Habibi A, Arlet J-B, Stankovic K, Gellen-Dautremer J, Ribeil J-A, Bartolucci P, Lionnet F
Unité des maladies génétiques du globule rouge (UMGGR), service de médecine interne, centre de référence de la drépanocytose, hôpital Henri-Mondor, AP-HP, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France; Université Paris-Est Créteil (Upec), avenue du Général-de-Gaulle, 94010 Créteil cedex, France; Équipe 2, IMRB, Inserm U955, Créteil, France.
Service de médecine interne, centre de référence de la drépanocytose, hôpital européen Georges-Pompidou, AP - HP, 20, rue Leblanc, 75908 Paris cedex 15, France; Faculté de médecine, université Paris Descartes, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
Rev Med Interne. 2015 May 11;36(5 Suppl 1):5S3-84. doi: 10.1016/S0248-8663(15)60002-9.
Sickle cell disease is a systemic genetic disorder, causing many functional and tissular modifications. As the prevalence of patients with sickle cell disease increases gradually in France, every physician can be potentially involved in the care of these patients. Complications of sickle cell disease can be acute and chronic. Pain is the main symptom and should be treated quickly and aggressively. In order to reduce the fatality rate associated with acute chest syndrome, it must be detected and treated early. Chronic complications are one of the main concerns in adults and should be identified as early as possible in order to prevent end organ damage. Many organs can be involved, including bones, kidneys, eyes, lungs, etc. The indications for a specific treatment (blood transfusion or hydroxyurea) should be regularly discussed. Coordinated health care should be carefully organized to allow a regular follow-up near the living place and access to specialized departments. We present in this article the French guidelines for the sickle cell disease management in adulthood.
镰状细胞病是一种全身性遗传疾病，会引发多种功能和组织改变。随着法国镰状细胞病患者的患病率逐渐上升，每位医生都有可能参与到这些患者的护理工作中。镰状细胞病的并发症可分为急性和慢性。疼痛是主要症状，必须迅速且积极地进行治疗。为降低与急性胸综合征相关的死亡率，必须尽早发现并治疗。慢性并发症是成人主要关注的问题之一，应尽早识别，以防止终末器官损伤。许多器官都可能受累，包括骨骼、肾脏、眼睛、肺部等。应定期讨论特定治疗（输血或羟基脲）的适应证。应精心组织协调的医疗保健，以便在患者居住地附近进行定期随访，并能转诊至专科科室。我们在本文中介绍了法国关于成人镰状细胞病管理的指南。
