نتایج جستجو برای: hyper igm

تعداد نتایج: 40595  

Journal: :Archives of disease in childhood 1969
G W Goldstein W J Krivit R Hong

Abnormalities of serum immunoglobulin levels are frequently associated with such haematological disorders as primary aregenerative anaemia, haemolytic anaemia, neutropenia, thrombocytopenia, and leukaemia (Good et al., 1962). Recently, Hermans et al. (1966) reported a group of patients with nodular intestinal lymphoid hyperplasia and hypoy-globulinaemia. We have now seen thrombocytopenia, immun...

2011
Aleksandra Szczawinska-Poplonyk Zdzislawa Kycler Barbara Pietrucha Edyta Heropolitanska-Pliszka Anna Breborowicz Karolina Gerreth

The hyper-IgE syndromes are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity, by particular susceptibility to staphylococcal and mycotic infections as well as by a heterogeneous genetic origin. Two distinct entities--the classical hyper-IgE syndrome which is inherited in an autosomal dominant pattern and the autosomal recessive hyper-IgE syndrome--have ...

Journal: :Blood 2000
A Solanilla J Déchanet A El Andaloussi M Dupouy F Godard J Chabrol P Charbord J Reiffers A T Nurden B Weksler J F Moreau J Ripoche

CD40 ligand (CD40L)/CD40 interactions play a central role in T-cell-dependent B-cell activation as previously shown by in vitro studies, the phenotype of CD40L knockout mice and the defective expression of CD40L in patients who have X-linked immunodeficiency with hyper-IgM. The distribution of CD40 in cells other than of myeloid and lymphoid lineages has suggested additional functions for this ...

Journal: :Monthly Notices of the Royal Astronomical Society 2022

The large opacity fluctuations in the $z > 5.5$ Ly$\alpha$ forest may indicate inhomogeneous progress of reionization. To explain observed scatter effective optical depth ($\tau_{\rm eff}$) intergalactic medium (IGM), fluctuation UV background ($\Gamma$ model) or IGM gas temperature ($T$ have been proposed, which predict opposite correlations between $\tau_{\rm eff}$ and galaxy density. In orde...

Journal: :British journal of rheumatology 1996
J Sibilia A Durandy T Schaeverbeke J P Fermand

We studied a patient who had a typical seronegative rheumatoid arthritis (RA) and an immunodeficiency with hyper-IgM (HIM syndrome). CD40L was normally expressed by activated T cells, but CD40-mediated signal transduction was defective in B cells, preventing heavy chain switching (CD40L+ type of the HIM syndrome). These data suggest that a typical RA can develop in at least some patients with d...

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