title: Hydroxyurea or chronic exchange transfusions in patients with sickle cell disease: role of transcranial Doppler ultrasound in stroke prophylaxis.
abstract: Hydroxyurea is increasingly used in patients with sickle cell disease and acts by several underlying mechanisms. Its usage has been reported to eliminate transfusion needs in children with beta-thalassemia major. It has also been used in sickle cell disease patients on stroke prophylaxis with exchange transfusions who develop transfusional iron overload and is now being considered as a possible alternative to chronic transfusions for secondary stroke prophylaxis. Our case demonstrates a pitfall when using hydroxyurea without monitoring intracranial cerebral vessels for vasculopathic changes; presence of which can predict stroke risk and alert the need for chronic blood transfusions as primary stroke prophylaxis. Transcranial Doppler is a crucial investigation that can reveal elevated cerebral arterial flow velocities.
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.
authors.forename: Heba
authors.initials: H
authors.lastname: Suliman
authors.forename: Yasser
authors.initials: Y
authors.lastname: Wali
authors.forename: Muna
authors.initials: M
authors.lastname: Al Saadoon
authors.forename: Mathew
authors.initials: M
authors.lastname: Zechariah
authors.forename: Ranjan R
authors.initials: RR
authors.lastname: William
authors.forename: Arunodaya
authors.initials: A
authors.lastname: Gujjar
authors.forename: Anil
authors.initials: A
authors.lastname: Pathare
mesh_terms: D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D000986:Antisickling Agents / Q000627:therapeutic use*; D001783:Blood Flow Velocity; D002536:Cerebral Arteries / Q000503:physiopathology; D002560:Cerebrovascular Circulation; D002675:Child, Preschool; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D020521:Stroke / Q000000981:diagnostic imaging* / Q000503:physiopathology; D017585:Ultrasonography, Doppler, Transcranial
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea