PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 568690

[Progressive combined immunity defect with ectomesodermal dysplasia (author's transl)].

K D Tympner·B Belohradsky·R Eife·P K Klose
View original source ↗Export metadata ↓

Abstract

A boy with dysplastic features had to be treated for recurrent diarrhoea and bronchitis since his tenth month of life. Defects in humoral and cellular immunity were found. The ecto-mesodermal abnormalities are so characteristic that in connection with the immunological changes it seems to be justified to assume a yet undescribed syndrome.

Explore related subjects

Antibody FormationChild, PreschoolEctodermal DysplasiaHLA AntigensHumansImmunity, CellularImmunoglobulin EImmunologic Deficiency SyndromesLymphocyte ActivationMalePedigreeCase ReportsEnglish AbstractJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

K D Tympner, B Belohradsky, R Eife, P K Klose. 1978. [Progressive combined immunity defect with ectomesodermal dysplasia (author's transl)].. https://pubmed.ncbi.nlm.nih.gov/568690/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2007

Analysis of HLA class I specific antibodies in patients with failed allografts.

BACKGROUND: The goals of this study are first to determine the epitope specificity of donor specific antibody (DSA) in the serum of alloimmunized transplant patients with a failed renal graft; and second to understand the correlation between the development of DSA and nondonor specific antibody (NDSA). METHODS: The sera of 35 pretransplant panel reactive antibody (PRA)-negative patients with failed allografts were examined with single-antigen (SA) luminex beads to identify human leukocyte antigen (HLA)-A and -B antibodies. Potential HLA antibody epitopes were identified by using computer software and verified by absorption and elution from single-antigen cell lines. RESULTS: Twenty-seven patients developed donor-specific HLA-A and/or -B antibodies, while the remaining eight patients had only nondonor-specific HLA-A and/or -B antibodies. The DSA-positive patients also had a long list of NDSA. Sixty-eight percent of the reactions found in 27 recipients with DSA were attributable to 66 epitopes on the mismatched donor HLA molecule. All 39 NDSA in eight patients with only NDSA shared 17 epitopes within positive allele specificities. By absorption and elution using recombinant cell lines having a single HLA specificity, we confirmed the epitopes involved in three patients. CONCLUSION: Development of most NDSA in patients with failed allografts is likely due to sharing epitopes with DSA and/or other NDSA.

Antibody Formation↗
PubMed2007

Human keratinocyte Toll-like receptors promote distinct immune responses.

It has been well established that Toll-like receptors (TLRs) are expressed by keratinocytes and respond to their respective ligands to initiate immune responses. However, it appears that keratinocytes, via differential activation of TLRs, may play a key role in determining the type of subsequent cutaneous immune response generated against a particular pathogen.

Antibody Formation↗