Cd154 Deficiency and Related Syndromes

Bonilla, Francisco A.; Geha, Raif S.

doi:10.1016/s0889-8561(05)70193-4

Cited by 10 publications

(2 citation statements)

References 145 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Both presented upper and lower respiratory tract infections, which have been described previously as the most common clinical manifestations of the disease in a multi‐centre study [19]. A high frequency of viral infections, including mild to severe herpes zoster infection, has been reported [14,17,20] . Patient 1 presented with severe varicella when she was 7 months old and a subsequent herpes‐zoster at age 8, despite IVIG therapy .…”

Section: Discussionmentioning

confidence: 92%

See 1 more Smart Citation

An imbalance of naive and memory/effector subsets and altered expression of CD38 on T lymphocytes in two girls with hyper-IgM syndrome

Costa‐Carvalho

Viana

Brunialti

et al. 2004

Clinical and Experimental Immunology

View full text Add to dashboard Cite

SUMMARYIn this report we evaluated CD4 + T, CD8 + T and natural killer (NK) cell counts, the levels of naive/ memory subsets within the CD4 + T lymphocyte population, expression of CD38 on T lymphocytes, and CD4 + and CD8 + T cell cytokine production in two girls with hyper-IgM (HIM) syndrome. Both girls developed recurrent infections early in infancy, presenting a wide spectrum of clinical manifestations, with a strikingly different disease severity between them. CD4 + T cell counts were low in both children (patient 1: 214 cells/mm 3 and patient 2: 392 cells/mm 3 ), and the CD4/CD8 T cell ratio was 0·4 for patient 1, the patient with the more severe disease, and 1·4 for patient 2. NK cell numbers were low in patient 1 (60 cells/mm 3 ) and borderline (286 cells/mm 3 ) with regard to normal levels in patient 2. An imbalance of naive and memory/effector cell subsets was found in both girls, with the percentage of CD45RA + 27 + (naive) CD4 + T lymphocytes being 5·8 and 12·4 for patients 1 and 2, respectively. Expression of CD38 on the surface of T lymphocytes was low in patient 1. Detection of intracellular interferon (IFN)-g and tumour necrosis factor (TNF)-a in CD4 + and CD8 + T lymphocytes upon PMA-Io stimulus was preserved in both children. In conclusion, we found low numbers of CD4 + T lymphocytes and a dramatic redistribution of naive and memory/effector CD4 + T lymphocytes in two girls with non-X-linked HIM syndrome. Furthermore, we found low expression of CD38 on T lymphocytes and low numbers of NK cells in the patient with the more severe disease, indicating a possible role for these cells in the pathogenesis of this immunodeficiency.

show abstract

Section: Discussionmentioning

confidence: 92%

“…A purified protein derivative (PPD) skin test performed some years after immunization was non‐reactive in both patients, possibly reflecting the HIM‐related T cell dysfunction. Such clinical complications following BCG immunization have been rarely reported [6,19,20].…”

Section: Discussionmentioning

confidence: 99%