Horst von Bernuth
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Explore the profile of Horst von Bernuth including associated specialties, affiliations and a list of published articles.
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122
Citations
4395
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Recent Articles
1.
Goetzke C, Massoud M, Frischbutter S, Guerra G, Ferreira-Gomes M, Heinrich F, et al.
Nature
. 2025 Mar;
PMID: 40074901
In a subset of children and adolescents, SARS-CoV-2 infection induces a severe acute hyperinflammatory shock termed multisystem inflammatory syndrome in children (MIS-C) at four to eight weeks after infection. MIS-C...
2.
Launspach M, Mindermann A, Schulz J, Alasfar L, Cyrull S, Zirngibl F, et al.
United European Gastroenterol J
. 2025 Feb;
PMID: 39955611
Exocrine pancreatic insufficiency (EPI) is suspected but remains understudied in immunosuppressed conditions such as post-hematopoietic stem cell transplantation (HSCT). This prospective observational study aimed to investigate the incidence, impact, and...
3.
Ransmayr B, Bal S, Thian M, Svaton M, van de Wetering C, Hafemeister C, et al.
Sci Immunol
. 2024 Nov;
9(101):eadq8796.
PMID: 39576873
Secondary lymphoid organs (SLOs) provide the confined microenvironment required for stromal cells to interact with immune cells to initiate adaptive immune responses resulting in B cell differentiation. Here, we studied...
4.
von Bernuth H, Gungor T
Bone Marrow Transplant
. 2024 Nov;
60(2):180-181.
PMID: 39496935
No abstract available.
5.
Eigemann J, Janda A, Schuetz C, Lee-Kirsch M, Schulz A, Hoenig M, et al.
J Clin Immunol
. 2024 Sep;
45(1):1.
PMID: 39264518
Purpose: Genetic hypomorphic defects in X chromosomal IKBKG coding for the NF-κB essential modulator (NEMO) lead to ectodermal dysplasia and immunodeficiency in males and the skin disorder incontinentia pigmenti (IP)...
6.
Berghuis D, Mehyar L, Abu-Arja R, Albert M, Barnum J, von Bernuth H, et al.
J Clin Immunol
. 2024 Aug;
44(8):182.
PMID: 39167297
Immunodeficiency-Centromeric instability-Facial dysmorphism (ICF) syndrome is an inborn error of immunity characterized by progressive immune dysfunction and multi-organ disease usually treated with antimicrobial prophylaxis and immunoglobulin substitution. Allogeneic hematopoietic stem...
7.
Variants in IGLL1 cause a broad phenotype from agammaglobulinemia to transient hypogammaglobulinemia
Soomann M, Bily V, Elgizouli M, Kraemer D, Akgul G, von Bernuth H, et al.
J Allergy Clin Immunol
. 2024 Aug;
154(5):1313-1324.e7.
PMID: 39147326
Background: Agammaglobulinemia due to variants in IGLL1 has traditionally been considered an exceedingly rare form of severe B-cell deficiency, with only 8 documented cases in the literature. Surprisingly, the first...
8.
Fasshauer M, Schuermann G, Gebert N, von Bernuth H, Bullinger M, Goldacker S, et al.
Immunotherapy
. 2024 Jul;
16(12):813-819.
PMID: 39073437
To assess a patient empowerment program (PEP) for children/adolescents with primary immunodeficiency (PID) on IgG replacement therapy regarding quality of life (QoL) in patients and proxy. Health-related QoL was assessed...
9.
Staudacher O, von Bernuth H
Front Pediatr
. 2024 Jul;
12:1384550.
PMID: 39005504
Chronic granulomatous disease (CGD) is caused by an impaired respiratory burst reaction in phagocytes. CGD is an X-linked (XL) (caused by pathogenic variants in ) or autosomal recessive inborn error...
10.
Fasshauer M, Dinges S, Staudacher O, Voller M, Stittrich A, von Bernuth H, et al.
Front Pediatr
. 2024 Jun;
12:1386959.
PMID: 38933494
In patients with severe and recurrent infections, minimal diagnostic workup to test for Inborn Errors of Immunity (IEI) includes a full blood count, IgG, IgA and IgM. Vaccine antibodies against...