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Urological complications of sickle cell disease in a pediatric population
Insights
Sickle cell disease can cause urinary tract infections and priapism in children. Early urological evaluation is recommended for infected patients, and priapism often resolves without surgery.
Area of Science:
- Pediatric Nephrology
- Hematology
- Urology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with various complications.
- Urological issues are recognized complications of SCD, but their frequency and management in pediatric patients require further elucidation.
Purpose of the Study:
- To investigate the spectrum and frequency of urological complications in children with sickle cell disease.
- To evaluate the outcomes of non-surgical interventions for priapism and the incidence of renal scarring in patients with urinary tract infections.
Main Methods:
- A retrospective survey of 321 pediatric patients (1-18 years) with SCD followed between 1970-1984.
- Review of medical records for urological complications including hematuria, urinary tract infections, and priapism.
- Analysis of radiographic evaluations and outcomes for renal scarring and priapism treatment.
Main Results:
- Common urological issues observed were hematuria, urinary tract infections, and priapism.
- A high frequency of renal parenchymal scarring was noted in patients with urinary tract infections, even without reported reflux.
- Priapism in young SCD patients frequently responded to non-surgical therapy and rarely led to impotence.
Conclusions:
- Pediatric patients with sickle cell disease experience various urological complications.
- The high rate of renal scarring in infected children suggests a need for increased urological evaluation in this population.
- Non-surgical management is often effective for priapism in young sickle cell patients, with a low risk of long-term sexual dysfunction.
Abstract:
We surveyed 321 patients 1 to 18 years old who were followed at the sickle cell clinic at the Children's Hospital of Philadelphia between 1970 and 1984 for urological complications of the disease. Mean followup was 5 years and all patients exhibited a typical spectrum of hemoglobin types. The urological problems encountered were those cited in the literature, namely hematuria, urinary tract infection and priapism. Surprisingly few of our patients experienced significant renal bleeding. Although the number of patients with infection evaluated radiographically was small, the frequency of renal parenchymal scarring was disturbingly high despite the reported rarity of reflux in black subjects. Our survey and a review of the literature indicate that most sicklemic children with urinary infection are not subjected to urological evaluation. We question the wisdom of that policy. Finally, we found that priapism responds most often to nonsurgical therapy and that it rarely results in impotence in young sickle cell patients.