Publications by authors named "H JANUKOWICZ LORENZ"
Introduction: Human Cytotoxic-T-lymphocyte-antigen-4 (CTLA-4) insufficiency caused by heterozygous germline mutations in is a complex immune dysregulation and immunodeficiency syndrome presenting with reduced penetrance and variable disease expressivity, suggesting the presence of disease modifiers that trigger the disease onset and severity. Various genetic and non-genetic potential triggers have been analyzed in CTLA-4 insufficiency cohorts, however, none of them have revealed a clear association to the disease. Multiple HLA haplotypes have been positively or negatively associated with various autoimmune diseases and inborn errors of immunity (IEI) due to the relevance of MHC in the strength of the T cell responses.
View Article and Find Full Text PDFBackground: Posterior vault distraction osteogenesis (PVDO) is an effective treatment for multisuture and syndromic craniosynostosis. It remains unclear how well the calvarium ossifies after PVDO, especially in older patients when they have plateaued in their cranial growth phase. The purpose of this study is to report outcomes associated with PVDO across a wide range of ages at our institution.
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- ENPP1/CD203a is an enzyme that breaks down ATP and other nucleotides, influencing purinergic signaling and immune responses.
- The study investigates ENPP1 expression on immune cells using new heavy-chain antibodies and finds high levels in specific cells like CD141 dendritic cells and natural killer cells, while most T cells and B cells show low expression.
- This detailed analysis of ENPP1 expression will aid in understanding its role in immune regulation and could help identify ENPP1-related conditions and tumors for targeted treatments.
Objective: The high potential of CD19.CAR-T cells to treat autoimmune diseases such as Systemic Sclerosis (SSc) supposedly relies on the disappearance of autoantibodies. Here we investigated effects of CAR-T cells on the innate immune system which is an important contributor to pathology in SSc.
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- The model advanced training regulations outline the necessary content for qualifying as a medical specialist in internal medicine and rheumatology.
- Currently, there are no established criteria for granting authorization in advanced training, which this position paper aims to address with proposed standards from the DGRh.
- The criteria aim to create a standardized and transparent process for issuing authorization across Germany, ensuring high-quality training in rheumatology that can adapt to future changes in the field.