Translation of the Pediatric Nausea Assessment Tool (PeNAT) Into Spanish and Evaluating Understandability Among Spanish-Speaking Hispanic American Children and Adolescents Receiving Chemotherapy.

Introduction: We aimed to create a Spanish-language version of the Pediatric Nausea Assessment Tool (PeNAT) and examine its understandability among Spanish-speaking, Hispanic American children.

Methods: : Forward and backward translations of the PeNAT documents were performed and verified by a bilingual panel. Four monolingual, Spanish-speaking dyads (child/parent) and four bilingual dyads piloted the Spanish-language PeNAT documents.

Employment and hospital support among pediatric surgeons.

Employment, either by an academic entity or a hospital, is increasingly becoming a feature of surgical practice. Independent practices receive indirect subsidies to support their revenue. A survey of the extent of employment and the forms of indirect subsidies by which hospitals support independent practices, not previously done, would be of interest to all clinicians.

Minimizing unnecessary parenteral nutrition after appendectomy in children.

Background: Consensus guidelines have indicated that postoperative parenteral nutrition (PN) might provide benefit when patients are expected to be nil per os (NPO) ≥7 d and when PN is administered ≥5 d. We hypothesized that most children receiving PN after appendectomy do not satisfy these criteria.

Methods: The medical records of the patients who had undergone appendectomy for perforated appendicitis from 2006-2011 were analyzed, and the proportion meeting the criteria for beneficial PN was determined.

Long-term safety and efficacy of deferasirox (Exjade) for up to 5 years in transfusional iron-overloaded patients with sickle cell disease.

To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, deferoxamine (DFO)-controlled study entered a 4-year extension, continuing to receive deferasirox, or switching from DFO to deferasirox. Average actual deferasirox dose was 19·4 ± 6·3 mg/kg per d.

Fertility potential in thalassemia major women: current findings and future diagnostic tools.

Preserving fertility, preventing early menopause, and predicting reproductive ability have become crucial for many adult thalassemia major females. Luteinizing hormone/follicle-stimulating hormone (LH/FSH) and estradiol, commonly used for assessment of fertility potential in thalassemia, have a poor predictive value. Current reproductive practice uses markers of ovarian reserve testing, which were not yet studied in thalassemia women.

Nutritional deficiencies in patients with thalassemia.

Optimal nutritional status is imperative for growth, development, immune function, and bone health. Patients with thalassemia are known to have poor growth, altered puberty, and immune function as well as reduced bone mineral acquisition. The etiology of these comorbidites is typically ascribed to the toxic effects of transfusion-related iron-overload.

Comprehensive profiling of the cell surface proteome of Sy5Y neuroblastoma cells yields a subset of proteins associated with tumor differentiation.

Neuroblastoma tumors are derived from the neural crest and exhibit substantial phenotypic heterogeneity and various degrees of differentiation and maturation. The identification of new cell surface markers in neuroblastoma has relevance to disease classification and therapy. As a means to categorize neuroblastomas based on cell surface protein expression, we have obtained a comprehensive profile of the cell surface proteome of the MYCN nonamplified SH-SY5Y neuroblastoma cell line.

Transvascular retrieval of a catheter remnant from the peripheral vein of a preterm neonate.

We report the first case of transvenous removal of a peripheral inserted central catheter (PICC) fragment embolized to a peripheral vein in a 32-week gestational age 1450 g infant. The technical aspect of this alternative method to surgery is discussed.

The link between homeless women's mental health and service system use.

Objective: With high rates of psychiatric and substance use problems, homeless women need a wide variety of services. This study, focusing on homeless women with and without symptoms of mental illness, examined the association of predisposing, enabling, and need factors (based on Aday-Andersen's health services utilization model) with use of behavioral, medical, and human services.

Methods: Data from 738 homeless women from the National Survey of Homeless Assistance Providers and Clients were analyzed.

Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemia.

Transfusional iron overload leads to gonadal failure and low bone mass in patients with thalassemia (Thal). However, gonadal failure is rarely reported in transfused patients with sickle cell disease (SCD) and the literature regarding fracture prevalence in SCD is limited. The objective of this study was to assess self-reported fracture prevalence and its relationship to endocrinopathy in transfused Thal or SCD subjects and compare to non-transfused subjects with SCD (NonTxSCD).

Clinical application of deferasirox: practical patient management.

Deferasirox (Exjade, ICL670) is a once-daily, oral iron chelation agent that is now widely available for the treatment of transfusional hemosiderosis in adult and pediatric patients aged > or =2 years of age. Clinical evaluation has established the efficacy and safety of this novel agent in patients with a variety of chronic anemias. Deferasirox represents a significant advance in the treatment of iron overload, as the availability of an effective oral therapy has the potential to relieve many patients from the burden of frequent parenteral therapy with the previous reference standard iron chelator, deferoxamine.

Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia.

Background: Iron chelation therapy (ICT) with deferoxamine (DFO), the current standard for the treatment of iron overload in patients with transfusion-dependent disorders such as beta-thalassemia, requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence, resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox is an orally administered iron chelator that has been approved for use in the United States, Switzerland, and other countries.

Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2.

Acute pulmonary injury is known as acute chest syndrome (ACS) in patients with sickle cell disease (SCD). Secretory phospholipase A2 (sPLA2) was found to predict those at risk for ACS and a trial was designed to determine if red blood cell transfusion can be used to prevent ACS. Patients with an elevated sPLA2 were randomised to either receive a single transfusion or standard care.

BAC clones generated from sheared DNA.

BAC libraries generated from restriction-digested genomic DNA display representational bias and lack some sequences. To facilitate completion of genome projects, procedures have been developed to create BACs from DNA physically sheared to create fragments extending up to 200 kb. The DNA fragments were repaired to create blunt ends and ligated to a new BAC vector.

Prevalence of HFE mutations in California newborns.

Advances in molecular diagnostics have led to an increased interest in expanding population-based screening to include genetic diseases that occur outside the newborn period. Hereditary hemochromatosis may be a candidate for large-scale screening in populations with a high prevalence of the common HFE mutations. To determine race-specific frequencies of the HFE mutations, C282Y and H63D, the authors applied an automated, high-throughput genotyping method to dried blood spot samples from a representative population of California newborns.

Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state.

The pathogenesis of pulmonary hypertension (PAH), a serious complication in thalassemia, is not well understood. Thromboembolism has been postulated as one of the causative factors; however, there are currently limited specific data on its role. To examine whether increased platelet activation and hypercoagulability are linked to PAH, 25 beta-thalassemia major and beta-thalassemia intermedia patients were evaluated with Doppler echocardiograms for estimation of pulmonary artery pressure and with laboratory assays for indications of a prothrombotic state.

Using qualitative and quantitative strategies to evaluate knowledge and perceptions about sickle cell disease and sickle cell trait.

Objectives: To evaluate knowledge, perceptions and the effectiveness of different sources of information about sickle cell trait (SCT) and sickle cell disease (SCD); to determine individual knowledge of SCT status.

Methods: 28 individuals participated in three focus groups (healthcare providers, people affected by SCD or SCT, and community members). Surveyors interviewed 282 respondents within their neighborhoods.

Pulmonary hypertension in thalassaemia major patients with normal left ventricular systolic function.

Pulmonary hypertension is common in adults with thalassaemia and other haemolytic anaemias. It was hypothesised that regular transfusions in thalassaemia major should both decrease the chronic haemolytic rate and be protective from pulmonary hypertension (PHT). To reduce the contribution of existing cardiac disease to PHT, the subjects were limited to patients with normal left ventricular shortening fractions.

New strategies for the treatment of pulmonary hypertension in sickle cell disease : the rationale for arginine therapy.

Nitric oxide (NO) is inactivated in sickle cell disease (SCD), while bioavailability of arginine, the substrate for NO synthesis, is diminished. Impaired NO bioavailability represents the central feature of endothelial dysfunction, and is a key factor in the pathophysiology of SCD. Inactivation of NO correlates with the hemolytic rate and is associated with erythrocyte release of cell-free hemoglobin and arginase during hemolysis.