One-third of patients with chronic idiopathic urticaria (CIU) have circulating functional autoantibodies against the high affinity IgE receptor FcepsilonRI, or IgE. The intradermal injection of autologous serum causes a weal and flare reaction in many patients with CIU, and this reaction forms the basis of the autologous serum skin test (ASST). We have determined the parameters of the ASST which define the optimal sensitivity and specificity for the identification of patients with autoantibodies. Two physicians (R.A. S. and C.E.H.G.) performed ASSTs in a total of 155 patients with CIU, 40 healthy control subjects, 15 patients with dermographism, nine with cholinergic urticaria and 10 with atopic eczema. Patients were classified as having functional autoantibodies by demonstrating in vitro serum-evoked histamine release from the basophils of two healthy donors. There were significant differences (P < 0.001) in the mean weal diameter, weal volume, weal redness and flare area of the intradermal serum-induced cutaneous responses at 30 min between patients with CIU with autoantibodies and either those without autoantibodies or control subjects. The optimum combined sensitivity and specificity of the ASST was obtained if a positive test was defined as a red serum-induced weal with a diameter of >/= 1.5 mm than the saline-induced response at 30 min. For R.A.S. and C.E.H.G., the ASST sensitivity was 65% and 71% and specificity was 81% and 78%, respectively. Using these criteria, the following subjects had positive ASSTs: none of 15 dermographics, none of 10 atopics, one of nine cholinergics and one of 40 controls.
93 (31%) of the 301 Haematology Departments in the U.K. responded to a questionnaire about the postnatal management of fetomaternal alloimmune thrombocytopenia (FMAIT). The number of reported cases of FMAIT was less than half than that estimated from its known incidence, suggesting that the condition is under-recognized. There was a consensus that the optimal approach to postnatal management is to transfuse compatible platelets promptly. However, a number of problems in the delivery of treatment were identified, including an apparent lack of awareness of the potential seriousness of the condition amongst clinical staff, and the availability of HPA-1a negative platelets.
Pemphigus vulgaris is an immunobullous disease affecting the skin and mucous membranes most commonly during the fifth and sixth decades of life. Its occurrence in pregnancy is rare. We now report two severe cases of the disorder presenting during pregnancy and discuss its potential effects on the foetus and its management in pregnancy.
Summary.The clinical manifestations of the antiphospholipid syndrome (APLS) include arterial and venous thrombosis, thrombocytopenia and fetal loss, but the pathogenic mechanisms remain unclear. It has been hypothesized that platelet activation by autoantibody may be a pathogenic mechanism. We studied IgG binding, microparticle (mp) formation and P-selectin expression by flow cytometry in normal platelets after incubation in serum from 11 patients with antiphospholipid antibodies and that from 10 normal healthy subjects. Levels of platelet-associated IgG were significantly higher after incubation in patient sera (mean 17·2, range 2·0-75·0%) compared with normal sera (mean 2·0, range 1·2-3·7%, P < 0·05). Incubation of normal platelets in serum led to increased microparticle formation (P < 0·01) and P-selectin expression (P < 0·05), compared with unstimulated platelets. There was no significant difference, however, between microparticle formation nor P-Selectin expression induced by patient serum (mp 3·0 (1·6-5·0)%; P-selectin 8·0 (4·0-16·6)%) versus normal serum (mp 3·2 (2·1-4·5)%; P-selectin 10·1 (4·0-15·6); median (range)). Pre-activation of platelets with subthreshold ADP concentrations or thrombin receptor activator peptide resulted in a small increase in microparticle formation, but there was still no significant difference between the effects of patient and control sera. Despite the presence of platelet membrane binding IgG in serum from 5/11 patients with antiphospholipid antibodies, there was no evidence for associated enhanced platelet-activating ability. This study supports antiplatelet reactivity in antiphospholipid syndrome, but not a direct platelet-activating role for platelet-directed autoantibodies.
Although botulinum toxin injection was well tolerated, these results using Dysport at a dose of 240 mouse units question its efficacy as a treatment for achalasia.
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