Pediatric hematology and oncology
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Pediatr Hematol Oncol · Feb 2010
Case ReportsA case of posterior reversible encephalopathy syndrome in a child with myelodysplastic syndrome following allogenic bone marrow transplantation.
Posterior reversible encephalopathy syndrome (PRES) is an uncommon but distinctive clinical-radiologic entity characterized by headache, seizures, visual disturbance, and altered mental function associated with reversible white matter edema affecting the posterior parietal and occipital lobes of the brain. Although PRES is caused by a variety of conditions, acute elevation of blood pressure, fluid retention, and treatment with immunosuppressive drugs and/or anti-neoplastic agents are the main causes. ⋯ Early recognition of PRES and appropriate management are needed to reduce the risk of permanent neurologic disability. The authors report a case of PRES in a girl who received an HLA-identical sibling bone marrow transplantation for myelodysplastic syndrome to emphasize the importance of early recognition and institution of appropriate management of PRES during HSCT.
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Pediatr Hematol Oncol · Sep 2009
Procalcitonin and C-reactive protein serum levels as markers of infection in a pediatric population with febrile neutropenia and cancer.
Procalcitonin and C-reactive-protein are inflammatory markers for sepsis. The authors evaluated their sensitivity and specificity in pediatric patients with cancer and febrile neutropenia. ⋯ Procalcitonin is an accurate predictor of bacterial infection in neutropenic children, while C-reactive-protein may be a better screening test in emergency settings.
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Pediatr Hematol Oncol · Sep 2009
Comparative StudySoluble endothelial protein C receptor level in children with sepsis.
Endothelial protein C receptor (EPCR) is primarily localized on the endothelial cells of large blood vessels. EPCR enhances activation of protein C by 5- to 20-fold. EPCR appears to be physiologically significant in the control of blood coagulation and inflammation and in the host response to gram-negative sepsis. ⋯ Escherichia coli and Klebsiella pneumonia were the most common pathogens. Among these children 16 had sepsis, 23 had septic shock, 15 had multiple organ dysfuntion syndrome, and 12 had disseminated intravascular coagulation. sEPCR levels of survivors were 96 (44-260) and those of nonsurvivors' were 82.5 (38-325) ng/mL (p > .05). Serum soluble endothelial protein C receptor level is not elevated in children with sepsis, and it is not a prognostic parameter in septic children.
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Pediatr Hematol Oncol · Jul 2009
Case ReportsA novel L218P mutation in NADH-cytochrome b5 reductase associated with type I recessive congenital methemoglobinemia.
The presence of central cyanosis that is unrelated to cardiopulmonary causes alerts clinicians to a possible diagnosis of methemoglobinemia. Congenital methemoglobinemia due to deficiency of nicotinamide-adenine dinucleotide (NADH)-cytochrome b5 reductase (cb(5)r) is an autosomal recessive disorder characterized by life long cyanosis. ⋯ Sequencing the CYB5R3 gene identified a homozygous T-->C transition at base c.653, which changed codon 218 from leucine to proline (L218P) in cb(5)r protein. Treatment with ascorbic acid relieved the cyanosis and returned methemoglobin levels to normal.
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Pediatr Hematol Oncol · Jun 2009
Comparative StudyGrowth status in children and adolescents with sickle cell disease.
To assess the BMI status of children and adolescents with sickle cell disease (SCD) and determine if zBMI status during adolescence is predicted by gender, childhood zBMI status, disease genotype, and healthcare utilization (emergency department visits or hospitalizations). ⋯ Children with SCD generally exhibit normal growth during childhood and adolescence, although 5-10% are at risk for poor growth or obesity. Prevention and intervention efforts should consider gender, average weight in childhood, and healthcare utilization factors.