Abstract
apism in children with HbS in order to determine if outcomes differed in patients who did and did not receive SMT. RESULTS: From July 2004 to July 2009, 15 patients with HbS (8HbSS, 3 HbSC, 3 HbSbeta thalassemia, 1 HbSF) experienced 20 priapism episodes. One patient experienced 2 episodes and 2 patients experienced 3 episodes each. 1 patient had undergone prior surgical shunting. Mean age at presentation was 13.2 years (range 1.5 17.8). Hematocrit at presentation was higher in patients with HbSbeta thalassemia (30.2) and HbSC (27) than in patients with HbSS (25.9) or HbSF (23.9). Mean episode duration at presentation was 38.2 hours (range 2.0 384); erections waxed and waned in 8 episodes and were continuous in 12. 7/20 (35%) episodes were associated with acute chest syndrome (ACS); these patients had lower room air oxygen saturation (92.7% vs 98.8%; p 0.04). All patients received treatment directed at the hemoglobinopathy with aggressive hydration (18/20 episodes), narcotic pain medication (19/20 episodes), and/or high-flow oxygen (5/20 episodes). No single therapeutic agent was associated with significant improvement in time to detumescence. Resolution of the priapism mirrored overall improvement in patients with ACS. In 5/20 (25%) episodes, patients required transfusion of packed red blood cells. One episode (5%) required a distal shunt after medical management failed. In 11 (55%) episodes, patients received adjunctive SMT; hematocrit (p 0.23) and episode duration at presentation (p 0.31) were similar in patients with and without SMT. Time to episode resolution was longer in patients who received SMT (76.8 vs 36.8 hours, p 0.09); however, the 1 patient who required shunting did not receive SMT. CONCLUSIONS: In patients with HbS, priapism is often a manifestation of the underlying disease process and rarely requires surgical intervention. Hemoglobinopathy directed therapy should be individually tailored for patients with HbS associated priapism, as no single agent has been shown to improve outcome. SMT use may be associated with delayed resolution of HbS associated priapism.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
