperatively and in 3 of 7 patients at 24 h. By 72 h, fentanyl was undetectable. Norfentanyl was present in larger quantities than fentanyl immediately postoperatively and was detected in all patients at 48 h and in 4 of 7 patients at 96 h. Despropionylfentanyl was not detected in any of the urine specimens tested. Neither fentanyl nor its metabolites could be detected consistently at any time in saliva. Saliva testing does not appear to be a viable alternative to urine testing based on this study. Urinary norfentanyl might be considered as the substance of choice when testing for fentanyl abuse.

Adult↗
PubMed1992

Treatment of venous thromboembolism in patients with congenital deficiency of antithrombin III.

The treatment course of all thromboembolic events in the patients with congenital deficiency of antithrombin III (AT III) in the national Swedish register was reviewed in order to assess the appropriate medical therapy in this situation. The medical treatment of 70 events of venous thromboembolism was evaluated. There were eight cases with signs of clinical progression. The risk of therapeutic failure with heparin could be as low as 1.5% or as high as 9.2%. It would not be cost-effective to substitute with concentrates of AT III in every case with congenital deficiency thereof in connection with acute venous thromboembolism. "Heparin resistance" does not seem to be a problem in the vast majority of these patients.

Adult↗