Sickle Cell Disease Pathophysiology and Related Molecular and Biophysical Biomarkers.

One of the challenges in sickle cell disease is its clinical variability. Our ability to identify the complications that a patient is at risk for is limited by a lack of validated diagnostic and prognostic biomarkers. Clinical care is limited by a lack of diagnostics to capture the biological variability needed to precisely direct patient care. Many biomarkers have been proposed, but few validated. We must make a concerted effort as a field to rigorously test proposed biomarkers to improve outcomes for our patients.

Postinduction Supportive Care of Pediatric Acute Myelocytic Leukemia: Should Patients be Kept in the Hospital?

Children with AML become profoundly neutropenic while they undergo remission induction chemotherapy. It is unknown whether these children should be kept in the hospital while they are severely neutropenic to prevent life-threatening complications associated with neutropenia and reduce fatality. We at our institution routinely discharge patients after completing remission induction chemotherapy in the presence of profound neutropenia, unless it is clinically contraindicated. We reviewed all AML patients who were consecutively treated at our hospital from 1989 to 2011. Thirteen patients were electively discharged after completion of induction I chemotherapy. Of the 13, 4 died due to relapse or complications of stem cell transplants (not due to neutropenia related complications). Another eight are long term survivors. In this very small series, discharge from the hospital even though patients were severely neutropenic did not adversely affect the survival.

Co-occurrence of Chiari malformations and sickle cell disease-a diagnostic dilemma: a report of 4 cases and review of literature.

The co-occurrence of sickle cell disease (SCD) and Chiari malformation (CM) poses clinical and diagnostic challenge since symptoms of both conditions may overlap. Although SCD and CM do not have a causal relationship, the overlapping neurologic symptoms may pose a diagnostic dilemma. To the best of our knowledge, the clinical manifestations and long-term consequence of CM in children with SCD has hitherto not been reported in the literature. We describe clinical manifestations of co-occurrence of SCD and CM in a case series of 4 African American children.

6-year-old girl with hydrocephalus.

With rare exceptions, pediatric tectal gliomas have been generally reported as low-grade tumors with relatively good prognosis. The patients are usually treated conservatively to manage the signs and symptoms of obstructive hydrocephalus. We report a case of a tectal glioma in a 6-years-old girl with histological features of anaplastic mixed oligoastrocytoma that continues to progress despite chemotherapy and radiation therapy.