Measurement of global haemostasis in severe haemophilia A following factor VIII infusion.

Patients with haemophilia requires different amounts of FVIII to prevent and treat bleeds. We hypothesise that this is because FVIII has variable effects on individual patients' global haemostasis. Twelve patients with severe haemophilia A were infused with 50 IU/kg FVIII and thrombin generation in platelet rich (PRP) and platelet poor plasma (PPP) and velocity of changing clot elasticity were measured preinfusion and at nine subsequent time points over 72 h. Despite a close correlation between median FVIII and median initial rate of thrombin generation (R(2) 0.94), endogenous thrombin potential (ETP; R(2) 0.94) and peak thrombin (R(2) 0.91) in PPP, there was wide inter-patient variability at each time point. There was, however, a highly predictable intra-patient relationship between FVIII level and thrombin generation. Inter-patient variability was due to both differences in FVIII levels and the variable effect FVIII had on an individuals' thrombin generation. The utility of PRP was limited because, at low-FVIII levels, only rate of thrombin generation was measurable. At low-FVIII concentrations, the rate of thrombin generation in PPP was the most useful test whilst at higher levels ETP and peak thrombin could also be used.

Formation and in situ sizing of gamma-Fe2O3 nanoparticles in a microwave flow reactor.

Nanocrystalline gamma-Fe2O3 particles were produced in a microwave flow reactor. The reaction of iron pentacarbonyl [Fe(CO)5] with the plasma gases Ar/O2 to form nanosized particles was followed by in situ particle mass spectrometry. The particle mass spectrometer combines a nonintrusive sampling technique with a calibration-free mass determination. The influence of process parameters like microwave power, precursor concentration, and pressure on the particle size was studied. The results reveal a mean particle diameter in the range of 4-5 nm with a slight dependence on the process parameter. The geometric standard deviation of the measured size distribution was always between 1.1 and 1.2.

Translocation trisomy 21 in CVS not found in embryoblast: three different cell lines in CVS, amnion- and placental culture.

Chorionic villus samples from a healthy pregnant female were obtained for first-trimester prenatal diagnosis. A translocation trisomy 21 was diagnosed. A consecutive amniocentesis revealed a normal male karyotype. At term a healthy boy was born. Cytogenetic analysis from cord blood showed a regular karyotype of 46,XY, whereas in term placenta a pathological karyotype of 47,XY, +mar was found.

Association of Behçet's syndrome with HLA-B5 in the Mexican mestizo population.

HLA typing done in 10 Mexican mestizo patients with Behcet's syndrome revealed that 7 of them had HLA-B5 antigen as compared to a frequency of 31% in a Mexican mestizo control population. These findings are akin to those made in Japan and Turkey but differ from those made in Caucasian populations in the United States and Great Britain. Ethnic groups with a high frequency of HLA-B5 in Asia and America may belong to the same ancestral trunk. The occurrence of Behcet's syndrome in them may be more frequently HLA-B5 positive than in other ethnic groups.

Behcet's disease. Report of twelve patients in the Mexican population.

Se presenta el analisis clinico e inmunologico de doce pacientes con enfermedad de Behcet Este ocurrio con mayor frecuencia en la tercera decada de la vida. Los sintomas iniciales mas frecuentes fueron los de la triada clasica de ulceraciones orales, genitales e iritis. Caracteristicamente, las ulceras orales fueron resistentes al tratamiento. La lesion ocular mas frecuente fue la conjuntivitis, seguida por iridociclitis. El hipopion se observo solo en un paciente y el sindrome de Sjogren en dos. Siete pacientes tuvieron sintomas articulares. Hubo tromboflebitis en cuatro pacientes y embolias pulmonares en dos. Otras manifestaciones incluyeron: pericarditis, fibrosis pulmonar, epididimitis y manifestaciones del sistema nervioso central.Se observo disminucion del C3 y aumento de la IgG y la IgA en cinco de ochos pacientes. Hubo linfopenia T en cuatro pacientes con enfermedad activa. La linfopenia T, la asociacion con sindrome de Sjogren y el infiltrado inflamativo perivascular apoyan la participacion del sistema inmune en la patogenia de la enfermedad

Heterogeneity of the spontaneously expanded and mitogen-induced generation of suppressor cell function of T cells on B cells in systemic lupus erythematosus.

Eighty percent of 31 untreated patients with systemic lupus erythematosus (SLE) had abnormalities in their spontaneously expanded and/or Con-A-induced suppressor cell function, but the association of defects detected with both systems was only 68%. Loss of spontaneous suppression related positively to disease activity (r = 0.641) and the number of T gamma cells (r = 0.624) whereas Con-A-induced suppression correlated negatively with disease activity (r = -0.456) and the number of T gamma cells (r = 0.089). Incubation of mononuclear cells from SLE patients in antiribonucleoprotein IgG caused further loss of suppression in some, but not all, instances. The suppressor cell dysfunction found in SLE may result from diverse mechanisms, including a basic defect in the generation of suppressor cells and the abrogation of suppressor function by autoantibodies.