The pros and cons of using SDL for creation of distributed services

In a competitive market for the creation of complex distributed services, time to market, development cost, maintenance and flexibility are key issues. Optimizing the development process is very much a matter of optimizing the technologies used during service creation. This paper reports on the experience gained in the Service Creation projects SCREEN and TOSCA on use of the language SDL for efficient service creation

HAE international home therapy consensus document

Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks ('self or assisted administration') and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE

Antibody response to the Haemophilus influenzae type b-tetanus toxoid conjugate vaccine in healthy and infection-prone individuals with IgG3 subclass deficiency

Searching for a possible explanation for the phenotypic heterogeneity in IgG3 deficiency, we studied the antibody response to a polysaccharide and a protein antigen in IgG3-deficient (IgG3d) adults after vaccination with Haemophilus influenzae type b capsular polysaccharide (Hib CP) conjugated to tetanus toxoid. Distribution of isotypes, idiotypes, clonotypes, and Gm allotypes were compared. All the vaccinated individuals, irrespective of the level of IgG3 and proneness to infections, developed protective levels of anti-Hib CP. Significantly lower prevaccination levels of IgG2 ( p < 0.05) and IgG4 anti-Hib CP ( p < 0.04 and p < 0.03) were noted among the infection-prone compared to the healthy IgG3d individuals and/or controls. Seventy percent of the IgG3d patients and none of the controls had the low responding Gm(ga-n/ga-n) genotype, while the majority of the controls had the alternative Gm(bfn/bfn) genotype. The conjugate ACT-HIB(R) vaccine efficiently overcomes the IgG3 subclass deficiency state and the genetic predisposition for lower responsiveness, providing protection against Hib and tetanus infections. The proneness to infection in some IgG3d individuals may relate to their low prevaccination antibody levels

European surveillance of immunoglobulin safety--results of initial survey of 1243 patients with primary immunodeficiencies in 16 countries.

A European multicenter study was conducted to obtain information on the current practices of immunoglobulin administration, the policies in use for the surveillance of the risk of hepatitis C virus (HCV) transmission, and the natural history of HCV infection in patients with hypogammaglobulinemia. Data from 1243 patients with primary immunodeficiencies in 16 countries demonstrated that 90% of patients with antibody deficiencies receive intravenous immunoglobulins in an inpatient setting, and 7% of patients are treated with subcutaneous immunoglobulins, mainly at home. Wide variations have been reported regarding the frequency and the type of tests monitored for the surveillance on the risk of viral hepatitis transmission. Only 60% of patients have been tested at least once for HCV RNA detection. Data from 71 HCV-infected patients demonstrated a rapid progression of HCV infection, with end-stage liver disease, in about 40% of patients. Ten percent of patients spontaneously cleared the virus, and about 30% are asymptomatic. Patients with CVID have a worse prognosis than patients with XLA

Common variable immunodeficiency disorders: division into distinct clinical phenotypes.

The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients. A total of 7 centers contributed patient data, resulting in the largest cohort yet reported. Patients (334), validated for the diagnosis, were followed for an average of 25.6 years (9461 patient-years). Data were used to define 5 distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy, and lymphoid malignancy. A total of 83% of patients had only one of these phenotypes. Analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times. Types of complications and clinical phenotypes varied significantly between countries, indicating the need for large, international registries. Ages at onset of symptoms and diagnosis were shown to have a Gaussian distribution, but were not useful predictors of phenotype. The only clinical predictor was polyclonal lymphocytic infiltration, which was associated with a 5-fold increased risk of lymphoid malignancy. There was widespread variation in the levels of serum immunoglobulin isotypes as well as in the percentages and absolute numbers of B cells, confirming the heterogeneity of these conditions. Higher serum IgM and lower circulating CD8 proportions were found to be predictive markers for polyclonal lymphocytic infiltration and autoimmunity, respectively