title: Clinical management of adult sickle-cell disease.
abstract: OBJECTIVE
  This review provides an overview of the clinical management of sickle-cell disease (SCD) with recently published findings.
  
  
  RESULTS
  Unfortunately, negative observations did not confirm the hope that therapies acting on nitric oxide-cyclic GMP signaling, NSAIDs or Gardos channel inhibitor would control SCD vaso-occlusive crises. The safety of hydroxycarbamide was further supported by two observational studies covering 20 years and over 2 years in young children. Concerning the management of chronic visceral complications of SCD, the STOP II trial showed the risk of discontinuing blood exchange transfusion for children with transcranial Doppler-assessed accelerated blood-flow velocities. The French multicenter Etendard study found that only 25% of SCD patients with tricuspid regurgitation velocity (TRV) 2.5 m/s or more on echocardiograms had catheterization-confirmed pulmonary hypertension. However, elevated TRV, regardless of its cause, was associated with higher mortality. Finally, recent results identified new therapeutic strategies for the treatment and prevention of renal dysfunction, priapism and skin ulcers, but prospective studies are needed to confirm those findings.
  
  
  CONCLUSIONS
  SCD treatment relies on concomitant preventive and curative measures to control its acute and chronic manifestations. Pathophysiologic advances have enabled better management, with new therapeutics highly likely in the near future.
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, Hôpital Henri-Mondor AP-HP, Université Paris Est, Créteil, Paris, France. pablo.bartolucci@hmn.aphp.fr
authors.forename: Pablo
authors.initials: P
authors.lastname: Bartolucci
authors.forename: Frédéric
authors.initials: F
authors.lastname: Galactéros
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy*; D000986:Antisickling Agents / Q000627:therapeutic use*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000517:prevention & control; D007674:Kidney Diseases / Q000209:etiology / Q000517:prevention & control; D012871:Skin Diseases / Q000209:etiology / Q000517:prevention & control; D020521:Stroke / Q000209:etiology / Q000517:prevention & control
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea