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Horst von Bernuth

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Articles 122
Citations 4395
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Recent Articles
11.
Strauss T, Korholz J, Kuehn H, Gil Silva A, Taube F, Trautmann-Grill K, et al.
Front Pediatr . 2024 May; 12:1345730. PMID: 38813543
Heterozygous germline variants in human encoding for IKAROS define an inborn error of immunity with immunodeficiency, immune dysregulation and risk of malignancy with a broad phenotypic spectrum. Growing evidence of...
12.
Busack L, Thee S, Liu Y, Allomba C, Ziegahn N, Tosolini A, et al.
ERJ Open Res . 2024 Mar; 10(2). PMID: 38469376
Background: Pulmonary manifestations are the major cause of morbidity and mortality in patients with inborn errors of immunity (IEI). New and more sensitive diagnostic methods can potentially lead to earlier...
13.
Materna M, Delmonte O, Bosticardo M, Momenilandi M, Conrey P, Charmeteau-De Muylder B, et al.
Science . 2024 Feb; 383(6686):eadh4059. PMID: 38422122
We describe humans with rare biallelic loss-of-function variants impairing pre-α T cell receptor (pre-TCRα) expression. Low circulating naive αβ T cell counts at birth persisted over time, with normal memory...
14.
Hagele P, Staus P, Scheible R, Uhlmann A, Heeg M, Klemann C, et al.
Lancet Haematol . 2024 Feb; 11(2):e114-e126. PMID: 38302222
Background: Lymphoproliferation and autoimmune cytopenias characterise autoimmune lymphoproliferative syndrome. Other conditions sharing these manifestations have been termed autoimmune lymphoproliferative syndrome-like diseases, although they are frequently more severe. The aim of...
15.
Bethke N, OSullivan J, Keller J, von Bernuth H, Gellert P, Seybold J
Appl Psychol Health Well Being . 2024 Feb; 16(3):1326-1348. PMID: 38299711
Vaccination rates for mumps, measles, and rubella (MMR) and tetanus, diphtheria, pertussis, and polio (Tdap-IPV) fall short of global targets, highlighting the need for vaccination interventions. This study examines the...
16.
Matuozzo D, Talouarn E, Marchal A, Zhang P, Manry J, Seeleuthner Y, et al.
Genome Med . 2024 Jan; 16(1):6. PMID: 38184654
No abstract available.
17.
Jhelum H, cerina D, Harbort C, Lindner A, Hanitsch L, Leistner R, et al.
J Leukoc Biol . 2023 Nov; 115(2):222-234. PMID: 37943843
Staphylococcus aureus strains that produce the toxin Panton-Valentine leukocidin (PVL-SA) frequently cause recurrent skin and soft tissue infections. PVL binds to and kills human neutrophils, resulting in the formation of...
18.
Unger L, Skoluda S, Backman E, Amulic B, Ponce-Garcia F, Etiaba C, et al.
EMBO Rep . 2023 Oct; 24(11):e57571. PMID: 37795769
The peptide toxin candidalysin, secreted by Candida albicans hyphae, promotes stimulation of neutrophil extracellular traps (NETs). However, candidalysin alone triggers a distinct mechanism for NET-like structures (NLS), which are more...
19.
Howley E, Golwala Z, Buckland M, Barzaghi F, Ghosh S, Hackett S, et al.
J Allergy Clin Immunol . 2023 Sep; 153(1):330-334. PMID: 37678573
Background: Newborn screening (NBS) programs for severe combined immunodeficiency facilitate early diagnosis of severe combined immunodeficiency and promote early treatment with hematopoietic stem cell transplantation, resulting in improved clinical outcomes....
20.
Tsilifis C, Torppa T, Williams E, Albert M, Hauck F, Soncini E, et al.
J Clin Immunol . 2023 Aug; 43(8):1964-1973. PMID: 37620741
X-linked chronic granulomatous disease (XL-CGD) is an inherited disorder of superoxide production, causing failure to generate the oxidative burst in phagocytes. It is characterized by invasive bacterial and fungal infections,...