New and old challenges in the management of immune disorders
Donnerstag 25. Mai 2017 / Thursday 25th of May
Morning: Pre-Meeting of Working Groups
AG Neugeborenenscreening (TREC/KREC)
PID-NET-Meeting
12:00 - 13:00 h
Registration for the API meeting
13:00 h Session 1:
Type I interferonopathies - A. Roesen-Wolff (Dresden) / H. Wittkowski (Münster)
Yanick Crow, Paris, INSERM
"What drives interferonopathies?"
Oral Presentations (10+4)
Stephan Ehl, Freiburg: Bone marrow is the key site of excessive IFN gamma production in primary hemophagocytic lymphohistiocytosis
Sandra Ammann, Freiburg: Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire
Katharina Wustrau, Hamburg: A Registry for Hemophagocytic Lymphohistiocytosis by the Histiocyte Society and the European Society for Immunodeficiencies
Sybille Landwehr-Kenzel, Berlin: In vivo survival and function of adoptively transferred natural regulatory T cells is supported by combination with Cyclosporine A therapy - new insights from a humanized GvHD mouse model
Case Reports (5+3)
Christian Klemann, Hannover: Hemophagocytic lymphohistiocytosis as presenting manifestation of profound combined immunodeficiency due to an ORAI1 mutation
Markus Seidel, Graz: A severe refractory autoinflammatory overlap syndrome with multiple genetic suspects - other options than transplantation?
Claas Hinze, Münster: NCF4 mutations associated with inflammatory bowel disease-like disease
15:15 - 15:45 h Coffee break
15:45 h Session 2:
How do I Diagnose? - K. Schwarz (Ulm) / F. Hauck (München)
Stephan Ehl, Freiburg, CCI
“Diagnosing immune disorders by functional testing“
Oral Presentations (10+4)
Sabine El-Helou, Freiburg: PID-Net Registry: The German Registry for Primary Immunodeficiency
Bernd Gruhn, Jena: New EBMT diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-‐occlussive disease in pediatric patients
Raul Jimenez Heredia, Wien: Next-generation sequencing targeted approach and analysis pipeline for Primary Immunodeficiencies and rare Hematological disorders
Kerstin Felgentreff, Ulm: Radiation Sensitivity Testing on Lymphocytes as a Diagnostic Tool to Identify DNA Repair Defects
Jana Pachlopnik Schmid, Zürich: Causes of low neonatal T-cell receptor excision circles (TRECs): a systematic review
Case Reports (5+3)
Alexander Blank, Ulm: Hyper IgE Syndrome-like Phenotype in a Patient with ARPC1B Deficiency
Rita Beier, Essen: Allogeneic monoclonal T cell lymphoproliferation presenting as severe immunodysregulation with granulomatous inflammation and life threatening vasculitis after heart transplantation in a 10 year old boy
19:00 h Dinner
Freitag 26. Mai 2017 / Friday 26th of May
8:00 h: Session 3:
New aspects of innate immune regulation - Ch. Hedrich (Dresden) / N. Wagner (Aachen)
James DiSanto, Paris, Institut Pasteur
"Innate lymphoid cell diversification and function in immunity"
Oral Presentations (10+4)
Christoph Kessel, Münster: An extracellular ionic milieu renders genulocytic S100A12 a pro inflammatory toll like receptor 4 binding alarmin
Emely Verweyen, Münster: Human monocytic interleukin 1b and interleukin 18 follow similar secretion but divergent gene expression kinetics
Jürgen Brunner, Innsbruck: Complement activation profiles in juvenile idiopathic arthritis
Case Reports (5+3)
Catharina Schütz, Ulm: ADA2 deficiency - spectrum of presentation in 3 siblings
Leif Hanitsch, Berlin: Free serum light chains in the differential diagnosis and prognosis of CVID patients and patients with unclassified antibody deficiency
Johann Greil, Heidelberg: Mutations in sphingosine-1-phosphate lyase cause nephrosis with ichthyosis and adrenal insufficiency
10:00 - 10:30 h Coffee break
10:30 h Session 4:
How do I prevent? - U. Baumann (Hannover) / A. Groll (Münster)
Thomas Lehrnbecher, Frankfurt a.M.
“Antibicrobials: Challenges in prevention and therapy of infections"
Oral Presentations (10+4)
Fabian Speth, Rostock: Varicella-Zoster-Virus vaccination in Immunosuppressed Children without suspension of immunosuppression: proposal for a practicable pre-vaccination checklist
Ellen Renner, München: Management of chronic lung disease in STAT3 hyper-IgE syndrome
Katharina Goessling, Düsseldorf: Anti- and proinflammatory properties of Aspergillus fumigatus cell wall chitin
Case Reports (5+3)
Maria Elena Maccari, Freiburg: Severe Toxoplasma gonii infection and immunological dysfunction in apatient of a NFKB2-deficiency family
Renate Krüger, Berlin: Scabies, periorbital cellulitis and recurrent skin abscesses due to Panton-Valentine Leukocidin positive S. aureus mimic hyper IgE syndrome in an infant
Michael Bauer, Berlin: Antibiotic prophylaxis, immunoglobulin substitution and supportive measures prevent infections in MECP2 Duplication Syndrome
12:30 - 13:30 h Lunch
13:30 h Session 5:
Common Variable Immunodeficiency - V. Wahn (Berlin) / H. v. Bernuth (Berlin)
Kaan Boztug, Wien
“Novel insights at the border of immunodeficiendy and immune dysregulation“
Oral Presentations (10+4)
Tiziana Lorenzini, Freiburg: NFKB1 mutations located downstream of the nuclear localization signal (NLS) lead to constitutive NF-KB activation in resting cells and a paradoxical suppression by TNF stimulation
Henner Morbach, Würzburg: Insights into the function of IgD on human B cells from individuals with heterozygous mutations in IGHD
Maria Faßhauer, Leipzig: Local tolerability of subcutaneous therapy - Results from PID-NET registry (support code: 01GM1517C) and LocTol pilot study of IDCL
Case Reports (5+3)
Gregor Dückers, Krefeld: Immunodeficiency in a patient with Smith Magenis Syndrome carrying a stop mutation in TACI beside classical monoallelic deletion of 17q11.2
Benedikt Spielberger, München: Novel STAT3 mutation results in diminished STAT3 function and autosomal-dominant Hyper-IgE Syndrome
Tuba Turul-Özgür, Ankara: Clinical features of chronic granulomatous disease: a series of 26 patients from a single center
15:30 - 16:00 h Coffee break
16:00 h Session 6:
How do I cure (now and in the future)? - C. Klein (München) / A. Schulz (Ulm)
Francesca Ferrua, Milan, IRCCS San Raffaele Hospital
„From basic research to gene therapy of immunologic disorders"
Oral Presentations (10+4)
Sujal Ghosh, Düsseldorf: T cell gene transfer corrects cytotoxicity defects in perforin deficiency
Georg Varga, Münster: Balancing JAK-signaling in monocytes allows for cross-talk to naive T-cells resulting in development of anti-inflammatory Tregs
Susan Farmand, Stockholm: AWMF-Guideline for diagnosing primary immunodeficiencies
Case Reports (5+3)
Katharina Wustrau, Hamburg: Symptomatic deficiency of the X-linked inhibitor of apoptosis (XIAP) due to extremely skewed X-inactivation in a four-year-old girl
Presentation of the Walter Hitzig Award 2017
18:00 h Member's general meeting
19:30 h Dinner
Samstag 27. Mai 2017 / Saturday 27th of May
8:00 h Session 7:
Unspecific immunomodulatory effects of vaccination - H. Morbach (Würzburg) / R. Seger (Zürich)
Tobias R. Kollmann, Vancouver (BC)
„The power of non-specific and heterologous effects of vaccination"
Oral Presentations (10+4)
Guilia Armaroli, Münster: S100A12 activates vascular endothelial cells via interleukin 1
Anne Sophie Kubasch, Dresden: Serum biomarkers for the diagnosis and monitoring of chronic recurrent multifocal osteomyelitis (CRMO)
Dorothee Viemann, Hannover: S100 alarmin-induced innate immune programming protects newborn infants from sepsis
Case Reports (5+3)
Anna Eichinger, München: Fatal and non-fatal properdin deficiency in monozygotic twins
Gregor Dückers, Krefeld: ATP6AP1 defciency causes immunodeficiency with hepatopathy, cognitive impairment and abnormal protein gycosylation
Tobias Rothoeft, Bochum: An adolescent with severe IL-36 receptor antagonist deficiency like disease
10:00 - 10:30 h Coffee break
10:30 h Session 8:
How do I treat? - S. Borte (Leipzig) / J. Brunner (Innsbruck)
Anders Fasth, Göteborg
„Immunoglobulines: Substitution, immunomodulation, alternatives"
Oral Presentations (10+4)
Eva-Maria Jacobsen, Ulm: Mixed donor chimerism of T-cells after HLA-haploidentical bone marrow transplantation with post-transplantation cyclophsphamide in nonmalignant disorders
Johanna Neubauer, Freiburg: Mutations in the zinc finger transcription factor ZNF341 cause STAT3 insufficiency associated with familiar hyper-IgE Syndrome (HIES)
Case Reports (5+3)
Renate Krüger, Berlin: Severe epistaxis due to nasal telangiectasia in a patient with ataxia telangiectasia
Stephan Borte, Leipzig: An ambiguous blended genotype leading to combined immunodeficiency in a young adult: clinical decision-making in a plot of multi-organ autoimmunity, susceptibility to respiratory tract infections and patient non-compliance
Volker Umlauf, Sankt Augustin: A lethal course of hereditary multiple intesintal atesisas (HMIA) with only mild immunodeficiency
13:00 h End of the Meeting
Zertifizierung
Die Veranstaltung ist bei der Bayerischen Landesärztekammer für Fortbildungspunkte zertifiziert. Bitte denken Sie an Ihre Barcode-Aufkleber.
This meeting is certified for CME Points by the physican's chamber of Bavaria. Please remember to bring your CME barcode labels with you.
Die Veranstaltung wird freundlich unterstützt von / This Event is kindly supported by:
PLATIN-SPONSOREN
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SILBER-SPONSOREN
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BRONZE-SPONSOREN
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gefördert von der
(Fördernummer FO 354/12-1)