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Priapism in children with sickle cell disease
S T Miller1, S P Rao, E K Dunn
1Division of Pediatric Hematology/Oncology, Children's Medical Center of Brooklyn, State University of New York, USA.
The Journal of Urology
|August 1, 1995
Summary
Priapism in pediatric sickle cell disease is rare but serious. Early interventions like hydration and exchange transfusion can be effective, though penile scans may not predict outcomes.
Area of Science:
- Pediatric Hematology
- Vascular Medicine
Background:
- Priapism is a known complication of sickle cell disease (SCD).
- Understanding priapism's clinical course and management in pediatric SCD patients is crucial.
Purpose of the Study:
- To review the incidence, clinical course, and management of priapism in pediatric patients with SCD.
- To evaluate the utility of 99mtechnetium penile scans in predicting clinical outcomes.
Main Methods:
- Retrospective review of hospital admissions over 80 months.
- Analysis of 8 pediatric male patients with SCD experiencing priapism.
- Inclusion of 99mtechnetium penile scan data (low/high flow) and treatment outcomes.
Main Results:
- Eight cases of priapism occurred in approximately 400 pediatric male SCD patients.
- Three cases resolved with hydration; five required exchange transfusion, with three needing shunt procedures.
- Severe clinical courses were observed in postpubertal patients, including temporary and persistent impotence.
- 99mtechnetium penile scans did not reliably predict clinical course in this cohort.
Conclusions:
- Priapism in pediatric SCD is infrequent but associated with significant morbidity, particularly in postpubertal males.
- While initial management strategies like hydration and exchange transfusion can be effective, long-term complications like impotence may occur.
- The predictive value of 99mtechnetium penile scans for clinical outcomes in pediatric SCD-related priapism appears limited based on this small study group.