Shocks, institutional change, and sustainability transitions.

The role of exogenous shocks in influencing transition processes is of significant interest to a wide variety of research in sustainability science (SS). Such events disturb and interrupt path-dependent processes in sociotechnical systems. Sometimes this can lead to radical departures from existing trajectories, while at other times existing systems can be more resilient, adapting, or reconfiguring in response to a shock.

Chronic lymphocytic leukemia disease progression is accelerated by APRIL-TACI interaction in the TCL1 transgenic mouse model.

Although in vitro studies pointed to the tumor necrosis factor family member APRIL (a proliferation-inducing ligand) in mediating survival of chronic lymphocytic leukemia (CLL) cells, clear evidence for a role in leukemogenesis and progression in CLL is lacking. APRIL significantly prolonged in vitro survival of CD5(+)B220(dull) leukemic cells derived from the murine Eμ-TCL1-Tg (TCL1-Tg [transgenic]) model for CLL. APRIL-TCL1 double-Tg mice showed a significantly earlier onset of leukemia and disruption of splenic architecture, and survival was significantly reduced.

p38 inhibition and not MK2 inhibition enhances the secretion of chemokines from TNF-α activated rheumatoid arthritis fibroblast-like synoviocytes.

Objectives: For many years the p38 MAP kinase (MAPK) has been a major anti-inflammatory target for the development of an oral therapy for rheumatoid arthritis (RA). However, disappointing results from Phase II clinical studies suggest that adaptations may occur, which allow escape from blockade of the p38 pathway. In this study we investigated whether p38 inhibition mediated JNK activation represents such an escape mechanism.

The RICHE Reference Architecture.

The RICHE Reference Architecture defines an open framework for health care information systems. Developed within the ESPRIT II programme, it is now the basis of several implementations in hospitals in Italy, Portugal, Ireland, France and the UK, and of follow-up initiatives such as EDITH (ESPRIT, no. 7508), NUCLEUS (AIM II, no.

Lhermitte-Duclos disease and Cowden disease: a single phakomatosis.

Two unrelated patients with macrocephaly, seizures, and mild cerebellar signs had a dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). Both also had autosomal dominant Cowden disease as evidenced by facial, oral, and acral papules. In the two families, 9 sibs demonstrated the mucocutaneous lesions, thyroid disease, breast tumors, and ovarian tumors compatible with the diagnosis of Cowden disease.

Genetics of two human monocyte antigens.

HMA-1 and HMA-2 are two serologically defined alloantigens that are present on human monocytes and granulocytes. Previous panel studies suggested that these two antigens comprise a diallelic system. We therefore performed segregation studies on 10 randomly chosen families and on 22 nucleus families belonging to one large pedigree.

X-linked immunodeficiency with hyperimmunoglobulinemia M appears to be linked to the DXS42 restriction fragment length polymorphism locus.

The gene involved in X-linked immunodeficiency with hyperimmunoglobulinemia M (XHM) was localized by the use of nine restriction fragment length polymorphic (RFLP) markers covering the entire X chromosome. Multipoint linkage analysis of RFLP data obtained in a three generation XHM pedigree indicates the Xq24-q27 area around the DXS42 RFLP locus as the most likely localization of the XHM locus.

Deficiency of immunity to Mycobacterium avium that can be restored by allogeneic lymphocytes.

A 3-year-old girl developed a disseminated Mycobacterium avium infection despite treatment with eight antimycobacterial drugs. She had no pre-existent general humoral or cellular immunodeficiency. In the course of the disease B lymphocyte areas in the lymphoid tissues were replaced by histiocytes and an IgM and IgA deficiency evolved.

Genetic heterogeneity in X-linked agammaglobulinemia complicates carrier detection and prenatal diagnosis.

X-linked agammaglobulinemia (XLA) is a severe antibody deficiency disease reflecting an arrest of B lymphocyte differentiation at the level of precursor B cells. The disease is inherited in an X-linked recessive mode. In a single eight-generation pedigree the XLA gene was mapped to the Xq21.

A comparison of two fluorescence-activated cell sorters, the FACSIV (laser) and the FACSTm (mercury lamp), as research analyzers for the quantification of T and B cell subsets in human peripheral blood.

T cell subset determinations were performed on 146 peripheral blood samples from healthy volunteers, and on 112 samples from immune deficient patients using two fluorescence-activated cell sorters (the FACSIV laser, and the FACSTm mercury lamp analyzer). The procedures necessary for the use and calibration of the FACSTm analyzer are discussed, and detailed. Using the FACSTm analyzer, counts were made of T and B cell subsets in 28 patients with multiple infections, 9 patients suffering from the acquired immune deficiency syndrome (AIDS) and 16 patients with a primary immunodeficiency disease.

Heterogeneity in the map distance between X-linked agammaglobulinemia and a map of nine RFLP loci.

In nine family pedigrees in which X-linked agammaglobulinemia (XLA) is segregating, a multi-point linkage analysis has been carried out. In each family, the map distance, d, between XLA and a fixed point in a known map of nine RFLP loci on the X chromosome was estimated by calculating the log likelihoods, L(d). Using a new method, the 10-point likelihood was approximated by appropriately combining three 4-point likelihoods.

Blood transfusion suppresses cutaneous cell-mediated immunity.

Cutaneous cell-mediated immunity (CMI) evoked by dinitrochlorobenzene (DNCB) was evaluated in end-stage renal disease patients on regular haemodialysis and before renal transplantation. Twenty-seven per cent of the patients had suppression of cutaneous CMI as shown by a negative response upon DNCB challenge. We analysed seven factors known or postulated to have an influence on renal allograft rejection for their effects on cutaneous CMI.

Mapping of a gene for X-linked agammaglobulinemia and evidence for genetic heterogeneity.

X-linked agammaglobulinemia (XLA) is a severe humoral immunodeficiency disease of man. The inheritance of the disease is X-linked recessive. Female carriers can not be distinguished by immunologic assays.

Immunoglobulin heavy chain gene rearrangements in X-linked agammaglobulinemia.

X-linked agammaglobulinemia (XLA) appears to involve a defect in human B lymphocyte differentiation which is manifested at the pre-B cell stage. The defect segregates as an X-linked recessive trait but is not a single genetic entity. IgM-producing B cell clones were established by Epstein-Barr virus transformation of peripheral blood mononuclear cells of patients with the XLA defect linked to the DXS3 and DXS17 chromosomal loci.

X-linked agammaglobulinemia and the red blood cell determinants Xg and 12E7 are not closely linked.

Studies on the segregation of the red blood cell determinant Xg in 12 families with X-linked inheritance of agammaglobulinemia (XLA) in 3-4 generations suggested linkage of Xg with XLA. One extensive pedigree of a Dutch family with XLA in eight generations was investigated for Xg and the quantitative polymorphism 12E7. LIPED analysis indicated linkage disproven up to 25cM distance within this pedigree.

The late bronchus-obstructive response to bronchial challenge with pigeon faeces and its correlation with precipitating antibodies (IgG) in the serum of patients having long-term contact with pigeons.

From a total of 160 patients with allergic bronchial asthma or chronic asthmatic bronchitis, eighteen (11.3%) patients reported being regularly exposed to pigeons. In these eighteen patients, bronchial provocation tests with pigeon faeces were performed and the precipitating antibodies (IgG) in their serum against pigeon faeces and serum were estimated.