Bilateral internal carotid artery occlusions in a pediatric patient with refractory acute myeloid leukemia.

Thromboembolism is a well-known complication of cancer including acute myeloid leukemia (AML) especially in patients with high myeloblast counts. However, spontaneous vaso-occlusion in the main arteries is very rare especially in patients with low blast counts and no pre existing vasculopathy. We report the case of a 3-year-old male with refractory AML who developed spontaneous bilateral internal carotid artery occlusion with diffuse cerebral infarcts. Strokes are rarely secondary to spontaneous carotid artery thrombosis and few cases have been reported in the literature.

Daily mood as a mediator or moderator of the pain-sleep relationship in children with sickle cell disease.

OBJECTIVE: To investigate mood as a mediator or moderator of the pain-sleep relationship in children with sickle cell disease (SCD). METHOD: Children with SCD (n = 20; aged 8-12 years) completed daily diaries assessing mood, sleep, and pain for up to 2 months. Data was analyzed using multilevel modeling. Results Results indicate that negative mood partially mediates the relationship between high daily pain and poor sleep that night as well the relationship between poor sleep and high daily pain the following day. The impact of poor sleep on high pain the following day was weakened at increasing levels of positive mood. CONCLUSION: Research is needed to fully explore the ways positive and negative mood may relate to pain and sleep characteristics. This information may be beneficial for developing more effective pain management and sleep interventions.

Brief report: sleep in children with sickle cell disease: an analysis of daily diaries utilizing multilevel models.

OBJECTIVE: To investigate the pain-sleep relationship in children with sickle cell disease (SCD) and the influence of stress and pain medication use on this relationship. METHOD: Children with SCD (n = 20; aged 8-12 years) completed daily diaries assessing sleep, pain, stress, and pain medication use for up to 2 months. Data analyzed using multilevel modeling. RESULTS: High daily pain was related to poor sleep quality that night and poor sleep quality was related to high pain the following day. High stress was related to less sleep. High same-day pain and pain medication attenuated the impact of pain on sleep quality. CONCLUSION: Results highlight the importance of sleep in addressing functioning in children with chronic pain, knowledge which may help patients and their families better manage the child's pain. Behavioral pain interventions may be improved by the inclusion of strategies to encourage proper sleep hygiene and address sleep issues.

Optimism predicting daily pain medication use in adolescents with sickle cell disease.

This study examined the influence of optimism on pain medication use in adolescents with sickle cell disease (n=27; 18 females, 9 males). Participants completed a baseline measure of optimism and an average of 100 daily-diary assessments of pain severity and medication use. Results indicated that adolescents who experienced more severe pain used more analgesic and opioid medications. Optimism was a significant moderator of the relation between pain and opioid-medication use. At medium and high levels of optimism, pain was positively related to opioid use, but at low levels of optimism, the same relation was not present, suggesting that more optimistic adolescents are better able to match their medication use to their pain severity. Future research should examine how other psychosocial factors might influence pain medication use in adolescents and adults who experience pain, and clinicians should take into account psychosocial factors when working with pain populations.

Daily stress and mood and their association with pain, health-care use, and school activity in adolescents with sickle cell disease.

OBJECTIVE: To determine the extent to which daily stress and mood are associated with pain, health-care use, and school activity in adolescents with sickle cell disease (SCD). METHOD: Adolescents with SCD (n = 37; aged 13 to 17 years) completed daily diaries assessing pain, stress, mood, activity, and health-care use for up to 6 months. Multilevel modeling was used to analyze the data. RESULTS: Daily increases in stress and negative mood were associated with increases in same-day pain, health-care use, and reductions in school and social activity. Increases in positive mood were associated with decreases in pain, less health-care use, and more activity participation. Notably, pain was predictive of higher stress and lower positive mood on subsequent days. CONCLUSION: Pain in adolescents with SCD is stressful and may lead to alterations in mood states. Understanding the way in which these variables relate to health-care use and activity may lead to improved pain management approaches.

Interpretation of fetal hemoglobin only on newborn screening for hemoglobinopathy.

Newborn screening for hemoglobinopathies rarely produces a fetal hemoglobin only result; it is most consistent with beta-thalassemia major, although other diagnoses are possible. The authors describe two unrelated African-American babies born in North Carolina whose newborn screening revealed fetal hemoglobin only. Both had a relatively benign clinical and hematologic picture. Molecular analyses indicated that both children are compound heterozygotes for beta-thalassemia and pancellular (deletional) hereditary persistence of fetal hemoglobin, a rare and apparently benign condition. Accurate interpretation of the fetal hemoglobin only result on newborn screening requires thorough evaluation, including family studies and molecular analysis.

Predictors of fetal hemoglobin response in children with sickle cell anemia receiving hydroxyurea therapy.

In the phase I/II pediatric hydroxyurea safety trial (HUG-KIDS), school-aged children with sickle cell anemia receiving hydroxyurea at the maximally tolerated dose (MTD) had variable increases in the percentage of fetal hemoglobin (%HbF). To identify predictors of the HbF response to hydroxyurea therapy, baseline clinical and laboratory values (age, sex, hemoglobin concentration, %HbF, reticulocytes, white blood cell [WBC], platelets, and serum chemistries), as well as treatment variables (number of toxicities, noncompliance, MTD dose, and MTD blood counts) were analyzed in 53 HUG-KIDS children who achieved MTD. Baseline %HbF values (P =.001), baseline hemoglobin concentration (P =.01), MTD dose (P =.02), and compliance (P =.02) were significantly associated with a higher %HbF at MTD; in contrast, age, sex, number of toxicities, and other baseline hematologic parameters were not. After adjusting for variations in baseline %HbF, the baseline reticulocyte count (P =.05) and baseline WBC count (P =.05) were also significantly associated with a higher %HbF at MTD. Hydroxyurea-induced increases in the hemoglobin concentration and mean corpuscular volume (both higher absolute values at MTD and larger positive changes from baseline values), as well as hydroxyurea-induced decreases in reticulocytes and WBC count, were significantly associated with a higher %HbF at MTD. These data suggest that selected baseline laboratory parameters, a higher MTD dose with attention to compliance, and greater therapy-related changes in blood counts may predict the HbF response to hydroxyurea therapy for children with sickle cell anemia. The HbF response to hydroxyurea is variable and complex, however, and even children with low baseline %HbF values can develop substantial increases in %HbF at MTD.