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  1. Article ; Online: Inborn Errors of Immunity Causing Pediatric Susceptibility to Fungal Diseases.

    Olbrich, Peter / Vinh, Donald C

    Journal of fungi (Basel, Switzerland)

    2023  Volume 9, Issue 2

    Abstract: Inborn errors of immunity are a heterogeneous group of genetically determined disorders that compromise the immune system, predisposing patients to infections, autoinflammatory/autoimmunity syndromes, atopy/allergies, lymphoproliferative disorders, and/ ... ...

    Abstract Inborn errors of immunity are a heterogeneous group of genetically determined disorders that compromise the immune system, predisposing patients to infections, autoinflammatory/autoimmunity syndromes, atopy/allergies, lymphoproliferative disorders, and/or malignancies. An emerging manifestation is susceptibility to fungal disease, caused by yeasts or moulds, in a superficial or invasive fashion. In this review, we describe recent advances in the field of inborn errors of immunity associated with increased susceptibility to fungal disease.
    Language English
    Publishing date 2023-01-22
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2784229-0
    ISSN 2309-608X ; 2309-608X
    ISSN (online) 2309-608X
    ISSN 2309-608X
    DOI 10.3390/jof9020149
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: STAT1 Gain-of-Function and Hidradenitis Suppurativa Successfully Managed with Baricitinib.

    Olbrich, Peter / Cortés, José Ivorra / Neth, Olaf / Blanco-Lobo, Pilar

    Journal of clinical immunology

    2023  Volume 43, Issue 5, Page(s) 898–901

    MeSH term(s) Humans ; Hidradenitis Suppurativa/diagnosis ; Hidradenitis Suppurativa/drug therapy ; Gain of Function Mutation ; Azetidines/therapeutic use ; Purines/therapeutic use ; STAT1 Transcription Factor
    Chemical Substances baricitinib (ISP4442I3Y) ; Azetidines ; Purines ; STAT1 protein, human ; STAT1 Transcription Factor
    Language English
    Publishing date 2023-03-07
    Publishing country Netherlands
    Document type Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-023-01454-2
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  3. Article ; Online: Eosinophilia Associated With Immune Deficiency.

    Olbrich, Peter / Ortiz Aljaro, Pilar / Freeman, Alexandra F

    The journal of allergy and clinical immunology. In practice

    2022  Volume 10, Issue 5, Page(s) 1140–1153

    Abstract: The differential diagnosis of eosinophilia is broad and includes infections, malignancies, and atopy as well as inborn errors of immunity (IEI). Certain types of IEIs are known to be associated with elevated numbers of eosinophils and frequently elevated ...

    Abstract The differential diagnosis of eosinophilia is broad and includes infections, malignancies, and atopy as well as inborn errors of immunity (IEI). Certain types of IEIs are known to be associated with elevated numbers of eosinophils and frequently elevated serum IgE, whereas for others the degree and frequency of eosinophilia are less established. The molecular defects underlying IEI are heterogeneous and affect different pathways, which highlights the complex regulations of this cell population within the immune system. In this review, we list and discuss clinical manifestations and therapies of immune deficiency or immune dysregulation disorders associated with peripheral blood or tissue eosinophilia with or without raised IgE levels. We present illustrative case vignettes for the most common entities and propose a diagnostic algorithm aiming to help physicians systematically to evaluate patients with eosinophilia and suspicion of an underlying IEI.
    MeSH term(s) Eosinophilia/diagnosis ; Humans ; Hypersensitivity, Immediate ; Immunoglobulin E ; Immunologic Deficiency Syndromes ; Primary Immunodeficiency Diseases
    Chemical Substances Immunoglobulin E (37341-29-0)
    Language English
    Publishing date 2022-02-25
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2022.02.016
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  4. Article ; Online: STAT1 and STAT3 mutations: important lessons for clinical immunologists.

    Olbrich, Peter / Freeman, Alexandra F

    Expert review of clinical immunology

    2018  Volume 14, Issue 12, Page(s) 1029–1041

    Abstract: Introduction: The transcription factors signal transducer and activator of transcription (STAT) 1 and STAT3 fulfill fundamental functions in nonimmune and immune cells. The description and follow-up of patients with germline mutations that result in ... ...

    Abstract Introduction: The transcription factors signal transducer and activator of transcription (STAT) 1 and STAT3 fulfill fundamental functions in nonimmune and immune cells. The description and follow-up of patients with germline mutations that result in either loss-of-function or gain-of-function have contributed to our understanding of the pathophysiology of these regulators. Depending on the type of mutations, clinical symptoms are complex and can include infection susceptibility, immune dysregulation as well as characteristic nonimmune features. Areas covered: In this review, we provide an overview about mechanistic concepts, clinical manifestations, diagnostic process, and traditional as well as innovative treatment options aiming to help the clinical immunologist to better understand and manage these complex and rare diseases. Clinical and research papers were identified and summarized through PubMed Internet searches, and expert opinions are provided. Expert commentary: The last several years have seen an explosion in the clinical descriptions and pathogenesis knowledge of the diseases caused by GOF and LOF mutations in STAT1 and STAT3. However, harmonization of laboratory testing and follow-up in international cohorts is needed to increase our knowledge about the natural history of these disorders as well as the development of curative or supportive targeted therapies.
    MeSH term(s) Allergy and Immunology/education ; Animals ; Education, Medical ; Genetic Predisposition to Disease ; Humans ; Immune System Diseases/drug therapy ; Immune System Diseases/genetics ; Immune System Diseases/immunology ; Immune System Diseases/metabolism ; Immunologic Factors/therapeutic use ; Mutation ; Phenotype ; Prognosis ; Risk Factors ; STAT1 Transcription Factor/genetics ; STAT1 Transcription Factor/immunology ; STAT1 Transcription Factor/metabolism ; STAT3 Transcription Factor/genetics ; STAT3 Transcription Factor/immunology ; STAT3 Transcription Factor/metabolism ; Signal Transduction
    Chemical Substances Immunologic Factors ; STAT1 Transcription Factor ; STAT1 protein, human ; STAT3 Transcription Factor ; STAT3 protein, human
    Language English
    Publishing date 2018-10-25
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 2274260-8
    ISSN 1744-8409 ; 1744-666X
    ISSN (online) 1744-8409
    ISSN 1744-666X
    DOI 10.1080/1744666X.2018.1531704
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  5. Article ; Online: Autoimmune lymphoproliferative syndrome (ALPS) due to a novel dominant negative germline mutation in the FAS gene.

    Rodríguez-Bayona, Beatriz / Lucena-Soto, José Manuel / Croché-Santander, Borja / Olbrich, Peter / González-Escribano, María Francisca / Neth, Olaf

    Immunologic research

    2023  Volume 72, Issue 1, Page(s) 162–166

    MeSH term(s) Humans ; Autoimmune Lymphoproliferative Syndrome/diagnosis ; Autoimmune Lymphoproliferative Syndrome/genetics ; Germ-Line Mutation ; Mutation ; Apoptosis/genetics
    Language English
    Publishing date 2023-08-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 632857-x
    ISSN 1559-0755 ; 0257-277X
    ISSN (online) 1559-0755
    ISSN 0257-277X
    DOI 10.1007/s12026-023-09411-2
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  6. Article ; Online: Severe Acute Respiratory Syndrome Coronavirus 2 Vaccination in Children with a History of Multisystem Inflammatory Syndrome in Children: An International Survey.

    Hoste, Levi / Soriano-Arandes, Antoni / Buddingh, Emilie Pauline / Whittaker, Elizabeth / Belot, Alexandre / Ulloa-Gutierrez, Rolando / Olbrich, Peter / Haerynck, Filomeen

    The Journal of pediatrics

    2022  Volume 248, Page(s) 114–118

    Abstract: The optimal severe acute respiratory syndrome coronavirus 2 vaccine strategy for patients with a history of multisystem inflammatory syndrome in children (MIS-C) is unclear. We performed an international survey (32 countries) and found substantial ... ...

    Abstract The optimal severe acute respiratory syndrome coronavirus 2 vaccine strategy for patients with a history of multisystem inflammatory syndrome in children (MIS-C) is unclear. We performed an international survey (32 countries) and found substantial variations in vaccine policies. Respondents did not report relapses of MIS-C or other severe inflammatory side effects after severe acute respiratory syndrome coronavirus 2 vaccination in 273 patients with a history of MIS-C.
    MeSH term(s) COVID-19/complications ; COVID-19/epidemiology ; COVID-19/prevention & control ; Child ; Humans ; SARS-CoV-2 ; Surveys and Questionnaires ; Systemic Inflammatory Response Syndrome ; Vaccination/adverse effects
    Language English
    Publishing date 2022-05-19
    Publishing country United States
    Document type Case Reports ; Research Support, Non-U.S. Gov't
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2022.05.028
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  7. Book ; Thesis: THz radiation induced spin polarized currents in low dimensional semiconductor structures

    Olbrich, Peter

    (Dissertationsreihe der Fakultät für Physik der Universität Regensburg ; 18)

    2010  

    Author's details Peter Olbrich
    Series title Dissertationsreihe der Fakultät für Physik der Universität Regensburg ; 18
    Keywords Spinpolarisation ; FIR ; Heterostruktur ; Photostrom
    Language English
    Size 113 S., Ill., graph. Darst, 24 cm
    Edition 1. Aufl.
    Publisher Univ.-Verl
    Publishing place Regensburg
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Univ., Diss.--Regensburg, 2010
    ISBN 9783868450682 ; 3868450688
    Database Library catalogue of the German National Library of Science and Technology (TIB), Hannover

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  8. Book ; Online ; Thesis: THz radiation induced spin polarized currents in low dimensional semiconductor structures

    Olbrich, Peter

    (Dissertationsreihe der Fakultät für Physik der Universität Regensburg ; 18)

    2010  

    Author's details Peter Olbrich
    Series title Dissertationsreihe der Fakultät für Physik der Universität Regensburg ; 18
    Language English
    Size Online-Ressource, Ill., graph. Darst, 24 cm
    Edition 1. Aufl
    Publisher Univ.-Verl. Regensburg
    Publishing place Regensburg
    Document type Book ; Online ; Thesis
    Thesis / German Habilitation thesis Univ., Diss.--Regensburg, 2010
    ISBN 9783868450682 ; 3868450688
    Database Library catalogue of the German National Library of Science and Technology (TIB), Hannover

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  9. Article ; Online: Long-term outcomes of an educational paediatric antimicrobial stewardship programme: a quality improvement study.

    Aboza Garcia, Marta / Goycochea-Valdivia, Walter / Peñalva, Germán / Falcon Neyra, Lola / Moleón Ruiz, Marta / Rodriguez-Villodres, Angel / Montero Valladares, Cristina / Olbrich, Peter / Sánchez-Valderrabanos, Elia / Jiménez, Francisco / Molina, Maria / Moreno Madueño, Gloria / Valencia Martin, Raquel / Gil Navarro, Maria Victoria / Molina, Jose / Neth, Olaf / Cisneros, Jose Miguel

    Archives of disease in childhood

    2024  Volume 109, Issue 2, Page(s) 144–151

    Abstract: Background and objectives: Antimicrobial stewardship programmes (ASPs) have resulted in antimicrobial consumption (AMC) reduction and quality of prescription (QOP) improvement. However, evidence of ASP impact in paediatrics is still limited. This study ... ...

    Abstract Background and objectives: Antimicrobial stewardship programmes (ASPs) have resulted in antimicrobial consumption (AMC) reduction and quality of prescription (QOP) improvement. However, evidence of ASP impact in paediatrics is still limited. This study aims to assess a paediatric ASP long-term outcomes.
    Methods: A quality improvement study assessed by a interrupted time series analysis was conducted in a paediatric tertiary hospital. QOP expressed as proportion of adequate prescriptions, AMC measured by defined daily dose incidence per 1000 occupied bed days, incidence density of bloodstream infections (BSIs) and its related all-cause crude death rate (CDR) were compared between pre (from January 2013 to December 2015) and post (from January 2016 to December 2019) ASP activities intensification, which included a dedicated paediatric infectious diseases physician to actively perform educational interviews with prescribers.
    Results: Inappropriate prescribing showed a significant downward shift associated to the intervention with a -51.4% (-61.2% to -41.8%) reduction with respect to the expected values. Overall AMC showed no trend change after the intervention. For neonatology a28.8% (-36.8% to -20.9%) reduction was observed. Overall anti-pseudomonal cephalosporin use showed a -51.2% (-57.0% to -45.4%) reduction. Decreasing trends were observed for carbapenem use, with a quarterly per cent change (QPC) of -2.4% (-4.3% to -0.4%) and BSI-related CDR (QPC=-3.6%; -5.4% to -1.7%) through the study period. Healthcare-associated multi-drug-resistant BSI remained stable (QPC=2.1; -0.6 to 4.9).
    Conclusions: Intensification of counselling educational activities within an ASP suggests to improve QOP and to partially reduce AMC in paediatric patients. The decreasing trends in mortality remained unchanged.
    MeSH term(s) Humans ; Child ; Anti-Bacterial Agents/therapeutic use ; Antimicrobial Stewardship/methods ; Quality Improvement ; Anti-Infective Agents/therapeutic use ; Carbapenems
    Chemical Substances Anti-Bacterial Agents ; Anti-Infective Agents ; Carbapenems
    Language English
    Publishing date 2024-01-22
    Publishing country England
    Document type Journal Article
    ZDB-ID 524-1
    ISSN 1468-2044 ; 0003-9888 ; 1359-2998
    ISSN (online) 1468-2044
    ISSN 0003-9888 ; 1359-2998
    DOI 10.1136/archdischild-2022-323802
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  10. Article ; Online: "Deficiency in ELF4, X-Linked": a Monogenic Disease Entity Resembling Behçet's Syndrome and Inflammatory Bowel Disease.

    Olyha, Sam J / O'Connor, Shannon K / Kribis, Marat / Bucklin, Molly L / Uthaya Kumar, Dinesh Babu / Tyler, Paul M / Alam, Faiad / Jones, Kate M / Sheikha, Hassan / Konnikova, Liza / Lakhani, Saquib A / Montgomery, Ruth R / Catanzaro, Jason / Du, Hongqiang / DiGiacomo, Daniel V / Rothermel, Holly / Moran, Christopher J / Fiedler, Karoline / Warner, Neil /
    Hoppenreijs, Esther P A H / van der Made, Caspar I / Hoischen, Alexander / Olbrich, Peter / Neth, Olaf / Rodríguez-Martínez, Alejandro / Lucena Soto, José Manuel / van Rossum, Annemarie M C / Dalm, Virgil A S H / Muise, Aleixo M / Lucas, Carrie L

    Journal of clinical immunology

    2024  Volume 44, Issue 2, Page(s) 44

    Abstract: Defining monogenic drivers of autoinflammatory syndromes elucidates mechanisms of disease in patients with these inborn errors of immunity and can facilitate targeted therapeutic interventions. Here, we describe a cohort of patients with a Behçet's- and ... ...

    Abstract Defining monogenic drivers of autoinflammatory syndromes elucidates mechanisms of disease in patients with these inborn errors of immunity and can facilitate targeted therapeutic interventions. Here, we describe a cohort of patients with a Behçet's- and inflammatory bowel disease (IBD)-like disorder termed "deficiency in ELF4, X-linked" (DEX) affecting males with loss-of-function variants in the ELF4 transcription factor gene located on the X chromosome. An international cohort of fourteen DEX patients was assessed to identify unifying clinical manifestations and diagnostic criteria as well as collate findings informing therapeutic responses. DEX patients exhibit a heterogeneous clinical phenotype including weight loss, oral and gastrointestinal aphthous ulcers, fevers, skin inflammation, gastrointestinal symptoms, arthritis, arthralgia, and myalgia, with findings of increased inflammatory markers, anemia, neutrophilic leukocytosis, thrombocytosis, intermittently low natural killer and class-switched memory B cells, and increased inflammatory cytokines in the serum. Patients have been predominantly treated with anti-inflammatory agents, with the majority of DEX patients treated with biologics targeting TNFα.
    MeSH term(s) Male ; Humans ; Behcet Syndrome/diagnosis ; Behcet Syndrome/genetics ; Inflammatory Bowel Diseases/diagnosis ; Inflammatory Bowel Diseases/genetics ; Arthralgia ; Arthritis ; Biological Products ; DNA-Binding Proteins ; Transcription Factors/genetics
    Chemical Substances Biological Products ; ELF4 protein, human ; DNA-Binding Proteins ; Transcription Factors
    Language English
    Publishing date 2024-01-17
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-023-01610-8
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