Sickle cell disease in California: sociodemographic predictors of emergency department utilization.

BACKGROUND: Patients with sickle cell disease (SCD) visit emergency departments (EDs) in rates leading to a significant health system burden. However, limited comprehensive evaluations of utilization patterns have been published using data connecting visits to patients across facilities. This study aims to examine sociodemographic predictors of ED utilization in SCD. PROCEDURE: This retrospective cohort study employed 2007 data from the California Office of Statewide Health Planning and Development (OSHPD). Data included all ED encounters from California hospitals; identifiers connected each visit to an individual patient, across all facilities in the state. Multivariate regression techniques evaluated sociodemographic predictors of utilization while adjusting for confounding variables. RESULTS: In 2007, 2,920 California patients with SCD made 16,364 ED visits. Adults ≥ 21 years of age had higher ED visit rates than children and were more likely to both be in the highest tier of users and visit multiple facilities. Patients living further from a self-identified provider of comprehensive SCD care had higher rates of ED visits and a lower likelihood of hospitalization from the ED. Publicly insured patients had higher rates of ED visits and were more likely to be in the highest tier of users than were the privately insured or uninsured. CONCLUSIONS: Adulthood ≥ 21 years of age, distance from comprehensive SCD care, and insurance status are significant predictors of ED utilization in SCD. As a routine source of care decreases ED utilization, these findings prompt concern that these factors act as barriers to accessing comprehensive SCD care.

Sun protection practices among children with a family history of melanoma: a pilot study.

The goal of this pilot study was to assess sun protection practices and correlates among children with a family history of melanoma, a high risk and understudied group. Sixty-eight melanoma cases, recruited through the Los Angeles County cancer registry, completed a survey. Survivors provided data on 110 children (mean age = 8.11 years). Although most children used sunscreen (79 %), half experienced a recent sunburn. The mean sun protection level for the sample was similar to levels observed among average risk children. Efforts to reduce sunburn frequency and improve sun protection among these vulnerable children appear warranted.

FondaKIDS: a prospective pharmacokinetic and safety study of fondaparinux in children between 1 and 18 years of age.

BACKGROUND: The incidence of thromboembolic disease is increasing in children. New anticoagulants have been licensed in adults and need to be studied in children. This report describes the first prospective study of fondaparinux in children. PROCEDURE: The purpose of the study was to determine the dosing, pharmacokinetics, and safety of fondaparinux in children with deep vein thrombosis (DVT) or heparin-induced thrombocytopenia (HIT). Hospitalized children between 1 and 18 years of age with DVT or HIT received fondaparinux 0.1 mg/kg once daily. Fondaparinux-based anti-factor Xa levels were assessed at 2, 4, 12, and 24 hr following the first dose, and peak levels were measured twice weekly thereafter. Detailed pharmacokinetic analyses were performed. RESULTS: Twenty four subjects in 3 age cohorts were enrolled and completed the study. Pharmacokinetic modeling demonstrated that a once-daily dose of fondaparinux at 0.1 mg/kg resulted in similar concentrations known to be efficacious in adults. Safety was demonstrated with only two bleeding events: one which may have pre-dated study drug administration and one which led only to temporary discontinuation of study drug. CONCLUSION: Dosing of fondaparinux at 0.1 mg/kg once daily in children resulted in PK profiles comparable to those in adults receiving standard dosing. Fondaparinux can be considered an attractive alternative to LMWH given its once-daily dosing, acceptable safety data, and other favorable properties.

Nutritional deficiencies in iron overloaded patients with hemoglobinopathies.

One of the hallmarks of both sickle cell disease (SCD) and thalassemia major (TM) is accelerated oxidative damage. Decreased antioxidant levels and increased oxidant stress biomarkers are found in both diseases. Although isolated vitamin deficiencies have been reported in TM and nontransfused SCD patients, a comprehensive evaluation of vitamin and trace mineral levels has never been performed in chronically transfused SCD or TM patients. As vitamins and trace minerals may be consumed as a result of chronic oxidative stress; we hypothesized that levels of these compounds would correlate with surrogates of iron overload, hemolysis, and inflammation in chronically transfused patients. Using a convenience sample of our group of chronically transfused patients we studied 43 patients with SCD (17 male, 26 female) and 24 patients with TM (13 male and 11 female). The age range for our patients varied from 1.5 to 31.4 years. Levels of vitamins A, thiamin, B6, B12, C, D, E as well as selenium, zinc, copper, and ceruloplasmin were measured. We found that 40-75% of the patients were deficient in A, C, D and selenium and 28-38% of the patients had low levels of B vitamins and folate. There was little association with iron overload, hemolysis, or inflammation. Although the precise mechanism of these deficiencies is unclear, they may contribute to the morbidity of chronically transfused hemoglobinopathy patients.

Vitamin D deficiency, cardiac iron and cardiac function in thalassaemia major.

Vitamin D25-OH and D1-25OH levels were compared with cardiac R2* (1/T2*), left ventricular ejection fraction (LVEF), age, ferritin and liver iron in 24 thalassaemia major patients. Vitamin D25-OH levels were reduced in 13/24 patients while vitamin D1-25OH levels were often elevated. Vitamin D25-OH levels decreased with age (r(2) = 0.48) and with liver iron (r(2) = 0.20). Cardiac R2* was inversely related with the ratio of D25-OH to D1-25OH levels (r(2) = 0.42). LVEF was also proportional to the D25-OH/D1-25OH ratio (r(2) = 0.49). Vitamin D deficiency may be associated with cardiac iron uptake and ventricular dysfunction in thalassaemia major patients.