Hemolysis After Medication Exposure in Pediatric Patients With G6PD Deficiency.

Hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficiency varies by mutation status and the oxidative stressor. Although classified by percent of enzymatic deficiency, variability in normal G6PD values clouds assessment of hemolysis risk by level. This was a retrospective, single institution, cohort study assessing risk of postexposure medication-induced hemolysis in G6PD deficient patients. Exposures occurred in 87 of 1415 deficient patients. Only 2 of 87 medication-exposed patients had hemolytic episodes and both had very low enzymatic activity. No hemolytic events occurred with G6PD levels >7 units/g hemoglobin. Correlation of levels with mutation may improve predictive capacity for hemolysis in G6PD deficiency.

Treatment of uncomplicated vaso-occlusive crises in children with sickle cell disease in a day hospital.

BACKGROUND: Day hospital management for patients with sickle cell disease experiencing uncomplicated vaso-occlusive pain crises has been described in adult populations as an alternative care delivery system. The objective of this study was to characterize and descriptively assess the benefits of a day hospital exclusively designed for children. PROCEDURE: We retrospectively studied all admissions to the Day Hospital at the Texas Children's Sickle Cell Center since its inception in 2000. A Day Hospital admission was defined as a minimum of two consecutive days of aggressive pain management as an outpatient, including intravenous hydration and analgesics, supported by home treatment over night with oral analgesic and anti-inflammatory agents. We gathered data on demographics, incoming pain score, provider type, opioid administration, length of stay, and needs for higher level care. RESULTS: A total of 35 patients, ages 2-19, accounted for 80 episodes during the study period. The median incoming pain score was 8 on a scale from 1 to 10. The median length of stay was 2 days. The return rate for acute care within 48 hr for persistent symptoms was 7%. Seventy-one percent of patients admitted to the Day Hospital were treated without requiring transfer to inpatient care for escalating pain or medical needs. CONCLUSIONS: We conclude that a dedicated Day Hospital facility has the potential to provide patient-centered, effective, and timely management of vaso-occlusive crises in children as well as adults.

Day hospital versus inpatient management of uncomplicated vaso-occlusive crises in children with sickle cell disease.

BACKGROUND: Day hospital (DH) management for patients with sickle cell disease (SCD) experiencing uncomplicated vaso-occlusive pain crises has been utilized as an alternative care delivery system to inpatient hospitalization. The objective of this study was to determine whether DH management results in shorter length of stay compared to inpatient care. PROCEDURE: We conducted a retrospective cohort study with 35 DH admissions and 35 inpatient admissions for children with SCD presenting with uncomplicated vaso-occlusive crises (VOCs). A DH admission was defined as a minimum of two consecutive days of aggressive pain management as an outpatient, including intravenous hydration and analgesics, supported by home treatment over night with oral analgesic and anti-inflammatory agents. We gathered data on demographics, pain scores, length of stay, admission charges, and needs for persistent care. RESULTS: DH care resulted in a 39% reduction of the average length of stay compared to inpatient admissions. Multivariate linear regression demonstrated that the location of patient care for VOCs was a significant predictor of length of stay (P < 0.0006) after controlling for patient characteristics, severity of illness, and disease history. CONCLUSIONS: We conclude that a dedicated DH facility has the potential to provide efficient and timely management of uncomplicated VOCs through reduction of length of stay. This delivery care system may be particularly relevant for children who are significantly impacted by inpatient hospitalization.

Anticoagulation in neonatal ECMO.

Despite advances made in technology and neonatal intensive care, the rate of hemorrhagic and thrombotic complications remains unacceptably high in patients undergoing extracorporeal membrane oxygenation (ECMO) and these complications negatively impact morbidity and mortality. Management of anticoagulation in neonates who have a developing hemostatic system is vastly different from adults and poses unique challenges. Variation in practice among ECMO centers regarding anticoagulation monitoring and titration reflects the lack of high-quality evidence. Novel anticoagulants may offer alternative options, though their impact on outcomes is yet to be demonstrated. In this chapter, we review the hemostatic alterations that occur during ECMO with a focus on current approaches and limitations to anticoagulation titration in neonates on ECMO.