Hydroxyurea effectiveness in children and adolescents with sickle cell anemia: A large retrospective, population-based cohort.

The clinical efficacy of hydroxyurea in patients with sickle cell anemia (SCA) has been well established. However, data about its clinical effectiveness in practice is limited. We evaluated the clinical effectiveness of hydroxyurea in a large pediatric population using a retrospective cohort, pre-post treatment study design to control for disease severity selection bias.

The other side of abnormal: a case series of low transcranial Doppler velocities associated with stroke in children with sickle cell disease.

The prevalence of cerebrovascular events in sickle cell disease (SCD) can be as low as 10% by the age of 18 for overt cerebral infarction or strokes, up to 35% for silent cerebral infarction, and as high as 43/100 patient years for acute silent cerebral ischemic events. These events typically occur during childhood with a peak incidence between the age of 4 and 7 years. The cumulative risk of central nervous system events in SCD increases with age.

Elevated circulating angiogenic progenitors and white blood cells are associated with hypoxia-inducible angiogenic growth factors in children with sickle cell disease.

We studied the number and function of angiogenic progenitor cells and growth factors in children aged 5-18 years without acute illness, 43 with Hemoglobin SS and 68 with normal hemoglobin. Hemoglobin SS subjects had at least twice as many mononuclear cell colonies and more circulating progenitor cell than Control subjects. Plasma concentrations of erythropoietin, angiopoietin-2, and stromal-derived growth factor (SDF)-1α were significantly higher in children with Hemoglobin SS compared to Control subjects.

Erythropoiesis and myocardial energy requirements contribute to the hypermetabolism of childhood sickle cell anemia.

Objectives: We hypothesized that an elevated hemoglobin synthesis rate (SynHb) and myocardial oxygen consumption (MVO2) contribute to the excess protein and energy metabolism reported in children with sickle cell anemia.

Patients And Methods: Twelve children (6-12 years old) with asymptomatic sickle cell and 9 healthy children matched for age and sex were studied. Measurements were whole-body protein turnover by [1-C]leucine, SynHb by [N]glycine, resting energy expenditure by indirect calorimetry and the systolic blood pressure-heart rate product used as an index of MVO2.

Opioid selection during sickle cell pain crisis and its impact on the development of acute chest syndrome.

Background: The hallmark of sickle cell disease (SCD) is recurrent, painful vaso-occlusive episodes (VOC) and is the most common reason for hospitalization in SCD patients. Narcotics, particularly morphine, along with fluid hydration are standard treatments for painful episodes but have been associated with the development of acute pulmonary events commonly referred to as acute chest syndrome (ACS). The development of ACS is often preceded by acute infections, painful episodes, rib infarction, bone marrow infarction, and fat embolism.

Proinflammatory cytokines and the hypermetabolism of children with sickle cell disease.

Sickle cell anemia (HbSS) includes chronic inflammation, but the origin is unclear. We hypothesized that in stable HbSS patients the inflammation was associated with hypermetabolism. We compared selected hypermetabolic and key immunomodulator indicators in HbSS versus control children and examined associations between measures of hypermetabolism and inflammation.