title: The diagnosis of hyper immunoglobulin e syndrome based on project management.
abstract: Hyperimmunoglobulin E Syndrome (HIES) is a complex primary immunodeficiency characterized by both immunologic and non-immunologic manifestations. High serum IgE level, eosinophilia, eczema, recurrent skin and lung infections constitute the immunologic profile of HIES, whereas characteristic facial appearance, scoliosis, retained primary teeth, joint hyperextensibility, bone fractures following minimal trauma and craniosynostosis are the main non-immunologic manifestations. The diagnosis of HIES cannot be made by routine immunologic tests. As the main characteristic laboratory abnormalities of this syndrome are highly elevated serum IgE levels and eosinophilia; both features have a broad spectrum of differential diagnosis. The purpose of this essay was presenting the best way for diagnosis management of HIES. Based on the genetic reports of patients of the Center for Chronic Immunodeficiency (CCI) as a single center experience, and applying project management (PM) in health care research projects, we sought the best way for a rapid diagnosis of HIES. The combination of project management principles with immunologic and genetic knowledge to better define the laboratory and clinical diagnosis lead to an improvement of the management of patients with HIES. These results are shown in one "Decision Tree" which is based on 342 genetic reports of the CCI during the past ten years. It is necessary to facilitate the diagnostic analysis of suspected HIES patients; applying project management in health care research projects provides a better and more accurate diagnosis eventually leading to a better patients' care. This Abstract was presented at 16th Biennial Meeting of the European Society for Immunodeficiencies (ESID 2014), Prague, Czech Republic.
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran shiva_saghafi@yahoo.com.
authors.forename: Shiva
authors.initials: S
authors.lastname: Saghafi
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran and Department of Immunology and Allergy, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran pourpakz@tums.ac.ir.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.affiliation: Center for Chronic Immunodeficiency (CCI), University Medical Center and University of Freiburg, Freiburg, Germany cristina.woellner@ucl.ac.uk.
authors.forename: Cristina
authors.initials: C
authors.lastname: Glocker
authors.affiliation: Center for Chronic Immunodeficiency (CCI), University Medical Center and University of Freiburg, Freiburg, Germany nussbaumer.franziska@gmail.com.
authors.forename: Franziska
authors.initials: F
authors.lastname: Nussbaumer
authors.affiliation: Health Management Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran rbt@bmsu.ac.ir.
authors.forename: Abdolreza
authors.initials: A
authors.lastname: Babamahmoodi
authors.affiliation: Center for Chronic Immunodeficiency (CCI), University Medical Center and University of Freiburg, Freiburg, Germany bodo.grimbacher@uniklinik-freiburg.de.
authors.forename: Bodo
authors.initials: B
authors.lastname: Grimbacher
authors.affiliation: Immmunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran and Department of Immunology and Allergy, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran mmoin@tums.ac.ir.
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
mesh_terms: D003663:Decision Trees; D006801:Humans; D007589:Job Syndrome / Q000175:diagnosis*