title: [Patient support for primary immunodeficiency with hypogammaglobulinemia and intravenous immunoglobulin substitution: result of a national survey].
abstract: BACKGROUND
  Primary Immunodeficiencies (PIDs) represent a heterogeneous group of rare diseases characterized by increased susceptibility to infections, often accompanied by a diverse immuno-pathological manifestations (autoimmunity, inflammation, benign or malignant lymphoproliferative disorders). The precise prevalence of PIDs in France is not known but is estimated to represent approximately 5 000 patients.
  
  
  METHODS
  To better understand the situation of PID in France and gain an insight as to the care of these patients, we conducted a national survey by sending a questionnaire to physicians potentially involved in the care of PID patients.
  
  
  RESULTS
  The majority of physicians follow only a few PID patients but the diagnostic and therapeutic attitudes are generally satisfactory.
  
  
  CONCLUSIONS
  These results underscore the need to coordinate the care of PID patients in France as part of national networks. The approach adopted by the CEREDIH (French reference center for hereditary immune deficiencies) and DEF-I french national cohort identifying patients with DIP, will optimize the management of PID by defining diagnostic and therapeutic guidelines. In addition, these networks will provide valuable data regarding the incidence of PID and their complications.
authors.affiliation: Service d'hématologie adultes, et CEREDIH (centre de Référence des Déficits Immunitaires Héréditaires), Assistance Publique- Hôpitaux de Paris, hôpital Necker- Enfants Malades et faculté de médecine Paris Descartes, 75015 Paris, France.
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Sophie
authors.initials: S
authors.lastname: Puget
authors.forename: Raphaelle
authors.initials: R
authors.lastname: Beaudet
authors.forename: Nilam
authors.initials: N
authors.lastname: Goulamhoussen
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D005602:France; D006801:Humans; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D010818:Practice Patterns, Physicians'; D011795:Surveys and Questionnaires
chemical_list: D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous