Reversible posterior leuko‐encephalopathy in children with sickle cell disease

Children with sickle cell disease (SCD) have high risk of neurologic morbidity and mortality, such as strokes, silent infarcts and TIA's. A retrospective review of magnetic resonance imaging and magnetic resonance angiography identified eight children with radiological and clinical characteristics of reversible posterior encephalopathy (RPLS). These patients had no evidence of previous cerebral infarcts or vasculopathy. Three have died during the 5‐year follow up; one developed a stroke and one a conditional TCD. RPLS needs to be considered in the differential diagnosis of children with SCD that present with acute neurological changes, especially if they are already been hospitalized. Pediatr Blood Cancer 2009;52:373–375. © 2008 Wiley‐Liss, Inc.     

Cochlear implantation in children with sickle cell disease

Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe‐to‐profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe‐to‐profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe‐to‐profound SNHL following acute vaso‐occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso‐occlusive crisis or unexplained vestibulocochlear event.     

Impact of proximity to comprehensive sickle cell center on utilization of healthcare services among children with sickle cell disease

Background

The impact of comprehensive care on utilization of healthcare services by children with sickle cell disease (SCD) has not been fully evaluated. We compared the medical care utilization and mortality in children less than 20 years of age with SCD in four regions in the state of Tennessee with and without a comprehensive sickle cell center (CSCC).

Methods

Rates of hospitalizations, outpatient and emergency department (ED) visits, and deaths were measured in a cohort of children aged <20 years with SCD, enrolled in TennCare, from January 1995 to December 2002. TennCare data linked to Tennessee vital records were used to define the population and identify the outcomes. The patients were classified into one of four regions based on their residential address on the day of their hospitalization or outpatient visit.

Results

The cohort consisted of 1,214 children with 6,393 person‐years of follow‐up. Fifty‐six percent of patients resided in the region with the CSCC. This region had the highest overall rates of hospitalization for all children (P < 0.001), while ED and outpatient visits were higher in other areas. The death rates ranged from 1.8 to 4.3 per 1,000 person‐years in the four regions and did not represent statistically significant differences.

Conclusion

No clear pattern of improved utilization of medical care services were identified in relation to proximity of residence to a CSCC. This cohort was not large enough to detect small differences in death rates. In addition, other outcomes that incorporate quality of life measures may be more sensitive to differences in medical care. Pediatr Blood Cancer 2008;50:66–71. © 2006 Wiley‐Liss, Inc.     

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.