Clinical Working Party
March 2023: Contemporary Management of DN-STAT3 / Job’s syndrome
Date: Tuesday, 14 March 2023, 17:00 CET
Please, click here to view the webinar video.
Contemporary Management of DN-STAT3 / Job’s syndrome.
AD-HIES / Job’s syndrome is a multisystem disorder with hematopoietic and extra-hematopoietic manifestations. Conservative treatment is based on skin and mucosal care, anti-infective drugs, immunoglobulin replacements, and more recently biologicals targeting allergic inflammation. Still, significant morbidity and mortality affect quality of live and overall survival of patients with DN-STAT3 and their families.
Allogeneic hematopoietic cell transplantation (HCT) at present is not routinely recommended because initial attempts have documented poor outcome. More recently, however, paralleling major improvements in HCT and supportive care, individual centers are beginning to offer HCT to select patients.
This webinar on contemporary management of DN-STAT3 / Job’s syndrome aims at providing most recent information on conservative treatment and HCT to facilitate patient counseling and treatment decision making.
After participating in this webinar, participants should be able to:
. Discuss clinical manifestations of DN-STAT3 / Job’s syndrome
. Describe conservative treatment of DN-STAT3 / Job’s syndrome
. Assess allogeneic hematopoietic cell transplantation for DN-STAT3 / Job’s syndrome
Moderator: Fabian Hauck, MD, PhD
@Div. of Pediatric Immunology and Rheumatology, Dr. von Hauner Children’s Hospital, Ludwig Maximilian University of Munich, DE
Fabian Hauck is a clinician scientist involved in inborn errors of immunity. He is heading the Division of Pediatric Immunology and Rheumatology at the Dr. von Hauner Children’s Hospital in Munich. He has done his post-doc in lymphocyte signaling at the Hôpital Necker – Enfants Malades in the group of Alain Fischer. His major research activities focus on cell signaling disorders of lymphatic and myeloid cells. By describing novel genetic etiologies and resulting pathophysiologies his work contributed to a better understanding, diagnosis and treatment of inborn errors of immunity.
Alexandra Freeman, MD
@National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, Maryland
Dr. Alexandra Freeman is a pediatric infectious diseases physician at the National Institute of Allergy and Infectious Diseases at the National Institutes of Health who focuses on the diagnosis and management of primary immunodeficiencies. Dr. Freeman received her medical training at Georgetown University Medical School, completed her pediatric residency training at Yale New Haven Children’s hospital and her pediatric infectious diseases fellowship at Northwestern’s program in Chicago. She then joined NIH as an attending physician, briefly focusing on pediatric HIV and then changing her focus to primary immunodeficiency. Her primary focus is Hyper IgE syndromes, and she has been involved in the initial genetic diagnosis of multiple of these syndromes, and is recognized worldwide as an expert in the management of these patients due to her large cohorts of patients followed at NIAID. She also directs the primary immunodeficiency clinic at NIAID.
Bodo Grimbacher, MD, PhD
@Medical Center, Faculty of Medicine, Albert-Ludwigs-University of Freiburg, Germany
Prof. Bodo Grimbacher is a physician scientist and the Vice-Director of the Institute for Immunodeficiency at the University Hospital Freiburg. Additionally, he is an honorary consultant for the Department of Immunology at the Royal Free Hospital, University College London, where until 2011 - when he returned to work in Freiburg (Germany) - he was a EU-Marie-Curie team leader. Previously, he was lecturer and senior scientist at the Department of Clinical Immunology, Freiburg, Germany. He completed his postdoc at the NIH, National Human Genome Institute. Amongst his awards and honors, he was granted the Emmy-Noether Fellowship of the Deutsche Forschungsgemeinschaft (DFG), the Marie-Curie Excellence Grant of the European Commission, the Georges Köhler Award of the German Immunology Society (DGfI), the Richard Farr Memorial Lectureship at AAAAI, the Rudolf-Schoen Prize from the Medizinische Hochschule Hannover, and the Thieme prize from the Leopoldina, the German Research Society.
Johannes Rädler, MD, Resident
@Dr. von Hauner Children’s Hospital, LMU Munich, Germany
Johannes Raedler is a fifth-year pediatrics resident at the Dr. von Hauner Children’s Hospital at the LMU Munich, Germany. Dr. Raedler received his medical training at the LMU Munich and completed his Doctoral thesis on innate RIG-I-like immune receptors. During his pediatrics residency he has gathered experiences in pediatric oncology, stem cell transplantation, intensive care and immunodeficiencies. Recently, Dr. Raedler joined the Hauck lab at the Comprehensive Childhood Research Center (CCRC) in Munich, Germany for his PhD Medical Research on a novel genetic cause for CVID with immune dysregulation.
Andrew Gennery, MD, PhD
@Newcastle University/Great North Children’s Hospital
Andrew Gennery is Professor in Paediatric Immunology and Haematopoietic Stem Cell Transplantation at the University of Newcastle upon Tyne and Honorary Consultant for the Northern Supra-Regional Bone Marrow Transplant Unit for SCID and related disorders, at the Great North Children’s Hospital, Newcastle upon Tyne. His research interests include immunoreconstitution following haematopoietic stem cell transplant for primary immunodeficiency, long-term outcomes of transplantation for primary immunodeficiency (and in particular Chronic Granulomatous Disease and Severe Combined Immunodeficiency), DNA repair disorders and their appropriate treatment and Di George Syndrome.
March 14, 2023 17:00 CET in Amsterdam, Berlin, Rome, Stockholm, Vienna