Adjusted dose continuous infusion of factor VIII in patients with haemophilia A.

Surgical interventions in patients suffering from haemophilia A require infusions of large doses of factor VIII (F VIII) concentrates. These are expensive and may pose a burden on the immune system, which is already compromised in many haemophiliacs. F VIII is usually given as bolus injections, although there are reports on fixed dose continuous infusion.

Effects of leukocyte depletion on the formation of anaphylatoxins in stored whole blood.

As reported earlier, factors of the complement cascade get activated in CPD-A1-stabilized whole blood. As early as after 10 days of storage under normal blood bank conditions the elevations of the concentrations of C3a-desArg and C4a-des-Arg were highly significant. By contrast, the concentration of the C3 activator complex C4b2b remained unchanged even after 3 weeks of storage.

Circumcision in hemophilia: the use of fibrin glue for local hemostasis.

Circumcision can be fatal in hemophilia patients unless they are treated with the missing coagulation factor. We describe 10 severe hemophilia patients in whom circumcision was performed with local fibrin glue instead of infusion of factor concentrate. Of the 10 patients 3 had postoperative bleeding and only 2 of them required infusion of factor VIII concentrate.

Evolution of a highly polymorphic human cytochrome P450 gene cluster: CYP2D6.

The CYP2D gene cluster on human chromosome 22 containing the functional cytochrome P450 gene CYP2D6 and two or three highly homologous pseudogenes is involved in a clinically important variation in the inactivation of drugs and environmental chemicals. Several mutant haplotypes of CYP2D6 have been identified by restriction analysis and by PCR-based allele-specific amplification. To understand the evolutionary sequence of mutational events as well as recently discovered interracial differences, we analyzed the arrangement of the CYP2D haplotype containing a common mutant allele of CYP2D6 associated with a XbaI 44-kb fragment.

[Coronary disease of transplanted heart. Physiopathology, prevention].

Coronary artery disease in cardiac transplant recipients is the main cause of mortality after the first postoperative year. This atheroma has unique anatomical features with widespread infiltration of the intima by inflammatory cells. The different physiopathological hypotheses are analysed.

McLeod syndrome: a distinct form of neuroacanthocytosis. Report of two cases and literature review with emphasis on neuromuscular manifestations.

McLeod syndrome was originally described on the basis of a specific blood group phenotype with weak expression of Kell antigens. This erythrocyte abnormality also causes acanthocytosis. The haematological findings are associated with abnormalities in other organ systems, including neuromuscular manifestations.

Complement activation during storage of blood under normal blood bank conditions. Effects of proteinase inhibitors and leukocyte depletion.

During storage of CPD-A1 preserved whole blood factors of the complement cascade become activated, as evidenced by a rapid increase in the concentrations of C3a-desArg and C4a-desArg. After 10 to 14 days of whole blood storage, the elevations of C3a and C4a levels were highly significant. This increase was paralleled by an increase in the concentration of the lysosomal proteinase elastase from polymorphonuclear (PMN) granulocytes.

Pupil's knowledge about health and illness.

Three hundred twenty high school pupils were evaluated concerning their knowledge about health and disease. The knowledge of 170 students in the first school in which they received lectures and had regular talks about health topics was higher in comparison with the knowledge of 150 students in the second high school who did not receive these lectures. We recommended that health topics be integrated in the school program starting at the first grade and continuing until graduation from high school.

[Spinal cord injuries caused by aviation accidents].

During the past 15 years fewer than 1% of those treated in the National Spinal Cord Injury Center were injured as a result of aviation accidents. In addition to 9 such patients treated at the center since 1973, another 6 were found among the many hundreds receiving ambulatory care in our clinics. 3 patients had survived a helicopter crash, 2 were injured while ejecting from combat aircraft, 3 were injured in crashes of light aircraft, 1 fell from a hand glider and 6 were injured in parachute drops.

Reflex sympathetic dystrophy in a child.

We describe a 9 1/2 year old girl who suffered from severe recurrent pain and functional limitation in her right leg with hyperesthesia, hyperalgesia, color change and edema as the presenting symptoms, during the previous two months. All laboratory tests were found to be normal and diagnosis of reflex sympathetic dystrophy was made.

[Lipid indicators of vascular risk. A cross-sectional study of a group of coronary patients, a group of subjects with normal coronary angiography and a control group].

During a transverse survey, 3 groups of men with the same weight and age were compared. Group I included 42 patients with coronary disease documented by coronarography, group 2 included 19 subjects with normal coronary angiograms, and group 3 included 27 healthy controls who had not undergone coronarography. Subjects presenting diabetes or any factor associated with secondary dyslipidemia or able to modify lipid levels were excluded from study.

Imaging rounds #112. Congenital short Achilles tendon.

Too often a relatively minor surgical procedure can prevent complications at a later stage. The diagnosis in children is often missed because of normal physiologic states that closely resemble the pathology. The early detection and correction of congenital short Achilles tendons can prevent further developmental abnormalities.

[Comparative study of LpAI lipoparticles, HDL cholesterol and apolipoprotein AI in a control population, in a group of subjects with coronary diseases, and in a group of subjects with angiographically normal coronary vessels].

A new method for directly measuring LpAI lipoparticles containing apolipoprotein AI, but not apolipoprotein AII, is now disponible for laboratories. Concentrations of LpAI were measured in serum from 158 presumably healthy normolipidemic subjects (72 male, 86 female), for the age group 30-60 years. Concentrations of LpAI were also measured in subjects with angiographically defined coronary artery disease (coro+) and without angiographically defined coronary artery disease (coro-).

Cyclic alternating chemotherapy of high-grade malignant non-Hodgkin lymphomas with VIM-Bleo and CHOP.

Between 1986 and 1988, 81 patients with high grade malignant non-Hodgkin lymphoma according to the Kiel classification were treated with the VIM-Bleo/CHOP-regimen: etoposide 100 mg/m2 intravenously on days 1-3, ifosfamide 1.5 g/m2 intravenously days 1-5 with mesna for prophylaxis of cystitis, methotrexate 30 mg/m2 intravenously on days 3, bleomycin 10 mg intravenously on days 8 and 15, cyclophosphamide 750 mg/m2 day 22, doxorubicin 50 mg/m2 day 22, vincristine 1.4 mg/m2 on day 22, and prednisolone 100 mg postoperatively on days 1-5 and 22-26.

Intravenous anti-D gammaglobulin for the prevention of rhesus isoimmunization caused by platelet transfusions in patients with malignant diseases.

Previous studies of sensitization to RhD by RhD-positive platelet transfusions in RhD-negative cancer patients have shown different frequencies of alloimmunization (max. 19%). We studied 37 RhD-negative patients who received RhD-incompatible platelet transfusions and simultaneously anti-D-immune globulin.

[Incidence of the detection of erythrocyte antibodies in relation to screening test cells].

A variety of antibody screening tests are available to detect immunization in patients' sera. However, antibodies can be observed only if corresponding antigens are present on test cells used by an antibody screen. By comparing available test cell kits of different manufacturers we revealed various specificities of antigens present or absent on these cells.

[Strong and weak histocompatibility antigens and immune response].

In blood samples of 88 healthy donors various red cell, HLA-A, -B, -Cw and -Dr antigens were determined by standard methods. Lymphocyte reactivity was measured by 3H-thymidine uptake into DNA of lymphoblastic cells stimulated by 14 different mitogens and antigens. Cluster analysis confirmed by t test and F test defined 3 distinct clusters of lymphocyte reactivity.

[Combination of a simple hollow fiber system with leukocyte filter for production of leukocyte depleted erythrocyte concentrates and plasma].

Leukocyte-depleted red cell concentrate (RCC) and plasma were separated by a hollow fiber filter system combined with a leukocyte filter without any additional devices. The RCC with 100 ml additive solution had a weight of 329 g; hematocrit was 0.55, free hemoglobin 16 mg/dl; leukocytes were (0.

Decreased formation of the complement component C4A in leukocyte-depleted stored whole blood.

As reported earlier, factors of the complement cascade get activated in CPD-A1-stabilized whole blood. While leukocyte depletion inhibited partially the activation of C4, it had no effect on C3a concentrations. Therefore cleavage of C4 during storage of whole blood seems to be partially leukocyte-dependent, whereas the activation of C3 is possibly due to the activation of the alternate pathway of the complement system by contact of blood to plastic surfaces.

[Graft versus host disease with fatal outcome after administration of filtered erythrocyte concentrates].

Transfusion-associated graft-versus-host disease (TA-GVHD) resulting from the engraftment of competent lymphocytes contained in blood products has been well described in immunocompromised patients and more recently in immunocompetent patients. Prophylactic irradiation of blood products prior to transfusion is the most efficient way to prevent TA-GVHD. Standard blood bank measures to reduce mononuclear cell contamination in red blood cell units, such as freezing, washing and filtration, may reduce the number of viable lymphocytes to prevent immunizations.

Pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVS) is an uncommon, usually monoarticular disorder encountered mainly in adults. A boy and a girl, both 7 years old, were referred because of recurrent knee effusions. Both were medically treated for other rheumatic disorders for five years.

Acetylation of serotonin in the rabbit pineal gland: an N-acetyltransferase with properties distinct from NAT1 and NAT2 is responsible.

Two rabbit arylamine N-acetyltransferases (NAT1 and NAT2, EC 2.3.1.

Debrisoquine/sparteine hydroxylation genotype and phenotype: analysis of common mutations and alleles of CYP2D6 in a European population.

Four different mutations of the cytochrome P450 CYP2D6 gene associated with the poor metabolizer phenotype (PM) of the debrisoquine/sparteine polymorphism were analyzed by Xba I restriction fragment length polymorphism (RFLP) analysis and a polymerase chain reaction (PCR)-based DNA amplification method in DNA of 394 healthy European subjects; 341 of these were phenotyped by sparteine or debrisoquine administration and urinary metabolic ratios (MR). Our study demonstrates the efficiency of the PCR-test for phenotype prediction; 96.4% of individuals were correctly predicted, i.