Journal of pediatric hematology/oncology
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J. Pediatr. Hematol. Oncol. · Apr 2018
Case ReportsA Mild Phenotype of Severe β+ Thalassemia in a 16-Month-Old Boy.
β thalassemia is characterized by a deficient production of functional β-globin chains and a relative excess of α-globin chains. An extremely diverse clinical spectrum-asymptomatic to transfusion-dependent-is primarily due to homozygosity or compound heterozygosity for the very large number of β-thalassemia-causing mutations, along with interacting mutations that affect the α-globin and γ-globin genes and their expression. We report a case of a 16-month-old boy who was initially diagnosed with iron deficiency anemia until he was later found to be homozygous for a severe β-thalassemia genotype with a mild hematologic phenotype. This was likely as a result of his ability to produce high levels of fetal hemoglobin.
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J. Pediatr. Hematol. Oncol. · Mar 2018
Randomized Controlled TrialFeasibility and Acceptability of Internet-delivered Cognitive Behavioral Therapy for Chronic Pain in Adolescents With Sickle Cell Disease and Their Parents.
Pain is a clinical hallmark of sickle cell disease (SCD), and is rarely optimally managed. Cognitive-behavioral therapy (CBT) for pain has been effectively delivered through the Internet in other pediatric populations. We tested feasibility and acceptability of an Internet-delivered CBT intervention in 25 adolescents with SCD (64% female, mean age=14.8 y) and their parents randomized to Internet CBT (n=15) or Internet Pain Education (n=10). ⋯ Our findings suggest that Internet-delivered CBT for SCD pain is feasible and acceptable to adolescents with SCD and their parents. Engagement and adherence were good. Next steps are to modify recruitment plans to enhance enrollment and determine efficacy of Internet CBT for SCD pain in a large multisite randomized controlled trial.
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J. Pediatr. Hematol. Oncol. · Mar 2018
Case ReportsAn Infant With Unusually High Unconjugated Hyperbilirubinemia Due to Coexistence of Hereditary Spherocytosis and Gilbert Syndrome.
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. ⋯ Here we present a 21-month-old girl with hereditary spherocytosis diagnosed at the age of 40 days with hyperbilirubinemia out of proportion of hemolysis which led to diagnosis of GS. Thereby, the diagnosis of GS should be considered in unexplained unconjugated hyperbilirubinemia in different age groups, including infants and toddlers.
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J. Pediatr. Hematol. Oncol. · Mar 2018
Treatment of Transient Peripheral Neuropathy During Chimeric 14.18 Antibody Therapy in Children With Neuroblastoma: A Case Series.
Children with high-risk neuroblastoma are currently treated with a chimeric monoclonal antibody against GD2 ganglioside (chimeric 14.18). The treatment improves survival but causes transient neuropathic pain-like syndrome. ⋯ Mean daily pain scores ranged from 0 to 5 and all children had a 0 pain score upon discharge. No major side effects were noted, suggesting morphine via nurse-controlled analgesia/patient-controlled analgesia is effective in controlling transient neuropathic pain in children receiving GD2 antibody therapy.
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J. Pediatr. Hematol. Oncol. · Jan 2018
Noninvasive Measurement of Hemoglobin Using Spectrophotometry: Is it Useful for the Critically Ill Child?
This study compared the accuracy of noninvasively measuring hemoglobin using spectrophotometry (SpHb) with a pulse CO-oximeter and laboratory hemoglobin (Hb) measurements. A total of 345 critically ill children were included prospectively. Age, sex, and factors influencing the reliabilityof SpHb such as SpO2, heart rate, perfusion index (PI), and vasoactive inotropic score were recorded. ⋯ The pulse CO-oximeter is a promising tool for measuring SpHb and monitoring critically ill children. However, PI may affect these results. Additional studies investigating the reliability of the trend of continuous SpHb values compared with simultaneously measured laboratory Hb values in the same patient are warranted.