title: [Sickle cell disease in adults: which emergency care by the internists?].
abstract: BACKGROUND
  Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the most prevalent genetic disease at birth in the Ile-de-France area. Internists are involved in the management of acute complications, particularly acute vaso-occlusive crisis.
  
  
  BACKGROUND
  Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to the ischaemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to the hospital and the main cause of death. It mainly manifests by osteoarticular pain but other clinical complications can be observed such as acute chest syndrome, priapism, ischaemic or haemorrhagic stroke, abdominal pain and acute multivisceral failure. The treatment of acute vaso-occlusive crisis is symptomatic. Simple transfusion or partial exchange transfusion is required in the more severe form of vaso-occlusive crisis.
  
  
  CONCLUSIONS
  The management of adult patients with sickle cell disease must be based on a multidisciplinary approach. At the present time, more than 50% of patients survive beyond the fifth decade. This better and longer life in developed countries has resulted from basic investigations and symptomatic treatments.
authors.affiliation: Service de médecine interne, hôpital Henri-Mondor, 51, avenue de-Lattre-de-Tassigny, 94010 Créteil, France. bertrand.godeau@hmn.ap-hop-paris.fr
authors.forename: B
authors.initials: B
authors.lastname: Godeau
authors.forename: V
authors.initials: V
authors.lastname: Noël
authors.forename: A
authors.initials: A
authors.lastname: Habibi
authors.forename: A
authors.initials: A
authors.lastname: Schaeffer
authors.forename: D
authors.initials: D
authors.lastname: Bachir
authors.forename: F
authors.initials: F
authors.lastname: Galactéros
mesh_terms: D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D001803:Blood Transfusion; D002423:Cause of Death; D019049:Developed Countries; D004638:Emergency Treatment / Q000379:methods*; D005260:Female; D005602:France / Q000453:epidemiology; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007239:Infections / Q000209:etiology* / Q000628:therapy*; D007388:Internal Medicine / Q000379:methods*; D008136:Longevity; D008297:Male; D010348:Patient Care Team; D010951:Plasma Exchange; D012307:Risk Factors; D016019:Survival Analysis; D014652:Vascular Diseases / Q000209:etiology* / Q000628:therapy*